- What is Neuroblastoma
- Causes of Neuroblastoma
- Signs and Symptoms of Neuroblastoma
- Diagnosis and Staging of Neuroblastoma
- Treatments for Neuroblastoma
I have written the piece below in an attempt to give some understanding of Neuroblastoma. I have written it reflecting on my reaction and ‘needs’ when Jack was diagnosed. There is nothing more ardently demanding than the need for answer when the word Neuroblastoma is uttered and its context is its entanglement with your child.
There is no current simple single answer to the totality of what Neuroblastoma can be and how it can be conquered. Please, the stress in the last sentence is placed on the word can. In many cases there are answers.
What’s important to know about Neuroblastoma? This in itself is quite subjective, different people, different parents, siblings, relatives and friends often have a differing priority amongst the areas of information. What’s highly relevant to one person, one carer, and one sufferer is less significant to another.
Why is the patient listed last in the above paragraph? Simply because of their likely age and understanding; they are the priority. One self-evident truism concerns those involved. The care of a child suffering Neuroblastoma requires a team. Those within a team may have different primary roles but each person must communicate in a meaningful way with the rest of the team. Communication is only meaningful if its content is understood. If it’s understood then it increases the chance of the best available decision being made. Treatment should not be dictatorial but inclusive. As a parent or carer your child needs a guardian; a supporter; an advocate.
What is Neuroblastoma?
Neuroblastoma is a form of cancer that mostly affects young children. How many children? Something like 100 a year in the UK. Often the age of the child at the point of diagnosis is between birth and five years. However, the illness does extend beyond the age of five. Although the diagnosis of the disease beyond five years of age becomes increasingly rare. It is most commonly found in young children because of the type of cell that becomes cancerous.
The cell is called a ‘neural crest cell’ or ‘neuroblasts’. This cell is much more relevant to young children and their development, specifically the development of the child’s nervous system and other tissues.The cells’ purposes are important to the normal development of the nervous system during their presence in the womb. Beyond the formation of the nervous system the cells that remain should have become redundant.
Neuroblastoma commonly presents in a child’s adrenal glands. These glands are located in the abdomen. Another location where the disease first establishes itself can be in nerve tissue that’s situated close to the spinal cord. Although, Neuroblastoma involves nerve cells it is not usual to find the disease present within the central nervous system at the point of diagnosis.
Neuroblastoma can spread to tissues such as the bone marrow, bone, lymph nodes, liver and skin.
It is very important to know that a diagnosis of Neuroblastoma is, in many ways, not the most significant piece of information. The most significant part of the information that accompanies the process is which ‘category’ or ‘stage’ does the Neuroblastoma form. Following diagnosis the assessment of the Neuroblastoma’s stage will be made.
What causes Neuroblastoma?
At the moment no-one knows what causes Neuroblastoma. There is nothing to indicate for instance that a particular aspect of environment or hereditary is relevant to the cancer’s onset.
What signs and symptoms occur when a child is suffering from Neuroblastoma?
Neuroblastoma effects children differently. This is because of where the cells form and how they develop. It may help to think of Neuroblastoma as invasive but not an invasion. The cells that cause the problem are your child’s cells. Your child is unique; you child’s cells are unique; your child is special; your child is incomparable.
Symptoms that can occur include:
- A swollen painful tummy, sometimes in association with constipation and difficulty passing urine
- Breathlessness and difficulty swallowing
- A lump in the neck
- Blueish lumps in the skin and bruising, particularly around the eyes
- Weakness in the legs and an unsteady walk, with numbness in the lower body, constipation and difficulty passing urine
- Fatigue, loss of energy, pale skin, loss of appetite and weight loss
- Bone pain, a limp and general irritability
- Rarely, jerky eye and muscle movements
A practical complication of the disease and its discovery is as a consequence of the age of the children affected by the illness. The children are not able articulate with clarity the totality of how they feel and where they are suffering pain and discomfort. Moreover, there is nothing unique about a particular symptom that enables someone to determine a child is suffering from Neuroblastoma. This is because the symptoms are only the outward generic display of a battle occurring in the child. The symptoms could arise from many causes that are a lot less concerning than Neuroblastoma.
Diagnosis of Neuroblastoma and what stage does it comprise?
Diagnosis requires specific tests. In any event in order to determine the extent and spread of the illness it is likely that these tests will be conducted within a short time of diagnosis.
- Biopsy of tissue
- Urine tests
- Blood tests
- MIBG scan, (this involves an infusion containing material that has an affinity to neuroblastoma cells. This affinity renders the ‘spread’ of the cells to be observed using a scan).
Once all the information arising from the scans an assessment of the cancer’s category or stage can be made. The stages of Neuroblastoma are referred to 1 to 4 and a 4s.
Stage 1 – The tumor is confined to where it started, (this means that there has been no spread, also known as metastasis), and the tumor can be completely removed by surgery.
Stage 2a – The tumor is confined to where it started but it cannot be completely removed by surgery.
Stage 2b –The tumor is confined to where it started. It may or may not be possible to completely remove the tumor by surgery. Neuroblastoma cells are present in the lymph nodes near to the tumor.
Stage 3 – The tumor cannot be completely removed by surgery and has one of the added factors described below:
It has spread from one side of the body to the other and may also have spread to nearby lymph nodes.
It has remained confined to where it started but the cancer has spread to lymph nodes on the other side of the body.
It has remained confined to the middle of the body and spread to lymph nodes on both sides of the body.
