Retinoblastoma Patient & Family Handbook

A Handbook for Families: Retinoblastoma

A Handbook for Families

Retinoblastoma

ONCOLOGY SERIES

A Handbook for Families

Retinoblastoma

ONCOLOGY SERIES

Retinoblastoma

A HANDBOOK FOR FAMILIES

AUTHORS Cheryl Fischer, MSN RN CPNP Mary Petriccione, MSN RN PPCNP-BC

CONTENT REVIEWERS APHON Steering Council PARENT REVIEWER Sherie Buell

ABOUT THIS COVER

This cover is specially designed for your child to color and personalize. When your child finishes decorating the cover, return it to the clinic or doctor’s office where you received the handbook.

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 retinoblastoma. 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 retinoblastoma, 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 grants unrestricted permission to photocopy or print the handbook for educational use by the care team or the care team’s institution. The user may not alter content or receive monetary gain from distributing photocopies of this product.

This resource may not be translated without written permission from APHON.

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INTRODUCTION

Hearing that your child has cancer can be shocking and overwhelming. At first it may be difficult to accept the diagnosis and to see your child in the hospital, or beginning treatment. However, the reality of the situation will become easier to accept with time and information. 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 resources available, may help ease some of the feelings surrounding the diagnosis and treatment.

WHAT IS RETINOBLASTOMA?

Retinoblastoma is a cancer of one or both eyes which usually occurs in children who are younger than 5 years of age. The tumor(s) occur in the retina (the innermost layer of the eyeball), which is the area of the eye containing nerves that control vision. Retinoblastoma is the most common intraocular (eye) childhood cancer and has an excellent prognosis, with a greater than 95% cure rate if the disease is contained within the retina when diagnosed. There are 2 forms of retinoblastoma, unilateral (one eye involved) and bilateral (both eyes involved). The majority of patients have unilateral disease and are diagnosed around 2 years of age. The remaining patients have bilateral tumors and are usually diagnosed at a younger age (often during the first year of life and rarely after 2 years of age).

STRUCTURE AND FUNCTION OF THE EYE

It is helpful to know the names, locations, and functions of the structures of the eye to understand your child’s tumor. The eye has three layers from front to back: Sclera: the white outer covering that protects the eye Choroid: the middle layer, which contains the blood vessels that bring nourishment to the eye Retina: the inner layer, which contains the nerves that send messages to the brain for sight Other structures of the eye include the following: Cornea: the clear portion at the front of the eye through which light rays pass Conjunctiva: a thin membrane that lines the outside of the eye Iris: the colored part of the eye

Pupil: the black opening in the middle of the iris that allows light into the eye Lens: the area behind the iris and pupil that focuses light onto the retina Vitreous humor: a gel-filled chamber of the eye that nourishes the eye and gives it shape Macula: the part of the retina that provides central vision Optic nerve: the nerve that carries messages from the eye to the brain

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WHAT ARE SOME OF THE SYMPTOMS OF RETINOBLASTOMA?

The most common signs of retinoblastoma are leukocoria and strabismus. Leukocoria is often called “cat eye reflex”. It is a white reflection (similar to a cat’s eye in the evening) seen in the pupil (black portion of the eye) when light shines in a child’s eye, instead of a red reflection (a red glow in dim light that is commonly seen in flash pictures). Strabismus is a wandering eye, or abnormal turning in or out of the eye, that does not go away. Other less common symptoms are changes in vision, eye irritation, and pain. When these symptoms are present, your child also may rub his or her eye frequently or have redness of the eye and tearing.

WHAT CAUSES RETINOBLASTOMA?

Retinoblastoma is caused by a mutation (abnormality, or variation) in the RB1 gene. Genes are instructions that help our body grow and function. All genes exist in pairs. Every person has two copies of each gene in every cell (building block) of their body, including the RB1 gene. Retinoblastoma occurs when a mutation develops in both copies of the RB1 gene inside the retinal cells of the eye. This allows the cells to grow out of control, causing retinoblastoma tumor(s). The reason for the mutation is unknown, and there is nothing a parent or child did to cause the mutation.

WHO GETS RETINOBLASTOMA?

Retinoblastoma is a rare form of cancer that occurs once in every 15,000–20,000 live births. It makes up only 3% of all childhood cancers.

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WHEN IS RETINOBLASTOMA INHERITED?

