Neuroblastoma Patient & Family Handbook

A Handbook for Families

Neuroblastoma

ONCOLOGY SERIES

A Handbook for Families

Neuroblastoma

ONCOLOGY SERIES

Neuroblastoma A HANDBOOK FOR FAMILIES

Author Linda D’Andrea, MSN RN PPCNP-BC CPHON®

Content Reviewers Steering Council

Parent Reviewer Stephanie McKeon

This handbook is published by the Association of Pediatric Hematology/Oncology Nurses (APHON) for educational purposes only. The material has been developed by sources believed to be reliable. The material is not intended to represent the only acceptable or safe treatment of neuroblastoma. Under certain circumstances or conditions, additional or different treatment may be required. As new research and clinical experience expand the sources of information available concerning the treatment of neuroblastoma, adjustments in treatment and drug therapy may be required. APHON makes no warranty, guarantee, or other representation, express or implied, concerning the validity or sufficiency of the treatments or related information contained in this handbook. APHON provides this handbook for educational use. Users may copy and distribute this material in unadapted form only, for noncommercial use, and with attribution given to APHON.

n INTRODUCTION Hearing that your child has cancer is shocking and overwhelming. At first, you may not believe it, or you may hope the diagnosis is wrong. However, the reality of the situation will be confirmed as you see the changes in your child and experience being in the hospital and beginning treatment. Educating yourself on your child’s diagnosis, treatment plan, and possible side effects and familiarizing yourself with the members of your child’s healthcare team and available resources may help ease some of these feelings. n WHAT IS NEUROBLASTOMA? Neuroblastoma is a rare childhood cancer that develops from nerve cells that are found throughout the body. Often, the tumor appears from branches of nerves that extend from the spinal cord. These branches reach many areas of the body, so tumors can be found in many different locations. In more than half of the cases of children with neuroblastoma, the tumors are found in the abdomen or belly and usually involve the adrenal gland, which sits on top of the kidney. Neuroblastoma also can be found in the head, chest, neck, pelvis, bone, and bone marrow.

n WHAT ARE SOME SIGNS AND SYMPTOMS OF NEUROBLASTOMA? The symptoms of neuroblastoma depend upon the location of the cancer. For example, if the tumor is in the abdomen, the child’s belly may appear enlarged or bloated, and he or she may complain of or show signs of abdominal pain, constipation, or diarrhea. If the cancer has spread to the bones, there may be bone pain that can result in the child’s inability or refusal to walk or use a certain arm

or leg. If tumor cells affect the bone(s) around the eyes, it often appears as if the child has a black eye. Skin tumors may occur and appear as blue or purple hardened areas under the skin that can be seen or felt. Some general signs of neuroblastoma may be loss of appetite (usually without significant weight loss), tiredness, minor aches and pains, unexplained fevers, bleeding, bruising, and difficulty breathing if the tumor is putting pressure on the lungs. More often, symptoms are subtle and difficult to put together, resulting in neuroblastoma not being diagnosed until the disease is in an advanced stage.

n WHAT CAUSES NEUROBLASTOMA? The cause of neuroblastoma is unknown. We do know, however, that it is not contagious and cannot be caught from another person. In very few instances, neuroblastoma may be caused by a change in the body’s genes that are passed from a parent to a child. Genes are forms of DNA. DNA is a collection of chemical information that carries instructions for making all the proteins a cell will ever need. However, it is extremely rare to have more than one person with neuroblastoma in a family. We also know that some infants are born with this cancer, which suggests that there is an accidental growth that occurs while the unborn child’s nerve cells are growing and maturing. The nerve cells remain immature and develop into neuroblastoma cells. Who Gets Neuroblastoma? Although neuroblastoma is the third most common type of cancer in children, it is still rare. Approximately 800 cases are diagnosed each year in the United States. In most cases, it is a disease of early childhood. About one third of children diagnosed with neuroblastoma are infants, and almost 90% of all children with neuroblastoma are younger than 5 years at diagnosis. Cases in children older than 10 years are extremely rare. What Is Metastasis? Metastasis refers to the spread of a tumor from its original location (primary site) to other parts of the body. Neuroblastoma metastasizes, or spreads, very easily, most commonly to the lymph nodes, bone and bone marrow (the inside of the bone where blood cells are made), liver, or skin. Neuroblastoma has been called a silent tumor because approximately 60% of children with this tumor already have metastases before any signs of the disease are noticed or diagnosed. What Is Staging and Risk Group Staging? Staging is the process of determining the location and amount of disease at the time of diagnosis. The International Neuroblastoma Staging System (INSS) divides neuroblastoma into four stages, ranging from a single tumor (stage 1) to a tumor plus disease that has spread to other parts of the body (stage 4). Another staging system, the International Neuroblastoma Risk Group (INRG), classifies your child’s disease as L1, L2, M, or Ms. In addition to determining the stage of neuroblastoma, your healthcare team will look at several additional factors to determine how aggressive the tumor is likely to be and determine the treatment your child will receive. These factors include the following: • whether the tumor is localized (a single tumor) or metastasized (spread to other parts of the body) • your child’s age at diagnosis • what the tumor looks like under the microscope • whether the tumor has more than 10 copies of the MYCN oncogene (a gene with the potential to cause cancer) • changes to chromosome 1p and 11q.

