When is Diagnostic Radiation Unsafe?

When it comes to modern medicine, many of the decisions that you make concerning your child’s health, involves a risk verses benefit factor.

The healthcare profession is extremely fortunate to have the diagnostics tools that are available for us to use. These powerful diagnostic tools have truly revolutionized our ability to accurately diagnose our patient’s condition. Consequently, this leads to prescribing the proper treatment modalities which results in successful outcomes for our patient’s. This is beneficial to the patient, am I right? However, these broadly used diagnostic tools also pose a danger to your child, thus we have our risk. What are the tools I am talking about? I’m talking about diagnostic tools such as CT scanners, X-ray machines, PET scanners, etc.  Although, these tools have revolutionized health care, your exposure to the high levels of radiation they emit can be hazardous to your health.  We’re talking about diagnostic medical radiation.

So, what exactly is diagnostic medical radiation? It is radiation that simply goes under our skin and reveals what a doctor’s skilled hands can’t palpate and what can’t be seen by the naked eye. Diagnostic radiation comes in different amounts and forms. Now, to address the amounts of radiation, an example of a small amount is the amount used for dental x-rays.  An example of a very large amount, is the amount your child could be expose to while undergoing a CT scan or PET scan procedure. Can you believe these scans can deliver the equivalent of up to 1000 or more chest x-rays?  Well, believe it.

But these tools are so valuable. They can spot precursors to certain disease processes and the early presence of so many others, such as, appendicitis, cancer, cardiovascular disease, infectious diseases, traumas, kidney stones and musculoskeletal disorders.  And because of their accuracy and speed, the need for some of the procedure once done routinely (exploratory surgeries, biopsies and other invasive procedures) has been curtailed.

Now the question you are probably asking at this point is, what is the down side to diagnostic radiation?  When it comes to radiation, all radiation creates what science calls “free radical.” Free radicals can cause damage to your child’s DNA. This can occur immediately but sometimes the damage can occur in the future. What becomes concerning is that the damage DNA is dangerous. What parents need to know is that radiation accumulates in the body. When your child is exposed to radiation, it doesn’t pass through them completely. A percentage of it will stay in their body and build up over time.

The question you may be asking right now is, how much radiation is too much? Well, there are two schools of thought involved with this question.  Some health care professionals believe that all diagnostic radiation is somewhat harmful, regardless of the amount.  But there is not any evidence to back this assumption.  When you look at this from a risk versus benefit perspective, particularly in the areas where radiation exposure is the highest, (i.e., CT scans, PET scans, etc.) the benefits outweigh the risk tremendously.  However, the other school of thought, is in complete contrast to the one, mentioned previously. Proponents of this school of thought believe that diagnostic radiation is extremely risky and have studies to back their claim.  There was a study involving CAT scans which concluded that the scan alone will increase the number of cancer cases in our nation by 2 percent (nearly 30, 000 cases). This could also result in, they concluded, about 14,500 deaths.  There was another study which concluded that the overuse of CT scans could lead to an estimated 3 million radiation-caused cancers over the next 20 to 30 years.

How is radiation measured?

Radiation can be measured by comparing radiation received from a medical procedure with natural background radiation. Background radiation is the radiation one receives simply from living on this planet. It has several sources. Cosmic rays (radiation from the Sun and stars), radiation from the earth (from the rocks and the soil), and radiation from Radon (odorless, colorless gas that is formed from the breakdown of radioactive elements in the ground).

Below, you will find profiles of some of the most common radiological procedures and how they compare to your child’s natural background radiation exposure:

Procedure                                                                                                                        Days of Natural Background Radiation                                                                                                                                                                                                                              

CAT scan (this procedure consists of hundreds of X-rays at different angles, which are   combined by a computer to produce a 3-d image)

 up to 2000

(5.5 years)

 

Chest X-ray

2.5

Dental X-ray
        <1
   Screening mammogram
Routine preventive screening/Diagnostic mammogram
(these are additional X-rays examining specific problem areas)PET Scan with this procedure, a tracer, that is radioactive, will be injected into the bloodstream or it is swallowed. As it moves through the body, radiation is emitted.
          5
PET Scan

(with this procedure a tracer that is radioactive, will be injected into the bloodstream or it is swallowed. As it moves through the body, radiation is emitted.