Retinoblastoma is often referred to as hereditary (inherited) or non-hereditary. This refers to the way your child developed the two RB1 gene mutations, and it impacts how your child presents with retinoblastoma, how they may be treated, and what type of follow-up they may need after treatment. Retinoblastoma is hereditary (inherited) when a child is born with a mutation in the RB1 gene. The mutation can be inherited from a parent, but most of the time it is a spontaneous, or new, mutation. Children with hereditary retinoblastoma typically have more than one tumor in the eye, have tumors in both eyes, and are of a younger age at diagnosis. If a child has the hereditary form of retinoblastoma, they can pass the RB1 gene mutation on to their future children. Retinoblastoma is non-hereditary when the mutation in the RB1 gene occurs after the child is born. The mutation only affects the cells in the eye, and cannot be passed on to children. Children with non-hereditary retinoblastoma typically have retinoblastoma in only one eye. Genetic testing (usually by taking a blood sample) is the only way to know for sure whether your child’s retinoblastoma is inheritable. Ask your healthcare team about genetic testing and a referral to a genetic counselor for your child, and possibly for yourselves and your other children. Genetic counselors investigate the health problems in the family, interpret information about the disorder, analyze inheritance patterns and risks of recurrence and review available options with the family. Genetic counselors also provide supportive counseling to families, serve as patient advocates, and refer individuals and families to community or state support services when appropriate.

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WHAT IS STAGING?

Staging is a very important process that is used to identify the location and size of the tumor(s) at the time of diagnosis. For several decades, the Reese-Ellsworth staging system was used as the primary International Staging System for Retinoblastoma. It was designed to predict the chance of controlling the disease and saving the vision in the affected eye(s). In the late 1990s, a new staging system called the International Classification of Intraocular Retinoblastoma (IIRC) was developed, and it has replaced the Reese-Ellsworth system at most treatment centers. The IIRC classifies eyes from groups A to E (ABCDE), with A being more favorable and E being the most severe. This system grades tumors by their size, number, and location in the retina. The advantage of this new system is that it more accurately describes the degree of disease in the eye, helping health care providers better determine the type of therapy needed for each child.

WHAT ARE METASTASES?

Metastases refer to the spread of the tumor from its original location to other parts of the body. Retinoblastoma can metastasize to other parts of the eye, the surrounding tissue of the orbit, and the lymph nodes. In more advanced stages retinoblastoma can spread either to distant areas of the body, such as the bone, bone marrow, and liver, or to the brain, spine, and spinal fluid. Metastatic retinoblastoma is rare.

HEALTHY EYE

RETINOBLASTOMA

RETINA

FOVEA CENTRALIS

BLIND SPOT

TUMOR

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WHICH TESTS AND PROCEDURES WILL MY CHILD NEED?

The following tests may be done to evaluate the location and extent of the cancer and determine the most appropriate treatment. All children should have an eye exam under anesthesia (EUA), an ultrasound of the eye(s), and either an MRI (magnetic resonance imaging) or CT (computed tomography) scan of the brain and orbits (eyes). Whenever possible an MRI is preferred over a CT scan for children with retinoblastoma. Blood tests may be done for genetic testing and to monitor side effects of treatment. Some children who have a higher-stage disease may need additional MRIs, bone marrow aspirate and biopsy, lumbar puncture, and/or a bone scan. Although advanced retinoblastoma is uncommon in the United States and other developed countries, these tests are still needed to ensure that the retinoblastoma has not spread to other parts of the body. MRI MRI (magnetic resonance imaging) uses radio waves and magnetic fields instead of X rays to create very detailed pictures of parts of the body. A computer translates the pattern of radio waves into images. MRI images are so clear that they are sometimes more useful than X rays or CT scans. This is true for areas of the brain and spinal cord, blood vessels, bones, and some organs of the body, like the eyes. Certain tumors are regularly checked by MRI during and after treatment to determine how effective the treatment is and if a relapse has occurred. For an MRI, a patient lies on a table that is rolled into a machine. The machine surrounds them on all sides, like a tube. (Open MRI machines exist for people who are very anxious in a regular MRI machine.) The MRI is painless, but the machine makes loud noises. Your child may use earplugs or a headset during the MRI. They must be able to lie completely still, and some children require sedation to do this. The MRI usually takes 1-2 hours. Often, a dye is injected into a vein to improve the MRI images. Because they use strong magnets, MRI machines cannot have metal objects inside them. Before an MRI, your child will be asked to remove all metal objects such as jewelry and clothes with metal snaps or zippers. An MRI does not expose your child to any form of radiation.

