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Copyright 2001-2008. All rights reserved. Text researched and written by Jeannie Wurz. Designed by McGuire Associates. Produced by the Kidney Cancer Association.


Acknowledgments We wish to thank the following individuals who reviewed and commented on the contents of this publication: Michael B. Atkins, M.D., Beth Israel Deaconess Medical Center, Boston, Massachusetts Ronald M. Bukowski, M.D., Cleveland Clinic, Cleveland, Ohio Janice P. Dutcher, M.D., Our Lady of Mercy Medical Center, Bronx, New York Robert C. Flanigan, M.D., Loyola University Medical Center, Maywood, Illinois Daniel M. Green, M.D., Roswell Park Cancer Institute, Buffalo, New York Elaine R. Morgan, M.D., Children’s Memorial Hospital, Chicago, Illinois Charlotte Omohundro, Editor Virginia Grimball Susan Mullane Joe Perusic Laura Randall

This book is made possible through the generosity of the

Edward and Carol Goldberg Family Foundation in Honor of

David Stulberg

Notes on the Text Both science and technology change rapidly, and developments in the treatment of Wilms tumor in the years following publication of this booklet are likely. This may make the text less up-to-date. More recent information may be available over the Internet (see On-Line Information). Changes in the addresses, telephone numbers, and Internet sites of the organizations listed in the booklet are to be expected, and we apologize for any inconvenience they may cause. Words defined in the Glossary of Technical Terms appear in italic type the first time they are used in the text. In some cases they are defined within the text as well.


Table of Contents 2 BACKGROUND What Is Wilms Tumor? How Do the Kidneys Work? What Is Cancer? How Is Research Conducted? 5 DIAGNOSIS Patrick’s Story by Susan Mullane Why My Child? What Causes Wilms Tumor? Evaluation Procedures Histology Staging 9 TREATMENT Matthew’s Story by Laura Randall Surgery Chemotherapy Radiation Therapy Immunotherapy 13 POST-TREATMENT Joe’s Story by Joseph Perusic Follow-Up Recurrent Disease Survival Statistics Lifetime Follow - Up 14 HELPING A CHILD WITH WILMS TUMOR Choosing a Hospital Advocacy The Hospital Experience Children and Pain Mental and Emotional Well-Being Resources for Children 21 SUPPORT FOR THE FAMILY Laura’s Realization Dealing with Stress The Needs of Siblings Counseling Sources of Information and Support Other Resources Dealing With Grief 24 LIVING EACH DAY 25 END NOTES Technical Publications General Publications On-Line Information 27 GLOSSARY OF TECHNICAL TERMS 30 JOINING KCA

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BACKGROUND You have recently learned that a child you care about has Wilms tumor, a form of kidney cancer. You are probably feeling dismayed and overwhelmed. Learning more about the disease is a first step in helping a child overcome it. This booklet is designed to give you information and contacts to help your child along the road to recovery. What Is Wilms Tumor? Wilms tumor, also known as a nephroblastoma, is a type of kidney cancer that occurs almost exclusively in children. It is the most common childhood tumor occurring within the abdomen, diagnosed each year in the U.S. in about 500 children under the age of 16, and occurs almost equally in girls and boys. Although tumors have been discovered in children ranging in age from newborn to young adult, the most common age of discovery is between 2 and 3 years. The child is usually initially brought in for medical attention after a caregiver notices a mass in the abdomen. The occurrence of Wilms has not changed substantially between 1975 and 1995, but the 5-year survival rate has improved from 81% to 92% during this period. Wilms tumor most commonly occurs in one kidney, which is termed a unilateral tumor, but may arise in both kidneys at the same time (a synchronous bilateral tumor) or first in one kidney and later in the other (a metachronous bilateral tumor). The unaffected or less affected kidney is termed the contralateral kidney, meaning it is located on the other side. How Do the Kidneys Work? The job of the kidneys is to filter the blood, collecting urea, salt, and other unneeded substances and passing them to the bladder, where they are stored until they leave the body in the form of urine. A kidney is curved like a kidney bean, with an indentation in the center where the renal artery, renal vein, and ureter connect. Blood enters the kidney through the renal artery and exits through the renal vein. Urine drains through the ureter, a tube connecting the filters in the kidney to the bladder. An adrenal gland, which affects circulation, muscles, and metabolism, is located on the top of each kidney. Each kidney is encased in a membrane called a capsule and is surrounded by fatty tissue. The majority of people have two kidneys, one located on each side of the body, toward the back at the bottom of the rib cage. What Is Cancer? Cancer is not a single disease, but more than 100 different diseases with a common characteristic: abnormal growth, division, and proliferation of cells, which spread from their site of origin to other parts of the body, invading and destroying normal organs and tissues as they spread. If rapidly growing, abnormal, out-of-control cells form a mass, the mass is referred to as a tumor. If the tumor invades and destroys the tissue it has grown from, and can spread to and attack other sites in the body, it is termed malignant. Malignant cells that break off from the main tumor, travel to other parts of the body, and begin to grow there are called metastases. The process by which these cells spread is called metastasis. If a tumor grows within a capsule, has no potential for spread, and can be removed with no chance of its recurring, it is referred to as benign. The human body reproduces about 300 billion cells every day. Sometimes something goes wrong. When abnormal cells are formed, presumably the body’s immune system tries to identify and destroy them. Sometimes the abnormal cells grow and form a tumor. Treatment of cancer involves controlling tumor growth by eliminating cancerous cells through surgery, radiotherapy, chemotherapy, and stimulation of the immune system. Often a combination of these therapies is used. How Is Research Conducted? Many of the improvements in survival are the result of collaboration among medical professionals. Large-scale research studies called clinical trials allow researchers to test new approaches and share information about the best ways to fight cancer. The National

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Wilms Tumor Study Group (NWTSG), formed in North America in 1969, and the International Society of Paediatric Oncology (SIOP), formed in 1969 in Europe, are two well-known groups of researchers. The U.S. Government’s National Cancer Institute urges parents and physicians to enroll children who have Wilms tumor in clinical trials. In order for advances to be made in treating these patients, therapy is best delivered in the context of a clinical trial at a major medical center with expertise in treating children. Only through entry of all eligible children with cancer into appropriate, well-designed clinical trials will progress be made against these diseases, NCI says. Trials in Wilms tumor can be found on the NCI web site at www.clinicaltrials.gov.