  2500 (6.8 years)

In view of these facts, your next question is probably, “when should I say no and what are my alternatives?” Good question. The answer to this question should be discussed with your doctor.  Your doctor would be aware of the risk involved in radiation-based diagnoses.  The doctor would be able to advise you as to when to say no. 

Sometimes, it just takes using some common sense.  For example, we all know that dental visits often require taking an x-ray of your teeth and jaw. From my experience, it is rare for the Dental Tech to ask you for permission.  Even though, it is a small amount of radiation, there is no such thing as a “small” amount of DNA damage. 

So, what about the procedures that emit a large amount of radiation, i.e., your CT scans, PET scans, etc.  Those in the medical community are aware of the fact, that many children who presents to the emergency room with abdominal pain or headache, have automatically bought themselves a CAT scan. The technology is so good that it has often been overused. It is estimated that 85 million CAT scans are performed every year.  So, what can a parent do to avoid the radiation based diagnostic tools?  There are a couple of alternatives that are as effective, if not more effective. The alternatives are ultrasounds and MRI’s. I need to point out that there are exceptions, where radiation-based procedures are required. My recommendation is that you mention your preference to avoid radiation based procedures to your child’s Pediatrician and allow the physician to advise you.

However, there are times when radiation based diagnostics are unavoidable. Fortunately, God has provided us ways to protect against radiation damage and some of them are delicious. What I am about to share with you are items that have high concentrations of antioxidants.  These antioxidants protect against DNA damage. These antioxidants can be found in teas made from the Chaga mushroom extract.

Blueberries is another natural food item that contains protective antioxidants and specialized anti-cancer compounds.  It is recommended that your child eats 45 berries a day or try a supplement containing the extract. If your child doesn’t like the taste of plain blueberries, you can always add it to pancake or muffin mixes. Have you ever added them to a homemade smoothie? Very delicious. Foods that contain vitamin A, vitamin C and vitamin D will have antioxidants, as well.  Or you can give them the supplemental equivalent. For Vitamin A, the recommended daily intake is 5,000 IU, for Vitamin C it is 1,000 mg and for Vitamin D, it is 5,000 mg. Lastly, the product, Curcumin is a powerful tumor-inhibiting product. If you get Curcumin in a supplemental form, 500 mg. should be suffice, just be sure it is in a “bioavailable” formulation.   And always, consult with your Pediatrician before considering any behavioral, dietary or supplemental changes.

Reference

Idiopathic Thrombocytopenia Purpura

Idiopathic Thrombocytopenia Purpura, or ITP, is a disorder characterized by excessive bruising and bleeding.  This bleeding and bruising is a result of an unusually low platelet count.  Platelets are the components in your blood, responsible for causing the blood to clot.  The normal platelet count is 150,000-450,000 platelets per microliter of blood. A child with ITP will have platelet counts below 20,000 platelets per microliter of blood. This disorder is also known as Immune Thrombocytopenia Purpura. It affects children and adults. With children the disease is preceded by a viral infection, usually acute in nature and recovery from it is completely.

Etiology or Causes

The definition of idiopathic is “of unknown cause” hence we get the labeling of this disorder.  The exact cause is unknown. What we do know, however, is that with this disorder, the immune system begins attacking the platelets as if they were foreign substances. The antibodies that the immune system produces, will attach themselves to the platelets. This is the means for marking the platelets for destruction.  The function of the spleen is to help the body fight off infection. When the spleen recognizes the antibodies, it will remove the antibody and platelet attached to it, from the circulatory system.

Signs and Symptoms

This disease may not have any overt symptoms. However, when they do occur, they may include easy bruising (purpura) and prolonged bleeding.  You might see superficial bleeding under the skin that appears as a red, pinpoint rash.  This is known as a petechial rash. Nose bleeds are common. You might have bleeding gums, particularly, after dental work.  You could see blood in the urine and stools. For teenage girls you may experience an unusually heavy menstrual flow.  Fatigue is not uncommon because as you bleed, you will become anemic.

Diagnostic Test

To diagnose this disorder, the doctor would begin with a physical examination. Your doctor will look for signs of bleeding under your child’s skin. You may be asked about recent episodes of bleeding, previous illnesses and medications your child has recently taken.

Complete blood cell count: this test is the gold standard for diagnosing this disease. The number of red and white blood cells and platelets are counted from a sample of blood. In this disorder, you may find the red and white blood cell counts may be normal, however, the number of platelets will be low.