These factors will help your healthcare team determine if your child is classified as being in the low-, intermediate-, or high-risk group. Each stage and risk group is associated with a different treatment plan and prognosis. Your healthcare team will speak to you in detail about this.

n WHAT TESTS AND PROCEDURES WILL MY CHILD NEED? Many tests and procedures are necessary to diagnose neuroblastoma and determine the extent of your child’s disease. These tests will be repeated to evaluate your child’s response to treatment. Some of the tests and procedures may include the following:

CT Scan The CT (computerized or computed tomography) scan—which is less frequently referred to as a CAT (computerized axial tomography) scan—shows detailed pictures of internal organs and tumors. It is a computer-assisted X ray. The CT scan machine is a table surrounded by a doughnut-shaped X-ray camera. Every second, that camera moves a very small distance around the patient while taking an X ray. A computer puts these X rays together to help the doctors see a cross-sectional, 3-D image from all angles. CT scans are very useful in diagnosing tumors, infections, blood clots, and areas of abnormal bleeding. Certain tumors are regularly checked by CT scan during and after treatment to determine the effectiveness of treatment or if there is a relapse. It is a painless test, but the child must be able to lie still during the scan. Some children require sedation to help them lie still. If the child is having a CT scan of the belly, they will be asked to drink a special dye that improves the images created during the scan. Some children may require a small amount of dye to be injected through an intravenous (IV) line as well. If your child receives the dye into their vein, they may have a warm, flushing feeling. Generally, there are no side effects from either type of dye; however, allergic reactions are possible. There is some radiation exposure with this test. Your child’s healthcare team will try to minimize how often CT scans are done, but they are very helpful in diagnosing and following the stage and location of the cancer. MRI MRI (magnetic resonance imaging) is a test that uses radio waves and magnetic fields instead of X rays. A computer translates the pattern of radio waves into very detailed pictures of the inside of the body. MRI is sometimes more useful than X rays or CT scans because it can create clearer images. This is true with areas of the brain and spinal cord, blood vessels, bones, and some organs of the body. Certain tumors are regularly checked by MRI during and after treatment to determine the effectiveness of treatment or if there is a relapse.

A person having an MRI lies on a table which is rolled into a machine that surrounds them on all sides, like a tube. There are open MRI machines available for people who find themselves very anxious in a regular MRI machine. The test is painless, but the machine makes quite a bit of noise. Your child may possibly use ear plugs or a headset during the test. They must be able to lie completely still, and some children require sedation to do this. The test usually takes one to two hours to finish. Often, a dye that will improve the pictures will be injected into the vein. Because it uses magnets, MRI machines cannot have metal objects inside them. Your child will be asked to remove all metal objects, including jewelry and clothes with metal snaps or zippers. An MRI does not expose your child to any form of radiation. MIBG A meta-iodobenzylguanidine (MIBG) scan is a very important test that looks for neuroblastoma throughout the body. MIBG is a clear liquid that is injected into a vein and absorbed by neuroblastoma tumor cells. The substance lights up on a nuclear scan so the tumor cells can be detected. MIBG is a radioactive substance, but it is given in such a small amount that your child receives less radiation than from an X ray. Potassium iodine drops are taken orally in the days surrounding the scan to protect the thyroid gland from radiation. The MIBG later leaves the body in the urine. A mom who is pregnant should speak to the nuclear medicine staff about any precautions she should take in caring for her child. The nuclear scan is done 1–3 days after the injection of MIBG. Your child may require sedation or anesthesia to stay still for the scan. Higher doses of MIBG may be used later in the treatment of your child’s neuroblastoma if first-line treatments are not 100% effective. Ultrasound Ultrasound uses the echoes from high-frequency sound waves to produce a picture of internal organs or tumors. It can help find tumors in the belly, reveal a lot about the health of the kidneys and bladder, or be used to measure the size of some organs, especially the liver and spleen. The ultrasound can show blood flow through the veins and whether the blood flow is blocked by a clot. It is a painless test and uses no radiation. The child lies on a table for a short time. A cold, clear jelly is smeared on the area being examined and wipes off easily after the test. A small, round handle is placed on the jelly and moved around to get a clear picture of the tissue or organ. If the ultrasound is of the belly, the child may not be permitted to eat or drink for several hours before the test. Urine Test Urine is tested for chemicals called catecholamines , specifically vanillylmandelic acid (VMA) and homovanillic acid (HVA), which are released by neuroblastoma tumors. Only a small amount of urine may need to be collected over a 24-hour period for this test. If your child is not toilet-trained, a simple collection bag can be placed over the penis or vaginal area or a catheter can be inserted into the bladder to obtain urine for testing. You may be asked not to allow your child to have certain foods, drinks, or medications that may affect the results of this test.

Blood Tests Blood tests are done to monitor your child’s blood cells, body salts, and chemistries. Blood tests are very helpful in diagnosing medical problems and monitoring for side effects of treatment. A specially trained nurse or technician uses a needle to enter a vein and collect a blood sample in tubes that are then sent to a laboratory where they are examined closely. In some cases, a finger prick may give enough blood for a test. These tests are usually done at the time of diagnosis to rule out other diseases and to serve as a baseline for your child. Blood tests are used throughout treatment to monitor for possible side effects and your child’s response to treatment. Blood tests such as creatinine and blood urea nitrogen (BUN) monitor changes in kidney function. Blood tests called hepatic function panel and bilirubin detect changes in liver function. Alkaline phosphate and lactic dehydrogenase (LDH) levels may be high in the blood due to bone growth from a tumor. Complete Blood Count Blood is made up of plasma (the liquid part of the blood) and cells that are floating in the plasma. There are three types of blood cells: white blood cells, red blood cells, and platelets. The white blood cells fight infection as part of the body’s immune system. The red blood cells carry oxygen to all cells of the body. The platelets stop bleeding by rushing to the site of an injury and forming a clot. A complete blood count , or CBC, is a blood test used to measure the amount and the health of these three types of cells. The CBC is useful in diagnosing blood disorders that a child has inherited or developed. A CBC’s results may change due to other things going on in the body, such as infections, nutritional problems, or as a response to some medications like chemotherapy. Venous Access Device (VAD) A venous access device (VAD), sometimes called a central venous cathete r, is a temporary or long-term intravenous (IV) tube that can be used for the duration of your child’s treatment. It is placed in a large vein in the chest or the arm. These long, flexible catheters empty into or near the heart, allowing necessary treatments to be given within seconds. It can be used to administer medications, IV fluids, chemotherapy, blood products, and nutritional support when needed, as well as to draw blood for testing. It is inserted during surgery while your child is sedated or has been given general anesthesia, either before chemotherapy begins or within the first month of treatment. Not all children need a VAD. This is determined by you and your healthcare team. The VAD will be removed when it is no longer needed. There are different types of VADs, such as an implanted port inserted under the skin near the collarbone, a tunneled central venous catheter (Broviac® or Hickman®) that has a tube, a lumen (either double or single) which comes out of your child’s chest, or a PICC line (a central line inserted through a vein in the arm). Your healthcare team will discuss each type with you and help you choose the best device for your child’s treatment. The care for each of these types of catheters is slightly different. A port requires almost no care at home but a special needle (noncoring) will need to be inserted through the skin