Inferior vena cava Aorta Adrenal gland

Renal artery

Kidney

Renal vein

Ureter

Bladder Location of the kidneys in the body

Urethra

Prostate (male only)

The Urinary System Reproduced with permission Copyright 1991 Tim Peters and Company Inc., Gladstone, NJ 07934 USA

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Patrick’s Story by Susan Mullane No one can ever prepare you for the word “cancer”, especially in your child. My child was diagnosed after a 6-month well-baby check when the pediatrician felt an obstruction in Patrick’s abdomen. When I was first told Patrick had tumors in both kidneys my whole body went numb. I totally blocked out all thoughts of cancer. Three hours later when my husband and I met with the doctor, an oncologist, we were told he suspected bilateral Wilms tumor. When I asked how often these tumors were cancerous his reply was, “Always”. I felt myself crumbling, reaching for the counter and thinking that my baby was going to die. I was overwhelmed with the extra duties that go along with having a seriously ill child. Patrick’s oncologist, Dr. Joe McNamara, said a few things that I will always remember. First, Dr. Joe explained that there were two types of balls in life: rubber and crystal. Rubber balls were able to be dropped because they could bounce back. Crystal balls were precious and needed to be held tightly. Letting go of the rubber balls when necessary greatly simplified my life and enabled me to be flexible. Secondly, Dr. Joe told me that even though the whole family is affected by cancer at the time of diagnosis, siblings are most affected. They have an overpowering need to feel loved and important as the ill child gets so much attention. Throughout Patrick’s 15 months of treatment I tried to keep my three-year-old daughter’s life as normal as possible. I kept school routines and activities consistent and planned private quality time with her as well. I also involved her in Patrick’s day-to-day treatment. My husband and I devoted one day a week exclusively to our family. We spent some time alone to discuss the week and our feelings and then spent the rest of the day with the children. The best thing Dr. Joe gave me was hope. He explained that children’s cancer is different from adult cancer. Children respond well to chemotherapy and, most hopeful of all, most children do get better.

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DIAGNOSIS Why My Child? It is natural to ask “Why?” when a child develops a life-threatening illness. If your child has been diagnosed with Wilms tumor, you may be feeling shocked, lonely, fearful, frustrated, angry, or hurt. It is okay to have these feelings, and to cry in response to them. But don’t let your feelings paralyze you. Your child will need your love, support, and advocacy in the months to come. What Causes Wilms Tumor? Wilms tumor is believed to result from changes in one or more of several genes. It occurs in two forms: sporadic and hereditary. Hereditary Wilms tumor is passed to children through their parents’ genes. Between 4% and 5% of patients with hereditary Wilms tumor have bilateral Wilms tumor, and 1% to 2% have a family history of the disease. Sporadic Wilms tumor is more common, and is not accompanied by unusual physical features or family history of cancer. Siblings of children with Wilms tumor have a low likelihood of developing Wilms. Tumors are thought to grow out of the metanephric blastema, which serves as the excretory organ of the fetus and later develops into the kidneys. Because lesions made up of abnormal cells from this organ called nephrogenic rests have been found in 40% of kidneys removed from children with unilateral Wilms tumor and nearly 100% of kidneys of children with bilateral Wilms tumor, it is believed that a disturbance occurs at a crucial time in the development of the fetal kidney in patients with Wilms. Several types of isolated birth defects, or congenital anomalies, tend to appear in association with Wilms tumor. Children who are born with these defects should be screened for Wilms periodically. Evaluation Procedures Many procedures and tests are used in the evaluation of Wilms tumor. Not all doctors use all procedures. More than one procedure may be used in order to collect the most complete information possible. Procedures used most frequently in diagnosing Wilms tumor are described below.

Physical examination The physician checks the child’s abdomen for the presence of pain, swelling, or an abdominal mass. The child is evaluated for the presence of associated genetic abnormalities. Laboratory evaluation A blood sample is taken. Laboratory tests determine how many blood cells are present (a complete blood count), the proportions of different kinds of white cells in the blood (a differential leukocyte count), how many platelets are present (a platelet count), and how well the kidneys and other organs are functioning. Radiographic evaluation An abdominal ultrasound is used to determine whether a mass is present within the kidney, whether a mass is a fluid-filled cyst or solid, whether at least one of the kidneys appears normal, and whether there is a tumor clot in the abdominal veins. Ultrasound uses sound waves to form electronic pictures of various structures in the abdomen. To create an image, a technician moves a hand-held device called a sonic transducer across the abdomen. The transducer sends out sound waves, and the echoes produce pictures which are displayed on a monitor. The test does not hurt. Chest radiographs are used to determine whether Wilms tumor cells have spread to the lungs. Pictures are created by passing X-rays through the chest. Radiographs are taken from the front and back and from the side. The test does not hurt.

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Computed tomography is used to define the origin of an abdominal mass or to identify tumor lesions that have metastasized to the lungs. A CAT scan or CT scan is a fancy, high-tech X-ray. The patient lies on a motorized table that rolls into a donut-shaped machine. Inside the hollow donut is an X-ray head mounted on a motorized track. As the patient and table roll through the donut, the X-ray head moves to various locations and a pencil-thin X-ray beam takes cross-section pictures of the body. A computer assembles these pictures and a series of X-ray images are created on film. Part of the procedure consists of taking pictures while a liquid called a contrast medium travels through the body. When the substance is introduced either orally or through the veins there may be an uncomfortable sensation of pressure and heat moving through the body, but no pain. Histology Histology is the study of the structure of tissues using special staining techniques and microscopes. It is used to confirm a diagnosis of Wilms tumor. If physical and radiographic evidence suggest that your child has Wilms tumor, a biopsy is performed or the tumor is removed, after which a pathologist will examine a sample of the tumor. When a sample shows the characteristic Wilms cells and patterns, your child is diagnosed with the disease. Some Wilms tumor cells display features associated with a poorer prognosis. These features are found in approximately 10% of Wilms tumor patients. Anaplasia, the loss of normal cell characteristics, is typical of rapidly growing malignant tumors. Anaplastic tumors are described as having unfavorable histology (UH). Another subtype of Wilms tumor with a less favorable prognosis is clear cell sarcoma of the kidney (CCSK). CCSK often metastasizes to the skeleton and is sometimes associated with brain tumors. Children with kidney tumors of unfavorable histology generally have survival rates of between 55% and 75%, compared with the almost 90% survival rate for children with tumors of favorable histology (FH). The use of more intensive therapy seems to lead to a significant improvement in outcome for many children with tumors of unfavorable histology. Staging Staging is a system used by doctors to determine the risk of local or distant recurrence of a tumor and to develop a treatment plan. Members of the National Wilms Tumor Study Group have developed a five-stage system based on the degree of tumor extension which can be seen with the naked eye or with a microscope. The stage is determined by a pediatric urologist or a pediatric surgeon during an exploratory operation and by a pathologist who studies samples taken from the tumor. A simplified version of the NWTSG staging system is shown on the following page.

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National Wilms Tumor Study Staging System (Simplified)

Stage I The tumor was found only in the kidney itself. The membrane enclosing the kidney was not broken. The tumor was not ruptured before or during removal. The tumor was completely removed.

Stage II The tumor was found in the kidney as well as in tissues outside of the kidney. The membrane enclosing the kidney was broken. Blood vessels outside the kidney contained tumor or tumor clots. The tumor was biopsied, or ruptured and spilled into the immediate area. The tumor appears to have been completely removed.