Bone Marrow test: Platelets are manufactured in the bone marrow, the soft, spongy tissue in the center of large bones. A procedure known as a bone marrow biopsy is performed which is the removal of solid bone marrow. The removal of the liquid portion of the bone marrow, known as a bone marrow aspiration, often accompanies the biopsy procedure. The two samples are sent to the lab for testing. With ITP, the bone marrow test will be normal.
If you have ITP, your bone marrow will be normal because your low platelet count is caused by the destruction of platelets in your bloodstream and spleen — not by a problem with the bone marrow

Treatment and Prevention

When treating ITP, the goal is to ensure a safe platelet count and prevent bleeding complications while minimizing treatment side effects.

If your child’s platelet count drops below 20,000 / microliters, they might receive corticosteroids or intravenous immunoglobulin (IVIG).  Prednisone, which is a steroid, could be administered for 2-3 weeks or until the platelet count increases above 30,000 / microliters. The IVIG is only given for 1-3 days.  Another drug, known as Rituximab (Rituxan) may be used in severe cases of ITP, when corticosteroids are ineffective. The mechanism of action for this drug is reducing the immune system response.   The newest medications approved to treat ITP are called Thrombopoietin Receptor Agonists. Romiplostim (Nplate) and Eltrombopag (Promacta) are two of these drugs. These drugs work by helping your bone marrow produce more platelets, which results in the cessation of bruising and bleeding.

There have been cases where those with ITP were also infected with Helicobacter Pylori.  This is the same bacteria responsible for most peptic ulcers. It has also been recognized that by eliminating the bacteria, the platelet count has increased. However, the outcome from this form of treatment has been inconsistent and more studies need to be done.

A transfusion of platelets is usually not indicated unless your child is facing a life-threatening condition. ITP is usually self-limiting. For those cases where ITP persists for a year or longer, the removal of the spleen (splenectomy) may be necessary.  The removal of your spleen results in the loss of the mechanism that is causing the rapid destruction of the platelets. However, when you remove the spleen you also increase your vulnerability to infections. Fortunately, if after having a splenectomy your child is otherwise healthy, the risk of an infection is low.

In regards to prevention, you should restrict your child from activities that could result in trauma, such as contact sports. Medications such as aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), and antihistamines should be avoided because these medications can precipitate the development of anemia in your child.

Prognosis

Idiopathic thrombocytopenic purpura usually runs its course without the need for treatment. About 80 percent of children with idiopathic thrombocytopenic purpura recover completely within six months. Even in children who develop chronic ITP, complete recovery may still occur, even years later.

When to see a doctor

If you or your child has abnormal bleeding or bruising, or develops a rash of pinpoint-sized red spots, see your doctor.  A referral to a Pediatric Hematologist is recommended, if your child develops ITP.  It’s also important to seek medical advice if you’re an adolescent who suddenly develops significantly increased menstrual bleeding, as this may be a sign of ITP. Serious or widespread bleeding indicates an emergency and requires immediate care.

Wilm’s Tumor

Wilms’ tumor, which is also known as Nephroblastoma, is the most common kidney tumor in children.  It most often affects children between the ages of 2-5 years of age. Peak incidence of occurrence is between the ages of 2-3 years. This is a tumor that grows very rapidly and is typically large at diagnosis. The tumor will metastasize to the peri-renal tissue, the liver, diaphragm, lungs, abdominal muscles and the lymph nodes.  Metastasis will occur through the bloodstream or by direct extension. Usually the tumor will occur in one of the kidneys, however, there are cases where both kidneys will have a tumor at the same time.

Etiology or Causes

The exact cause of Wilms’ tumor is not known. What we do know, however, is that cancer begins when there is an error in the DNA of cells. This leads to the cells growing and dividing uncontrollably, even in situations where normal cells would die. These rogue cells accumulate to form a tumor.  There are cases where the DNA that causes the Wilms Tumor is passed from a parent to the child. However, those cases are rare.

