I thought you would be interested in this. This "write up" is THE MOST
conclusive account of our experience. It is amazing how accurate and "text
book" this write up explains the overall experience of a child with
neuroblastoma. It actually comes from an Italian research website.
http://www.neuroblastoma.org/nb/inglese/

Jeff Tubo

This write-up
(http://www.neuroblastoma.org/neuro.OLD/engl/Neuroblastoma.html) on
neuroblastoma which uses several “examples”, a “glossary” and discusses a
variety of aspects of the illness neuroblastoma, intends to provide
comprehensive information to, not only parents and relatives of children
with this disease, but also to anyone who wants to become more informed
about the disease so that they may be able to confront it with more
awareness.
We should like to emphasis that these notes are not claiming to answer all
questions and will certainly not substitute the vital daily dialogue between
parents and the medical team, but by reading our notes, we would like to
think that you will then be encouraged to seek the further information that
you require from both doctors and nurses.

&Mac183; General details on Neuroblastoma
&Mac183; Diagnosis
&Mac183; The stages of Neuroblastoma
&Mac183; Treatment
&Mac183; How to speak to your child
&Mac183; Home Sweet Home - returning home
&Mac183; Asking for help
&Mac183; After treatment
&Mac183; Conclusion


How Neuroblastoma presents itself

Example 1.
Mario, a newborn baby of 20 days, has had a somewhat swollen abdomen since
birth and for a couple of days, small subcutaneous “nodules" have been
observed. For this reason, the Pediatrician has advised his admittance to
hospital. Blood tests reveal only a slight condition of “anemia”, but on
carrying-out an ultra-scan of the abdomen, an unusually large liver is shown
up with small rounded nodulary lumps and an enlarged right suprarene.
Several days after being admitted to hospital, a biopsy is carried out on
the subcutaneous nodule and the histological results prove it to be
neuroblastoma.
The precocious age and the presence of multiple centres of infection in the
liver clearly define a very particular form of neuroblastoma, the so-called
4-S stage (S standing for special). This “special” particularity comes from
the fact that the tumour lesions, after a growth phase lasting from a couple
of weeks to several months, in the case of 8 patients out of 10, tend to
regress spontaneously, that is to say, without any need for therapy.

Example 2.
Maria is a 5 year old girl who has suffered a fractured arm in a fall from
her bicycle. Since the fracture is put to rights under a general
anaesthetic, a radiogram of her chest is carried out, the results of which
are quite surprising because a rounded shaded area near to the vertebral
column is discovered. Subsequently, an ultascan (TAC) of the chest detects
an oval-shaped mass, with distinct limits, well-defined by large vessels,
from the lungs and heart.
In the urine an abnormal quantity of vanilmandelic acid, a substance which
derives from adrenaline and which is produced by the sympathetic nervous
system and also from tumours where it originates, is calculated (in fact, it
is known that neuroblastoma comes from residues that have not been correctly
developed by the sympathetic nervous system).
It is then decided to intervene with a surgical operation in order to remove
the mass completely. During the operation, no lymph-nodes of particular
pathology are found by the surgeon and 4 medullary aspirations and 2 bone
biopsies are carried out in order to exclude the possibility of infiltration
of the tumour into the bone marrow.
The histological results prove that it is a neuroblastoma which shows
aspects of maturation (ganglioneuroblastoma).
The complete removal of the tumour and the absence of the metastasis give a
definition of Phase 1, that is to say, a localized tumour, for which no
other therapy is necessary, but only periodic controls over 3 years.

Example 3.
Mario is a toddler of 2 and a half years old who has always shown good
health and has grown at a regular rate, but in about the past two or three
months, has shown a certain loss in appetite and vague and irregular stomach
ache. The Paediatrician has visited him quite thoroughly, on more than one
occasion, without finding anything unusual; however, in the last couple of
days, the child has become very palid, irritable and seems to climb steps
with some difficulty. The parents, becoming anxious about this, have brought
him to the hospital.

>From the clinical tests carried out, a small mass within the left abdominal
area is found and a reduced strength in the inferiore limbs is noted. An
abdominal RMN is carried out which shows a retro-peritonale mass on the left
para-vertebrale with lymph eonomegalie surrounding it and a small branching
of this mass into the speco vertebra which compresses the spinal cord. The
reduced strength in the inferior limbs worsens in the space of only a few
hours and it is decided to undergo surgery with a laminotomia operation to
take the pressure off the spinal cord and to carry out an histological
examination of the tumour which has infiltrated into the vertebral canal.
During the operation, a vascular catheter is put into position. The
diagnosis of the neuroblastoma is defined as stage 3 with spinal epidural
compression (the aspirations and the bone marrow biopsies carried out during
the operation give negative results, as does the skeletral radiogram carried
out a couple of days later). The child then undergoes several cycles of
chemotherapy, and the mobility of the limbs are recovered and the abdominal
mass becomes reduced in volume whilst the linfoadenomegalie disappears
completely so that after some months of his admittance into hospital, the
doctors carry out another operation in order to remove the abdominal mass
completely. No other treatment is necessary but the child will receive
regular check-ups in order to also identify early on any such possible
relapse or alteration in the vertebral column linked to the first operation.


Example 4.
Maria is a child of 4 years old who has had a fever for several weeks, which
has, during short periods, been also rather high with vague and migrating
aches in her bones. Antibiotics have not changed this condition. The
Paediatrician, even though blood tests have shown to be normal, suspecting
that it could be “rheumatic fever” asks for the child to be admitted to a
University Hospital Clinic. Once again, blood tests prove normal, but the
fever and aches persist and so the doctors carry out a midullary withdrawal,
suspecting that it could be a form of leukemia. This examination reveals
however, typical neuroblastoma cells and a further immunological
investigation on these, with monoclonal antibodies, confirms the diagnosis.
The next step is to search for the primary tumour, but the ultrascan of the
abdomen and the radiograph of the chest gives negative results. Then a
radiogram with MIBG is carried out which shows up various bone metastases
and a small hyper retro-vesicular ipercaptante retrovescicale lesion. The
ultrascan (TAC) of the abdomen, centred on the pelvic region, reveals a
pre-sacral mass. The diagnosis is neuroblastoma, stage 4. The child is
operated on in order to remove the primary tumour, to confirm the
histological diagnosis and to carry out the molecular biological
examinations (oncogenes, citogenetic) and a central vascular catheter is put
into place.
Immediately after, chemotherapy is begun, the aches and fever disappear and,
after several cycles, the cardiogram with MIBG is carried out once more,
together with midullary withdrawals and a TAC of the abdomen. As all the
tests carried out are negative, the child is considered to be in remission
but the treatment cannot be interrupted because the illness has taken hold
and is practically certain: it is necessary to resort to a very strong
treatment of chemotherapy which requests the extracting of perifial staminal
cells or explant of the bone marrow, and successively, a block treatment of
chemotherapy at a very much higher dosage than the previous, followed by the
re-infusion of that cryo-preserved. Having concluded this period of toxicity
of mass therapy, thanks also to the use of hematopoietic factors of growth,
the child is able to be discharged from the hospital.
Now it is necessary to repeat an extensive evalution of the state of the
disease and return periodically in order to control the illness and to
identify any possible late effects of the chemotherapy carried out.


General details on Neuroblastoma

Neuroblastoma represents about 10% of tumours found in children and is the
third most common tumour after leukemia and brain tumours. Around 110 new
cases of neuroblastoma are diagnosed yearly in our country.
Neuroblastoma occurs where cells of the sympathetic nervous system are
situated and therefore can be found in the adrenal cortex system and in
anyone of the sympathetic nervous system glands in the neck, chest, abdomen
and pelvis, even if, usually, the larger part of neuroblastoma is more
frequently found in the abdomen and, in particular, in the adrenal system
surrene.
The symptoms manifested by Neuroblastoma are varied, some being inspecific
and difficult to pinpoint, like fever, loss of strength, pallor, vomiting,
and mood changes; others depend on where the primary tumour is situated:
coughing and difficulty in breathing, when the tumour is in the chest,
stomach pains or a swelling of the abdomen, when it is in the abdomen.

As with other tumours to be found in children, parents are often astonished
that neither they nor their Paediatrician have realised before, but tumours
are a rare event in children and, unfortunately, the symptoms are vague and
lead one to think of other illnesses, also banal illnesses which are very
much more frequent. In some cases, the discovery of a tumour can occur quite
by chance, for example, during a simple check-up or by way of investigating
the possibility of another illness. It should also be mentioned, however,
that in the case of neuroblastoma, a early diagnosis does not always mean
less spread of the disease or more possibility of curing it.


Diagnosis

The diagnosis of neuroblastoma is confirmed and finalised in a specialised
centre, with indispensible tests and investigations for planning the most
suitable treatment and cure.
A bone radiogram is carried out by injecting a radioactive isotope into the
blood which becomes absorbed by any parts of the bone where a lesion might
be present which requires a bone repair, as in the case of a possible
metastasis. In the case of a radiogram with MIBG, a substance, guanidine, is
injected. This substance is connected to radioactive iodine and becomes
caught specifically by the neuroblastoma cells, identifying the presence of
either the tumour or its metastases.
In the images which one obtains from this investigation, the mass of tumour
cells are seen as "luminous points". This is neither a painful nor dangerous
examination which lasts at least 20 minutes and requires a specific
preparation of administering the iodine (in the form of Lugol drops) orally
in order to avoid the radioactive iodine contained in the MIBG becoming
absorbed by the thyroid which could then alter the quality of the results of
the examination. The Lugol solution is very bitter but can be rendered
acceptable by diluting it with water in a plastic teaspoon. This test is of
utmost importance because it gives us the precise information of the
location and extension of the tumour, if not also, though less precisely,
the tumour's size.
It should be stressed that the carrying out of these investigations are not
harmful nor painful and can be repeated without causing any harm. The TAC
and RMN require nevertheless a state of immobility for quite a long time and
often are carried out under sedation or general anaesthetic.
Another fundamental stage in the study of the spread of the tumour is that
of a medullary evaluation which consists of carrying out of one or two bone
biopsies, that is the withdrawal of bone and bone marrow fragments using a
high calibre needle in order to exclude the presence of tumour cells. This
evaluation is carried out under general anaesthetic, usually following a
surgical operation or after the positioning of a vascular catheter. Such an
investigation could be repeated during the treatment so as to give a clear
picture of the effect of the therapy. If the evaluation is limited to only
one bone withdrawal, then in this case it may be carried out under a simple
local anaesthetic.

Sometimes, it could be necessary to resort to particular investigations or
to repeat some due to unsatisfactory first results. Other times, the results
of the tests may require more time than first expected, bringing about a
delay in the diagnostic time and creating anxiety and even dissatisfaction.
For the family, these days of waiting are, of course, difficult moments,
even if the staff who is looking after the child is most willing to discuss
any problems which could arise, but it is important to remember how
essential it is to acquire, from the beginning, all the results which could
then influence the medical team's judgement of the extention of the illness
and to consequently decide the best way forward for the most suitable cure.
For the diagnosis, it may be necessary to carry out a surgical operation on
the tumour. In some cases, an operation will eliminate the tumour completely
with the possibility of cure without resorting to therapy, being higher than
90%. More frequently, however, is the case of the operation being limited to
removing only a small part of the tumour so as to gain important information
on its histological-biological characteristics (for example, the presence of
a genetical anomaly).
When the illness requires treatment with anti-tumour drugs, it is necessary
to put a central venous catheter in place, i.e. the insertion of a very
little thin and soft rubber tube which remains in a large blood vessel for
months, allowing one to carry out, without pain, all the necessary
examinations and treatments which would normally require repetitive venous
injections and a long period of immobility in the child.


The Stages of Neuroblastoma

At the end of the investigations mentioned above and often only after a
surgical operation, it shall be possible to obtain a clear and precise
picture of the most suitable treatment needed. The type of therapy and the
prognostic verdict of the illness depends, on the whole, on two factors:
one, the age of the child (children of under one year at the time of
diagnosis have, generally speaking, a more favourable prognosis) and two,
the extent to which the disease has spread, or rather, the study of the
tumour itself.