Stage III The tumor has spread into the abdomen but has not been carried further by the blood: 1. Biopsy showed tumor in some lymph nodes 2. The tumor has extensively penetrated the lining of the abdomen, either as a result of spillage before or during surgery or as a result of growth 3. There are tumor implants in the lining of the abdomen 4. The tumor was not completely removed by surgery, and can be seen either with the naked eye or the microscope; and/or 5. The tumor cannot be completely removed by surgery because it has grown into structures which are vital for life

Stage IV The tumor has metastasized through the blood to other sites such as the lung, liver, bone, and/or brain.

Stage V Both kidneys had tumor at the time of the diagnosis. The extent of disease before biopsy is determined separately on each side according to the list above.

Table adapted from Table 1, National Wilms Tumor Study Clinicopathological Staging System, Green et al., CA-A Cancer Journal for Clinicians 1996: 46, p. 49. Stage and histology are used in combination to describe a patient’s chances for recovery. Thus your child could have a Stage I/favorable histology tumor, a Stage I/unfavorable histology tumor, a Stage II/favorable histology tumor, and so on.

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Matthew’s Story by Laura Randall On Monday, November 13, 1995, my biggest concern was planning Matthew’s first birthday party for the upcoming weekend. We were at Matthew’s one-year checkup at our pediatrician’s office. I had almost canceled the appointment because our insurance didn’t cover well-baby care and we already owed over $500. When the doctor felt his tummy, he exclaimed, “Whoa, what’s this?” Sure enough, a large lump could be felt in the middle of his abdomen. And if you looked at it, you could see it! How could I have not seen that before! We later found out that Wilms tumors double in size every month, so there was almost no way that our pediatrician could have seen that two months earlier. During surgery, the doctor came out and showed us the tumor. He held it in a jar. I was shocked at how large it was. It was not some black, ugly evil-looking thing as I had expected. There was barely any kidney tissue left. It had been taken over by the tumor. The good news was that the other kidney looked fine. Three days later we learned that Matthew had a favorable histology tumor. Surgery was followed by six months of outpatient chemo and six days of radiation. It was a difficult time. Sometimes when I think about what Matthew has gone through, I wonder if it was worth it. Then I give him a hug and see his beautiful smile and hear his silly laugh, and the answer is an overwhelming “YES!”

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TREATMENT Surgery In the United States, treatment of Wilms tumor typically begins with laparotomy, a type of abdominal surgery. General anesthesia is used. An incision is made in your child’s abdomen and the surgeon examines the affected kidney and neighboring abdominal structures to determine the extent of disease. The guidelines of the National Wilms Tumor Study Group call for exploration of the opposite kidney as well, because the presence of tumor on both sides greatly influences the treatment approach. In Europe and in some North American locations, treatment of Wilms tumor routinely begins with chemotherapy rather than surgery. This approach is not taken by NWTSG because there is some concern that it may lead to incorrect staging of the tumor and thus under or over treatment of the disease. If it is determined that only one kidney is affected, the diseased kidney and tumor and some associated structures are removed. This procedure is called nephrectomy. In addition, samples are taken from suspicious-looking areas of the liver and from the lymph nodes, especially those nodes that are enlarged. Sometimes a tumor is too large to remove immediately or involves structures preventing immediate removal. In this case a sample of the tumor is taken for evaluation in the lab and your child begins chemotherapy. After the tumor has shrunk significantly a second operation is performed and the tumor can be more easily removed. If it is found that both kidneys contain tumors, which occurs in 4.4% to 7% of cases, the current surgical approach is to individualize therapy, often taking tissue samples from both kidneys and following with chemotherapy and then surgery to remove all remaining tumor. This approach is described as renal parenchymal-sparing. It is designed to avoid later kidney dysfunction and failure, which occurs more often in patients with synchronous bilateral tumors. Patients with massive bilateral tumors that do not respond to chemotherapy and radiation therapy undergo removal of both kidneys, and eventually require dialysis and kidney transplant. Renal parenchymal-sparing surgery has also been used in some children with unilateral tumors. However, very few children meet the criteria established for undergoing such surgery. And because renal failure is so uncommon in this group-typically occurring in less than 1% of children with unilateral tumors and a normal contralateral kidney-the entire diseased kidney is usually removed. Chemotherapy In the U.S., chemotherapy is used in conjunction with surgery and radiotherapy. Using drugs after surgical removal of a primary tumor in order to keep tumors from recurring or developing in the future is termed adjuvant therapy. Chemicals referred to as cytotoxic drugs are used to damage tumor cells so that they can’t multiply. Several drugs are given in combination, either because they kill through different mechanisms, thus reducing the chance of resistance, or because they are synergistic, which means that using them together makes each one stronger.

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The types of drugs used, how often they are given, and what doses are required depend on the stage and histology of your child’s tumor and how much of the tumor has been removed surgically. The combination of vincristine and dactinomycin (also known as actinomycin D) has been shown to be effective in treating children with stage I/FH favorable histology, stage I/anaplastic, and stage II/FH favorable histology tumors. Doxorubicin is added to vincristine and dactinomycin to treat patients with stage III/FH, stage IV/FH, and stages II-IV/UH unfavorable histology. Other drugs are available for tumors which do not respond to conventional chemotherapy. Unfortunately, chemotherapy damages healthy cells along with cancerous ones. As a result, your child may experience side effects such as nausea, vomiting, diarrhea, chills, rash, and allergic-type reactions. The severity of these effects depends on drug dosage, the specific drug used, the patient, and how long and how often chemotherapy is administered, among other factors. The effects may last anywhere from a few hours to a few weeks. Chemotherapy, like radiation therapy, can sometimes cause new malignant cancers to form. In addition, some cytotoxic drugs can cause complications long after they are given. Doxorubicin, for example, if given in high doses, may increase the risk of heart rhythm disturbances or heart failure anywhere from a year to 20 or more years later. Radiation Therapy For some patients, radiation therapy is used following surgery to eliminate any remaining tumor in the region of the primary tumor and to help reduce the likelihood that the cancer will return. It may also be used to treat cancer cells that have metastasized to such areas as the lung, the liver, the lymph nodes, the bones, and the brain. Radiation therapy consists of using X-rays to damage cancerous cells so that they can’t reproduce. The X-rays are directed to the area where tumor cells are believed to be present. Radiation to the primary site may be directed just at the tumor bed after the kidney is removed or to the whole abdomen if the tumor has ruptured. For metastases to the lung and the brain, radiation is usually directed at the entire area. Although radiation therapy was once given to all children with Wilms tumor following the removal of their diseased kidney, that is no longer the case. Clinical trials have shown that radiation is not necessary for children with stage I-II/FH favorable histology or stage I/anaplastic tumors, as long as they receive adequate chemotherapy with vincristine and dactinomycin. Stage III/FH patients who are treated with vincristine, dactinomycin, and doxorubicin receive reduced doses of radiation to the abdomen. Radiation therapy also has short-term and long-term side effects, which are usually confined to the area beneath the radiation beam. Common short-term effects include a sunburn-like patch on the skin, nausea, diarrhea, and stomach cramps. Long-term effects which occur in only 3% to 10% of children, varying with the effect, may include abnormal development of the spine; underdevelopment of soft tissue such as abdominal and back muscles; new cancers, such as bone, breast, and thyroid cancers, which may occur in irradiated areas; and damage to the heart, lungs, liver, ovaries, or the remaining kidney, if they are irradiated.