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CT SCAN A CT (computerized or computed tomography) scan is a computer-assisted X ray that creates detailed pictures of internal organs and tumors. It used to be called a CAT (computerized axial tomography) scan. The CT machine is a doughnut-shaped X-ray camera that moves around a table that the patient lies on. Every second, the 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 helpful in diagnosing and following the stage and location of cancer. CT scans are very useful in diagnosing tumors, infections, blood clots, and areas of abnormal bleeding. Some tumors are regularly checked by CT scan during and after treatment to see how effective the treatment has been and if a relapse has occurred. CT scans are painless, but the patient must lie still during the scan. Some children require sedation to help them lie still. If your child’s belly is being scanned, they may be asked to drink a dye that allows organs to be seen more clearly. Some patients may be given a small amount of dye through an intravenous (IV) line, which puts the dye right into their veins. The dye can cause a warm, flushing feeling. Generally, there are no side effects from either type of dye; however, allergic reactions are possible. Because there is some radiation exposure with this test, your child’s healthcare team will try to minimize how often it is done. EYE EXAM UNDER ANESTHESIA (EUA) Retinoblastoma is unlike other tumors because a biopsy of the tumor cannot be obtained by surgically removing a piece of it from the eye for examination. Instead, a thorough eye exam under anesthesia (EUA) is performed. During EUA, your child is put into a deep sleep, and the ophthalmologist (eye doctor) examines the inside of the eye(s) after they are dilated (opened widely) with special eye drops. Retinoblastoma can be diagnosed based on how the tumor(s) look inside the eye. The stage of retinoblastoma is based on the number, size, and location of the tumor(s) that are seen by the ophthalmologist during the EUA. The type and intensity of treatment is based on the stage of the disease. Repeat EUAs will be performed treatment to evaluate the response to therapy, and after treatment to monitor your child’s health.

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ULTRASOUND Ultrasound is a technique that uses the echoes from high-frequency sound waves to produce a picture of internal organs or tumors. In children with retinoblastoma Ultrasound is used to produce a picture of the inside of the eye. The picture can tell your doctor about the size and number of tumors. It is helpful for staging the tumor and monitoring response to treatment. An ultrasound is a painless test that does not use radiation. The child lies on a table for a short time, and a small, round handle is moved around to get a clear picture of the tissue or organ being examined. Sometimes a clear jelly may be applied to the eyelids (the jelly wipes off easily after the test). BONE MARROW ASPIRATION AND BIOPSY A bone marrow biopsy is a test done to determine whether tumor cells are in the bone marrow. Not all children with retinoblastoma will need a bone marrow aspiration and biopsy. If they do, it may be done only once, or may need to be repeated to monitor the effect of treatment. A needle is inserted into the middle of a large bone (usually the hip bone). Some of the bone marrow (the biopsy) and the fluid in the bone marrow (the aspirate) are collected. Patients undergoing bone marrow aspirations and biopsies may experience pain, so children are often sedated for this procedure. If your child is uncomfortable after the test, a mild pain medication such as acetaminophen (Tylenol®) may be given, but not ibuprofen (Motrin® or Advil®) or aspirin. A specially trained doctor (a pathologist or hematologist) examines the biopsy or aspirate under a microscope to look for any abnormal cells, such as tumor cells. 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 how well the treatment is working. LUMBAR PUNCTURE A lumbar puncture is a procedure done to obtain a small sample of cerebral spinal fluid (CSF), which is the fluid that surrounds and protects the brain and spinal cord. CSF is made continually by our bodies, so the small amount removed is quickly replaced. The central nervous system (brain, spinal cord, and CSF) can be a hiding place for retinoblastoma cells and this test can help determine if any cancer cells have spread to the brain or spinal cord. Not all children with retinoblastoma will need a lumbar puncture. If they do, it may be done only once, or may need to be repeated to monitor the effect of treatment. In a lumbar puncture, also referred to as a spinal tap, a needle is inserted into the back between the spaces of the spinal bones (vertebrae) to obtain a sample of CSF. The needle enters the spinal column below the spinal cord to avoid injuring the cord. This test can be uncomfortable, so some children are sedated to help them lie still. The results will help the physicians determine the type of treatment your child will receive.

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BONE SCAN A bone scan is used to look for abnormalities in bones. In cases where there is concern for retinoblastoma outside of the eye, a bone scan can be used to look for tumor cells that may have spread to the bones. Not all children with retinoblastoma will need a bone scan. It may be done only once, or it may need to be repeated to monitor the effect of treatment. A small amount of radioactive dye (isotope) is given through an IV line and travels around the body. This isotope is picked up by tumor cells, which allows for the tumor’s location in the body to be seen. Pictures are taken about 2-3 hours after the injection. The test is painless, but some children require sedation to lie still for the scan. The radioactive isotope leaves the body in the urine over 2–3 days. There is very little radiation exposure from a bone scan.

BLOOD TESTS

Blood tests help diagnose medical problems and monitor for side effects of treatment. A specially trained nurse or technician uses a needle to collect blood from a vein. The blood samples are collected in tubes that are sent to a laboratory to be closely examined. 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 serve as a baseline for your child. COMPLETE BLOOD COUNT Blood is made up of plasma (the liquid part of the blood) and cells 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 throughout the body. The platelets stop bleeding by rushing to the site of an injury to form a clot. A complete blood count, or CBC, is a blood test that measures the amount and the health of these three types of cells. Your child’s CBC results may change with each test because of other things going on in the body, such as infections or nutritional problems, or in response to some medications like chemotherapy. BLOOD CHEMISTRY A blood chemistry test measures the balance of fluid, electrolytes, and blood sugar. These measurements can provide information about the health and function of the kidneys and liver and about the body’s metabolism; they can also help diagnose certain diseases, such as diabetes. They can indicate how a disease is responding to treatment and monitor for possible side effects. Blood tests may be done to: Measure creatinine and blood urea nitrogen (BUN) to monitor changes in kidney function Detect changes in liver function by measuring bilirubin, alanine transaminase (ALT), and aspartate transaminase (AST) Common chemistry tests are a basic metabolic panel (BMP) or a comprehensive metabolic panel (CMP). These test results may change due to other things going on in the body, such as nutritional changes or side effects of treatment.