Signs and Symptoms

The most obvious sign is a firm mass in the abdominal cavity. Other symptoms that might appear with this disorder is abdominal pain and swelling, constipation, vomiting, weight loss, loss of appetite, fever and difficulty breathing

Diagnostic Test

To diagnose a Wilms Tumor, a physical examination would be your best starting point.  Your doctor may order imaging test such as an ultrasound (sonogram), computerized tomography (CT scan), or an MRI (magnetic resonance imaging) in order to visualize the kidney and detect a tumor. If a tumor is discovered, your doctor might order a chest x-ray and a nuclear medicine bone scan to determine the extent of the tumor. This is necessary in order to stage the tumor and to offer some guidance in determining the best treatment modality for dealing with the tumor. A 24 hour urine collection could be ordered to measure the levels of HVA (homovanillic acid) and VMA (vanillylmandelic acid).  In Neuroblastomas, the levels are elevated, but with Wilms tumor the levels are not elevated.

Staging of the Wilms Tumor

  • Stage I: unilateral, limited to kidney, and can be resected from the kidney.
  • Stage II: unilateral, with the tumor extending beyond the kidney, however, it can be resected from the kidney.
  • Stage III: unilateral, with the tumor extending beyond the kidney and located in the  lymph nodes, as well as, other structures in the abdominal cavity. The tumor may not be completely removed with surgery.
  • Stage IV: unilateral with metastasis occurring in the liver, lung, bone or brain
  • Stage V: bilateral kidney involvement

Treatment and Prevention

Surgical removal of the tumor and the affected kidney is the usual treatment. Some cases will require chemotherapy and radiation therapy. This will occur either before or after the surgery.

Radiation therapy uses high-energy beams, such as X-rays, to kill cancer cells.

Chemotherapy uses powerful drugs to kill cancer cells throughout the body. Chemotherapy treatment usually involves a combination of drugs that work together to kill cancer cells. Chemotherapy can be administered through a vein or taken in pill form.

Chemotherapy may be used before surgery to shrink tumors and make them easier to remove. Chemotherapy may be used after surgery to kill any cancer cells that may remain in the body.

Radiation therapy may be used after surgery to kill any cancer cells that weren’t removed during the operation. Radiation therapy may also be an option to control cancer that has spread to other areas of the body, depending on where the cancer has spread.

Prognosis

The survival rate is as high as 90%. However, there are potential complications which include metastasis.  With radiation therapy, there is the potential for female sterility, bowel obstruction, pneumonia, scoliosis, and damage to the liver or kidneys.

Sickle Cell Anemia

Definition

Sickle Cell Anemia (SCA) is an inherited disorder characterized by a diminished amount of healthy red blood cells (RBC’s) to adequately supply your body with oxygen. Normally, your red blood cells are round and flexible. They travel through your blood vessels very smoothly.  In SCA, some of the red blood cells are shaped like a sickle. Some have described them to look like the shape of a crescent moon.  These irregularly shaped RBC’s can become stuck in small blood vessel and cause the flow of blood to be slower or even obstruct the vessel.

Etiology or Causes

The sickling of the red blood cells can be triggered by stress or traumatic events such as fever, dehydration, physical exertion, infection, excessive cold exposure, hypoxia and acidosis. As the red blood cell sickle, they become viscous or sticky. This causes the blood cells to clump together and to become stuck in the blood vessel, resulting in decreased flow of blood to the tissues. This blood vessel occlusive process can lead to lack of oxygen to the body tissue, which results in tissue injury and quite possibly, tissue death.

Signs and Symptoms

The signs and symptoms of SCA may often not manifest themselves until the child is at least 4 month of age. Those symptoms are as follows:

Pain – Commonly known as sickle cell crisis, these periodic episodes of pain is a major symptom of this disorder. Pain occurs when these sickled RBC’s block blood flow to the body tissue. Pain can occur in any part of the body, but the most common areas affected are the chest, abdomen and bone joints. The pain will vary in intensity and also in duration, lasting from a few hours and up to a few weeks. These pain episodes may be experience by the person only a few times during their lifetime or they can occur as often as a dozen times per year. A person in crisis may have to be hospitalized if the crisis is severe enough.

Anemia – The anemia is a result of the fragility of the sickle cell. These cells will break apart and die. The red blood cell has a normal lifetime of 120 days. The sickle cell will live for an average of 20 days. The premature deaths of the red blood cell result in a deficiency of red blood cells circulating in your body. It affects your body by limiting the amount of oxygen being carried in your circulation, which results in lack of energy / fatigue.

Delay Growth – Your red blood cells are responsible for supplying your body with oxygen and nutrients needed for growth. A deficiency in red blood cells can result in retarded growth in children and delayed puberty in teenagers.