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A type of radiation treatment called brachytherapy is currently being tested in children with bilateral Wilms tumor. This therapy, most often used to treat cervical cancer, involves implanting radiation seeds along the single remaining kidney at the point where the tumor emerged. The procedure allows high amounts of radiation to be concentrated in a small area with little tissue damage and little or no damage to other organs. It is currently being used experimentally. Immunotherapy Immunotherapy consists of using manufactured substances called biological response modifiers to make the immune system more effective in fighting disease. Although it has been successful in treating adult kidney cancer and is used for many adult tumors, it is not effective in treating Wilms.

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Joe Perusic’s Story I was two when I was diagnosed with Wilms tumor. My most lasting memory is a fear of needles. However, I am now 38 and without recurrence. I am the very proud father of one son. I had genetic counseling and know that I did not have a genetic form of Wilms. The genetic form of Wilms is very rare, but the counseling was reassuring.

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POST-TREATMENT Follow-Up After initial treatment of his disease, your child will return at regular intervals for a variety of procedures to determine whether the tumor has recurred. These may include palpation, in which the hands and fingertips are used to examine the abdomen; chest X-ray; abdominal ultrasound; bone scan; magnetic resonance imaging (MRI) of the brain; and skeletal X-ray of irradiated sites. All children who received lung irradiation should have routine thyroid function tests for 5 years and yearly palpation of the gland for life. Recurrent Disease Sometimes a tumor reappears after a patient seems to have recovered. This is called a relapse. The most frequent site of Wilms tumor recurrence is the lung. Additional sites are the liver, the opposite kidney, the original tumor bed, and other areas of the abdomen. Bone, brain, and other sites are only occasionally involved. Most first relapses are diagnosed within two years after nephrectomy, but they have been known to occur as long as 11 years afterward. Which treatments a child receives for a recurring tumor and how effective those treatments are depends on many factors, including the site of recurrence, histology of the tumor, length of time from removal of the kidney to recurrence, and how many cytotoxic drugs were given in the first round. Patients who initially received only two drugs (dactinomycin and vincristine), who initially had stage I disease, who have a recurrence only in the lungs, who have a recurrence in the abdomen without previous radiation therapy, or who have a recurrence more than 12 months after diagnosis have a better prognosis. These children may benefit from the use of alternative chemotherapy, surgical excision of the recurrent tumor, or radiation therapy. Less can be done for patients with unfavorable histology tumors, abdominal recurrence after treatment with radiotherapy, recurrence within 6 months of nephrectomy, or recurrence after initial three-drug therapy. These children may be given new or experimental therapies. Survival Statistics Many factors play a role in the survival of a cancer patient. Among them are the makeup of the tumor, whether a tumor has metastasized, completeness of surgical removal, response to chemotherapy and radiation therapy, and whether proper follow-up treatment is received. You can improve your child’s chances of survival by doing all you can to ensure access to the best possible medical care. Your child is considered a Wilms tumor survivor if he or she has not had a relapse and is disease-free four years after diagnosis, since very few relapses occur after this time. A patient is also generally considered a survivor if he or she has had a relapse but is disease-free five years after diagnosis and two years after completion of secondary therapy. Lifetime Follow-Up The following guidelines for follow-up after Wilms tumor are suggested by the National Wilms Tumor Study Group in the “Frequently Asked Questions� section of their web site, updated on May 11, 1999. = All patients should have tests of kidney function and a urinalysis at least once a year. = Those patients who received abdominal radiation should be examined closely for possible growth irregularities. = Patients who received radiation to the chest should have detailed tests of lung function if there is a clinical suspicion of lung disease or if there are plans for a vigorous exercise program. = Patients who were treated with Adriamycin should undergo an echocardiogram and 24-hour Holter Monitor evaluation before initiating a vigorous exercise program, and should be questioned regularly regarding evidence of heart disease. = All women should undergo detailed cardiac evaluation prior to becoming pregnant and during the course of pregnancy. = Women who have received radiation therapy to part of their abdomen are more likely to have a baby born early or of small size, and should inform their doctor of their past medical history. However, there is no evidence of an increase in birth abnormalities among the children of men and women who have been treated for Wilms tumor. 13


HELPING A CHILD WITH WILMS TUMOR Where should we go for treatment? How can I help my child? How do I know that my child is getting the best possible care? Will I be able to participate in decision making? Where can I find more information? As the parent of a child with Wilms tumor, you probably have many questions. In the beginning, you may feel as if you are entering a maze where every turn leaves you more bewildered. Fortunately, there are people out there who want to help. Choosing a Hospital According to the American Cancer Society, “Children with cancer and their families have special needs that can be best met by children’s cancer centers. (A major medical center that has a large pediatric department is next best.) Treatment in specialized children’s cancer centers takes advantage of a team of specialists who understand the differences between adult and childhood cancers, and the unique needs of children with cancer. This team usually includes pediatric oncologists, surgeons, radiation oncologists, pediatric oncology nurses, and nurse practitioners. Many professionals other than doctors and nurses are also involved. Children’s cancer centers have psychologists, social workers, child life specialists, nutritionists, rehabilitation and physical therapists, and educators who can support and educate the entire family. Supportive care is essential to the success and continuation of therapy. By controlling chemotherapy-induced nausea and vomiting, and giving proper nutrition, adequate pain control, and prophylaxis against infection, the complications of treatment can be reduced and the quality of life for childhood cancer patients can be restored. In addition, maintenance of a normal social setting and schooling will relieve some of the stress associated with the alterations and limitations imposed by the treatment.” The right hospital for your toddler may end up being far from home, but getting the best care possible, when it’s possible, is almost always worth the trip. Advocacy Children often have little control over what happens to them as a result of their illness. This means it is up to adults to ensure that they receive the best possible care. Advocating for the rights of small patients is the responsibility not only of the child’s parents and pediatrician, but also of the many hospital personnel who contribute to the child’s welfare. Medical personnel have many patients to care for, however. It is important not to allow your child to be a number, says Susan Mullane, whose child was diagnosed at age 6 months with bilateral Wilms tumor. You are your child’s best advocate. Try to work with the staff and doctors to help your child. Become active advocates for your child’s care. Search the Internet using the tools provided here. Be informed about your child’s disease. The Hospital Experience You are probably just beginning your fight against Wilms tumor. Eventually, the hospital will become a familiar place for your child. But for every cancer patient there is a first hospital experience, and that experience can set the tone for future visits. Parents and hospital staff should do all they can to help prepare a child for an upcoming hospital stay. If the hospital is viewed as a place where friendly people work to help children get well, then a sick child may find future visits more tolerable.