into the port each time it is used. Tubing can be attached to the needle as needed to give medication or IV fluids. An external central line requires routine dressing changes to prevent infection and to care for the skin around the tube. The external lines also are routinely flushed with a solution to keep them working properly. You will be taught how to care for the device at home. Bone Marrow Aspiration and Biopsy A bone marrow biopsy is a test done to determine whether tumor cells are in the bone marrow. A needle is inserted through into the marrow inside a bone. Some of the bone marrow (the biopsy) and the fluid in the bone marrow (the aspirate) are collected. It usually is necessary to obtain bone marrow samples from both hip bones. Patients undergoing bone marrow aspirates and biopsies may experience pain; children are often sedated for this procedure. If your child is uncomfortable after the test, a mild pain medication such as Tylenol® may be given, but not aspirin or ibuprofen (Motrin® or Advil®). A specially trained doctor (a pathologist or hematologist) examines the bone marrow under a microscope to see if white blood cells, red blood cells, and platelets are being produced normally. The doctor also looks for any abnormal cells, such as immature blood-forming cells (blasts), which can indicate leukemia; tumor cells which can indicate a different type of cancer; or dysmorphic (abnormal-looking) cells, which can indicate a different disease. The bone marrow also is assessed for protein expression of malignant (cancer) cells, which can indicate leukemia, and chromosomal abnormalities, which can diagnose other diseases. Other tests include immune-phenotyping, cytogenetics, and fluorescent in situ hybridization (FISH). During treatment the bone marrow is checked regularly to determine response to the treatment. The number of cancer cells can be measured and will indicate if and how well the treatment is working. Tumor Biopsy A biopsy of the tumor can help your child’s healthcare team make the diagnosis and choose the proper treatment. Biopsy is routinely done by removing an enlarged lymph node or a small part of a tumor. The biopsy is usually performed by a surgeon in an operating room, but some medical centers do biopsies in the radiology department. In some cases, the biopsy sample is obtained through a needle that is guided into the lymph node or tumor (fine needle aspiration biopsy). In other cases, a surgical incision is made. Sedation is often used for biopsies. Your child’s comfort and anxiety level will always be considered in planning procedures that involve needles.

n HOW IS NEUROBLASTOMA TREATED? Your child’s treatment for neuroblastoma will be based on their staging and risk group: low risk, intermediate risk, or high risk.

For infants with low-risk neuroblastoma, whose disease has not spread to the bone, the tumor sometimes breaks down on its own. In these instances, your healthcare team may observe your child closely without treatment. Neuroblastoma that consists of a single tumor or mass (low risk) that can be removed totally with surgery may not require further treatment. Intermediate-risk neuroblastoma may be treated by surgical removal of the tumor with or without moderate doses of chemotherapy. High-risk neuroblastoma most often is treated with a combination of chemotherapy, surgery, high-dose chemotherapy with peripheral blood stem cell rescue (often referred to as a stem cell transplant), radiation therapy, immunotherapy, retinoic acid, and possibly targeted radionuclides (MIBG therapy). Your healthcare team will speak to you in detail about your child’s expected treatment course. Surgery Surgery to remove the tumor usually is only done at the time of diagnosis in low-risk neuroblastoma that will not require further treatment. In high-risk neuroblastoma, the complete or partial removal of the tumor through surgery is more successful after the tumor has been shrunk by chemotherapy. Using chemotherapy first minimizes the risk during surgery of injuring normal organs that the neuroblastoma may be attached to. Chemotherapy Most children with neuroblastoma will receive chemotherapy (anticancer drugs), which involves giving specific drugs to kill cancer cells. Several chemotherapy medications are known to be effective in killing neuroblastoma cells. Cyclophosphamide, topotecan, doxorubicin, vincristine, cisplatin, and etoposide are the agents most commonly used in induction (the initial phase of treatment that aggressively tries to get rid of the disease). Other agents that may be used to treat neuroblastoma include ifosfamide, carboplatin, irinotecan, temozolomide, and several others. No single drug can control this disease by itself, so the drugs are given in a special combination, most often into a vein through a venous access device (VAD), which was discussed earlier in this booklet. Each treatment lasts several days and is usually given in the hospital or a clinic. When chemotherapy is needed, close monitoring of your child will be a priority. Possible side effects of the specific chemotherapy your child requires will be explained to you in detail by your child’s healthcare team.