Infection – Sickled red blood cells can cause damage to your spleen, a vital organ in the fight against infections. If your spleen is damaged, your body becomes more vulnerable to infections. A common practice in the health care community is for the PCP (primary care provider) to vaccinate the child and to prescribe antibiotic to prevent life-threatening illnesses.

Hand-Foot Syndrome – When sickled shaped red blood cells circulate in your bloodstream they can have a tendency to block the flow of blood out of the hands and feet of tiny blood vessels. This can result in the hands and feet becoming swollen. This symptom tends to be more prevalent in infants and may present as the first sign of SCA.

Problems with Vision – This is another case where the tiny blood vessel of an organ, in this case the eyes, become plugged up by the sickled red blood cell. This results in damage to the retina, which is the vision processing component of the eye.

Potential Problems

Acute Stroke – The sickle cells may obstruct the flow of blood to the brain either partially or completely, causing a cerebral vascular accident or stroke. The following are signs and symptoms of a stroke: facial drooping, numbness and weakness in the arms and legs, speech difficulties, seizures and loss of consciousness. If you child is displaying these symptoms, you should seek medical attention immediately.

Damage to organs – With SCA, the blood is typically low in oxygen content. You also have the problem of the sickle cell obstructing the flow of blood to vital organs. When your organs, such as the kidneys, liver, and spleen, are chronically deprived to oxygen-rich blood, they can be damaged.

Acute Stroke– The sickle cell may obstruct the flow of blood to the brain either partially or completely, causing a cerebral vascular accident or stroke.  The signs and symptoms of a stroke includes, facial drooping, numbness and weakness in your extremities, slurred speech

Acute Chest Syndrome – Another potential problem with SCA patients is a condition known as acute chest syndrome. This condition is a result of sickle cells blocking the flow of blood to parts of the lungs. It is characterized by fever, chest pain and difficulty breathing. This is another situation that requires immediate medical attention.

Gallstones – The development of gallstones is a potential problem which is a result of the red blood cells breaking down and producing a substance known as bilirubin.

Pulmonary Hypertension – This is a condition which is more common in adults than with children who have Sickle Cell Anemia. It is characterized by the blood pressure in the lungs becoming elevated. Symptoms manifested are shortness of breath and fatigue. It can become life-threatening, so seek immediate medical attention, if this occurs.

Priapism – Sickle cells can block the flow of blood in the blood vessels of the penis, as it does with other body organs. This can lead to a very painful, long lasting erection known as priapism. This condition can cause damage to the penis and eventually lead to impotence.

Stasis ulcers – SSA can cause open sores to develop on the legs, also known as, stasis ulcers.

Diagnostic Test

Sickle cell disease usually is diagnosed at birth with a blood test during routine newborn screening tests. If a child tests positive on the screening test, a second blood test (called a hemoglobin electrophoresis) should be performed to confirm the diagnosis. Other findings of the blood test will include the following:

Reticulocyte count – this will be elevated.

Hemoglobin – The baseline is usually 7-10 mg / dl. This will be significantly lower.

Peripheral Blood Smear – there will be a presence of sickle-shaped cells.

Platelet count – This will be increased.

Erythrocyte Sedimentation Rate – This will be elevated.

Abnormal Liver Function Test – Elevated Bilirubin levels in the blood.

Because kids with sickle cell disease are at an increased risk of infection and other health complications, early diagnosis and treatment to prevent problems is important. Currently, all 50 states require newborn screening programs for sickle cell disease by law or by rule.

Treatment and Prevention

Blood Transfusions – A blood transfusion is a common treatment modality for the management of SSA. With a transfusion, you are essentially decreasing the amount of red blood cells that can sickle by adding normal red blood cells. This treatment have been proven to be efficacious in the reducing the risk for strokes in children.

Bone marrow transplant offers the only cure for SCA. The procedure is complex and has some risks involved. Finding the appropriate donor offers some unique challenges in itself. A bone marrow transplant, also known as, a stem cell transplant is a multi-step process. The first step involves finding a person, a donor (often a sibling), who will agree to contribute their bone marrow stem cells to the diseased person. The donor will then be tested to determine if their stem cells will be a match to the recipients. If the donor and the recipient share the same Human Leukocyte Antigens (abbreviated as HLA), they are said to be a “match.” This means that their tissues are immunologically compatible with each other. Even if an appropriate donor can’t be found and the patient never has a bone marrow transplant, kids with SCA can still lead a relatively normal life.