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Preparing for a hospital stay Many pediatric hospitals have pre-admission programs which give children scheduled for surgery the chance to become familiar with the hospital before they check in. These programs may include a tour of the operating room, the chance to put on a hospital gown and an anesthesia mask, role playing with doctor kits, puppet shows, and a variety of other child-oriented activities. Check with your hospital for details. If your child is young, you can also help prepare him or her by doing some role-playing at home with a toy medical kit. Books designed to prepare children for hospitalization are available from public libraries, bookstores, and healthcare organizations. Picture books and coloring books may be helpful for younger children. Older children may wish to read fiction about a child with an illness or a nonfiction account of how the body works. Some titles are listed under “Resources for Children” on page 17. Keeping a scrapbook or journal to document the hospital stay may also help your child. Preparing for surgery The medical community is divided over the issue of whether parents should be allowed to be with their child while anesthesia is given before an operation. Child advocacy groups generally believe that the participation of a parent is beneficial for the child psychologically. Ask your doctor whether the hospital will permit you to be present. A video produced by the Australian Association for the Welfare of Child Health, titled “Parental Involvement in their Child’s Anaesthetic,” offers the following tips for a positive surgical experience: = inform child and parent in advance about what will happen = promote a child-friendly environment = provide age-appropriate operating room clothing = allow the child to wear underpants = reduce fasting times before operation = use oral premedication = avoid needles if possible = use anesthetic cream when I.V. needles are needed = walk with or carry the child to the operating area where possible = use scented masks for application of anesthesia = use distraction techniques = encourage parental support of the child Children and Pain Little more than a decade ago, infants were commonly operated on without anesthesia because it was believed that their nerves were not sufficiently developed for them to feel pain. Now it is known that even very premature babies feel pain. Today, preventing and managing pain in seriously ill and hospitalized children is an essential part of patient care. Pain can slow healing, disrupt treatment, and cause further medical problems. Uncontrolled pain makes children afraid. The Association for the Care of Children’s Health (ACCH) distributes a booklet titled Pain, Pain, Go Away: Helping Children with Pain. It is designed to teach parents about pain in children so that they can help their child receive better care. Written in simple language, it briefly describes sources of pain, measuring pain in children, pain management, treatment of postoperative pain, cancer pain, advocating pain relief for children, and what to do if your child is in pain.

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Two distinct types of pain that a child may encounter in the hospital are short, sharp pain and postoperative pain. Pain, Pain, Go Away... offers the following suggestions for dealing with pain:

Short, sharp pain = provide simple, accurate information about what is going to happen = encourage your child to ask questions = give your child some control over the treatment = help your child express his or her feelings = teach deep and steady breathing = distract your child or absorb his imagination = touch (stroke, swaddle, hold, rock, caress, cuddle, or massage) your child = teach relaxation techniques = encourage play Short, sharp pain resulting from a needle stick can often be avoided with the use of an anesthetic cream which is applied to the child’s skin one to two hours before a procedure is performed. Postoperative pain Most postoperative pain can be prevented, or at the very least reduced. Reduction of postoperative pain generally requires pain medication, which should be given before pain occurs or becomes severe. Such medication is generally given by hospital personnel according to a regular schedule. Sometimes pain medication is not given in a timely manner, however, and the patient begins to suffer from and even anticipate pain. In response to this problem, a method of self-medication called patient-controlled analgesia (PCA) has been developed. With PCA, a patient who starts to feel pain administers a dose of pain medication by pushing a button that is connected to a computer, which then delivers the medicine through the intravenous (I.V.) line. PCA has been used successfully by patients as young as 6 years, and gives them the sense that they are in control of their pain. Ask your child’s doctor whether PCA is available at your hospital. Mental and Emotional Well-Being Mental processes and states of mind can contribute to survival and healing among cancer patients. Although very young children may not be able to actively work at maintaining a positive attitude, there are steps that can be taken by parents and caregivers to help children of all ages deal with their disease.

Stress reduction Research shows that stress can alter immune system function, which in turn can alter tumor growth and response. Children with cancer should be offered age-appropriate methods of dealing with stress. For a young child, one of the most stressful aspects of having cancer may be the contact with unfamiliar people and surroundings. This may particularly apply to a hospital admission. The authors of Your Child in the Hospital encourage parents to stay in the hospital with their child both during the day and overnight. If you stay at your child’s side, you can provide comfort, protection, and advocacy, they say. This is not always possible, however. Another way to lessen the unfamiliarity of a hospital room is to bring along a little bit of home. Familiar items such as pajamas, a favorite blanket or stuffed animal, and pictures or posters can be especially comforting.

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Young children also need to be able to play. Ask your hospital whether you can bring along toys, games, audio- and videocassettes, and other entertainment for your child’s hospital stay. Putting together a kit with special items and activities to be used only at the hospital can give your child something positive to associate with each visit. In addition, many children’s hospitals are equipped with playrooms and offer activities led by play therapists. Play allows children to deal with emotions such as anger, sadness, and anxiety. For older children, stress may arise from general anxiety over the disease, or from the discomfort of treatment. These children may benefit from guided imagery or the use of audio cassettes for relaxation. A temporary escape from the illness can be provided by computers, movies, and books. And talking with peers can be valuable. Research has shown that patients who actively participate in a support group survive longer than comparable patients who don’t.

Dealing with fears Young children need simple, accurate information about what will happen to them. Ask your child to tell you what he thinks will happen during a procedure; you may be surprised at his answer. Encouraging your child to ask questions will give you a chance to check his understanding and allay some of his fears. Older children and teenagers may fear dying. You can encourage your child to express his or her fears with other cancer patients in discussion groups, by keeping a journal, through artwork or poetry, through formal counseling, or in another way that works for him or her. Resources for Children

Publications There are numerous books, both fiction and nonfiction, designed to introduce young children to the hospital. Fewer titles are available on the topic of dealing with a serious illness. Books about death written for a younger audience often deal with the death of an animal. Your local librarian may be able to steer you toward appropriate titles, as well as provide copies of books which are out of print. The titles listed on the following page are not meant to be recommended over other books which are not listed, and a book which appeals to one child may not appeal to another. A bookstore can also give you an idea of the range of titles which are available. If you would like to sample some of the books listed below, an on-line book ordering service located at http://www.amazon.com is an excellent resource. The Amazon Books web site provides synopses and reviews of books on a wide range of topics. You can search the on-line database for the specific types of books which interest you, and if the book you want is out of print, the company will even search its network of used book stores for a copy.