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GENETIC TESTS

If your child is referred to a geneticist as part of their retinoblastoma care, the geneticist may obtain a blood sample for genetic testing.

CENTRAL VENOUS ACCESS DEVICE (VAD) A central venous access device (CVAD), sometimes called a central line, is a temporary or long-term intravenous (IV) tube that can be used for the duration of your child’s therapy (keep in mind that “therapy” may be used interchangeably with “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. They can be used to administer medications, fluids, chemotherapy, blood products, and nutritional support as needed and to draw blood for testing. Surgery is performed to insert the CVAD, and your child will be sedated or given general anesthesia for the procedure. It will be placed either before chemotherapy begins or within the first month of treatment. Not all children will need a CVAD. This will be determined by you and your child’s healthcare team. The CVAD will be removed when it is no longer needed. There are different types of CVADs, 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 single or double) which comes out of your child’s chest; or a PICC (peripherally inserted central catheter) line, a central line inserted through a vein in the arm. Your child’s healthcare team will discuss each type with you and help you choose the best CVAD for your child’s treatment. Each type of catheter requires slightly different care. A port requires almost no care at home, but a special needle will need to be inserted through the skin into the port each time it is used. Tubing can then be attached to the needle if needed to give medication or IV fluids. An external central line requires routine changes to the dressing that covers the exit site of the line and keeps it in place to prevent infection and to care for the skin around the tube. The external lines also must be routinely flushed with a solution to keep them working properly. You will be taught how to care for the device at home.

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HOW CAN RETINOBLASTOMA BE TREATED?

Depending on the institution, several experts may be involved in the discussion regarding your child’s specific treatment plan. They may include the pediatric ophthalmologist expert in the care of retinoblastoma, a pediatric oncologist, a pediatric nurse specialist/nurse practitioner and a social worker. The type of therapy offered to your child will depend on information about your child’s disease. Your team will explain if the retinoblastoma is: 1.) intraocular (within the eye) a.) affects one or both eyes b.) the stage (refer to the staging section) of the retinoblastoma in each affected eye 2.) extraocular (outside the eye) a.) disease has spread beyond the eye b.) need to determine exactly where in the body the disease has spread to. Treatment options include the following: surgery, focal therapies, chemotherapy, radiation therapy, and the availability of clinical trials. A combination of these therapies may be recommended to maximize the destruction of the retinoblastoma cells. Treatment decisions will be made collaboratively by you (the child’s parents) and the medical team. Each treatment will be discussed in more detail. It is optimal to be treated in a center with a dedicated retinoblastoma team. SURGERY Surgery is indicated for the treatment of retinoblastoma if only one eye is affected and if the tumor is large enough to cause little or no useful vision of the affected eye. It can also be used in children with bilateral disease if one eye has little or no useful vision because of tumor size. The remaining eye with lower stage disease is then treated. In some cases, both eyes are removed because vision cannot be salvaged in either eye. The operation for the removal of the eye is called enucleation and is performed as an outpatient surgical procedure. With this surgery, the entire globe (the eyeball, which resembles a marble) and part of the optic nerve is removed by the ophthalmologist in the operating room. During the procedure, the ophthalmologist places a temporary implant called a conformer inside the orbit once the globe has been removed. The implant looks like part of a small golf ball and permanently takes the place of the globe. The implant maintains the shape of the eye. Your child will be unaware that the conformer is in place; it cannot be removed, other than by surgery, and is not visible. After the surgery, your child’s eyelids may be bruised and swollen for several days, but these local side effects will go away. Within 6–8 weeks after surgery, your child will be fitted with a prosthetic (artificial) eye, which resembles a large contact lens. A special artist called an ocularist will create your child’s prosthesis by custom fitting the prosthetic eye and painting it to match your child’s remaining eye. The prosthetic eye will be placed inside the eye socket behind the eyelids and, if fitted properly, should not come out easily. It will look almost as natural as the remaining eye. It will move in coordination with the natural eye but will not have full movement. Caring for the prosthetic eye will require gentle washing at home every few weeks and scheduled appointments with the ocularist for professional cleaning and polishing. Children will need their prosthesis replaced as they grow. Children generally adapt easily to the prosthetic eye with age-appropriate explanations and support. Play therapy with young children can be especially helpful when explaining what a prosthetic eye is, how it will look and function.