Medications -There are medications that are available to assist the child to manage the pain. Immunizations and daily doses of antibiotics can help prevent life threatening infections. The most common drug prescribed is penicillin and those in the penicillin family, such as amoxicillin. These are usually prescribed until the child is at least 5 years of age. Along with the regularly scheduled child immunizations, the pneumococcal, meningococcal and influenza vaccines are often added to the child’s vaccination schedule. Hydroxyurea is a drug that has been approved by the Food and Drug administration for use in adults with SCA. However, some pediatricians and pediatric hematologist are prescribing this drug in certain circumstances. This medication works by reactivating fetal hemoglobin production in place of the production of hemoglobin S. It also disrupts the sickling process and makes red blood cells less sticky. Consequently, this leads to fewer painful episodes, as well as, diminished intensity of them.

Exchange Transfusion – Another treatment modality is the use of exchange transfusion. This complex, procedure entails the exchanging of the patient’s red blood cells with normal red blood cells, resulting in a decrease in the percentage of sickled cells circulating.

Prognosis – The survival rate has drastically improved over the years. It is estimated that approximately 90% of patient’s survive to the age of 20 and 50% to the age of 50.

Neuroblastoma

Neuroblastoma is a type of cancer that develops from embryonic neural crest cells. These are immature nerve cells that can be found in several areas of the body. It is the second most frequently occurring tumor in children, the most common type of cancer in infancy and the most common extracranial (outside of the cranium or  skull)  tumor in childhood. Ninety percent of the cases are children younger than 5 years old.

Etiology or Causes

The etiology or cause of neuroblastoma is not very well understood.  What we do know, however, is that cancer in general begins with a genetic mutation whereas normal, healthy cells will continue to grow and not respond to the body’s signal to stop growing. They will continue to grow and multiply indiscriminately. This accumulation of abnormal cells will grow into a tumor.  Neuroblastoma begins its journey in neuroblast, which are immature nerve cells in the fetus. As the fetus matures, these cells become a part of the nerve cells and fibers, as well as, part of the cells that comprises the adrenal gland. Most of these neuroblast will mature by birth, however, some remain immature. Those immature cells will either disappear or will form a tumor, known as a neuroblastoma. There isn’t enough evidence to suggest a familial component since only 1-2% of case have that genetic component to it.

Signs and Symptoms

The signs and symptoms of neuroblastoma is contingent upon the origin of the tumor and the extent of metastasis. The most common site of origin is the abdomen.  Symptoms seen are abdominal pain, change in bowel habits (diarrhea), swollen abdomen, a non-tender abdominal mass, vomiting, and swelling in the legs. If the tumor is located in the chest, you could have wheezing, chest pain, and changes to the eyes, including drooping eyelids and unequal pupil size. Other signs that are prevalent with this condition includes back pain, bone pain, fever, unexpected weight loss, lumps of tissue under the skin, dark circles around the eyes and protruding eyeballs.

Diagnostic Test 

A thorough health history is often the catalyst for obtaining a diagnosis of neuroblastoma.  The parents are usually the first to notice a swollen or asymmetrical abdomen. Your child will undergo a CT scan or MRI to determine the site of the tumor and evidence of metastasis.  A chest x-ray, a bone scan and a skeletal survey may be performed to identify metastasis. Bone marrow aspirations and biopsies are performed to determine if the disease has migrated to the bone marrow. Elevated levels of catecholamines or their metabolites, such as dopamine, homovanillic acid (HVA) and vanillylmandelic acid (VMA) can be found in the urine or blood in 90% of the cases of neuroblastoma.   Neuroblastomas can also be detected by the use of the mIBG scan (meta-iodobenzylguanidine).  This nuclear medicine scan involves the injection of a radioactive liquid substance into the veins. This radioactive material is taken up by the tumors in up to 95% of cases and can be visualized with the use of a special gamma camera. This sophisticated scan is also utilized to monitor response to treatment of the disease.

Staging of Neuroblastoma – Once a diagnosis of Neuroblastoma has been made, your child’s physician will want to know, if the cancer has migrated (metastasized) to other organs and the lymph nodes. This can be determined by a series of imaging test and biopsy. The test utilized will include x-rays, bone scans, CT (computerized tomography) scans, MRI (magnetic resonance imaging) and MIBG scans and the process is known as staging.  Staging of the tumor is important because it determines the prognosis and the course of treatment.