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Books about hospitals and hospitalization: A Visit to the Sesame Street Hospital, by Dan Elliott and Deborah Hautzig (for young children) Barney Is Best, by Nancy White Carlstrom (for young children) Behind the Scenes at the Hospital, by Marilyn Miller (for young children) Coping With a Hospital Stay, by Sharon Carter and Judy Monnig (for teenagers) Curious George Goes to the Hospital, by Maragaret Rey and H. A. Rey (for young children) Going to the Hospital, by Fred Rogers (for young children) The Hospital Book, by James Howe (for young children) The Teenage Hospital Experience: You Can Handle It, by Elizabeth Richter (for teenagers) When Molly Was in the Hospital: A Book for Brothers and Sisters of Hospitalized Children, by Debbie Duncan (for young children) Why Am I Going to the Hospital? by Claire Ciliotta and Carole Livingston (for young children) Books about coping with cancer: Dear Bruno, by Alice Trillin (letters to a young child with cancer) I Want to Grow Hair, I Want to Grow Up, I Want to go to Boise, by Erma Bombeck (a novel for teenagers; out of stock in spring 2000) Chemo Girl: Saving the World One Treatment at a Time, by Christina Richmond (written by a 12-year-old cancer patient) Books about death: Badger’s Parting Gifts, by Susan Varley (for young children) The Fall of Freddie the Leaf, by Leo F. Buscaglia (for readers 9-12) Gran-Grans Best Trick: A Story for Children Who Have Lost Someone They Love, by L. Dwight Holden (deals with a girl’s reaction to her grandfather’s cancer) I’ll Always Love You, by Hans Wilhelm (for young children) The Tenth Good Thing About Barney, by Judith Viorst (for young children) When Dinosaurs Die: A Guide to Understanding Death, by Laurie Krasny Brown and Marc Tolon Brown (for young children) Publications for parents are available free from the National Cancer Institute, Office of Cancer Communications, Building 31, Room 10A24, Bethesda, MD 20892. Or call 1-800-4-Cancer, (1-800-422-6227). Young People with Cancer Managing Your Child’s Eating Problems When Someone in Your Family Has Cancer

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Internet sites More and more resources are becoming available via computer over the Internet. Selected sites are listed below. Kids’Home is a web site provided by the National Cancer Institute. It gives children with cancer and other illnesses a place to share their original art, stories, and poems, and provides parents with information on topics such as how to talk to kids about cancer. The site also contains links to Fun Places to Visit on the Web. Internet address: http://cancernet.nci.nih.gov/occdocs/KidsHome.html Matthew Randall’s Story is a personal Web site recounting how Matthew and his family dealt with Wilms. Internet address: http://www.geocities.com/Heartland/Garden/5195. You can also send an e-mail message to Matthew’s parents, Laura and Chris Randall (CLRandall@aol.com), who will answer questions about Wilms tumor and talk with parents about their experience. Laura is a nurse and active in Candlelighters as well.

Videocassettes A 200-member children’s choir called Kids Healing Kids has created a number of videos featuring lively songs designed to cheer up children undergoing treatment for cancer. Tapes are available free of charge to individuals and children’s cancer hospitals nationwide. Telephone: (616) 786-1892. Fax: (616) 786-1991. Plastic Eggs or Something!?: Cracking Hospital Life and What Am I, Chopped Liver?: Communicating With Your Doctor are the first two videos in a series called “Videos With Attitude” produced by Steven Spielberg and the Starbright Foundation. The 15-minute videos are available free to teenagers and preteens with serious and chronic illnesses. They can be purchased by healthcare professionals. Address: Starbright Foundation, 1990 S. Bundy Drive, Suite 100, Los Angeles, CA 90025. Telephone: (310) 442-1560 or (800) 315-2580 x3.

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Laura Randall’s Realization If you have a child in treatment it can seem like everyone is in treatment or relapsing or even dying. That is because you are seeing only those children who are coming to the hospital. The survivors are not at the hospital! A sick child is not the only one who needs help dealing with illness: having a child with a life-threatening disease can put enormous strain on you and your family. You may become so involved with your sick child’s medications, operations, and treatments that you forget there is a world outside the hospital. For your own sake and for the well-being of your family make an extra effort to communicate with your spouse, your other children, close friends, and relatives. Don’t try to shoulder every burden yourself: turn to others for support. In the words of John Donne:

“ No man is an island, entire of itself; every man is a piece of the continent, a part of the main . . .

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SUPPORT FOR THE FAMILY Dealing with Stress You’ll find that people cope with stress in different ways. Relationships within your family as well as with friends and co-workers will change. Your daily schedule may alter drastically, and you may feel you can’t keep up with the many demands on your time, energy, and emotions. It will probably be necessary to cut back on commitments. Try to decide what is most important to you. Don’t neglect your own health as you worry over the health of your child. Remember to eat and sleep. At the same time, don’t ignore your need for mental and physical relaxation. Whenever possible, make time for yourself. Have coffee with a friend. Swim laps at the local pool. Get a massage. Go for a walk in the woods. Watch a movie. Work in your garden. Find some pastime that will help relieve the pressure that is bound to build up in you. The Needs of Siblings If your child has siblings, do your best to meet their needs as well. Answer their questions honestly and encourage them to share their feelings. Reassure them that you love them. And consider bringing them to the hospital with you. According to the authors of Your Child in the Hospital, many parents say bringing the siblings along helps everyone. The sibling gains an accurate understanding of hospital procedures, the sick child is comforted by the presence of the sibling, and parents get to spend time with all their children. Counseling A child’s illness causes stress for the entire family. Anger, jealousy, fear, and guilt are common reactions. For some family members, the stress may be too great to handle alone. Professional counseling can help them address and cope with the many issues which accompany the serious illness of a child. If you feel that you or another family member would benefit from professional assistance, ask your doctor to refer you to a mental health professional. Many cancer centers have psychologists and social workers who specialize in assisting cancer patients and their families. Sources of Information and Support As you and your child fight the battle against Wilms tumor, remember that you are not alone. Accept support from relatives, friends, and neighbors. Contact organizations for information and referrals. Share your experience with other families who currently have or at some point have had the same problems. A range of options are available, from local meetings to telephone and computer-based support. There are numerous organizations devoted to helping people who are ill and their families. Some cover a broad spectrum of illnesses, while others specialize in a specific type of illness or a particular age group. Selected organizations are listed below. Cancer Information Service - An excellent source of information on every aspect of cancer. Questions answered free of charge over the telephone by trained staff members of the National Cancer Institute. Telephone: 1-800-4-CANCER (1-800-422-6237). Web site: www.clinicaltrials.gov lists clinical trials for all kinds of cancer by type of cancer and location of trial. The Candlelighters Childhood Cancer Foundation - An international nonprofit membership organization founded in 1970 by parents of children with cancer. Its mission is to educate, support, serve, and advocate for families of children with cancer, survivors of childhood cancer, and the professionals who care for them. Among the services it provides are publications, regional and national conferences, advocacy, medical referrals, member links, and referrals to local support groups. Address: Candlelighters Childhood Cancer Foundation, 3910 Warner Street, Kensington, MD 20895. Telephone: 1-800-366-2223 (toll-free information) or (301)962-3520. Fax: (301) 962-3521. E-mail: info@candlelighters.org. Internet address: http://www.candlelighters.org