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FOCAL TREATMENTS Focal treatment is the term used to describe direct treatments to the eye(s) that try to kill or shrink the size of the tumor. These treatments can be used alone or in conjunction with other treatments. There are several different types of focal treatments including: cryotherapy (the use of extreme cold to destroy cancer cells), laser therapy (the use of a direct source of heat to the pupil and surrounding vasculature to destroy the tumor cells) and thermotherapy (similar to laser therapy but uses a lower temperature infrared laser). Other therapies that can be administered focally include chemotherapy that will be injected directly into different areas of the eye to attack cancer cells that may have spread. These focal treatments usually are performed during the exam under anesthesia (EUA) when the child is asleep. After the procedure, your child may complain of some mild pain or have local swelling or redness of the treated eye. CHEMOTHERAPY Chemotherapy is treatment with medications that are known to kill retinoblastoma tumor cells throughout the body. There are many different types of chemotherapy drugs that can be given. Some drugs can be administered alone, and others are given in combination with other chemotherapy drugs. Each drug kills the cancer cells in a different way which is why some of these drugs are used together. There are also different ways to administer chemotherapy. The type of chemotherapy drugs that are used and the way they are administered depends on the stage of the retinoblastoma disease and whether it has traveled beyond the original site in the eye. The pediatric oncologist (cancer doctor) will decide the most effective chemotherapy agents to use, the schedule for the drugs Systemic chemotherapy which can be given by mouth, through a vein or a CVAD. Chemotherapy medications used for retinoblastoma are usually given through a vein or a CVAD. The oncologist will guide you as to which type of access is most appropriate for the treatment planned for your child. Systemic chemotherapy can be used alone or in combination with various focal therapies. OAC (Ophthalmic Artery Chemosurgery) is another way that chemotherapy can be given; this may also be referred to as IAC (intra-arterial chemotherapy). Chemotherapy is administered through an artery that leads to the ophthalmologic artery so that the chemotherapy is delivered directly to the tumor in the eye. This treatment is done under anesthesia and can only be administered at certain retinoblastoma specialty centers. Intrathecal chemotherapy is administered directly into the cerebrospinal fluid (CSF) if the retinoblastoma cells have spread there. Chemotherapy can also be administered directly into certain areas of the eye. This is done while the child is receiving anesthesia. and the length of time needed to treat your child. The different types of chemotherapy include: The chemotherapy plan for your child will be carefully discussed with you. The oncologist and your treatment team will explain the reason why the particular treatment was chosen as well as potential side effects and their management.

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RADIATION Radiation therapy uses high energy x-rays to kill cancer cells and can be effective in treating retinoblastoma. It is used either in combination with other therapies or after other therapies have failed. There are two types of radiation therapy used for retinoblastoma: brachytherapy and external beam radiation therapy. Plaque radiotherapy is a form of brachytherapy that uses a custom designed plaque (that is placed directly around the affected area of the eye. The plaque is a thin piece of metal (usually gold) that has radioactive seeds on one side of it. The side of the plaque with the seeds faces the eye. The radioactive seeds deliver the radiation to the tumor site. The plaque helps protect surrounding tissue from the radiation. It is sewn to the skin that covers the eye while the child is under general anesthesia in the operating room. The plaque remains in place for 3–5 days, depending on the dose of radiation that needs to be delivered, and then is removed while the child is under general anesthesia in the operating room. This form of treatment delivers concentrated radiation therapy only to the tumor without affecting the rest of the eye or the eye socket (bone). The child remains in the hospital during this process. External beam radiation therapy (EBRT) focuses radiation therapy on the cancer cells from a machine outside the body. The radiation dose is calculated by a radiation oncologist who will plan how much radiation therapy is needed and how long it will take. Newer types of radiation therapy are able to target the cancer cells more precisely and spare surrounding tissue. Two types of EBRT radiation therapy include: (1) IMRT (intensity modulated radiation therapy) which allows the doctor to aim the radiation at different angles of the tumor while using different strengths of radiation that is needed, and (2) Proton beam iradiation therapy. With Proton beam therapy, the radiation machine delivers protons (tiny invisible charged particles) directly to the tumor. Depending on the age of the child, anesthesia may be required with each treatment to ensure that the child stays in the correct position which is crucial to the delivery of the radiation to the tumor area.

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WHAT NEW METHODS OF TREATMENT ARE THERE?

Your provider and treatment team will discuss all available treatment options with you and may present a clinical trial that is available for the treatment of your child’s retinoblastoma. In clinical trials, the best known (standard therapy) treatment for retinoblastoma is compared with a new treatment plan that is believed to be at least as good as, and possibly better than, the standard treatment. Clinical trials allow providers to determine whether promising new treatments are safe and effective. Participation in clinical trials is voluntary. Because clinical trials involve research with new treatment plans, all of the risks cannot be known ahead of time, and unknown effects may occur. However, children who participate in clinical trials can be among the first to benefit from new treatment approaches. Before making a decision about your child’s participation in a clinical trial, you will discuss the risks and potential benefits with your child’s provider and treatment team. A useful website for information about clinical trials for retinoblastoma is ClinicalTrials.gov (NCI website).