  • Stage I – Tumor is confined to an organ or structure of origin
  • Stage IIA – The tumor extends beyond the organ or structure, but not beyond the midline. No lymph node involvement
  • Stage IIB – The tumor extends beyond the organ or structure, but not beyond the midline. Lymph node involvement on the same side of the tumor
  • The tumor has metastasized beyond the midline including bilateral lymph node involvement.
  • Stage IV – The cancer has metastasized (spread) to bones, bone marrow, other organs  and distant lymph nodes
  • Stage IV S – This is a special category of neuroblastoma, that applies only to children less than 1 year of age.  Metastasis has occurred to one or more sites except for the bones.

Treatment and Prevention

The treatment for the disease is dependent upon the staging of the tumor. It can be as conservative as observation due to the fact that some neuroblastomas will disappear without treatment.  However, in most cases of neuroblastoma some form of treatment is required. What is so unfortunate about neuroblastoma is that once the diagnosis is made, in many cases, metastasis has already taken place. Therefore, the surgical removal of the tumor, as well as, chemotherapy is warranted.  Radiation therapy and stem cell transplantation are other treatment modalities that are used to combat this disease.  There are other treatment options that are new which include immunotherapy using monoclonal antibodies and tumor vaccine.

When to see a doctor

Contact your child’s doctor if your child has any signs or symptoms that worry you. Mention any changes in your child’s behavior or habits.

Prognosis

With the proper treatment, your child’s chance of survival is very good. However, it is contingent on where your child ranked in the classification of the disease. If your child was classified as having low or intermediate risk disease your chances of a favorable prognosis is good. Their course of treatment is milder and the chance of a post disease sequela is lessened.    A child with high risk disease (stage 3 and 4) has a guarded prognosis.  Many survivors of the disease have post disease sequela which could include hearing loss, thyroid function disorder, stunted growth, learning difficulties, behavioral problems, and a greater risk of secondary cancer.

Leukemia

Leukemia is primarily a disorder of the body’s blood-forming tissues in which the normal elements are replaced with abnormal white blood cells. It involves the bone marrow and the lymphatic system. It accounts for one third of all childhood cancers. It is seen more prevalently in caucasian children than in any other race and it occurs more in males than in females.

Classification

Leukemia is classified as acute or chronic, lymphocytic or myelogenous and its classification is based on the type of cells involved and on the speed of progression.

With acute leukemia, you have a rapidly progressing disease involving abnormal, immature blood cells known as blast. These blood cells multiply so rapidly and are so immature, and consequently the disease worsens because the cells cannot carry out their normal work.   With chronic leukemia, the blood cells are more mature. The cells replicate and tend to accumulate more slowly and can even function normally for a short time. With chronic leukemia, the symptoms may not present themselves initially and in fact, may not manifest themselves for several years.

Lymphocytic Leukemia is a type of leukemia that involves the lymphoid cells (T-cells, B-cells, and pre-B cells) known as lymphocytes. These cells form lymphatic tissue which is part of your immune system.

Myelogenous Leukemia is a type of leukemia that involves the cells which arise from the blood forming tissue of the bone marrow. Myeloid cells help form your red blood cells, white blood cells and platelet-producing cells.

Etiology or Causes

Your white blood cells are the components in the blood that fight infection. Typically, they will grow and divide in an orderly way, to fit the needs of your body.  Lymphoid cells will grow and develop into lymphocytes and myeloid cells will grow and develop into red blood cells, granulocytes, monocytes and platelets. Lymphoid cell, granulocytes and monocytes are a type of white blood cell. However, with leukemia, the bone marrow produces abnormal white blood cells, which do not function in the proper manner. With the displacement of normal bone marrow cells with the higher number of immature white blood cells, the blood becomes deficient in the number of blood platelets which is an important component in the blood clotting process. Consequently, this will lead to easier bruising and bleeding.  With the immature white blood cells, you have dysfunction and the immune system becomes incompetent in its ability to fight off infection.  And finally, with the red blood cell deficiency, you can expect to see some fatigue, dyspnea (difficulty breathing) and pallor that you will typically see when a person is anemic.

Leukemia can develop at any time during the stages of normal blood cell development. The exact cause of leukemia remains unknown, however, genetics factors and chromosomal abnormalities may play a part in its development. One school of thought is that leukemia occurs when blood cells acquire a mutation in their DNA. Other changes within the cells, which are not completely understood, may contribute to the development of leukemia.