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Children’s Hospice International - Provides a network of support and care for children with life-threatening conditions and their families. Address: 2202 Mt. Vernon Avenue, Suite 3C, Alexandria, VA 22301. Telephone: (703) 684-0226 or 800-2-4-CHILD. Internet address: http://www.chionline.org Kidney Cancer Association - A nonprofit membership organization made up of kidney cancer patients, families, physicians, nurses, and other healthcare professionals. KCA provides information to patients and physicians, works to generate and finance research on Wilms tumor and kidney cancer, and advocates for patients before government agencies. Address: Kidney Cancer Association, 1234 Sherman Avenue, Suite 203, Evanston, IL 60202. Telephone: (800) 850-9132, (847) 332-1051. E-mail: office@kidneycancer.org Internet address: http://www.kidneycancer.org Make-A-Wish Foundation of America works to grant the wishes of children with terminal or life-threatening illnesses, anything from a ride in a race car to a meeting with a movie star. Address: Make-A-Wish Foundation, 3550 N. Central Ave., Ste 300, Phoenix, AZ 85012. Telephone: (800) 722-WISH (9474). E-mail: MAWFA@wish.org. Internet address: http://www.wish.org National Cancer Institute - The organization that coordinates the U.S. Government’s cancer research program. In addition to producing publications, NCI provides CancerNet, a wide range of information via the Internet. Sites include CANCERLIT (a bibliographic database) and PDQ, a comprehensive cancer database containing summaries of cancer treatments, screening, prevention, and supportive care; a registry of cancer trials; and directories of physicians, genetic counselors, and organizations that provide cancer care. Address: National Cancer Institute, Office of Cancer Communications, Building 31, Room 10A24, Bethesda, MD 20892. Internet address: http://cancernet.nci.nih.gov National Childhood Cancer Foundation - A nonprofit charitable foundation that supports pediatric cancer treatment and research projects at more than 115 pediatric medical institutions throughout North America. Research is conducted by a network of 2800 pediatric cancer specialists called the Children’s Cancer Group (CCG). Address: 440 E. Huntington Drive, Suite 300, P.O. Box 60012, Arcadia, CA 91066-6012. Telephone: 1-800-458-6223. Fax: 1-800-723-2822. Internet address: http://www.nccf.org The National Wilms Tumor Study Group (NWTSG) - A federally funded, multi-institutional study of the treatment of patients with Wilms tumor. The study involves the collaboration of a large number of pediatric surgeons, pediatric oncologists, pediatric radiation oncologists, pediatric urologists, and allied health professionals with a goal of developing more effective treatments for children with Wilms tumor, as well as looking for the causes of this cancer. The NWTSG web site contains a bibliography of several hundred scholarly articles and publications related to Wilms tumor, as well as answers to questions frequently asked by Wilms tumor survivors and parents of children with Wilms tumor. Internet address: http://www.nwtsg.org Pediatric Oncology Group Foundation - A nonprofit charitable foundation that supports pediatric cancer treatment and research projects at more than 100 pediatric medical institutions throughout North America. Research is conducted by a network of more than 2000 pediatric cancer specialists called the Pediatric Oncology Group (POG). Address: 645 North Michigan Avenue, Suite 910, Chicago, Illinois 60611. Telephone: (312) 482-9944. Internet address: http://www.pog.ufl.edu

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Editor’s Note: Effective January 1, 2002, the Children’s Cancer Group, National Wilms Tumor Study Group, Pediatric Oncology Group, and Intergroup Rhabdomyosarcoma Study Group will all merge to form the Children’s Oncology Group, so that research and treatment for all pediatric cancers will fall under one umbrella in the U.S. In the interim, information about the Children’s Oncology Group may be directed to the Pediatric Oncology Group at (312) 482-9944, or their web site at cog@nccf.org

Other Resources Several organizations offer help for families who must travel to get the best care for a sick child. AirLifeLine - Provides free flights nationally to patients and their families. Address: AirLifeLine National Office, 50 Fullerton Court, Suite 200, Sacramento, CA 95825. Toll-free telephone: 1-877-AIRLIFE (877-247-5433) or 916-641-7800. E-mail address: staff@AirLifeLine.org. Internet address: http://airlifeline.org Angel Flight East - Provides free transportation on private aircraft for people who need access to medical care and cannot afford regular commercial transportation. Based in Pennsylvania, it serves states in a 600-mile radius. Address: Angel Flight East, 600 W. Germantown Pike, Suite 400, Plymouth Meeting, PA 19462. Telephone: (610) 940-1717. E-mail: angeladmin@aol.com. Internet address: http://www.angelflightpa.org National Association of Hospital Hospitality Houses - Gives referrals for lodging for families of hospital inpatients and outpatients. Address: National Association of Hospital Hospitality Houses, P.O. Box 18087, Asheville, NC 28814. Toll-free telephone: 1-800-542-9730. Telephone: (828) 253-1188. E-mail address: helpinghomes@nahhh.org. Internet address: http://nahhh.org National Patient Travel Center - Provides information about free or discount air travel for patients who must travel for care. 4620 Haygood Rd., Ste 1, Virginia Beach, VA 23455. Toll-free telephone: 1-800-296-1217 (in the U.S.). Telephone: (757) 318-9145 (outside the U.S.). E-mail address: mercymedical@erols.com. Internet address: http://www.PatientTravel.org Ronald McDonald Houses - Provides free or low-cost housing for the families of hospitalized children at 197 sites in 16 countries. Address: Ronald McDonald House Charities, 1 Kroc Drive, Oak Brook, IL 60523. Telephone: (630) 623-7048. Internet address: http://www.rmhc.com Dealing With Grief The Compassionate Friends - Offers worldwide and computer-based support groups for families who are grieving the death of a child. Information: The Compassionate Friends, Inc., P.O. Box 3696, Oak Brook, IL 60522-3696. E-mail address: tcf_national@prodigy.com. Internet address: http://www.compassionatefriends.org GriefNet - A computer World Wide Web site that sponsors e-mail support groups and provides links to other Internet sites dealing with grief and loss. Address: GriefNet, P.O. Box 3272, Ann Arbor, MI 48106-3272. E-mail address: Visibility@griefnet.org. Internet address: http://www.rivendell.org

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LIVING EACH DAY Parenting a seriously ill child is an enormous challenge. It is also an opportunity. Try to make the most of each day that you spend with your child. Celebrate simple things together: a sunrise, a rainbow, a snowfall. Record good experiences with film, audio cassettes, videocassettes, or a journal, so that when your child grows up you can look back together and remember the good times you shared.