HOW LONG WILL MY CHILD’S THERAPY LAST?

The length of your child’s therapy depends on the stage and extent of your child’s disease at the time of diagnosis. Treatment time will vary depending on the amount of therapy that is needed. Follow-up blood tests and check-ups between and following treatments generally are done in the clinic or doctor’s office. Timing of these visits will depend on the treatment given and will be planned carefully by your team. Your child will require close follow-up initially after completion of therapy with longer time intervals between visits as the time from diagnosis lengthens. Surveillance exams will continue throughout his or her entire life. LIFE-LONG RECOMMENDATIONS Maintaining lifelong surveillance visits (the schedule will vary between those with the inherited disease vs non inherited) with an ocular oncologist and a pediatric or adult team that specializes in screening and managing long term issues for a retinoblastoma survivor is very important. Your team will guide you as to what long term effects may be associated with specific treatments and how best to monitor for them. Striving to lead a “healthy lifestyle” and being vigilant about seeking attention for any new health issues is critical Most survivors of retinoblastoma will lead a happy and productive life.

WHAT ARE MY CHILD’S CHANCES OF GETTING ANOTHER CANCER?

Children who have the germline, or hereditary, form of retinoblastoma are at a greater risk of developing cancer in other parts of the body. The most common types of second cancers are tumors of the bone, muscle, and skin. It is important that retinoblastoma patients continue to receive annual physical exams throughout their lives. If a second cancer occurs, the chance of cure increases the earlier it is found and treated. A genetic counselor can help you understand your child’s risk for secondary cancers and what type of screening tests they should have throughout their life.

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HOW CAN I WORK WITH THE HEALTHCARE TEAM?

The care of your child 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. If there is anything you are not sure about, ask the team. Write down your questions as you think of them, so you’ll remember to ask them during your next meeting with your child’s healthcare team. Important questions you may want to ask: What stage is the disease and what does that mean? What treatment choices are available? What treatment do you recommend and why?

What are the risks or side effects during the treatment? What are the risks and side effects after the treatment? What are my child’s chances for survival? What are the chances for recurrence/relapse? What financial or social resources are available to help my family?

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ARE MY FEELINGS NORMAL AND WHAT CAN I DO ABOUT THEM?

Hearing that your child has cancer is shocking and overwhelming. Many parents say, “I didn’t remember anything after the words, ‘Your child has cancer.’” Parents often feel numb and have a hard time believing the diagnosis. It is important to know this is normal and expected. Most families have difficulty processing all of the information the healthcare team is providing in the beginning. However, over time, the information will be repeated and can be absorbed. Do not be afraid to ask questions. Many families feel somehow responsible for their child’s disease. Feelings of guilt are also common because they could not protect their child from illness or because of the amount of time it took to diagnose the child. This disease is not caused by anything that you did or did not do. The cause of childhood cancer is not known. It often takes time for the cancer diagnosis to be made because symptoms are often the same as those of many childhood illnesses. 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 that 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. If you are interested in speaking with a therapist, you can ask your child’s oncologist whether this is a service provided through the treatment center or whether your oncologist has any specific recommendations. It may also be helpful to find a therapist for siblings to speak with.

HOW CAN I HELP MY CHILD?

Children often think that something they did caused their cancer. You can help your child by reinforcing 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 you keep a close relationship with your child. Your child will need to share their feelings with someone that they trust, and they may choose someone other than a parent to avoid upsetting the parent, which is OK. Don’t be afraid to ask your child about their feelings—it may be what your child is waiting for. Keep in mind that your child is still a child first in spite of the disease. As children go through treatment, they still have all the needs of a growing child. All children need love, attention, the opportunity to learn and try new skills, and limits. Do not avoid talking to your child about therapy. Use explanations that your child will understand. Children tolerate treatment better if they understand it and are allowed to help make decisions about their care, when appropriate. The things children can imagine on their own are often more frightening than what is actually happening. As a parent, you may find it difficult to watch your child go through treatment. Your child may sometimes seem sicker during treatment than before treatment. Your feelings about what your child is going through must be balanced with the knowledge that treatment provides the chance of curing the disease and making it possible for your child to live a full and meaningful life. It’s difficult to accept how cancer and treatment change your child, but most of these changes are temporary. Despite outward changes, your child is still the same person on the inside. Most healthcare centers have a psychosocial team that can support you and your child through their diagnosis and treatment.

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IS MY CHILD’S DIET IMPORTANT DURING THE TREATMENT?