There are certain risk factors associated with this disease.

Those factors are:

  • Age 2-5 years old
  • Male gender
  • X-ray exposure in utero
  • Previous radiation-treated cancer
  • Ataxia telangiectasia, Down’s Syndrome and Shwachman Syndrome
  • Signs and Symptoms

Some of the more common signs and symptoms you will see includes fever, frequent infections, fatigue, listlessness, pallor, bone pain, abdominal pain, nausea and vomiting, bruising, tiny red spots on the skin (petechiae), recurrent nose bleeds, weight loss, swollen lymph nodes, possibly an enlarged spleen, and night sweats.

Diagnostic Test

Complete Blood Count:  You can expect to see an elevated, normal or decreased white blood cell count. Other findings will include low hemoglobin and hematocrit levels, a decreased platelet count and a decreased red blood cell count.

Bone Marrow Aspiration: The bone marrow aspirate is examined for the percentage of lymphoblast (> 25%), and also to examine for immuno-phenotyping.  Phenotyping is the process of classifying the type of cells involved, as well as, determining the level of mature cancer cells.  Cytogenetic analysis is the process used to determine abnormalities in chromosome structure and number.  Both cytogenetic analysis and immunophenotyping are used to determine the type of Leukemia, which is necessary to formulate a treatment plan.

Lumbar Puncture:  This procedure involves inserting a needle in the lower spinal column to obtain cerebrospinal fluid from the subarachnoid space. The fluid is examined to determine if leukemic cells have infiltrated the central nervous system.

Peripheral Blood Smear: The blood smear is examined for the presence of blast.

Comprehensive Metabolic Panel:  More specifically, doctors are looking at the liver function test components, as well as, the blood urea nitrogen and creatinine levels (both components used to assess kidney function). If the test results are abnormal, this may prohibit the use of certain chemotherapeutic agents. 

Treatment and Prevention

Treatment of leukemia can involve chemotherapy, radiation therapy, targeted therapy and possibly, bone marrow transplantation. Targeted therapy involves the use of an oral medication called “tyrosine kinase inhibitors or TKI’s. Tyrosine kinase is an enzyme that functions as an on/off switch for many cellular functions. The mechanism of action involves targeting tyrosine kinase and blocking its action. This slows or stops the growth of cancer cells.

Bone Marrow Transplantation

A Bone marrow transplant is a multi-step process. The first step involves finding a person, a donor (often a sibling), who will agree to contribute their bone marrow stem cells to the diseased person. The donor will then be tested to determine if their stem cells will be a match to the recipients.  If the donor and the recipient share the same Human Leukocyte Antigens (abbreviated as HLA), they are said to be a “match.”  This means that their tissues are immunologically compatible with each other. Next, the recipient’s bone marrow is depleted through radiation treatments and chemotherapy. Then, the donor’s stem cells are filtered from their blood and later injected intravenously into the recipient’s bloodstream.  These healthy stem cells will migrate into the bone marrow cavities where they will begin producing new blood cells. This process involves a lengthy hospital stay and typically, the patient is placed in isolation. There are risks involved with this procedure.  The recipient may reject the transplanted stem cells which can lead to life-threatening complications.  The treatment also may involve the use of drugs that will suppress the immune system. These drugs known as immune-suppressants, will aid in preventing the rejection of the donated stem cells.

Treatment of the child with leukemia also focuses on managing the symptoms and complications associated with this disease.  Antibiotics, pain medications and anti-emetics are used to fight infections, relieve the pain and control the nausea and vomiting.  Many children will require transfusions of blood products to treat the anemia and low platelet counts.  The blood products should be leukodepleted, irradiated, and cytomegalovirus (CMV)negative.  This is required to decreases the amount of antibodies in the blood (with blood transfusions, you are introducing the donor blood antibodies). Consequently, if a stem cell transplant becomes necessary, you diminish the chance of the child developing Graft Verse Host Disease, complication of the transplantation treatment.

Prognosis

The success of treatment is dependent on several factors; the type of leukemia and the age of the person. According to statistics, the average five-year survival rate is 57% in our country. For children, less than 15 years old, the five-year survival rate can be as high as 85%, contingent on the type of leukemia. In most cases of acute leukemia, if the child is cancer free for 5 years, the disease is unlikely to return.