High-Dose Chemotherapy with Peripheral Blood Stem Cell Rescue (Stem Cell Transplant) Chemotherapy can be given in low doses, standard doses, high doses, and even very high doses. When very high doses are used, this is called ablative therapy. Special procedures are required to ensure that a child recovers from ablative therapy. Early in the treatment phase, your child’s stem cells will be collected from the peripheral blood using a special machine that filters out stem cells and returns the remaining blood to your child. These important cells are frozen for later use. After the induction therapy has been given and the tumor has been removed by surgery, very high doses of chemotherapy may be given to overwhelm and destroy the remaining neuroblastoma. An unavoidable result of this very high-dose therapy is the weakening of the body’s blood-producing organ, the bone marrow. Your child may be given back his or her own stored stem cells to promote normal body and organ function when the treatment is complete. This is often referred to as stem cell transplant . The process of administering high-dose chemotherapy with peripheral blood stem cell rescue may be done once or twice. When a patient receives their own stem cells back twice, this is referred to as tandem transplant . Radiation Neuroblastoma cells often are very sensitive and easily killed by radiation. Radiation treatment is very precise and given in specially measured amounts by radiation therapy experts. If your child will receive radiation treatment, the radiation oncologist will discuss with you exactly how the radiation will be given and how long the treatments will last. In general, the side effects of radiation are directly related to the area of the body receiving the radiation treatment. Overall, children experience few side effects while they are getting radiation therapy. The more common side effects include tiredness, decreased appetite, nausea, vomiting, diarrhea, and skin irritation. There may be side effects later, again depending on which area receives the radiation treatment. Possible side effects will be discussed with you in detail by your child’s healthcare team. Immunotherapy Immunotherapy may be part of the treatment for your child’s neuroblastoma. Generally, it is used after tumors have been surgically removed, when there is only minimal disease remaining or no evidence of disease, meaning so few neuroblastoma cells are present that they are not visible on scans. This treatment is designed to train the body’s own immune system to find and kill neuroblastoma cells that have survived the treatment already administered. Immunotherapy involves the injection of a substance called a monoclonal antibody into the bloodstream. The monoclonal antibody attaches to a marker on the surface of neuroblastoma cells, which causes the body to see the neuroblastoma cell as foreign and destroy it. The two most common monoclonal antibodies used in the treatment of neuroblastoma are dinutuximab (Unituxin®) and naxitamab. These antibodies usually are given in combination with other agents that stimulate the immune system and make the antibodies more effective. Your team will talk to you about potential side effects your child may experience.

Retinoic Acid Retinoic acid (isotretinoin) is a type of vitamin A derivative. It may be effective in the treatment of neuroblastoma when there is little to no evidence of disease. This medication works by taking immature, rapidly dividing neuroblastoma cells and teaching them to become mature nerve cells. It is given by mouth for 2 weeks each month for 6 months. The most common side effects include sensitivity to light; dry skin; and dry, cracked lips. Less common side effects may include irritability, mood swings, bone pain, nausea and abdominal discomfort, and diarrhea. Targeted Radionuclides Previously, we discussed how MIBG is absorbed by neuroblastoma tumors and cells and used to detect neuroblastoma throughout a child’s body at diagnosis and during follow-up testing. Higher doses of MIBG have been used as treatment for high-risk neuroblastoma that has not completely responded to standard therapies. Studies are underway that are looking at using this treatment option earlier in treatment, in induction therapy. If this is a treatment option for your child, your healthcare team will talk to you about potential side effects your child may experience. n HOW LONG WILL MY CHILD’S TREATMENT LAST? The length of time your child will receive treatment will be based on how his or her disease responds to the treatment. Generally induction chemotherapy, surgery, ablative chemotherapy, and radiation treatment take 6–9 months. The duration of immunotherapy varies, but is usually between 4–6 months, followed by retinoic acid for an additional 6 months. Some of these treatments may overlap. Follow-up blood tests and physical exams between chemotherapy treatments are done on an outpatient basis. However, your child may have to stay in the hospital if a fever develops while blood counts are low and the immune system is weakened. n WHAT IS MY CHLD’S PROGNOSIS? Prognosis varies greatly based on your child’s risk level. Neuroblastoma has an increased chance of being permanently cured if it is low or intermediate risk. High-risk neuroblastoma is more difficult to control, and the cure rate is lower, although it can be successfully treated. New therapies are being developed to improve treatment success. It is important to keep in mind that neuroblastoma may present differently in each child, with different characteristics, and may respond differently to treatment.

n WHAT NEW THERAPIES ARE AVAILABLE? Most of the advances made in the treatment of childhood cancer have been gained through a process known as clinical trials. In clinical trials, the best known (standard) treatment for a specific cancer is compared with a new (experimental) treatment that is believed to be at least as good as the standard. Clinical trials allow doctors to determine whether promising new treatments are safe and effective. Clinical trials test new chemotherapy combinations and regimens, alternative ways of giving radiation therapy, updated procedures for stem cell rescue following high-dose chemotherapy, advances in immunotherapy, targeted radionuclides, genetic testing and medications that target abnormal gene growth, and much more. Participation in clinical trials is voluntary. On one hand, because clinical trials involve research into new treatment plans, all risks cannot be known ahead of time, and unknown side effects may occur. On the other, children who participate in clinical trials can be among the first to benefit from new treatments. Before making a decision about your child’s participation in a clinical trial, discuss the risks and the potential benefits with your child’s doctor and treatment team. More information about clinical trials is available in the free booklet Taking Part in Cancer Treatment Research Studies (National Cancer Institute Publication P105). To get this booklet, go to https://pubs.cancer.gov/ncipl/detail.aspx?prodid=P105. n HOW CAN I WORK WITH MY CHILD’S HEALTHCARE TEAM? Your child’s care requires a team approach. You are a key player on your child’s healthcare team. You know your child better than anyone else and your input is important. Other members of your child’s healthcare team include doctors, nurses, pharmacists, and social workers. Always communicate openly with the members of your child’s healthcare team. Ask questions when there is anything you are not sure about. Write down your questions as you think of them to help you remember them when the healthcare team is there. Here are some examples of questions to ask: • What kind of cancer does my child have? • Has the cancer spread beyond the primary site? • What is the stage and risk group of the cancer, and what does that mean? • Is the MYCN gene amplified in my child? • What tests need to be done before starting treatment?