Stage 4 – The tumor has spread to distant lymph nodes, bone marrow, bone, liver, or other organs (except as defined by stage 4S).
Stage 4s –– Stage 4S is applicable only to children who are younger than one year. The tumor is confined to the area in which it arose, as in stages 1 and 2, but some cells have spread to the liver, skin, or bone marrow. Stage 4S has characteristics that mean it may be possible for the illness to be treated with little complication.
There are other factors, which affect the way in which the cancer has developed and how it might be fought. The structure of a cell is far more complicated than I can comprehend. Factors associated specifically with your child’s Neuroblastoma cells and how they work are important to prognosis.
‘Your child’s neuroblastoma cells’ and this is thought to be an important concept by many. Your child is different than any other ergo you child’s neuroblastoma cells differ. They may even differ throughout the child and throughout their therapies.
What treatments are available for Neuroblastoma?
There are a number different treatments which are used and usually these are used in combination or in sequence in order to try and rid cancer cells from the patient. The range of treatment will depend on the determination of the cancer’s stage. Jack had all the types of treatment described below, although not necessarily in that order.
The surgeon will try to remove the whole tumour, or as much of it as possible. The exact type of surgery depends on where the tumour is in the body. If the tumour hasn’t spread, and it’s possible to remove it all, surgery may be the only treatment the patient needs. All surgery carries risk and it is important to discuss with your doctor and the surgeon what the surgery entails and the risks associated with it.
Chemotherapy involves the use of drugs to kill cancer cells and it is usual in Neuroblastoma for chemotherapy to involve more than one active drug. This is because different drugs have an ability to attack different aspects of the Neuroblastoma cells. The combination of damage to the Neuroblastoma cells tends to be more effective that a singular blow. Chemotherapy is a balancing act meaning the drugs also damage some healthy cells, which share characteristics of the cancer cells. Chemotherapy has side effects and these may include tiredness, feeling sick, and a reduced immune system that can leave the patient vulnerable to infections. Sometimes sores occur and often there is hair loss.
As part of the regimen your child is likely to have a central line or port inserted while they are having treatment. This is a tube or access point that is put into one of the large veins in the chest. They have their chemotherapy through it and can have blood taken from it. It means that they need fewer needles. They keep it in throughout their treatment. A central line requires care as it introduces its own vulnerability in terms being a potential site for infection to occur or to be introduced. If your child is to have a central line you will receive detailed instructions about how to best care for and protect against infection.
Some children need more intensive or high dose chemotherapy. High doses of chemotherapy can damage the bone
marrow and kill off the cells that make blood cells. These cells are called stem cells. So, as part of this high dose treatment they need to have treatment to replace these blood stem cells.
This means early on in the treatment the doctors collect some blood stem cells and store them until after they have had the high dose chemotherapy. Then after the high dose treatment they have the stem cells back. This is called a stem cell transplant.
This is the use of high energy X-rays to kill cancer cells. This is usually external beam radiotherapy. This means radiotherapy aimed at the cancer from outside the body. Your child is most likely to have this after surgery to reduce the risk of the cancer coming back. The radiotherapy aims to ‘blast’ the former bed of the tumor in order to destroy residual cancer cells. If your child’s neuroblastoma is advanced and chemotherapy is not working they may need targeted radiotherapy. Or, they may need it if their neuroblastoma has come back after earlier treatment.
Targeted radiotherapy is similar to the MIBG scan used to diagnose neuroblastoma, but gives a higher dose of radioactive iodine. The MIBG is picked up by the cancer cells.
The dose of radioactive iodine attached to the MIBG is high enough to kill the neuroblastoma cells, but not to cause serious side effects to surrounding tissues. But this treatment can damage the bone marrow and kill off the cells that make blood cells. So, children having this treatment also need a stem cell transplant.
Radiotherapy does carry risk most commonly associated with the increased potential for secondary cancers later in life.
Immunotherapy is a type of drug treatment to help the immune system kill cancer cells. It is a type of biological therapy. Monoclonal antibodies are used in order to stimulate the patient’s immune system to recognise and kill the cancer cells.
This therapy can be painful and it will be important to understand this and how the team will monitor and control pain. It is also important to understand what the pain is associated with and why it occurs. Jack had this treatment and although painful he was able to play and associate with his peers within a few hours.
What is a clinical trial? Medicine progresses through research and practice. In order to determine whether how effective or not a new therapy maybe it is necessary to trial the process. It is important to understand that this is the purpose of a trial. Trials are conducted in stages. Eligibility for a trial is a complication. One patient may be deemed suitable because of their illness and the preceding treatments another may not be.
Stage one’s purpose is designed to determine safety and boundaries. A simple example might be the amount of a drug that is tolerable in a patient; so three different ‘amounts’ of a drug might be tested in a small group of patients. What were the side effects?
Stage two normally involves a larger group of patients and the purpose now does swing towards efficacy. What effects does it have on the patients and their disease? Neuroblastoma is rare and many phase two trials require long periods because it takes a long time to amass the number of patients to produce data that can result in a conclusion as to the benefit of the therapy.
Stage three in effect means that there has been a conclusion and the therapy is believed to be of benefit to some patients.
Medicine and doctors and hospitals do share expertise and results but a therapy or trial that is considered valid in one country or region of the world may not yet be valid in another. Further the validation of a therapy in one country does not lead to automatic replication in another. Often, if not always, the therapy will require the process of ‘staged trial’ to be repeated in a different ‘jurisdiction’.