Stage 1: The tumour is normally small and confined to a sole location
(primary tumour) and the surgeon is able to remove it completely. All
medical investigations reveal the absence of any malignant cells in any
other organs.
This condition does not require any post-operational therapy.

Stage 2: The tumour is more extended, but again remains confined to a sole
location. The removal by the surgeon leaves a minimum residual, which
sometimes may penetrate the spinal cord. Tumour cells can infiltrate the
lymph-nodes which drains the area where the tumour is located.
Only patients aged over 1 year old at the time of diagnosis, with
infiltration of the lymph-nodes or in which the tumour has become "broken
up" during the operation, receive a brief period of chemotherapy in order to
reduce the risk of relapse.

Stage 3: The tumour is confined to the region of origin and there are no
metastases in other locations. It is large in size and/or infiltrates the
surrounding areas and for this reason cannot be completely removed during a
first surgical operation. The lymph-nodes of the area in which the tumour
has its origin are areas of metastases of the primary tumour. In this case,
therapy to try to reduce the tumour is necessary, allowing it then to be
completely removed by the surgeon at a later date.


Stage 4: The tumour, regardless of its dimension and original nature, has
spread its diseased cells to other organs, such as to the bone marrow, the
bone structure itself, the liver and the lymph-nodes, all at a distance from
the location of the primary tumour. This bears witness to its biological
aggressivity and an intense treatment is necessary in order to eliminate the
disease.


Stage 4 S: This stage is a special form of neuroblastoma which occurs during
the first months of the life of a baby, with a particular spread which
involves, above all, the skin and the liver, but not the bones and which can
regress completely in an absolutely spontaneous way. In some cases,
particularly when the involvement of the liver is vast, a helping hand in
making it regress might be necessary by treating it with a brief cycle of
therapy. The primary tumour, often small, can be removed immediately or when
the metastatic lesions have at least partially regressed.
Other important elements as regards to diagnosis other than age, stages and
the genetical characteristics (whether the oncogene MYCN has become modified
or not) is the histology and the results obtained from the therapy.


Neuroblastoma: The tumour is composed exclusively of malignant cells which
are very undifferentiated.

Ganglioneuroblastoma: The tumour is composed of cells which are, once again,
malignant but are less undifferentiated with characteristics which are
similar to the mature cells of the nervous system ganglions; there is also
an abundant periplast, or in other words, a component of sheathed nerve
fibres and tissues interposed between the tumour cells.


Ganglioneuroma: The tumour is composed of completely mature cells, incapable
of forming metastases. In this case, the tumour is benign and only surgery,
and no therapy, is necessary.

Metastasis: Are quite large masses of a variable number of malignant cells
which have invaded the organs and structures of the body, other than that of
the nervous system, of which the primary tumour had its origin. The organs
which are more frequently inflicted are the bone marrow, the bone structure,
the lymph-nodes and the liver.

Complete Remission: Is the situation in which the disease, following
treatment, is no longer detected by either specific tests or diagnostic
examinations using images or biochemical methods. The disappearance of the
disease in some cases is real and lasts, whereas in others, it is only
apparent and the tumour may reappear once again after a certain period.

Relapse: Is the re-growth of the tumour within the location of the primary
tumour or in other areas after treatment or surgery have previously induced
their reduction or disappearance.
This can occur also some months after an apparant complete cure and the
possibilities of cure are very low.


Treatment

At the moment there are three types of treatment against neuroblastoma which
have been well consolidated and experimented; other treatments, more recent
and still under experimentation, shall be outlined later. Below, we briefly
discuss surgery, radiotherapy and chemotherapy.

Surgery
This entails an operation to remove the tumour totally or partially. When
the tumour is removed completely one speaks of a radical operation, which
forms the sole and sufficient therapy required in the cases in which no
metastatic lesions are present. (see Stages).
However, when the operation is limited to removing only a part of the tumour
or only a small fragment, one speaks of a biopsy. Sometimes, a biopsy is
decided upon for diagnostic reasons or to evaluate the impossibility of
removing the entire mass before or during the operation.
In other cases, a surgical operation is decided upon at the moment following
diagnosis and following some cycles of chemotherapy carried out in order to
reduce the tumour mass when it is considered impossibile to remove the
tumour due to its proximity with other vital organs (deferred surgery).
Surgery tends to avoid impairment i.e. the non- removal of organs, but
neverthless sometimes it is a preferred practice to sacrifice a kidney in
order to render surgery radical, since the advantage, at a prognostic and
therapeutic level, is greater than that of the disadvantage of the
detraction of an organ.


Radiotherapy
Is a method by which the tumour cells are destroyed by administering
radiation from outside the body. In the case of neuroblastoma, the
radiotherapy is carried out in a number of ways. Infact, whilst externally
administered radiation does not produce acceptable results, the technique of
administering radiation from within the body, by intravenously introducing a
radioactive isotope (MIBG) offers encouraging results. One uses this
technique because the particular "attraction" that the isotope has for the
tumour cells allows the administering of the radiotherapy to be more precise
and better aimed. The therapy of MIBG requests particular attention in its
preparation as the iodine has to be adminstered orally and is carried out in
special rooms within the department of nuclear medicine because for a couple
of days after the treatment, the patient emits a small quantity of
radiation.

Chemotherapy
Is based on the administering, almost always intravenously, of the so-called
anti-blastic drugs that, using various mechanisms, destroy all types of
cells which multiply rapidly. Amongst these, in addition to the tumour
cells, also healthy cells can become destroyed, for example, blood cells and
those from the gastro-intestinal mucoid.
The anti-blastic drugs are administered for a brief period only, and in
doses which are not elevated, for cases where it is necessary to destroy a
small tumour residue following an operation which has resulted in being
almost radical. When this is not possible, because the tumour is too
extensive or when metastases are present, the therapeutic practice, commonly
used, is that of administering antibiotics at a considerable dosage level
for a brief period (three to four months) in the attempt to reduce it.
Subsequently, after having reduced the primary tumour and its metastases to
a minimum with all the therapeutic practices possible and after having
carried out accurate and specific medical investigations in order to
identify more precisely the quantity of the residual tumour which exists,
the next step is to use very strong doses of antiblastic drugs
(megatherapy), possibly linked to radiotherapy with the MIBG therapy. In
order to overcome the toxic effects of this treatment, it is necessary to
undergo a bone-marrow transplant. Very briefly, this means that before
exposing the child to megatherapy, it is necessary to remove a quantity of
hematopoietical staminal cells (i.e. those which are capable of regenerating
blood cells) from the child. This can be brought about by way of removing a
considerable quantity of bone-marrow using multiple aspirator-needles from
the iliac crest under general anesthetic, or by using repetitive removal of
perifial blood cells (aferesi of perifial staminal cells). These are
progenitric cells of blood elements which will be "saved" by a process of
cyro-preservation (that is to say, freezing them) and, once the megatherapy
is administered to the child, they shall be re-infused intravenously and
spontaneously shall return to the bone-marrow where they will regenerate the
normal cell components of the blood. The collection of perifial blood cells
is possible using growth factors which increase the proliferation and the
circulation of these cells within the perifial areas.

The most frequently pharmaceutical drugs used for neuroblastoma are
Ifosfamide, Adriamicina, Endoxan, Carboplatino, VP16, Thiotepa, Melfalan,
Busulfano amongst others. These drugs, which are administered exclusively
intravenously, are used together in block treatment, with necessary
intervals of non-use so that the organism does not become damaged and has
the time and opportunity to rid itself of the toxic effects. As already
mentioned, the treatment for each individual child affected by neuroblastoma
will be decided on the basis of many controlling factors, i.e. the stage of
the disease, the patient's age, and in some cases, the biological
characteristics (oncogenes, citogenetics) of the tumour and its reaction to
the therapy. Every child, therefore, can be treated in a different way and
this should be explained to you in a calm way, as should the side effects of
the drugs and the precautions to take in order to limit harmful
complications.
At this point, we would like to briefly speak about the more common
side-effects namely, nausea, vomiting, loss of hair, mucosite and midullary
reduction.
Nausea and vomiting are immediate side-effects brought about by the
administering of many antiblastic drugs but, today, there are also several
anti-emetic drugs in existence which are very effective against these
symptoms. It should be remembered that, since every child has his own
particular level of sensitivity, it is opportune that you report accurately,
just how each cycle-phase is coped with.
A particularly difficult side-effect of antiblastic drugs to come to terms
with, for both the family and the child, particularly if he is quite old, is
that of the loss of hair.
This occurs rapidly after the first phase of chemotherapy, but it should be
emphasized that it is a transitional effect which will only last whilst the
chemotherapy is being administered. It is of utmost importance to prepare
the child, cutting the hair short and explaining the facts, but also
necessary, however, for the family to accept the situation; some children
ask to wear a hat, or, on some rare occasions, a wig: whatever, it is
important that this is the child's request and not a "suggestion" of the
parent. Infact, most children affected by this phenomenom, fortunately, are
not even interested in hiding their "little bald heads".
The mucosity of the oral tract is a quite frequent side effect of the
antiblastic drugs and expresses itself, more or less, a couple of days after
the termination of the treatment and lasts for several days; a good oral
hygiene, following the prescribed indications, can help to reduce and
relieve this problem.

Midullary reduction, or rather the reduction of blood cells, is considered
the most important and harmful toxic effect caused by chemotherapy. It is
linked to the toxic damage of the antiblastic drugs upon the progenitor
leukocytes, erythrocytes and the thrombocytes. It is a transitory effect
because our reserve of staminal cells is enormous, but, in some cases, it
can bring about a considerable reduction in the number of blood cells.
The reduction of the leukocytes weakens our defence system against
infections and so it is likely that a high fever accompanies this condition.
It is, therefore, necessary to take some precautions in regards to the food
the child eats and to keep the child a little more "protected" than usual in
order to avoid infections which could result in being very dangerous to the
child's health. Today, however, it is possible, in the case of a prolonged
and severe reduction of the leukocytes, to administer certain factors of
stimuli which induce an increase in the number of leukocytes. They are the
same factors which are used for the collection of the hemapoietic staminal
cells.
Also the thrombocytes, the cells whose purpose is to repair small vascular
damage and to impede bleeding, can become remarkedly reduced, rendering it
necessary to carry out a transfusion of thrombocytes; similarly, for the
same reason, it could also be necessary to carry out a transfusion of the
erythrocytes.

Protocols and Experimental Treatment
At present, all children affected by neuroblastoma who are treated in AEIOP
centres (those consented by the Italian Association of Paediatric Hematology
and Oncology), as in many other European and American centres, undergo
certain "therapeutic protocols", or rather, in accordance with the
characteristics of their illness, are treated with common and collective
definite therapeutic models so that the largest amount of information
possible can be collected together in order to able to gradually modify the
treatment on the basis of consistent data.
The committment presently being undertaken by many groups operating in
centres of various nations takes the form of adopting criteria of common
stages of the illness, in such a way to favour the international exchange of
information and, in some cases trying to define a common European
therapeutic protocol.

In some situations, new pharmaceutical antiblastic drugs are under
experimentation, together with experimenting with new associations, or
different therapeutic models, like differentiated drugs (i.e. those capable
of modifying the biological characteristics of the tumour, transforming it
from malignant to benign) or with immune-stimolants (those capable of
increasing the defence systems of the organism against tumours).
However, whilst we have a large amount of information regarding these drugs,
unfortunately, we have not yet been tried sufficiently for us to be able to
define their true therapeutic potential.
All this and more, shall be discussed with you in an attempt to keep you,
not only well-informed, but to also allow you to be able to give your
consent to the various treatment for your child. We would ask you to
remember that this is a collaboration which we retain to be of utmost
importance in our aim to identify new methods for our struggle against
tumours and to improve the cure of your child and others who are inflicted
with this disease.