• Will genetic testing be performed on my child’s tumor? • What treatment choices are available? • What treatment do you recommend and why? • What is the order of treatment for my child? • How long will my child be in treatment?

• What risks or side effects does the recommended treatment have? • What are the long-term risks from the disease and the treatment? • What should we do to prepare for treatment?

• What is my child’s outlook for survival? • What are the chances of a recurrence?

• Who are the members of my child’s healthcare team and what are their roles? • What financial, psycho-social, and other resources are available for my child and me? • Will all treatments be given in the hospital? • How many cases of neuroblastoma have you treated in the past year? Use this space to write down any additional questions.

n ARE MY FEELINGS NORMAL, AND WHAT CAN I DO ABOUT THEM? Many families feel that they are somehow responsible for their child’s disease. They may feel guilty that they could not protect their child from illness or about the amount of time it took to diagnose their child. This disease is not caused by anything that you did or did not do. The cause of childhood cancer is not known. Symptoms are often the same as many childhood illnesses, so it often takes some time for the diagnosis of cancer to be made. Feelings of sadness, anger, and helplessness about your child’s diagnosis also are common. These feelings are normal. Each member of the family may express these emotions in different ways and at different times. Talking honestly with each other about these feelings, emotions, and reactions will help everyone in the family. Keep in mind there is no right or wrong way to feel. Each member of the family needs the chance to express their feelings when they are ready and in their own way. Talking to friends, family, and members of the healthcare team can be difficult at times— however, sharing your feelings can help you cope. Your child will benefit from family and friends showing their care through communication and support.

n HOW CAN I HELP MY CHILD? Children often think that something they did caused their cancer; it will help if you reinforce that this is not the case. Make sure your child understands that your feelings of anger and sadness are directed at the cancer, not at them. This will help to keep your relationship straightforward and maintain closeness. Your child will need to share their feelings with someone that they trust. Sometimes, children choose to share feelings with someone other than a parent because they are afraid they might upset the parent. Don’t be afraid to ask your child about their feelings—it may be what your child is waiting for. Also, don’t be afraid to share information with your child about what is happening and why. Sometimes, the things children imagine on their own are more frightening than what is actually happening. In spite of your child’s disease, they are still a child first. All children need love, attention, the opportunity to learn and try new skills, and limits. As your child goes through cancer treatment, they still have all the needs of a growing child. Do not avoid talking to your child about treatment. Use explanations your child will understand. Children tolerate treatment better if they understand it and if they are allowed to help make decisions about their care, when appropriate. It is difficult for parents to watch their children go through cancer treatment. Your child may sometimes seem sicker than before the treatment. Your feelings about what your child is going through during treatment must be balanced with the knowledge that treatment provides the chance of curing the disease and having your child live a full and meaningful life. It is often difficult to accept the changes your child experiences from cancer and treatment, but many of the changes, like hair loss, are temporary. Always remember: Despite outward changes your child is still the same person. n IS MY CHILD’S DIET IMPORTANT DURING TREATMENT? Yes. Research shows that well-nourished children tolerate cancer treatment better and experience fewer treatment delays. Nausea and vomiting can be a distressing side effect of treatment, and your child’s healthcare team will prescribe medications to help prevent and treat these side effects. Your child may have difficulty eating normal meals during treatment. Foods that are appealing to your child will be tolerated better. Offer foods that are high in calories, protein, and carbohydrates. Frequent small meals are often better tolerated than three larger meals. When possible, avoid junk food and foods that are high in salt. Choose more nutritious foods. If your child is on steroid medications such as prednisone or Decadron (dexamethasone), their appetite will likely be increased. While on these medicines, your child may be constantly hungry, crave unusual foods, or want one particular food constantly. Try to keep healthy snacks on hand. Steroids may cause your child to gain weight and develop a round face and puffy appearance; these side effects are temporary.

Your child needs to stay hydrated, too. Offer your child fluids to drink frequently throughout the day. If your child is not drinking enough fluids throughout the day, they may become dehydrated. Signs of dehydration include decreased urine output, dizziness, and a dry mouth. Multivitamins, herbs, and other supplements should be avoided unless approved by your child’s healthcare team to avoid interactions with your child’s chemotherapy. Please ask your healthcare team if you are considering any of these types of treatments. A dietitian may be part of your child’s healthcare team. Trained in the calorie and nutritional needs of children, the dietitian can explain your child’s dietary needs and ideas about how to meet these needs during treatment. Your child’s healthcare team will monitor your child’s nutritional status, height, and weight during and after treatment to try to prevent problems. When there is a concern about nutrition, your child’s healthcare team will talk with you about ways to improve your child’s nutrition. n CAN MY CHILD ATTEND SCHOOL DURING TREATMENT? Your child’s ability to go to school during treatment will depend on the intensity of the treatment and your child’s response to treatment. There may be extended periods when your child is unable to go to school because of hospitalizations or treatments. However, it is important for your child to keep up with their schoolwork even when they cannot go to school. Federal laws ensure that your child can receive the same education as their peers. Talk with your child’s healthcare team and school about these laws. Then work with them to arrange for a tutor or other ways to keep your child on track with schoolwork. Discuss your child’s ability to attend school with the healthcare team. Some children are able to attend school between hospitalizations or treatments. Many hospitals and clinics have resources such as educational liaisons who will assist in coordinating school services and preparing for your child’s return to school by educating teachers and classmates.