Yes, your child’s diet is important. Research has shown that well-nourished children tolerate treatment better and experience fewer treatment delays. If your child is receiving chemotherapy it may cause nausea and vomiting. These can be distressing side effects 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. Small frequent meals are often better tolerated than three larger meals. When possible, choose more nutritious foods. Your child’s nurse or dietitian can help you put together a list of possible food choices. It is important to offer your child fluids to drink frequently throughout the day so that dehydration is avoided. 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 because they can interact with your child’s treatment. Please ask your healthcare team if you are considering having your child take any of these types of supplements. A dietitian may be a member of your child’s healthcare team. Dietitians are trained in the calorie and nutritional needs of children. The dietitian can provide you with education regarding your child’s dietary needs and give you 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 a concern about nutrition arises, your child’s healthcare team will discuss with you the ways to manage and improve your child’s nutrition.

CAN MY CHILD ATTEND SCHOOL DURING TREATMENT?

Your child’s ability to go to school during therapy will depend on the intensity of the treatment and your child’s response to the treatment. There may be extended periods when your child is unable to go to school because of hospitalizations or treatments. Your child may have vision problems ranging from limited sight to complete blindness. As an infant or toddler, vision loss can affect your child’s natural development and adjustment to normal activities. In school-age children, vision loss can affect their ability to complete school work or easily move around their school. Your child, regardless of age, should have his or her vision checked to determine vision level and be referred for services for children with vision limitations. This could include mobility training, early intervention services, and individualized education plans (IEPs). The earlier your child begins receiving vision services, the better they will adjust to any vision impairments. It is important that your child keep up with schoolwork. Federal laws protect children with disabilities or illnesses to ensure that they receive the same education as their peers. Talk with your child’s healthcare team and school staff about these laws. Then work with them in arranging for a tutor or finding other ways to help your child stay on track with schoolwork. Discuss with your child’s healthcare team your child’s ability to attend school. Some children are able to attend school between hospitalizations or treatments. Many hospitals and clinics have resources such as educational liaisons to assist in coordinating school services and educating teachers and classmates to prepare them for your child’s return to school.

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WHERE CAN I GO FOR MORE INFORMATION ABOUT RETINOBLASTOMA?

The following websites have a wealth of information about retinoblastoma: https://www.cancer.org/cancer/retinoblastoma.html https://www.cancer.gov/types/retinoblastoma https://medlineplus.gov/genetics/condition/retinoblastoma

www.cancerindex.org www.curesearch.org Clinical Trials.gov (NCI website)

REFERENCES

Dimaras H, Corson TW, Cobrinik D, et al. Retinoblastoma. Nat Rev Dis Primers. 2015;1:15021. Published 2015 Aug 27. doi:10.1038/nrdp.2015.21 Mallipatna, Ashwin, Marino, Meghan, Singh, Arun. Genetics of Retinoblastoma. Asia-Pacific j. ophthalmol.. 2016;5(4):260-264. doi:10.1097/APO.0000000000000219 Ortiz,M.V., Dunkel I.,J.,( 2016). Retinoblastoma. Journal of Child Neurology, 31(2),227-236. Fabian, I.D., Onadim, Z., Karaa,E., et al. ( 2018). The management of Retinoblastoma. 37, 1551-1560. Wyse,E.,Handa,J.,T., Friedman,A.,D.,Pearl, M.,S. ( 2016). A review of the literature for intra-arterial chemotherapy used to treat retinoblastoma. Pediatric Radiology, 46: 1223-1233. Abramson,D.,H., Francis, J.,H., Gobin,Y.,P.,( 2019). What’s new in Intra-Arterial Chemotheray for Retinoblastoma. 59(2), 87-94. Belson,P.J., Eastwood,J.,A., Brecht, M.,L.,Hays, R,D., Pike,N.,A., Journal of Pediatric Oncology Nursing. (2020). 37(2) 116-127.

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IMPORTANT PHONE NUMBERS

NOTES

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The Association of Pediatric Hematology/Oncology Nurses (APHON) was formed as a 501(c)(3) public charity (EIN 23-7446224) to develop educational content, deliver informative programs, promote the latest evidence-based practice guidelines, and provide resources to the pediatric, adolescent, and young adult communities and their families. The Handbook for Families series is made possible through the generous support of our APHON Star Fund donors and our Industry Relation Council Members.

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Manual para la familia

Retinoblastoma

SERIE ONCOLOGÍARIE

A Handbook for Families

Retinoblastoma

SERIE ONCOLOGÍARIE

Retinoblastoma

MANUAL PARA LA FAMILIA

AUTORES Cheryl Fischer, MSN RN CPNP Mary Petriccione, MSN RN PPCNP-BC

REVISOR DE CONTENIDO APHON Steering Council

REVISOR POR PARTE DE LOS PADRES Sherie Buell

ACERCA DE ESTA PORTADA Esta portada está especialmente diseñada para que tu hijo(a) la ilumine y personalice. Cuando tu hijo(a) termine de colorearla, devuélvela a la clínica o al consultorio médico donde te dieron el manual. El encargado de la atención médica de tu hijo(a) la enviará a APHON para que la publiquen en su sitio web. Este manual ha sido publicado por 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 retinoblastoma. Bajo ciertas circunstancias o condiciones, es posible que se requiera un tratamiento adicional o diferente. A medida que nuevas investigaciones y experiencias clínicas amplíen las fuentes de información disponibles sobre el tratamiento para el retinoblastoma, es posible que sea necesario hacer ajustes en el tratamiento y la terapia con fármacos. 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 otorga al comprador de este manual un permiso ilimitado para fotocopiar o imprimir el manual para uso educativo del equipo de cuidado o de la institución del equipo de cuidado. El comprador no puede alterar el contenido ni recibir ninguna ganancia económica por la distribución de fotocopias de este producto.