Speaking to your child

Knowing how to speak to a child regarding his illness and the future
treatment that he must undergo, together with what approach to use with him
when he shows fear and worries, is always a terrible dilemma and enigma for
most parents with ill children.
Often a parent's initial reaction, and we must stress, a very natural
reaction, is to want to hide the illness from the child, firstly because one
wants to protect him/her from something which just from its name “cancer”
implies a really quite terrifying illness, and in the second place, because
one believes their child is too young to be able to understand the meaning
of the diagnosis. Nevertheless, whatever the age of the child, he will
understand that something is not quite right: that he has been taken into
hospital, that his parents are concerned, and that he is being spoilt more
than usual; in addition, he will be able to hear the conversations of
grown-ups and therefore he himself will also begin to feel worried and
concerned, guessing that something is being kept from him. And so it is be
very important that he is given a suitable explanation which is appropriate
for his age and that the normal everyday education remains the same.
One must remember at this stage that it is you, his parents, who are the
people he knows best and on whom he will rely to give him the trust he
needs.

He will sense, from the reactions of the people around him, that something
difficult to understand, is happening; in hospital he will see other
children who are like him and who are undergoing the same kind of treatment.
It is fundamental that he feels that he can rely totally on you for the
explanations to his worries. We must remember that if he doesn't receive the
trust and support he needs, the risk is to make him feel insecure and this
lack of confidence could show itself in an outwardly aggressive manner and a
regressive infantile behaviour.
We should not underestimate our children's sensitivity - also a very young
child can become depressed and distant. Many parents fear that, to a child,
the knowledge of having cancer can really frighten him and therefore they
try to avoid him hearing the word cancer, not realising that infact he will
come to know of it anyway, by way of hearing other children talking, hearing
adult discussions or even from the television. It is because tumours can be
cured that there are always more and more people who undergo chemotherapy
and almost all of us know someone who has lost their hair due to the
treatment and so it is not something at all costs to hide from a child.
The important thing to realise is that we cannot hide the truth from a
child. It is better that a child knows about his illness from us and from
the doctors treating him, rather than by listening to others who could well
give wrong or distorted information which in the long run could be upsetting
or more worrying. The best way is to simply tell your child that he has a
particular type of tumour which inflicts only children and that the doctors
have the medicine to cure it. The child in this way will receive a
beneficial comfort from your calm and reassured presence and will then worry
much less about the details of the treatment itself.

If the child is older, say pre-adolescent, he will, of course, have a more
adult concept of cancer and it could be more difficult to be sincere, but it
must be remembered that for a child of this age, or an adolescent, it is
however more important for them to be well-informed of the details of their
illness in order to be able to prepare themselves and understand what they
will have to face. If you try to hide certain facts or indeed distort the
facts, the child could certainly remain seriously troubled, losing the vital
trust which he needs to have in you and the doctors, and consequently one
could risk an increase in the difficulties to be faced with the treatment,
together with behavioural problems.
For you as parents it is advised that you talk with the staff of the
department about the questions your child could put to you and to ask the
doctor in charge of your child's treatment to talk to him, deciding together
with a psychologist, just "how" to talk to the child and the best approach
to be adopted.
A child of scholastic age may have heard of the word cancer and therefore it
is of importance that he knows that he is able to talk about his worries and
fears, regarding, for example, certain changes in his appearance, and that
he also feels free to talk about how he should behave at school, together
with knowing what will happen during and after the treatment that he has to
undergo.
Moreover, one must remember that the members of the medical team responsible
for the treatment of your child, the doctors, nurses and psychologists, are
there to talk to both you and your child, whenever necessary.
The pre-adolescent patient, like very small babies, strongly need, to not
only feel your physical presence and support, but to also have a certain
independence which can sometimes mean they may feel the need to speak in
private with the assisting staff or with other children who have been
inflicted with the same illness.


Facing the medical examinations
Whatever the age of your child, the moment in which you really come to terms
with what is happening is at the moment when you must help prepare himself
for the various medical analysis and examinations that he must undergo.
It is somewhat absurd to try to calm your frightened child reassuring him
that it will "not be painful". If this is not the case, your child could
then lose the vital trust that he previously had in you and could become
withdrawn. A better approach is to look for the positive side of a difficult
situation. For example, you could say: "the injection will hurt, but Mummy
will be here by your side to help you to be brave and then afterwards we
will go and play, or go to drink something, or Mummy will read you a story."
To plead with him to be a big boy or to be good doesn't help when he is
frightened, but giving him courage by saying something like “We'll scream
together when they give you the injection" can help.
Sometimes pyschologists and school teachers, using books and suitable toys,
are able to help the child to become familiar with the diagnostic procedures
and to get him to express his feelings through playing.
“Playing hospitals” for example, means firstly creating a protected change
of environment in which the child feels that he is in control of what he is
doing and all that occurs, reducing or eliminating anxiety, and secondly,
introducing familiarity once again into an unknown environment with an aim
to reinstate a possible threatened sense of security. The creativity of the
child will be safeguarded by the hospital teacher who aim is to give the
child ample space to express himself whilst keeping in mind the natural
development of the child. Drawing and artwork, structured and unstructured
activities, and methodical observation are all elements which help to
understand and help a hospitalised child.
A favourite game for the youngest of children is often “the hospital corner’
in the playroom, or for children confined to their beds, a game of "doctors
and nurses" with a teddy bear who is ill like them, and has to have an
injection. This game helps the child to understand the scenario better and
to feel more in control over the things that are happening to him.


You and your family
After you have been in hospital for some days and the routine has become a
little more familiar, it is then when you can turn your thoughts to how you
feel, how to face the situation and how the consequences of everything will
influence you, your family and your way of living.
The first impact and the feelings associated with this, after having been
informed that your child is inflicted with cancer, will probably be the same
as any other person receiving the same terrible shocking news; one of
incredibility, of fear, helplessness, sadness, anger and even a sense of
guilt. It is likely that you will have difficulty in taking in what is being
said to you; many parents describe a similar feeling to that of a very bad
dream hoping to suddently wake up from it and to return to how life was
before. Others may feel actually physically ill due to the shock, and other
may express themselves with anger, saying: “Why has it happened to us? What
have we done to deserve it?”.
It is certainly a profoundly deep-rooted feeling that brings us to think of
this situation as a punishment, but you must be reassured that there is no
correlation between what parents do, the food the child has been given to
eat, his daily habits, the people around him, nor banal accidents whilst
playing - none of these factors cause neuroblastoma: it is certain too that
it cannot be an inherited condition and does not come from any side of the
family nor be inherited by the family. There is also no known evidence which
proves there is a connection between the disease and habits or occurances
during pregnancy.

The incidence of the illness seems more or less stable, whether in regards
to the frequency and where it is found in the world, in so much that we can
say that a certain number of children each year will develop neuroblastoma
and that this number remains approximately constant in the world, regardless
of the environment and race.
Nevertheless, the psychological need to give the blame to someone or
something is always tempting because it is hard to accept a fact when the
cause is unknown. Some parents give the blame to doctors for "not having
discovered the disease in time", whilst many blame themselves for not having
informed a doctor earlier of the first mimimal disturbances or symptoms seen
in the child. But we must remember that this type of cancer is somewhat
"devious" and that the dimension and presence of the metastases are not
influenced by the time taken before diagnosis.
It is important, however, to openly express your doubts to those who are
taking care of your child. Doctors and nurses fully understand that you are
having to face a situation in which you feel lost and out of your depth with
often too little information or more than often distorted information. Above
all, a pschologist may be able to offer help. With his active participation
in sharing the situation with him, for example, at the moment of being told
the diagnosis; helping you, together with your child, in clarifying your
doubts and queries, your anxieties and expectations connected to the
duration of the illness, of the therapy and of the hospitalisation;
assisting you psychologically during the check-ups with the Day Hospital and
helping you in familiarising your child into the hospital environment, or in
the return back home, in the relationships of school, with brothers and
sisters and with friends. The psychologist is to be found in all
departments, in the Marrow Bone Transplant Unit, and in the Day Hospital,
and it is possible to speak with him in the presence of a doctor or
otherwise request to have his help directly by way of a personally sitting,
set up specifically for you. The psycologist is, infact, a relatively new
hospital figure who has been integrated with the medical team and who can
provide professional help for you, your child and your family in this
delicate and often difficult relationship between family members and
hospital staff.

It is advised to have someone to accompany you during the initial meetings
so as to listen to what is being communicated about the disease and its
treatment, so that you are not alone with the stress of understanding and in
facing the responsibility of taking in everything that is said or repeating
it to others.
Another piece of helpful advice is to not repress your feelings. Undoubtedly
both of you will try to hide certain emotions from one another but this is
thought to only make matter worse. Many people wrongly believe that having a
good cry is something to be ashamed of. However, fathers cry just as much as
mothers do when being told about the illness of their child; it must be
stressed that it is not a sign of weakness, neither of potential incapacity
when dealing with the situation, but a natural reaction and a necessary
liberation for both family and friends. Many parents also believe that they
must not show their own anxiety infront of the child, but sometimes you will
find that you are unable to hide your feelings and emotions and your child
will not be so alarmed if you give a simple but honest explanation to him
of: "Mummy is sorry that you are not feeling well."
In addition, speaking with relatives and friends admittedly can be tiring.
It often requires a real effort to repeat and relay back exactly what the
doctors have told you to understandably anxious and enquiring close ones. In
this situation, the presence of one or two understanding relatives or
friends at these meetings can be of great comfort and help. Sometimes, both
parents and the patient can also become quite exhausted by the influx of
visitors and by the constant telephone calls and so, for this reason, many
parents have found it useful to have a family member at home with the sole
task of informing relatives of what is happening and to generally put them
in the picture after having put in a daily call to the hospital. Close
relatives of the family, for example, the grandparents, can also often find
it useful to have a meeting with doctors so as to becomed better informed of
the well-being of the child and to have the same information and
understanding of the course of the illness and the proposed cures: do not
hesitate to ask for this type of meeting.

Siblings
It is only natural that brothers and sisters of the child with the illness
want to see him and know what is happening.
Many parents believe that to see an ill brother or sister in hospital is a
traumatising event. Nevertheless, the imagination of children can be such
that sometimes they imagine something worse than reality and that often
brothers and sisters can be reassured by seeing exactly the place where
their sibling is being taken care of.
It can also be of help in explaining to younger members of the family what
is happening to the ill child by actually showing them the hospital and the
equipment used to treat the child. It is suggested in this case to ask the
nurse if it may be possible to have a "tour" of the department or day
hospital, obviously following the necessary precautions and explaining
beforehand what they are going to see.
Because of their age and despite a most careful explanation, siblings will
naturally be alittle sceptical at the beginning about the things which are
happening.
They will notice that the child has changed, that perhaps he is paler, more
fragile or thinner, that he has lost his hair and that he cannot always play
with them and join in with the activities that previously they enjoyed
together. It is opportune to check on their playing activities, taking part
in them rather than to ask the siblings of the ill child to treat him with
care.
It is also necessary to give a suitable explanation, according to their age,
of neuroblastoma - if possible, one which is similar to that given to the
ill child; the ill child, himself, if he is old enough, could even explain
his illness, the therapy and the new routine to his brother or sister.
Something to bear in mind is that the siblings of the ill child may
sometimes become worried that what is happening to their brother or sister,
could happen to them. They must obviously be reassured that a tumour will
not develop in them and that, in the case that it could happen, they would
be cured and looked after with the same love and attention that their
sibling is receiving. Regarding this point, it should also be stressed to
them that it will not happen either to you or your other relatives.
Occasionally children can feel guilty about the illness of their brother or
sister. They will attach importance, in some way, to the responsibility of
the occurance, perhaps because at times they have shown particular jealously
towards their brother or sister, or they could even be jealous of the ill
sibling, even if you have well-informed and prepared them about the illness
and its treatment. After all, the child in care, has earnt your complete
attention, has probably also received presents and new toys from relatives,
friends and neighbours, and when he returns home, he will have more things
and more attention than before. It will be very difficult for you when you
are tired and worried to find the necessary resources and energy for your
other children, but it will help in avoiding the problem of jealously by
making a special effort to not favour the ill child too much and to find, at
the end of the day, a little slot to exclusively dedicate to your other
children or to give them a reassuring phone call from the hospital and to
remind them how much you love them and how much you are thinking of them.