n ONLINE RESOURCES CureSearch, the website of the National Childhood Cancer Foundation and the Children’s Oncology Group , www. curesearch.org National Cancer Institute , www.cancer.gov/cancerinfo New Approaches to Neuroblastoma Therapy (NANT) , www.nant.org

IMPORTANT PHONE NUMBERS

NOTES

n IN RECOGNITION OF PREVIOUS CONTRIBUTORS Kathleen Adlard, MN RN CPON® Kellei Agostinelli, RN CPON® Linda D’Andrea, MSN RN PNP-BC CCRN Martha Funkhouser, PHN BSN RN Susan Kreissman, MD Lynn Wirick, MSN RN CPON®

8735 W. Higgins Road, Suite 300 Chicago, IL 60631 847.375.4724 • Fax 847.375.6478

info@aphon.org www.aphon.org

Manual para la familia

Neuroblastoma

SERIE ONCOLOGÍA

Manual para la familia

Neuroblastoma

SERIE ONCOLOGÍA

Neuroblastoma MANUAL PARA LA FAMILIA

Autor Linda D’Andrea, MSN RN PPCNP-BC CPHON®

Revisor de contenido Steering Council

Revisor por parte de los padres r Stephanie McKeon

Este manual ha sido publicado por la Association of Pediatric Hematology/Oncology Nurses (APHON) con fines educativos únicamente. El contenido ha sido desarrollado por fuentes confiables y no pretende ser el único tratamiento aceptable o seguro para el neuroblastoma. Es posible que bajo ciertas circunstancias o condiciones se requiera un tratamiento adicional o diferente. A medida que las nuevas investigaciones y la experiencia clínica amplían las fuentes de información disponibles sobre el tratamiento del neuroblastoma, es posible que sea necesario ajustar el tratamiento y la farmacoterapia. APHON no garantiza ni asegura, ni hace ninguna otra declaración, expresa o implícita, con respecto a la validez o suficiencia de los tratamientos o a la información relacionada contenida en este manual. APHON proporciona este manual para uso educativo. Los usuarios pueden copiar y distribuir este material únicamente en su forma no adaptada, para uso no comercial y con atribución a APHON.

n INTRODUCCIÓN Enterarte de que tu hijo(a) tiene cáncer es impactante y abrumador. Al principio, es posible que no lo creas o que esperes que el diagnóstico sea incorrecto. Sin embargo, la realidad de la situación se confirmará a medida que veas los cambios en tu hijo(a), su estancia en el hospital y el comienzo del tratamiento. Mantenerte informado sobre el diagnóstico, el plan de tratamiento y los posibles efectos secundarios, así como familiarizarte con los miembros del equipo de atención médica y con los recursos disponibles, puede ayudarte a sobrellevar algunos de estos sentimientos. n ¿QUÉ ES UN NEUROBLASTOMA? El neuroblastoma es un cáncer infantil poco común que se desarrolla a partir de células nerviosas que se encuentran en todo el cuerpo. A menudo, el tumor se desarrolla a partir de ramas de nervios que se extienden desde la médula espinal. Estas ramas llegan a muchas áreas del cuerpo, por lo que los tumores se pueden encontrar en varios lugares diferentes. En más de la mitad de los casos de niños con neuroblastoma, los tumores se encuentran en el abdomen o vientre y generalmente afectan la glándula suprarrenal, que se encuentra en la parte superior del riñón. El neuroblastoma también se puede encontrar en la cabeza, el pecho, el cuello, la pelvis, los huesos y la médula ósea.

n ¿CUÁLES SON ALGUNOS SIGNOS Y SÍNTOMAS DEL NEUROBLASTOMA?

Los síntomas del neuroblastoma dependen de la ubicación del cáncer. Por ejemplo, si el tumor está en el abdomen, éste se puede ver agrandado o hinchado, y tu hijo(a) puede quejarse o mostrar signos de dolor abdominal, estreñimiento o diarrea. Si el cáncer se ha diseminado a los huesos,

puede haber dolor en los huesos que haga que no pueda o no quiera caminar o a usar cierto brazo o pierna. Si las células tumorales afectan el(los) hueso(s) alrededor de los ojos, puede parecer como si tuviera un ojo morado. Los tumores de la piel pueden aparecer como áreas endurecidas de color azul o púrpura debajo de la piel que pueden verse o sentirse. Algunos signos generales del neuroblastoma pueden ser pérdida de apetito (generalmente sin una pérdida significativa de peso), cansancio, dolores y molestias leves, fiebre inexplicable, sangrado, hematomas y dificultad para respirar si el tumor ejerce presión sobre los pulmones. Muy a menudo, los síntomas son sutiles y difíciles de relacionar, lo que hace que el neuroblastoma no se diagnostique hasta que la enfermedad se encuentra en una etapa avanzada.