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INTRODUCCIÓN

Escuchar que tu hijo(a) tiene cáncer puede ser algo impactante y abrumador. Al principio puede resultar difícil aceptar el diagnóstico y ver a tu hijo(a) en el hospital y comenzar el tratamiento. Sin embargo, la realidad de la situación será más fácil de aceptar con el tiempo y con la obtención de información. El estar informado sobre su diagnóstico, el plan de tratamiento y los posibles efectos secundarios, así como estar familiarizado con los miembros del equipo de atención médica, y con los recursos disponibles, puede ayudarte a calmar algunos de los sentimientos relacionados con el diagnóstico y el tratamiento.

¿QUÉ ES RETINOBLASTOMA?

El retinoblastoma es un cáncer en uno o ambos ojos que suele aparecer en niños menores de 5 años. El tumor, o tumores, se producen en la retina (la capa más interna del globo ocular), que es la zona del ojo que contiene los nervios que controlan la visión. El retinoblastoma es el cáncer intraocular (en el ojo) infantil más común y tiene una tasa de curación superior al 95 % si la enfermedad se encuentra restringida al interior de la retina en el momento del diagnóstico. Existen dos formas de retinoblastoma: unilateral (un ojo afectado) y bilateral (ambos ojos afectados). La mayoría de los pacientes tienen la forma unilateral y se les diagnostica alrededor de los 2 años de edad. El resto de los pacientes tiene tumores bilaterales y se les suele diagnosticar a una edad más temprana (a menudo durante el primer año de vida y rara vez después de los 2 años de edad).

ESTRUCTURA Y FUNCIÓN DEL OJO

Es importante conocer el nombre, la ubicación y la función de las estructuras del ojo para entender el tumor de tu hijo(a). El ojo tiene tres capas de adelante hacia atrás: Esclerótica: la cubierta blanca del exterior que protege el ojo. Coroides: la capa intermedia, que contiene los vasos sanguíneos que nutren el ojo. Retina: capa interna que contiene los nervios que envían mensajes al cerebro para la visión. Otras estructuras del ojo incluyen: Córnea: parte transparente en la parte frontal del ojo por la cual pasan los rayos de luz. Conjuntiva: membrana delgada que recubre el exterior del ojo. Iris: la parte coloreada del ojo.

Pupila: la abertura negra en el centro del iris que permite que la luz entre al ojo. Cristalino: el área detrás del iris y la pupila que enfoca la luz sobre la retina. Humor vítreo: espacio lleno de un gel transparente que nutre al ojo y le da forma. Mácula: la parte de la retina que proporciona la visión central. Nervio óptico: el nervio que lleva mensajes desde el ojo hasta el cerebro.

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¿CUÁLES SON ALGUNOS DE LOS SÍNTOMAS DEL RETINOBLASTOMA?

Los signos más comunes del retinoblastoma son la leucocoria y el estrabismo. La leucocoria suele denominarse “reflejo o pupila de gato”. Consiste en un reflejo blanco (similar al ojo de un gato por la noche) que se observa en la pupila (la parte negra del ojo) cuando la luz brilla sobre el ojo del niño, en lugar de un reflejo rojo (el brillo rojo en la luz tenue que se ve comúnmente en las fotografías con flash). El estrabismo es un ojo desviado, o un giro anormal del ojo hacia adentro o hacia afuera, que no desaparece. Otros síntomas menos comunes son cambios en la visión, irritación ocular y dolor. Cuando estos síntomas están presentes, tu hijo(a) también puede frotarse el ojo con frecuencia o tener el ojo enrojecido y con lagrimeo.

¿QUÉ CAUSA EL RETINOBLASTOMA?

El retinoblastoma es causado por una mutación (anormalidad o variación) en el gen RB1. Los genes son instrucciones que ayudan a nuestro cuerpo a crecer y funcionar. Todos los genes existen en pares. Cada persona tiene dos copias de cada gen en cada célula (bloques estructurales) de su cuerpo, incluido el gen RB1. El retinoblastoma se produce cuando se desarrolla una mutación en ambas copias del gen RB1 en las células de la retina del ojo. Esto hace que las células crezcan sin control, causando uno o más tumores de retinoblastoma. Se desconoce la razón de la mutación y no hay nada que haya hecho uno de los padres o el niño para causar la mutación.

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