Home Sweet Home - Returning Home

When the medical diagnostic tests and the first stages of the treatment have
been concluded, it is likely that your child can return home. Despite the
obvious relief of knowing that you can leave the hospital and the good news
of being able to be back with the rest of the family, don't be alarmed that
it could take you a considerable time to pick up the normal daily routine as
before.
Probably, during the recovery period, you began to put much hope into the
fact of seeing your child once again up and about, eating normally and
temporarily forgetting the hospital. One should bear in mind though that all
these things on which you have built up your hopes will, at least at the
beginning, require much patience.

After having been in hospital for a certain period of time, both you and
your child could find some difficulty in returning to the old daily routine
back home. Perhaps you will find it difficult, at the start, to eat and
sleep normally. You may feel the need to avoid the company of relatives and
close ones and try to reproduce the lifestyle that you had in hospital in
order to seek the feeling of security that the hospital offered you. Your
child may be more attached to you than usual and ask you, when you focus
your attention on other family members, if you have forgotten him. Many
parents, who were previously very sure of their way of bringing up their
children and looking after then, all of a sudden become fearful also in
applying the more common of common household rules.
This question of discipine is a rather prickly topic to discuss. Many
parents say that they cannot imagine, even if they are in the conditions to
do so, to shout or punish their ill child and have a tendency to be
super-indulgent to the point of spoiling them, creating in this way an inner
fear in the child of not having been cured.
An ill child should be reminded of the certain limits established by the
parents for him. If he is allowed everything all of a sudden, he shall not
be reassured that his life is proceeding normally, also regarding his
illness and moreover, this will not help him integrate with his siblings and
friends.
On either hospital visits or after hospital visits, where one could expect
changes in mood of character under conditions of great stress, you should
be, for example, neither too tolerating nor show extreme severity or
aggression. Often, on such occasions, the difficulties of behaviour are the
results of fear and insecurity and it could be helpful to talk about this
issue with a psychologist or another member of the medical team.
It is of utmost importance that the child is helped by way of frank and
reassuring explanations.

It is neither a good idea to indulge your child with mountains of presents
on his home return nor to promise expensive or extravagant gifts. However,
if relatives or visitors do want to bring some little something for the ill
child, they must be persuaded to also bring a similar present for the other
children in the family. Moreover, it is debatable whether to induce a child
to expect some sort of present every time he goes to hospital or offering
him a present as compensation for having undergone a visit or some medical
treatment.
.
If the child constantly had his mother by his side during his stay in
hospital, he could well be even more attached to her once home and could be
reluctant to sleep in his own bed. Nevertheless, it is better to convince
him in a kind and caring, but firm manner, reminding him and reassuring him
that the habits in hospital and home are two distinct things and that at
home he has his own clothes, his toys and his own bed. Even if, at the
beginning, he may feel the need to constantly check for the presence of his
mother, calling out for her or going into her room, he will soon take up his
previous routine of being at home, when he knows that the habits which
existed before his illness must be followed also after his illness. Needless
to say, that if you were to concede to special treatment of an ill child,
for example, letting him sleep in his parent's bed, this would undoubtedly
reflect badly in the eyes of the siblings and probably provoke as a
consequence gelosy and other related family problems.

In the case of neuroblastoma, there are no particular restrictions in the
child's diet, neither a need for particular medicine between the various
stays in hospital, and as the the treatment advances, the child often gains
more energy and his appetite together with the joy for the usual previous
activities, between one phase of chemotherapy and another.
Nevertheless, alimentation is often an issue which causes anxiety in many
parents. It is inevitable that your child will have lost weight, first of
all, due to the neuroblastoma itself, and then due to the treatment which
can cause poor health and a lack of appetite, as can a decrease in the
child's physical activities which brings about a reduced expediture of
energy.
Some children do not regain their appetites as before, at least not until
the whole course of chemotherapy has been concluded. It is, however, quite
natural to want to feed your child up; to want to prepare nutritious meals
and see your child eat them can sometimes be very positive but remember that
it can also become counterproductive if every mealtime becomes a fierce
battleground in trying to coax him to eat "just one more spoonful".

Needless to say one should not be too rigid in this phase and try to offer
your child what he wants and to not feel offended if he tries only little
amounts at a time.
Provided that the doctors are not concerned about a particularly excessive
weight loss, a fluctuating appetite and often a prolonged loss of appetite
during treatment should be accepted as quite normal. It could be of help to
consult a hospital dietician who will be able to suggest various ways to
prepare a nutritious diet or to advise supplementary foods, and in the case
where the main treatment is given in hospital, added nutrients or possibly,
the Parental Total Nutrition (NPT) can be provided by way of the Broviac
catheter.


Returning to normality

There is a strong but natural tendency to become over-protective with an ill
child.
However, you have probably been informed by the medical team responsible for
the child's treatment that you can allow him to do all that he wants and
feels up to doing. Infact, it is a positive thing to encourage him in,
little by little, as he becomes stronger and healthier to regain his
previous capabilities and habits, bearing in mind to keep an eye on him,
particularly in regards to a lowered defence system and/or if he has a
Broviac catheter. On the whole, the child will draw once again from the
benefits of mixing with other children and joining in their activities,
provided that any specific precautions which need to be considered are
followed.
All this can come about mainly by playing outside with friends, or for an
older child, returning to doing certain activities, for example, with the
Scouts or going out on some school trip.
Many of the desires to protect your child could have come from the
suggestions given by the medical team to keep him away from possible sources
of infection and for this reason, many parents are worried for the
consequences of letting their child mix with other children. Obviously, you
must follow the indications given by your pediatrician.

Generally speaking, common sense should be followed. Naturally, one
shouldn't allow their child to play in a group of children where almost
every child has a cold. It is more advisable and cannot harm him if one
invites a neighbour's child in or if you take your child out to the park
with a friend. Certainly you have been informed that measles and chicken pox
are infectious; your child must avoid, if possible, these two diseases and
even though it is not easy, one should try to have a helping hand from the
support of neighbours, friends, teachers and social assistants in order to
be told if children in the community have contracted any such infectious
diseases. This goes without saying also in regards to the child's teachers
at school. Obviously, it shouldn't become a disturbance, but one must be
cautious with an ill child if, in the brother's or sister's class, there is
a child who has contracted, for example, measles or chicken pox.
In the case that your child has come in contact with such an illness, do not
panic, but immediately inform your doctor, so that it is possible to give a
special injection to lessen the attack or even prevent it completely;
telephone your hospital or your Paediatrician to let them know; however, in
the case that your child does contract an infectious disease, do not take
him to hospital without warning the hospital beforehand in order to avoid
passing it to other children.

Please note that a child is unable to have normal vaccinations while under
chemotherapy, and so it is crucial that you inform the paediatrician or the
National Health Unit (ASL) responsible in the case where a vaccination is
needed.

School
Returning to school or nursery school can pose yet another problem for a
child who has been diagnosed as having neuroblastoma. However, it is
important to remember that your child has the right and the need to come
into normal contact with his friends.
One shouldn't believe that, at 5 years old, a regular and committed
schooling is not of importance, or that at 10 years old, the educational
activities at school are top priorities above all others, because for a
child who already feels different and somewhat isolated from his friends due
to spells in hospital and often lengthy periods of treatment, it is very
important that he once again takes up his normal activities and intellectual
stimulation as soon as possible, in such a way that he can get back into a
routine with his friends and school without having the added disadvantage of
having lost precious studying time.
Regular contact with his school friends or nursery school companions can be
of great help even whilst he is unable to actually attend school full time
and the hospital teacher, who will have contact with the school or the
nursery school, will be able to obtain suitable material for doing his
homework and activities from home.

A helpful and good collaboration between the two sets of teachers (the
hospital teacher and the regular school teacher) would be something to hope
for, so that the child does not feel "different" at the moment when he
returns amongst his school friends (it is not enough just to exchange
opinions on the child or the curriculum: it would be useful to take
photographs, draw pictures of similar themes, of specific moments, events of
the day etc which are common to all children).
If returning to school for a certain period is really impossibile, then one
should see to it that the local education authorities set to organising some
home visits by a teacher, a service which should be taken advantage of.
Even if the child cannot attend school full time, perhaps a couple of half
days could be considered or to attend those lessons which are of particular
interest or to take part in some special activity during the week. In order
to help the re-integration into the school, it is necessary to get into
contact with the Head or a teacher of the school.
Many schools provide a sympathetic and constructive approach for the
particular problems met by children affected with cancer, but do not be
surprised if they have never heard about neuroblastoma and if they require
your guidance regarding the limits and possibilities of your child. In
addition, your child will have to also "teach" his classmates something
about his illness and its therapy and the reason he has lost his hair, etc
in order to prevent negative behaviour which is usually generated by
ignorance and fear.
Once the children know why one of their friends seems different, they will
often become protective and understanding.

If your child is very small, you will find yourself having to explain to the
other parents and neighbours about what has happened: some alarming
prejudices and old wives' tales about cancer are often the cause of great
fear and ignorance for which reason certain people could be embarrassed to
give a help hand. A simple explanation and reassurance of the condition not
being infectious normally brings about the change, making them become
affectionate and willing to offer their help.



Asking for help

You have probably received much appreciated help from close friends but have
discovered that some people are too imbarrassed or reticent to offer help
for fear of interfering. However, if asked, they will no doubt show
sensitivity and will be capable of doing something to help you and your
child. You should try not to be too proud to ask for help which could make
both your life and the organisation of the family easier. If, for example,
you need to take your other children to school or you need a lift to the
hospital, or some time to do the shopping, ask for help; if someone nearby
offers their help, do give them the opportunity to show their solidarity. In
addition, use the services of your Paediatrician, who probably will know you
well and, if you feel that it could be of help, do not hesitate to ask help
from a local social assistant. They could well become a valuable friend and
you could rely on them to help you sort out certain red tape practices or
financial contributions with the aim of helping your child. At the same
time, in this way, you will feel a relief in the reduction of the things you
have to do.
Even though much has been said to arrive at a point of having a more or less
normal life, certainly every parent can affirm that life will never quite
return to how it was before. In the back of your mind there is always the
illness and sometimes it will be quite a battle to manage to look after all
the family, concentrate on the hospital visits, the blood tests, the visits
to the clinic etc.
If it is at all possible, it is necessary to have moments “to switch off
completely”, to have time for yourself and to dedicate some time to your
partner and to your close family and friends. It is also important to find
time to relax a little. Even if your child is ill, no-one expects you to
remain all the time in a sad and sorry state and a "break" from your child
can be good, not only for you but also for your child, so that at other
times, when you have regained your strength and positive attitude, you can
be even more united, stronger and more willing. Even when you are in
hospital with your child, you mustn't feel guilty about going for a coffee
or a meal or simply leaving the hospital so as to have a break or to get
some fresh air. There is no reason on earth why you cannot leave your child
in the safe and caring hands of an efficient babysitter or with a relative
for a brief period.
In line with traditions, family events and celebrations where possible
should continue and everyone should take part in parties and anniversaries,
including an ill child.
Some parents understandably worry that their marriage can succumb to a
certain amount of tension due to the stress and in fact there is an increase
in separations in families where a child has cancer. Nevertheless, what is
likely is that marriages which are already unstable can experience an
acceleration towards its breakdown when under the effects of stress; as a
compensation, though, many couples who are concerned and worried for their
marriage actually feel stronger due to the necessity of sharing their
anxieties for their ill child.
Probably this is the first time, as parents, that you do not have the total
control over everything which happens to your child.
Even though the person who is responsible for the care of the child does his
utmost to keep you informed with the best methods about the illness, the
cures, the treatment and the child's progress etc, the sense of desperation
you can feel really can be very frustrating and also cause even a loss of
confidence in yourself. There may even be occasions in which you may feel so
worried, depressed and tired that you begin to ask yourself if you are
giving way or going mad.
Some parents describe moments of panic, nightmares and almost all, at a
certain point, believe in the possibility of the death of their child. At
times, many admit to thinking thoughts of details and events relative to
their child's imaginary death.
These thoughts may well be involuntary and parents often fear to becoming in
some way wicked. Instead it is not like this: these images are ways to face
the worst that could happen in an attempt to confront the possibilities of
the future.
Moreoever, it is very common for parents to see wherever they glance, e.g.
on TV, in newspapers, etc, something on cancer or on children and to feel
extremely distressed about this. This kind of feeling and an infinite number
of other thoughts and fears can be astonishing and it is only natural that
you try to forget about them. If you fear that you are the only person with
such thoughts, you could even try to hide them as part of your
vulnerability. Instead, it is advised to trust in someone: your husband or
another relative, a friend, the person looking after your child; in this way
you will feel comforted and reasurred and discover that other people also
have had very similar thoughts and fears as your own and that they are just
all part and parcel of the normal process of adapting to the news that your
child is ill and to the stress and tiredness of looking after a child with
such an illness.