n ¿QUÉ CAUSA EL NEUROBLASTOMA? Se desconoce la causa del neuroblastoma. Sin embargo, se sabe que no es contagioso y que no puede adquirirse a través de otra persona. En muy pocos casos, el neuroblastoma puede ser causado por un cambio en los genes que se transmiten de padres a hijos. Los genes son formas de ADN, que es una colección de información química que contiene instrucciones para producir todas las proteínas que necesita una célula. Sin embargo, es extremadamente raro que haya más de una persona con neuroblastoma en la familia. Se sabe que algunos bebés nacen con este cáncer, lo que sugiere que hay un crecimiento accidental que ocurre mientras las células nerviosas del feto crecen y maduran. Las células nerviosas permanecen inmaduras y se convierten en células de neuroblastoma. ¿Quién puede contraer neuroblastoma? Aunque el neuroblastoma es el tercer tipo de cáncer más común en niños, sigue siendo raro. Cada año se diagnostican en los Estados Unidos unos 800 casos. En la mayoría de los casos, es una enfermedad de la primera infancia. Aproximadamente un tercio de los niños diagnosticados son bebés y casi 90% de todos los niños con neuroblastoma son menores de 5 años al momento del diagnóstico. Los casos en niños mayores de 10 años son extremadamente raros. ¿Qué es una metástasis? La metástasis es la dispersión de un tumor desde su ubicación original (sitio primario) a otras partes del cuerpo. El neuroblastoma hace metástasis, o se disemina, muy fácilmente, con frecuencia a los ganglios linfáticos, los huesos y la médula ósea (el interior del hueso donde se producen las células sanguíneas), el hígado o la piel. Se le llama tumor silencioso porque aproximadamente 60% de los niños con este tumor ya tienen metástasis antes de que se diagnostique o se note los signos de la enfermedad. ¿Qué son la estadificación y la estadificación de grupos de riesgo? La estadificación es el proceso para determinar la ubicación y la magnitud de la enfermedad en el momento del diagnóstico. El INSS (International Neuroblastoma Staging System / Sistema Internacional de Estadificación del Neuroblastoma) divide el neuroblastoma en cuatro etapas, que van desde un solo tumor (etapa 1), hasta un tumor, más la enfermedad diseminada a otras partes del cuerpo (etapa 4). Otro sistema de estadificación es el del INRG (International Neuroblastoma Risk Group / Grupo Internacional de Riesgo de Neuroblastoma), que clasifica la enfermedad como L1, L2, M o Ms. Además de determinar la etapa en que está el neuroblastoma, el equipo de atención médica analizará varios factores adicionales para determinar qué tan agresivo puede ser el tumor y así decidir el tratamiento que recibirá tu hijo(a). Estos factores incluyen lo siguiente: • si el tumor está localizado (un solo tumor) o ha hecho metástasis (diseminado a otras partes del cuerpo) • la edad de tu hijo(a) en el momento del diagnóstico

• cómo se ve el tumor bajo el microscopio • si el tumor tiene más de 10 copias del oncogén MYCN (un gen con el potencial de causar cáncer) • cambios en los cromosomas 1p y 11q. Estos factores ayudarán al equipo de atención médica a determinar si el grupo de riesgo de tu hijo(a) es bajo, intermedio o alto. Cada etapa y cada grupo de riesgo requiere un plan de tratamiento y un disgnódstico diferente. El equipo de atención médica te hablará en detalle sobre esto. n ¿QUÉ PRUEBAS Y PROCEDIMIENTOS NECESITARÁ MI HIJO(A)? Se necesitan muchas pruebas y procedimientos para diagnosticar el neuroblastoma y determinar la extensión de la enfermedad. Estas pruebas se repetirán para evaluar la respuesta de tu hijo(a) al tratamiento. Algunas de las pruebas y procedimientos pueden incluir lo siguiente: Tomografía computarizada (CT) La TC (tomografía computarizada), que se conoce con menos frecuencia como exploración CAT (tomografía axial computarizada), muestra imágenes detalladas de los órganos internos y de los tumores. Es una radiografía asistida por computadora. La máquina de tomografía computarizada es una mesa rodeada por una cámara de rayos X con forma de rosquilla o dona. Segundo a segundo, la cámara avanza una distancia muy pequeña alrededor del paciente mientras toma una radiografía. Una computadora reúne estos rayos X para ayudar a los médicos a ver una imagen tridimensional en sección transversal desde todos los ángulos. Las tomografías computarizadas son muy útiles para diagnosticar tumores, infecciones, coágulos de sangre y áreas con sangrado anormal. Ciertos tumores se checan periódicamente mediante una tomografía computarizada durante y después del tratamiento para determinar la eficacia del tratamiento o si hay una recaída. Es una prueba indolora, pero los niños deben permanecer quietos durante la exploración. Algunos necesitan ser sedados para poder permanecer quietos. Si la CT es del abdomen, se le dará a tu hijo(a) a beber un tinte especial que mejora las imágenes creadas durante la tomografía. Algunos niños también pueden requerir que se les inyecte una pequeña cantidad de tinte a través de una línea intravenosa (IV). Si tu hijo(a) recibe el tinte en la vena, es posible que tenga una sensación de calor y enrojecimiento. Generalmente, no hay efectos secundarios por ninguno de los tintes; sin embargo, puede haber reacciones alérgicas. Esta prueba conlleva una cierta exposición a la radiación, por lo que el equipo de atención médica tratará de minimizar la frecuencia con la que se realiza, aunque es muy útil para diagnosticar y seguir el estadio y la ubicación del cáncer.