Obviously, hearing it be said that these thoughts are very common will not
so easity reduce the anxiety that these thoughts bring; but it does rather
get you to see them as something more “normal". The people who are treating
your child at present, know only full well that you think in this way and
they understand that many aspects of the disease of your child and the
treatment can worry you and that as a consequence you may feel depressed or
exhausted, and that you may need to talk about yourselves and that you may
find it of help to talk with hospital volunteers or with a psychologist;
nobody will judge you or your capability as parents or think that you child
is one of those who has special problems or difficulties. Each one of the
medical team has his own particular area of experience and everyone is ready
to share it with you in an attempt to help your child and family as much as
possible and in any way possible. This subdivision of experience is
particularly important, whether before or during the treatment of your
child. Here, unfortunately, we don't have the appropriate space to go into
more detail of the many reactions, fears, anxieties, not forgetting the joy
and satisfaction, that many parents feel while their child is being cured
either at home or in hospital; but you know your child better than anyone
else and the medical staff who have the responsibility of the treatment need
to share your knowledge, your responses, preferences and character of your
child and whatever other problem which could arise.


After treatment

The months of therapy are a long and anxious-making period of time but the
relief in knowing that you are about to conclude the treatment can be
contradicted by the continuous fear of the illness taking hold again and
your child having a relapse. Also if the treatment has proved difficult and
life in the hospital is tiring and a problem, the treatment has probably
created some sort of reassurance for you as parents due to the fact that it
has uprooted the illness or that it has been completely and firmly destroyed
it following the medicine administered. Especially if your child has, like
many others, showed a speedy recovery and a quick return to a normal state
of health during the course of the chemotherapy, it is not uncommon to feel
that, since the drugs seem to being doing your child good, the withdrawal of
them, means leaving things in the hands of providence. To reassure yourself,
do clarify with your doctor the reason why he has retained it better to stop
the treatment after a certain period of time.

Parents describe the moment of concluding the treatment as a moment in which
the safefy net is removed and therefore a moment of entering into a period
of major uncertainty. Some who managed to find the necessary courage and
high morale during the treatment period, find themselves surprised to now
feel depressed. They often describe their feeling like those of a father who
said "my daughter now is perfectly well, seems cured but we have to wait
another 5 years before this can be confirmed - it is like living with a time
bomb ……...”
This uncertainty can be however alleviated by regular check-ups that your
child will undergo. The visits and tests carried out may well bring some
anxiety for the days in which you are awaiting results, but one must keep in
mind that there will also be the reassurance up to when the next tests are
to be carried out. Your natural instinct is probably as sensitive as the
ultrascan or blood tests in as much as telling you whether your child is
getting better. A good appetite, a good mood and high energy levels are all
reliable indicators that things are going well. At this point, you will feel
less frustrated and gladdened by your child's health day after day.
If you should feel worried, try applying the same rules as to when your
child was having his therapy. You could put yourself in contact with the
hospital, ask for explanations and plan some check-ups for him.
Infact even though, like any other child, he could succumb to the normal
moments of being ill, it is only natural that nobody will be able to
reassure you that a high temperature or stomach upset is only a passing bug.

If your child shows once again signs and symptoms of neuroblastoma your
anxiety and your suffering shall be completely shared and received. There
will not be protocols for a second treatment, but the best therapy for your
child must be totally discussed and planned on an individual basis, as was
done during the previous phases. There are many medicines and procedures
which can be suggested and there are always finalised treatments for
relieving the symptoms and discomforts that your child could have.

Conclusions

This write up does not have the intention of coming between you and the
curing medical team but is to be used as a useful tool, not only for
parents, but for everyone who wants to understand what neuroblastoma is.
By way of these notes, we have tried to give you a helping hand in becoming
more aware of the illness so that you can face this challenge in your life
in a better way and to help all of us in understanding this illness in order
to defeat it and to assist everyone who is involved or comes into contact
with it.






This write-up on neuroblastoma which uses several “examples”, a “glossary”
and discusses a variety of aspects of the illness neuroblastoma, intends to
provide comprehensive information to, not only parents and relatives of
children with this disease, but also to anyone who wants to become more
informed about the disease so that they may be able to confront it with more
awareness.
We should like to emphasis that these notes are not claiming to answer all
questions and will certainly not substitute the vital daily dialogue between
parents and the medical team, but by reading our notes, we would like to
think that you will then be encouraged to seek the further information that
you require from both doctors and nurses.

&Mac183; General details on Neuroblastoma
&Mac183; Diagnosis
&Mac183; The stages of Neuroblastoma
&Mac183; Treatment
&Mac183; How to speak to your child
&Mac183; Home Sweet Home - returning home
&Mac183; Asking for help
&Mac183; After treatment
&Mac183; Conclusion


How Neuroblastoma presents itself

Example 1.
Mario, a newborn baby of 20 days, has had a somewhat swollen abdomen since
birth and for a couple of days, small subcutaneous “nodules" have been
observed. For this reason, the Paediatrician has advised his admittance to
hospital. Blood tests reveal only a slight condition of “anaemia”, but on
carrying-out an ultra-scan of the abdomen, an unusually large liver is shown
up with small rounded nodulary lumps and an enlarged right suprarene.
Several days after being admitted to hospital, a biopsy is carried out on
the subcutaneous nodule and the histological results prove it to be
neuroblastoma.
The precocious age and the presence of multiple centres of infection in the
liver clearly define a very particular form of neuroblastoma, the so-called
4-S stage (S standing for special). This “special” particularity comes from
the fact that the tumour lesions, after a growth phase lasting from a couple
of weeks to several months, in the case of 8 patients out of 10, tend to
regress spontaneously, that is to say, without any need for therapy.

Example 2.
Maria is a 5 year old girl who has suffered a fractured arm in a fall from
her bicycle. Since the fracture is put to rights under a general
anaesthetic, a radiogram of her chest is carried out, the results of which
are quite surprising because a rounded shaded area near to the vertebral
column is discovered. Subsequently, an ultascan (TAC) of the chest detects
an oval-shaped mass, with distinct limits, well-defined by large vessels,
from the lungs and heart.
In the urine an abnormal quantity of vanilmandelic acid, a substance which
derives from adrenaline and which is produced by the sympathetic nervous
system and also from tumours where it originates, is calculated (in fact, it
is known that neuroblastoma comes from residues that have not been correctly
developed by the sympathetic nervous system).
It is then decided to intervene with a surgical operation in order to remove
the mass completely. During the operation, no lymph-nodes of particular
pathology are found by the surgeon and 4 medullary aspirations and 2 bone
biopsies are carried out in order to exclude the possibility of infiltration
of the tumour into the bone marrow.
The histological results prove that it is a neuroblastoma which shows
aspects of maturation (ganglioneuroblastoma).
The complete removal of the tumour and the absence of the metastasis give a
definition of Phase 1, that is to say, a localized tumour, for which no
other therapy is necessary, but only periodic controls over 3 years.

Example 3.
Mario is a toddler of 2 and a half years old who has always shown good
health and has grown at a regular rate, but in about the past two or three
months, has shown a certain loss in appetite and vague and irregular stomach
ache. The Paediatrician has visited him quite thoroughly, on more than one
occasion, without finding anything unusual; however, in the last couple of
days, the child has become very palid, irritable and seems to climb steps
with some difficulty. The parents, becoming anxious about this, have brought
him to the hospital.

>From the clinical tests carried out, a small mass within the left abdominal
area is found and a reduced strength in the inferiore limbs is noted. An
abdominal RMN is carried out which shows a retro-peritonale mass on the left
para-vertebrale with lymph eonomegalie surrounding it and a small branching
of this mass into the speco vertebra which compresses the spinal cord. The
reduced strength in the inferior limbs worsens in the space of only a few
hours and it is decided to undergo surgery with a laminotomia operation to
take the pressure off the spinal cord and to carry out an histological
examination of the tumour which has infiltrated into the vertebral canal.
During the operation, a vascular catheter is put into position. The
diagnosis of the neuroblastoma is defined as stage 3 with spinal epidural
compression (the aspirations and the bone marrow biopsies carried out during
the operation give negative results, as does the skeletral radiogram carried
out a couple of days later). The child then undergoes several cycles of
chemotherapy, and the mobility of the limbs are recovered and the abdominal
mass becomes reduced in volume whilst the linfoadenomegalie disappears
completely so that after some months of his admittance into hospital, the
doctors carry out another operation in order to remove the abdominal mass
completely. No other treatment is necessary but the child will receive
regular check-ups in order to also identify early on any such possible
relapse or alteration in the vertebral column linked to the first operation.


Example 4.
Maria is a child of 4 years old who has had a fever for several weeks, which
has, during short periods, been also rather high with vague and migrating
aches in her bones. Antibiotics have not changed this condition. The
Paediatrician, even though blood tests have shown to be normal, suspecting
that it could be “rheumatic fever” asks for the child to be admitted to a
University Hospital Clinic. Once again, blood tests prove normal, but the
fever and aches persist and so the doctors carry out a midullary withdrawal,
suspecting that it could be a form of leukemia. This examination reveals
however, typical neuroblastoma cells and a further immunological
investigation on these, with monoclonal antibodies, confirms the diagnosis.
The next step is to search for the primary tumour, but the ultrascan of the
abdomen and the radiograph of the chest gives negative results. Then a
radiogram with MIBG is carried out which shows up various bone metastases
and a small hyper retro-vesicular ipercaptante retrovescicale lesion. The
ultrascan (TAC) of the abdomen, centred on the pelvic region, reveals a
pre-sacral mass. The diagnosis is neuroblastoma, stage 4. The child is
operated on in order to remove the primary tumour, to confirm the
histological diagnosis and to carry out the molecular biological
examinations (oncogenes, citogenetic) and a central vascular catheter is put
into place.
Immediately after, chemotherapy is begun, the aches and fever disappear and,
after several cycles, the cardiogram with MIBG is carried out once more,
together with midullary withdrawals and a TAC of the abdomen. As all the
tests carried out are negative, the child is considered to be in remission
but the treatment cannot be interrupted because the illness has taken hold
and is practically certain: it is necessary to resort to a very strong
treatment of chemotherapy which requests the extracting of perifial staminal
cells or explant of the bone marrow, and successively, a block treatment of
chemotherapy at a very much higher dosage than the previous, followed by the
re-infusion of that cryo-preserved. Having concluded this period of toxicity
of mass therapy, thanks also to the use of hematopoietic factors of growth,
the child is able to be discharged from the hospital.
Now it is necessary to repeat an extensive evalution of the state of the
disease and return periodically in order to control the illness and to
identify any possible late effects of the chemotherapy carried out.


General details on Neuroblastoma

Neuroblastoma represents about 10% of tumours found in children and is the
third most common tumour after leukemia and brain tumours. Around 110 new
cases of neuroblastoma are diagnosed yearly in our country.
Neuroblastoma occurs where cells of the sympathetic nervous system are
situated and therefore can be found in the adrenal cortex system and in
anyone of the sympathetic nervous system glands in the neck, chest, abdomen
and pelvis, even if, usually, the larger part of neuroblastoma is more
frequently found in the abdomen and, in particular, in the adrenal system
surrene.
The symptoms manifested by Neuroblastoma are varied, some being inspecific
and difficult to pinpoint, like fever, loss of strength, pallor, vomiting,
and mood changes; others depend on where the primary tumour is situated:
coughing and difficulty in breathing, when the tumour is in the chest,
stomach pains or a swelling of the abdomen, when it is in the abdomen.

As with other tumours to be found in children, parents are often astonished
that neither they nor their Paediatrician have realised before, but tumours
are a rare event in children and, unfortunately, the symptoms are vague and
lead one to think of other illnesses, also banal illnesses which are very
much more frequent. In some cases, the discovery of a tumour can occur quite
by chance, for example, during a simple check-up or by way of investigating
the possibility of another illness. It should also be mentioned, however,
that in the case of neuroblastoma, a early diagnosis does not always mean
less spread of the disease or more possibility of curing it.


Diagnosis

The diagnosis of neuroblastoma is confirmed and finalised in a specialised
centre, with indispensible tests and investigations for planning the most
suitable treatment and cure.
A bone radiogram is carried out by injecting a radioactive isotope into the
blood which becomes absorbed by any parts of the bone where a lesion might
be present which requires a bone repair, as in the case of a possible
metastasis. In the case of a radiogram with MIBG, a substance, guanidine, is
injected. This substance is connected to radioactive iodine and becomes
caught specifically by the neuroblastoma cells, identifying the presence of
either the tumour or its metastases.
In the images which one obtains from this investigation, the mass of tumour
cells are seen as "luminous points". This is neither a painful nor dangerous
examination which lasts at least 20 minutes and requires a specific
preparation of administering the iodine (in the form of Lugol drops) orally
in order to avoid the radioactive iodine contained in the MIBG becoming
absorbed by the thyroid which could then alter the quality of the results of
the examination. The Lugol solution is very bitter but can be rendered
acceptable by diluting it with water in a plastic teaspoon. This test is of
utmost importance because it gives us the precise information of the
location and extension of the tumour, if not also, though less precisely,
the tumour's size.
It should be stressed that the carrying out of these investigations are not
harmful nor painful and can be repeated without causing any harm. The TAC
and RMN require nevertheless a state of immobility for quite a long time and
often are carried out under sedation or general anaesthetic.
Another fundamental stage in the study of the spread of the tumour is that
of a medullary evaluation which consists of carrying out of one or two bone
biopsies, that is the withdrawal of bone and bone marrow fragments using a
high calibre needle in order to exclude the presence of tumour cells. This
evaluation is carried out under general anaesthetic, usually following a
surgical operation or after the positioning of a vascular catheter. Such an
investigation could be repeated during the treatment so as to give a clear
picture of the effect of the therapy. If the evaluation is limited to only
one bone withdrawal, then in this case it may be carried out under a simple
local anaesthetic.

Sometimes, it could be necessary to resort to particular investigations or
to repeat some due to unsatisfactory first results. Other times, the results
of the tests may require more time than first expected, bringing about a
delay in the diagnostic time and creating anxiety and even dissatisfaction.
For the family, these days of waiting are, of course, difficult moments,
even if the staff who is looking after the child is most willing to discuss
any problems which could arise, but it is important to remember how
essential it is to acquire, from the beginning, all the results which could
then influence the medical team's judgement of the extention of the illness
and to consequently decide the best way forward for the most suitable cure.
For the diagnosis, it may be necessary to carry out a surgical operation on
the tumour. In some cases, an operation will eliminate the tumour completely
with the possibility of cure without resorting to therapy, being higher than
90%. More frequently, however, is the case of the operation being limited to
removing only a small part of the tumour so as to gain important information
on its histological-biological characteristics (for example, the presence of
a genetical anomaly).
When the illness requires treatment with anti-tumour drugs, it is necessary
to put a central venous catheter in place, i.e. the insertion of a very
little thin and soft rubber tube which remains in a large blood vessel for
months, allowing one to carry out, without pain, all the necessary
examinations and treatments which would normally require repetitive venous
injections and a long period of immobility in the child.


The Stages of Neuroblastoma

At the end of the investigations mentioned above and often only after a
surgical operation, it shall be possible to obtain a clear and precise
picture of the most suitable treatment needed. The type of therapy and the
prognostic verdict of the illness depends, on the whole, on two factors:
one, the age of the child (children of under one year at the time of
diagnosis have, generally speaking, a more favourable prognosis) and two,
the extent to which the disease has spread, or rather, the study of the
tumour itself.

Stage 1: The tumour is normally small and confined to a sole location
(primary tumour) and the surgeon is able to remove it completely. All
medical investigations reveal the absence of any malignant cells in any
other organs.
This condition does not require any post-operational therapy.

Stage 2: The tumour is more extended, but again remains confined to a sole
location. The removal by the surgeon leaves a minimum residual, which
sometimes may penetrate the spinal cord. Tumour cells can infiltrate the
lymph-nodes which drains the area where the tumour is located.
Only patients aged over 1 year old at the time of diagnosis, with
infiltration of the lymph-nodes or in which the tumour has become "broken
up" during the operation, receive a brief period of chemotherapy in order to
reduce the risk of relapse.

Stage 3: The tumour is confined to the region of origin and there are no
metastases in other locations. It is large in size and/or infiltrates the
surrounding areas and for this reason cannot be completely removed during a
first surgical operation. The lymph-nodes of the area in which the tumour
has its origin are areas of metastases of the primary tumour. In this case,
therapy to try to reduce the tumour is necessary, allowing it then to be
completely removed by the surgeon at a later date.


Stage 4: The tumour, regardless of its dimension and original nature, has
spread its diseased cells to other organs, such as to the bone marrow, the
bone structure itself, the liver and the lymph-nodes, all at a distance from
the location of the primary tumour. This bears witness to its biological
aggressivity and an intense treatment is necessary in order to eliminate the
disease.


Stage 4 S: This stage is a special form of neuroblastoma which occurs during
the first months of the life of a baby, with a particular spread which
involves, above all, the skin and the liver, but not the bones and which can
regress completely in an absolutely spontaneous way. In some cases,
particularly when the involvement of the liver is vast, a helping hand in
making it regress might be necessary by treating it with a brief cycle of
therapy. The primary tumour, often small, can be removed immediately or when
the metastatic lesions have at least partially regressed.
Other important elements as regards to diagnosis other than age, stages and
the genetical characteristics (whether the oncogene MYCN has become modified
or not) is the histology and the results obtained from the therapy.


Neuroblastoma: The tumour is composed exclusively of malignant cells which
are very undifferentiated.

Ganglioneuroblastoma: The tumour is composed of cells which are, once again,
malignant but are less undifferentiated with characteristics which are
similar to the mature cells of the nervous system ganglions; there is also
an abundant periplast, or in other words, a component of sheathed nerve
fibres and tissues interposed between the tumour cells.


Ganglioneuroma: The tumour is composed of completely mature cells, incapable
of forming metastases. In this case, the tumour is benign and only surgery,
and no therapy, is necessary.

Metastasis: Are quite large masses of a variable number of malignant cells
which have invaded the organs and structures of the body, other than that of
the nervous system, of which the primary tumour had its origin. The organs
which are more frequently inflicted are the bone marrow, the bone structure,
the lymph-nodes and the liver.

Complete Remission: Is the situation in which the disease, following
treatment, is no longer detected by either specific tests or diagnostic
examinations using images or biochemical methods. The disappearance of the
disease in some cases is real and lasts, whereas in others, it is only
apparent and the tumour may reappear once again after a certain period.

Relapse: Is the re-growth of the tumour within the location of the primary
tumour or in other areas after treatment or surgery have previously induced
their reduction or disappearance.
This can occur also some months after an apparant complete cure and the
possibilities of cure are very low.


Treatment

At the moment there are three types of treatment against neuroblastoma which
have been well consolidated and experimented; other treatments, more recent
and still under experimentation, shall be outlined later. Below, we briefly
discuss surgery, radiotherapy and chemotherapy.

Surgery
This entails an operation to remove the tumour totally or partially. When
the tumour is removed completely one speaks of a radical operation, which
forms the sole and sufficient therapy required in the cases in which no
metastatic lesions are present. (see Stages).
However, when the operation is limited to removing only a part of the tumour
or only a small fragment, one speaks of a biopsy. Sometimes, a biopsy is
decided upon for diagnostic reasons or to evaluate the impossibility of
removing the entire mass before or during the operation.
In other cases, a surgical operation is decided upon at the moment following
diagnosis and following some cycles of chemotherapy carried out in order to
reduce the tumour mass when it is considered impossibile to remove the
tumour due to its proximity with other vital organs (deferred surgery).
Surgery tends to avoid impairment i.e. the non- removal of organs, but
neverthless sometimes it is a preferred practice to sacrifice a kidney in
order to render surgery radical, since the advantage, at a prognostic and
therapeutic level, is greater than that of the disadvantage of the
detraction of an organ.


Radiotherapy
Is a method by which the tumour cells are destroyed by administering
radiation from outside the body. In the case of neuroblastoma, the
radiotherapy is carried out in a number of ways. Infact, whilst externally
administered radiation does not produce acceptable results, the technique of
administering radiation from within the body, by intravenously introducing a
radioactive isotope (MIBG) offers encouraging results. One uses this
technique because the particular "attraction" that the isotope has for the
tumour cells allows the administering of the radiotherapy to be more precise
and better aimed. The therapy of MIBG requests particular attention in its
preparation as the iodine has to be adminstered orally and is carried out in
special rooms within the department of nuclear medicine because for a couple
of days after the treatment, the patient emits a small quantity of
radiation.

Chemotherapy
Is based on the administering, almost always intravenously, of the so-called
anti-blastic drugs that, using various mechanisms, destroy all types of
cells which multiply rapidly. Amongst these, in addition to the tumour
cells, also healthy cells can become destroyed, for example, blood cells and
those from the gastro-intestinal mucoid.
The anti-blastic drugs are administered for a brief period only, and in
doses which are not elevated, for cases where it is necessary to destroy a
small tumour residue following an operation which has resulted in being
almost radical. When this is not possible, because the tumour is too
extensive or when metastases are present, the therapeutic practice, commonly
used, is that of administering antibiotics at a considerable dosage level
for a brief period (three to four months) in the attempt to reduce it.
Subsequently, after having reduced the primary tumour and its metastases to
a minimum with all the therapeutic practices possible and after having
carried out accurate and specific medical investigations in order to
identify more precisely the quantity of the residual tumour which exists,
the next step is to use very strong doses of antiblastic drugs
(megatherapy), possibly linked to radiotherapy with the MIBG therapy. In
order to overcome the toxic effects of this treatment, it is necessary to
undergo a bone-marrow transplant. Very briefly, this means that before
exposing the child to megatherapy, it is necessary to remove a quantity of
hematopoietical staminal cells (i.e. those which are capable of regenerating
blood cells) from the child. This can be brought about by way of removing a
considerable quantity of bone-marrow using multiple aspirator-needles from
the iliac crest under general anesthetic, or by using repetitive removal of
perifial blood cells (aferesi of perifial staminal cells). These are
progenitric cells of blood elements which will be "saved" by a process of
cyro-preservation (that is to say, freezing them) and, once the megatherapy
is administered to the child, they shall be re-infused intravenously and
spontaneously shall return to the bone-marrow where they will regenerate the
normal cell components of the blood. The collection of perifial blood cells
is possible using growth factors which increase the proliferation and the
circulation of these cells within the perifial areas.

The most frequently pharmaceutical drugs used for neuroblastoma are
Ifosfamide, Adriamicina, Endoxan, Carboplatino, VP16, Thiotepa, Melfalan,
Busulfano amongst others. These drugs, which are administered exclusively
intravenously, are used together in block treatment, with necessary
intervals of non-use so that the organism does not become damaged and has
the time and opportunity to rid itself of the toxic effects. As already
mentioned, the treatment for each individual child affected by neuroblastoma
will be decided on the basis of many controlling factors, i.e. the stage of
the disease, the patient's age, and in some cases, the biological
characteristics (oncogenes, citogenetics) of the tumour and its reaction to
the therapy. Every child, therefore, can be treated in a different way and
this should be explained to you in a calm way, as should the side effects of
the drugs and the precautions to take in order to limit harmful
complications.
At this point, we would like to briefly speak about the more common
side-effects namely, nausea, vomiting, loss of hair, mucosite and midullary
reduction.
Nausea and vomiting are immediate side-effects brought about by the
administering of many antiblastic drugs but, today, there are also several
anti-emetic drugs in existence which are very effective against these
symptoms. It should be remembered that, since every child has his own
particular level of sensitivity, it is opportune that you report accurately,
just how each cycle-phase is coped with.
A particularly difficult side-effect of antiblastic drugs to come to terms
with, for both the family and the child, particularly if he is quite old, is
that of the loss of hair.
This occurs rapidly after the first phase of chemotherapy, but it should be
emphasized that it is a transitional effect which will only last whilst the
chemotherapy is being administered. It is of utmost importance to prepare
the child, cutting the hair short and explaining the facts, but also
necessary, however, for the family to accept the situation; some children
ask to wear a hat, or, on some rare occasions, a wig: whatever, it is
important that this is the child's request and not a "suggestion" of the
parent. Infact, most children affected by this phenomenom, fortunately, are
not even interested in hiding their "little bald heads".
The mucosity of the oral tract is a quite frequent side effect of the
antiblastic drugs and expresses itself, more or less, a couple of days after
the termination of the treatment and lasts for several days; a good oral
hygiene, following the prescribed indications, can help to reduce and
relieve this problem.