Resonancia magnética (MRI) La MIR (escaneo por resonancia magnética) es una prueba que utiliza ondas de radio y campos magnéticos en lugar de rayos X. Una computadora traduce el patrón de ondas de radio en imágenes muy detalladas del interior del cuerpo. A veces la MIR es más útil que los rayos X o las CT porque puede crear imágenes más claras, como en el caso de áreas como las del cerebro y la médula espinal, vasos sanguíneos, huesos y algunos órganos del cuerpo. Ciertos tumores se controlan periódicamente mediante la resonancia magnética, durante y después del tratamiento, para determinar la eficacia del tratamiento o verificar si hay una recaída. La persona se coloca sobre una mesa que se desliza dentro de una máquina en forma de tubo que la rodea por todos lados. Existen máquinas de MRI abiertas para las personas que se ponen muy ansiosas dentro de una máquina de resonancia magnética normal. La prueba es indolora, pero la máquina hace bastante ruido. Es posible que tu hijo(a) use tapones para los oídos o auriculares durante la prueba. Los niiños deben permanecer completamente quietos y algunos necesitan ser sedados para lograrlo. La prueba suele tener una duración de una o dos horas. A menudo se inyecta en la vena un tinte que mejora las imágenes. Ya que las máquinas de resonancia magnética utilizan imanes, no puede haber objetos metálicos en su interior. Se le pedirá a tu hijo(a) que se quite todos los objetos metálicos, incluyendo joyas y ropa con broches o cierres metálicos. La MIR no expone a tu hijo(a) a ninguna forma de radiación. Gammagrafía con MIBG La exploración con metayodobencilguanidina (MIBG) es una prueba muy importante que busca la presencia de neuroblastoma en todo el cuerpo. El MIBG es un líquido transparente que se inyecta en una vena y es absorbido por las células tumorales del neuroblastoma. La sustancia se ‘ilumina’ durante la exploración nuclear para detectar las células tumorales. Es una sustancia radiactiva, pero se administra en una cantidad tan pequeña que tu hijo(a) recibe menos radiación que con los rayos X. Se administran gotas de yodo de potasio vía oral en los días previos a la exploración para proteger la glándula tiroides de la radiación. La sustancia MIBG sale del cuerpo con la orina. Las mujeres embarazadas deben hablar con el personal de medicina nuclear sobre las precauciones que deben tener para proteger al bebé. La gammagrafía nuclear se realiza 1 o 3 días después de la inyección de MIBG. Es posible que tu hijo(a) necesite sedación o anestesia para permanecer quieto(a) durante la prueba. Se usarán dosis más altas de MIBG durante el tratamiento del neuroblastoma si los tratamientos de primera línea no son 100% efectivos. Ultrasonido o ecografía El ultrasonido usa los ecos de ondas sonoras de alta frecuencia para producir una imagen de los órganos internos o de los tumores. Ayuda a encontrar tumores en el abdomen, revela el estado de salud de los riñones y la vejiga y puede medir el tamaño de algunos órganos, especialmente el hígado y el bazo. El ultrasonido muestra el flujo de la sangre a través de las venas y puede detectar si hay algún coágulo bloqueándolas. Es una prueba indolora y no

utiliza radiación. Tu hijo(a) se coloca sobre una mesa y se le aplica un gel transparente y frío en el área que se va a examinar; este gel se limpia fácilmente después de la prueba. Se coloca un perilla pequeña y redonda sobre el gel y se mueve para obtener una imagen clara del tejido u órgano. Si el ultrasonido es del abdomen, es posible que tu hijo(a) no pueda comer ni beber durante varias horas antes de la prueba. Examen de orina La orina se analiza para buscar sustancias químicas llamadas catecolaminas , específicamente ácido vanililmandélico (VMA) y ácido homovanílico (HVA), que son liberados por los tumores del neuroblastoma. Sólo se necesita recolectar una pequeña cantidad de orina durante un período de 24 horas para esta prueba. Si tu hijo(a) aún no sabe ir al baño, se puede colocar una bolsa de recolección sobre el pene o el área vaginal o insertar un catéter en la vejiga para obtener la orina para el análisis. Es posible que no le puedas dar a tu hijo(a) ciertos alimentos, bebidas o medicamentos que pueden afectar los resultados de esta prueba. Análisis de sangre Los análisis de sangre se realizan para monitorear las células sanguíneas, las sales corporales y la composición química de tu hijo(a). Éstos son muy útiles para diagnosticar problemas médicos y controlar los efectos secundarios del tratamiento. Una enfermera o un técnico capacitado inserta una aguja en una vena para recolectar una muestra de sangre en tubos que luego se envían a un laboratorio donde se analizan. En algunos casos, un pinchazo en el dedo puede dar suficiente sangre para una prueba. Por lo general, estas pruebas se realizan en el momento del diagnóstico para descartar otras enfermedades y servir como referencia. Los análisis de sangre se utilizan durante todo el tratamiento para controlar los posibles efectos secundarios y la respuesta de tu hijo(a) al tratamiento. Los análisis de sangre como la creatinina y el nitrógeno ureico en sangre (BUN) controlan los cambios en la función renal. Los llamados panel de función hepática y bilirrubina detectan cambios en la función hepática. Los niveles de fosfato alcalino y deshidrogenasa láctica (LDH) pueden ser altos en la sangre debido al crecimiento óseo de un tumor. Hemograma completo La sangre está formada por plasma (la parte líquida de la sangre) y por células que flotan en el plasma. Hay tres tipos de células sanguineas: glóbulos blancos, glóbulos rojos y plaquetas. Los glóbulos blancos son parte del sistema inmunológico del cuerpo y combaten las infecciones. Los glóbulos rojos transportan oxígeno a todas las células del cuerpo. Las plaquetas detienen el sangrado al formar un coágulo en el lugar de la lesión. Un hemograma completo , o CBC, es un análisis de sangre que mide la cantidad y la condición de salud de estos tres tipos de células. El CBC es útil para diagnosticar trastornos sanguíneos que han sido heredados o desarrollados. Los resultados de un CBC pueden cambiar debido a otras cosas que suceden en el cuerpo, como infecciones, problemas nutricionales o como respuesta a algunos medicamentos como la quimioterapia.

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