Midullary reduction, or rather the reduction of blood cells, is considered
the most important and harmful toxic effect caused by chemotherapy. It is
linked to the toxic damage of the antiblastic drugs upon the progenitor
leukocytes, erythrocytes and the thrombocytes. It is a transitory effect
because our reserve of staminal cells is enormous, but, in some cases, it
can bring about a considerable reduction in the number of blood cells.
The reduction of the leukocytes weakens our defence system against
infections and so it is likely that a high fever accompanies this condition.
It is, therefore, necessary to take some precautions in regards to the food
the child eats and to keep the child a little more "protected" than usual in
order to avoid infections which could result in being very dangerous to the
child's health. Today, however, it is possible, in the case of a prolonged
and severe reduction of the leukocytes, to administer certain factors of
stimuli which induce an increase in the number of leukocytes. They are the
same factors which are used for the collection of the hemapoietic staminal
cells.
Also the thrombocytes, the cells whose purpose is to repair small vascular
damage and to impede bleeding, can become remarkedly reduced, rendering it
necessary to carry out a transfusion of thrombocytes; similarly, for the
same reason, it could also be necessary to carry out a transfusion of the
erythrocytes.

Protocols and Experimental Treatment
At present, all children affected by neuroblastoma who are treated in AEIOP
centres (those consented by the Italian Association of Paediatric Hematology
and Oncology), as in many other European and American centres, undergo
certain "therapeutic protocols", or rather, in accordance with the
characteristics of their illness, are treated with common and collective
definite therapeutic models so that the largest amount of information
possible can be collected together in order to able to gradually modify the
treatment on the basis of consistent data.
The committment presently being undertaken by many groups operating in
centres of various nations takes the form of adopting criteria of common
stages of the illness, in such a way to favour the international exchange of
information and, in some cases trying to define a common European
therapeutic protocol.

In some situations, new pharmaceutical antiblastic drugs are under
experimentation, together with experimenting with new associations, or
different therapeutic models, like differentiated drugs (i.e. those capable
of modifying the biological characteristics of the tumour, transforming it
from malignant to benign) or with immune-stimolants (those capable of
increasing the defence systems of the organism against tumours).
However, whilst we have a large amount of information regarding these drugs,
unfortunately, we have not yet been tried sufficiently for us to be able to
define their true therapeutic potential.
All this and more, shall be discussed with you in an attempt to keep you,
not only well-informed, but to also allow you to be able to give your
consent to the various treatment for your child. We would ask you to
remember that this is a collaboration which we retain to be of utmost
importance in our aim to identify new methods for our struggle against
tumours and to improve the cure of your child and others who are inflicted
with this disease.



Speaking to your child

Knowing how to speak to a child regarding his illness and the future
treatment that he must undergo, together with what approach to use with him
when he shows fear and worries, is always a terrible dilemma and enigma for
most parents with ill children.
Often a parent's initial reaction, and we must stress, a very natural
reaction, is to want to hide the illness from the child, firstly because one
wants to protect him/her from something which just from its name “cancer”
implies a really quite terrifying illness, and in the second place, because
one believes their child is too young to be able to understand the meaning
of the diagnosis. Nevertheless, whatever the age of the child, he will
understand that something is not quite right: that he has been taken into
hospital, that his parents are concerned, and that he is being spoilt more
than usual; in addition, he will be able to hear the conversations of
grown-ups and therefore he himself will also begin to feel worried and
concerned, guessing that something is being kept from him. And so it is be
very important that he is given a suitable explanation which is appropriate
for his age and that the normal everyday education remains the same.
One must remember at this stage that it is you, his parents, who are the
people he knows best and on whom he will rely to give him the trust he
needs.

He will sense, from the reactions of the people around him, that something
difficult to understand, is happening; in hospital he will see other
children who are like him and who are undergoing the same kind of treatment.
It is fundamental that he feels that he can rely totally on you for the
explanations to his worries. We must remember that if he doesn't receive the
trust and support he needs, the risk is to make him feel insecure and this
lack of confidence could show itself in an outwardly aggressive manner and a
regressive infantile behaviour.
We should not underestimate our children's sensitivity - also a very young
child can become depressed and distant. Many parents fear that, to a child,
the knowledge of having cancer can really frighten him and therefore they
try to avoid him hearing the word cancer, not realising that infact he will
come to know of it anyway, by way of hearing other children talking, hearing
adult discussions or even from the television. It is because tumours can be
cured that there are always more and more people who undergo chemotherapy
and almost all of us know someone who has lost their hair due to the
treatment and so it is not something at all costs to hide from a child.
The important thing to realise is that we cannot hide the truth from a
child. It is better that a child knows about his illness from us and from
the doctors treating him, rather than by listening to others who could well
give wrong or distorted information which in the long run could be upsetting
or more worrying. The best way is to simply tell your child that he has a
particular type of tumour which inflicts only children and that the doctors
have the medicine to cure it. The child in this way will receive a
beneficial comfort from your calm and reassured presence and will then worry
much less about the details of the treatment itself.

If the child is older, say pre-adolescent, he will, of course, have a more
adult concept of cancer and it could be more difficult to be sincere, but it
must be remembered that for a child of this age, or an adolescent, it is
however more important for them to be well-informed of the details of their
illness in order to be able to prepare themselves and understand what they
will have to face. If you try to hide certain facts or indeed distort the
facts, the child could certainly remain seriously troubled, losing the vital
trust which he needs to have in you and the doctors, and consequently one
could risk an increase in the difficulties to be faced with the treatment,
together with behavioural problems.
For you as parents it is advised that you talk with the staff of the
department about the questions your child could put to you and to ask the
doctor in charge of your child's treatment to talk to him, deciding together
with a psychologist, just "how" to talk to the child and the best approach
to be adopted.
A child of scholastic age may have heard of the word cancer and therefore it
is of importance that he knows that he is able to talk about his worries and
fears, regarding, for example, certain changes in his appearance, and that
he also feels free to talk about how he should behave at school, together
with knowing what will happen during and after the treatment that he has to
undergo.
Moreover, one must remember that the members of the medical team responsible
for the treatment of your child, the doctors, nurses and psychologists, are
there to talk to both you and your child, whenever necessary.
The pre-adolescent patient, like very small babies, strongly need, to not
only feel your physical presence and support, but to also have a certain
independence which can sometimes mean they may feel the need to speak in
private with the assisting staff or with other children who have been
inflicted with the same illness.


Facing the medical examinations
Whatever the age of your child, the moment in which you really come to terms
with what is happening is at the moment when you must help prepare himself
for the various medical analysis and examinations that he must undergo.
It is somewhat absurd to try to calm your frightened child reassuring him
that it will "not be painful". If this is not the case, your child could
then lose the vital trust that he previously had in you and could become
withdrawn. A better approach is to look for the positive side of a difficult
situation. For example, you could say: "the injection will hurt, but Mummy
will be here by your side to help you to be brave and then afterwards we
will go and play, or go to drink something, or Mummy will read you a story."
To plead with him to be a big boy or to be good doesn't help when he is
frightened, but giving him courage by saying something like “We'll scream
together when they give you the injection" can help.
Sometimes pyschologists and school teachers, using books and suitable toys,
are able to help the child to become familiar with the diagnostic procedures
and to get him to express his feelings through playing.
“Playing hospitals” for example, means firstly creating a protected change
of environment in which the child feels that he is in control of what he is
doing and all that occurs, reducing or eliminating anxiety, and secondly,
introducing familiarity once again into an unknown environment with an aim
to reinstate a possible threatened sense of security. The creativity of the
child will be safeguarded by the hospital teacher who aim is to give the
child ample space to express himself whilst keeping in mind the natural
development of the child. Drawing and artwork, structured and unstructured
activities, and methodical observation are all elements which help to
understand and help a hospitalised child.
A favourite game for the youngest of children is often “the hospital corner’
in the playroom, or for children confined to their beds, a game of "doctors
and nurses" with a teddy bear who is ill like them, and has to have an
injection. This game helps the child to understand the scenario better and
to feel more in control over the things that are happening to him.


You and your family
After you have been in hospital for some days and the routine has become a
little more familiar, it is then when you can turn your thoughts to how you
feel, how to face the situation and how the consequences of everything will
influence you, your family and your way of living.
The first impact and the feelings associated with this, after having been
informed that your child is inflicted with cancer, will probably be the same
as any other person receiving the same terrible shocking news; one of
incredibility, of fear, helplessness, sadness, anger and even a sense of
guilt. It is likely that you will have difficulty in taking in what is being
said to you; many parents describe a similar feeling to that of a very bad
dream hoping to suddently wake up from it and to return to how life was
before. Others may feel actually physically ill due to the shock, and other
may express themselves with anger, saying: “Why has it happened to us? What
have we done to deserve it?”.
It is certainly a profoundly deep-rooted feeling that brings us to think of
this situation as a punishment, but you must be reassured that there is no
correlation between what parents do, the food the child has been given to
eat, his daily habits, the people around him, nor banal accidents whilst
playing - none of these factors cause neuroblastoma: it is certain too that
it cannot be an inherited condition and does not come from any side of the
family nor be inherited by the family. There is also no known evidence which
proves there is a connection between the disease and habits or occurances
during pregnancy.

The incidence of the illness seems more or less stable, whether in regards
to the frequency and where it is found in the world, in so much that we can
say that a certain number of children each year will develop neuroblastoma
and that this number remains approximately constant in the world, regardless
of the environment and race.
Nevertheless, the psychological need to give the blame to someone or
something is always tempting because it is hard to accept a fact when the
cause is unknown. Some parents give the blame to doctors for "not having
discovered the disease in time", whilst many blame themselves for not having
informed a doctor earlier of the first mimimal disturbances or symptoms seen
in the child. But we must remember that this type of cancer is somewhat
"devious" and that the dimension and presence of the metastases are not
influenced by the time taken before diagnosis.
It is important, however, to openly express your doubts to those who are
taking care of your child. Doctors and nurses fully understand that you are
having to face a situation in which you feel lost and out of your depth with
often too little information or more than often distorted information. Above
all, a pschologist may be able to offer help. With his active participation
in sharing the situation with him, for example, at the moment of being told
the diagnosis; helping you, together with your child, in clarifying your
doubts and queries, your anxieties and expectations connected to the
duration of the illness, of the therapy and of the hospitalisation;
assisting you psychologically during the check-ups with the Day Hospital and
helping you in familiarising your child into the hospital environment, or in
the return back home, in the relationships of school, with brothers and
sisters and with friends. The psychologist is to be found in all
departments, in the Marrow Bone Transplant Unit, and in the Day Hospital,
and it is possible to speak with him in the presence of a doctor or
otherwise request to have his help directly by way of a personally sitting,
set up specifically for you. The psycologist is, infact, a relatively new
hospital figure who has been integrated with the medical team and who can
provide professional help for you, your child and your family in this
delicate and often difficult relationship between family members and
hospital staff.

It is advised to have someone to accompany you during the initial meetings
so as to listen to what is being communicated about the disease and its
treatment, so that you are not alone with the stress of understanding and in
facing the responsibility of taking in everything that is said or repeating
it to others.
Another piece of helpful advice is to not repress your feelings. Undoubtedly
both of you will try to hide certain emotions from one another but this is
thought to only make matter worse. Many people wrongly believe that having a
good cry is something to be ashamed of. However, fathers cry just as much as
mothers do when being told about the illness of their child; it must be
stressed that it is not a sign of weakness, neither of potential incapacity
when dealing with the situation, but a natural reaction and a necessary
liberation for both family and friends. Many parents also believe that they
must not show their own anxiety infront of the child, but sometimes you will
find that you are unable to hide your feelings and emotions and your child
will not be so alarmed if you give a simple but honest explanation to him
of: "Mummy is sorry that you are not feeling well."
In addition, speaking with relatives and friends admittedly can be tiring.
It often requires a real effort to repeat and relay back exactly what the
doctors have told you to understandably anxious and enquiring close ones. In
this situation, the presence of one or two understanding relatives or
friends at these meetings can be of great comfort and help. Sometimes, both
parents and the patient can also become quite exhausted by the influx of
visitors and by the constant telephone calls and so, for this reason, many
parents have found it useful to have a family member at home with the sole
task of informing relatives of what is happening and to generally put them
in the picture after having put in a daily call to the hospital. Close
relatives of the family, for example, the grandparents, can also often find
it useful to have a meeting with doctors so as to becomed better informed of
the well-being of the child and to have the same information and
understanding of the course of the illness and the proposed cures: do not
hesitate to ask for this type of meeting.

Siblings
It is only natural that brothers and sisters of the child with the illness
want to see him and know what is happening.
Many parents believe that to see an ill brother or sister in hospital is a
traumatising event. Nevertheless, the imagination of children can be such
that sometimes they imagine something worse than reality and that often
brothers and sisters can be reassured by seeing exactly the place where
their sibling is being taken care of.
It can also be of help in explaining to younger members of the family what
is happening to the ill child by actually showing them the hospital and the
equipment used to treat the child. It is suggested in this case to ask the
nurse if it may be possible to have a "tour" of the department or day
hospital, obviously following the necessary precautions and explaining
beforehand what they are going to see.
Because of their age and despite a most careful explanation, siblings will
naturally be alittle sceptical at the beginning about the things which are
happening.
They will notice that the child has changed, that perhaps he is paler, more
fragile or thinner, that he has lost his hair and that he cannot always play
with them and join in with the activities that previously they enjoyed
together. It is opportune to check on their playing activities, taking part
in them rather than to ask the siblings of the ill child to treat him with
care.
It is also necessary to give a suitable explanation, according to their age,
of neuroblastoma - if possible, one which is similar to that given to the
ill child; the ill child, himself, if he is old enough, could even explain
his illness, the therapy and the new routine to his brother or sister.
Something to bear in mind is that the siblings of the ill child may
sometimes become worried that what is happening to their brother or sister,
could happen to them. They must obviously be reassured that a tumour will
not develop in them and that, in the case that it could happen, they would
be cured and looked after with the same love and attention that their
sibling is receiving. Regarding this point, it should also be stressed to
them that it will not happen either to you or your other relatives.
Occasionally children can feel guilty about the illness of their brother or
sister. They will attach importance, in some way, to the responsibility of
the occurance, perhaps because at times they have shown particular jealously
towards their brother or sister, or they could even be jealous of the ill
sibling, even if you have well-informed and prepared them about the illness
and its treatment. After all, the child in care, has earnt your complete
attention, has probably also received presents and new toys from relatives,
friends and neighbours, and when he returns home, he will have more things
and more attention than before. It will be very difficult for you when you
are tired and worried to find the necessary resources and energy for your
other children, but it will help in avoiding the problem of jealously by
making a special effort to not favour the ill child too much and to find, at
the end of the day, a little slot to exclusively dedicate to your other
children or to give them a reassuring phone call from the hospital and to
remind them how much you love them and how much you are thinking of them.



Home Sweet Home - Returning Home

When the medical diagnostic tests and the first stages of the treatment have
been concluded, it is likely that your child can return home. Despite the
obvious relief of knowing that you can leave the hospital and the good news
of being able to be back with the rest of the family, don't be alarmed that
it could take you a considerable time to pick up the normal daily routine as
before.
Probably, during the recovery period, you began to put much hope into the
fact of seeing your child once again up and about, eating normally and
temporarily forgetting the hospital. One should bear in mind though that all
these things on which you have built up your hopes will, at least at the
beginning, require much patience.

After having been in hospital for a certain period of time, both you and
your child could find some difficulty in returning to the old daily routine
back home. Perhaps you will find it difficult, at the start, to eat and
sleep normally. You may feel the need to avoid the company of relatives and
close ones and try to reproduce the lifestyle that you had in hospital in
order to seek the feeling of security that the hospital offered you. Your
child may be more attached to you than usual and ask you, when you focus
your attention on other family members, if you have forgotten him. Many
parents, who were previously very sure of their way of bringing up their
children and looking after then, all of a sudden become fearful also in
applying the more common of common household rules.
This question of discipine is a rather prickly topic to discuss. Many
parents say that they cannot imagine, even if they are in the conditions to
do so, to shout or punish their ill child and have a tendency to be
super-indulgent to the point of spoiling them, creating in this way an inner
fear in the child of not having been cured.
An ill child should be reminded of the certain limits established by the
parents for him. If he is allowed everything all of a sudden, he shall not
be reassured that his life is proceeding normally, also regarding his
illness and moreover, this will not help him integrate with his siblings and
friends.
On either hospital visits or after hospital visits, where one could expect
changes in mood of character under conditions of great stress, you should
be, for example, neither too tolerating nor show extreme severity or
aggression. Often, on such occasions, the difficulties of behaviour are the
results of fear and insecurity and it could be helpful to talk about this
issue with a psychologist or another member of the medical team.
It is of utmost importance that the child is helped by way of frank and
reassuring explanations.

It is neither a good idea to indulge your child with mountains of presents
on his home return nor to promise expensive or extravagant gifts. However,
if relatives or visitors do want to bring some little something for the ill
child, they must be persuaded to also bring a similar present for the other
children in the family. Moreover, it is debatable whether to induce a child
to expect some sort of present every time he goes to hospital or offering
him a present as compensation for having undergone a visit or some medical
treatment.
.
If the child constantly had his mother by his side during his stay in
hospital, he could well be even more attached to her once home and could be
reluctant to sleep in his own bed. Nevertheless, it is better to convince
him in a kind and caring, but firm manner, reminding him and reassuring him
that the habits in hospital and home are two distinct things and that at
home he has his own clothes, his toys and his own bed. Even if, at the
beginning, he may feel the need to constantly check for the presence of his
mother, calling out for her or going into her room, he will soon take up his
previous routine of being at home, when he knows that the habits which
existed before his illness must be followed also after his illness. Needless
to say, that if you were to concede to special treatment of an ill child,
for example, letting him sleep in his parent's bed, this would undoubtedly
reflect badly in the eyes of the siblings and probably provoke as a
consequence gelosy and other related family problems.

In the case of neuroblastoma, there are no particular restrictions in the
child's diet, neither a need for particular medicine between the various
stays in hospital, and as the the treatment advances, the child often gains
more energy and his appetite together with the joy for the usual previous
activities, between one phase of chemotherapy and another.
Nevertheless, alimentation is often an issue which causes anxiety in many
parents. It is inevitable that your child will have lost weight, first of
all, due to the neuroblastoma itself, and then due to the treatment which
can cause poor health and a lack of appetite, as can a decrease in the
child's physical activities which brings about a reduced expediture of
energy.
Some children do not regain their appetites as before, at least not until
the whole course of chemotherapy has been concluded. It is, however, quite
natural to want to feed your child up; to want to prepare nutritious meals
and see your child eat them can sometimes be very positive but remember that
it can also become counterproductive if every mealtime becomes a fierce
battleground in trying to coax him to eat "just one more spoonful".

Needless to say one should not be too rigid in this phase and try to offer
your child what he wants and to not feel offended if he tries only little
amounts at a time.
Provided that the doctors are not concerned about a particularly excessive
weight loss, a fluctuating appetite and often a prolonged loss of appetite
during treatment should be accepted as quite normal. It could be of help to
consult a hospital dietician who will be able to suggest various ways to
prepare a nutritious diet or to advise supplementary foods, and in the case
where the main treatment is given in hospital, added nutrients or possibly,
the Parental Total Nutrition (NPT) can be provided by way of the Broviac
catheter.


Returning to normality

There is a strong but natural tendency to become over-protective with an ill
child.
However, you have probably been informed by the medical team responsible for
the child's treatment that you can allow him to do all that he wants and
feels up to doing. Infact, it is a positive thing to encourage him in,
little by little, as he becomes stronger and healthier to regain his
previous capabilities and habits, bearing in mind to keep an eye on him,
particularly in regards to a lowered defence system and/or if he has a
Broviac catheter. On the whole, the child will draw once again from the
benefits of mixing with other children and joining in their activities,
provided that any specific precautions which need to be considered are
followed.
All this can come about mainly by playing outside with friends, or for an
older child, returning to doing certain activities, for example, with the
Scouts or going out on some school trip.
Many of the desires to protect your child could have come from the
suggestions given by the medical team to keep him away from possible sources
of infection and for this reason, many parents are worried for the
consequences of letting their child mix with other children. Obviously, you
must follow the indications given by your pediatrician.

Generally speaking, common sense should be followed. Naturally, one
shouldn't allow their child to play in a group of children where almost
every child has a cold. It is more advisable and cannot harm him if one
invites a neighbour's child in or if you take your child out to the park
with a friend. Certainly you have been informed that measles and chicken pox
are infectious; your child must avoid, if possible, these two diseases and
even though it is not easy, one should try to have a helping hand from the
support of neighbours, friends, teachers and social assistants in order to
be told if children in the community have contracted any such infectious
diseases. This goes without saying also in regards to the child's teachers
at school. Obviously, it shouldn't become a disturbance, but one must be
cautious with an ill child if, in the brother's or sister's class, there is
a child who has contracted, for example, measles or chicken pox.
In the case that your child has come in contact with such an illness, do not
panic, but immediately inform your doctor, so that it is possible to give a
special injection to lessen the attack or even prevent it completely;
telephone your hospital or your Paediatrician to let them know; however, in
the case that your child does contract an infectious disease, do not take
him to hospital without warning the hospital beforehand in order to avoid
passing it to other children.

Please note that a child is unable to have normal vaccinations while under
chemotherapy, and so it is crucial that you inform the paediatrician or the
National Health Unit (ASL) responsible in the case where a vaccination is
needed.

School
Returning to school or nursery school can pose yet another problem for a
child who has been diagnosed as having neuroblastoma. However, it is
important to remember that your child has the right and the need to come
into normal contact with his friends.
One shouldn't believe that, at 5 years old, a regular and committed
schooling is not of importance, or that at 10 years old, the educational
activities at school are top priorities above all others, because for a
child who already feels different and somewhat isolated from his friends due
to spells in hospital and often lengthy periods of treatment, it is very
important that he once again takes up his normal activities and intellectual
stimulation as soon as possible, in such a way that he can get back into a
routine with his friends and school without having the added disadvantage of
having lost precious studying time.
Regular contact with his school friends or nursery school companions can be
of great help even whilst he is unable to actually attend school full time
and the hospital teacher, who will have contact with the school or the
nursery school, will be able to obtain suitable material for doing his
homework and activities from home.

A helpful and good collaboration between the two sets of teachers (the
hospital teacher and the regular school teacher) would be something to hope
for, so that the child does not feel "different" at the moment when he
returns amongst his school friends (it is not enough just to exchange
opinions on the child or the curriculum: it would be useful to take
photographs, draw pictures of similar themes, of specific moments, events of
the day etc which are common to all children).
If returning to school for a certain period is really impossibile, then one
should see to it that the local education authorities set to organising some
home visits by a teacher, a service which should be taken advantage of.
Even if the child cannot attend school full time, perhaps a couple of half
days could be considered or to attend those lessons which are of particular
interest or to take part in some special activity during the week. In order
to help the re-integration into the school, it is necessary to get into
contact with