Look around and choose your own ground For long you live and high you fly And smiles you’ll give and tears you’ll cry And all you touch and all you see Is all your life will ever be Lyrics by Pink Floyd

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END NOTES The research articles, book chapters, on-line database, booklets and books listed below were used in the production of Wilms Tumor: What Now? They range from highly technical, scholarly articles published in peer-reviewed medical journals to books written for a general reader with little medical knowledge. They were chosen because they contained pertinent information. There are undoubtedly many other publications which were not consulted but are also valuable. Technical Publications The following research articles and book chapters are written for a medical audience. Each of the articles also includes a list of references to other articles on topics related to Wilms tumor, childhood cancer, or cancer in general. In addition, a bibliography of almost 300 scholarly articles and publications related to Wilms tumor can be found at the National Wilms Tumor Study Group web site (Internet address: http://www.nwtsg.org/public/bibliography.html). Birch JM, Breslow N. Epidemiologic features of Wilms tumor. Hematology/Oncology Clinics of North America 1995: Vol. 9, No. 6, pp. 1157-1178. Breslow NE, Olson J, Moksness J, et al. Familial Wilms tumor: A descriptive study. Medical and Pediatric Oncology 1996: Vol. 27, pp. 398-403. Clericuzio CL, Johnson C. Screening for Wilms tumor in high-risk individuals. Hematology/Oncology Clinics of North America 1995: Vol. 9, No. 6, pp. 1253-1265. Green DM, DAngio GJ, Beckwith JB, et al. Wilms tumor. CA-A Cancer Journal for Clinicians 1996: Vol. 46 No. 1, pp. 46-63. Green DM, Coppes MJ, Breslow NE, Grundy PE, Ritchey ML, Beckwith JB, Thomas PRM, D’Angio GJ. Wilms Tumor. In: Pizzo PA, Poplack DG (editors). Principles and Practice of Pediatric Oncology (3rd Edition). Philadelphia: Lippincott-Raven Publishers 1997, pp. 733-759. Haase GM. Current surgical management of Wilms tumor. Current Opinion in Pediatrics 1996: Vol. 8, pp. 268-275. Heuch JM, Heuch I, Kuale G. Birth characteristics and risks of Wilms tumor: a nationwide prospective study in Norway. British Journal of Cancer 1996: Vol 74, pp. 1148-1151. Marina NM, Bowman LC, Pui C, Crist WM. Pediatric Solid Tumors. In: Murphy GP, Lawrence WL, Lenhard Jr., RE (editors). American Cancer Society Textbook of Clinical Oncology. Atlanta, GA: American Cancer Society; 1995: 524-551. Paulino AC. Current issues in the diagnosis and management of Wilms tumor. Oncology 1996: Vol. 10, No. 10, pp. 1553-1565. Thomas P. (Review of Paulino’s Current issues in the diagnosis and management of Wilms tumor.) Oncology 1996: Vol. 10, No. 10, pp. 1569-1570. Zantinga AR and Coppes MJ. Historical aspects of the identification of the entity Wilms tumor, and its management. Hematology/Oncology Clinics of North America 1995: Vol. 9, No. 6, pp. 1145-1156.

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General Publications The Bantam Medical Dictionary, Market House Books Ltd., ©1990. Better Homes and Gardens New Family Medical Guide, edited by Edwin Kiester, Jr. Meredith Corporation, ©1989 (out of print). Pain, Pain, Go Away: Helping Children with Pain, by Patrick J. McGrath, G. Allen Finley, and Judith Ritchie. Association for the Care of Children’s Health, ©1994. What to Expect the Toddler Years, by Arlene Eisenberg, Heidi E. Murkoff, and Sandee E. Hathaway. Workman Publishing, New York, ©1994, pp. 588-590, “In the Hospital.” We Have Kidney Cancer. Kidney Cancer Association, ©2000-2008 Your Child in the Hospital: A Practical Guide for Parents, by Nancy Keene and Rachel Prentice. O’Reilly and Associates, Inc., ©1997. On-Line Information If you would like to read a layperson’s version of the most recent recommendations for treating Wilms tumor, look up the National Institute of Health’s PDQ database (http://cancernet.nci.nih.gov/pdq.htm) and select Treatment Summaries for Patients. Basic information is also available from the National Wilms Tumor Study Group through its list of Frequently Asked Questions (http://fhcrc.org/science/phs/nwtsg/faq.html). If you don’t have access to a computer, or are not familiar with the Internet, ask your librarian for help.

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GLOSSARY OF TECHNICAL TERMS Below are some technical terms used in this booklet. You will come across many of them in scientific articles about Wilms tumor. However, this list is by no means complete, and you may wish to invest in a medical dictionary if you plan to do technical reading. An attempt has been made here to define terms as simply as possible. Most definitions are adapted from The Bantam Medical Dictionary, Š1990 by Market House Books Ltd. actinomycin D - a cytotoxic drug used to treat Wilms tumor, usually in combination with other drugs; also known as dactinomycin anaplasia (anaplastic) - loss of normal cell characteristics; typical of rapidly growing malignant tumors adjuvant therapy - drug treatment given to patients after surgical removal of their primary tumor to keep tumors from recurring or developing benign - refers to a tumor that grows within a capsule, has no potential for spread, and can be removed with no chance of its recurring biological response modifiers - manufactured substances which make the immune system more effective in fighting disease; used to treat adult kidney cancer biopsy - the removal of a small piece of living tissue from an organ or a part of the body for evaluation in the lab capsule - a membrane that surrounds each kidney chemotherapy (chemotherapeutic) - treatment of cancer with one or more cytotoxic drugs clear cell sarcoma of the kidney (CCSK) - a type of childhood kidney cancer similar to Wilms tumor but associated with a poorer prognosis; see also sarcoma clinical trials - large-scale research studies that allow researchers to test new approaches and share information complete blood count - a test to determine how many red and white blood cells are present in the blood computed tomography (CT scan, CAT scan) - a procedure in which an X-ray beam takes cross-section pictures of the body, which are then assembled by a computer to create a series of X-ray images on film. congenital anomalies - abnormalities present at birth contralateral kidney - the kidney on the other side (i.e., the one unaffected by cancer) contrast medium - a substance used to improve the visibility of structures in the body during a procedure using X-rays such as computed tomography cyst - an abnormal fluid-filled or semi-solid mass that may appear in a variety of locations in the body cytotoxic drug - a drug that damages or destroys cells and is used to treat cancer. It destroys cancer cells by inhibiting cell division, but also affects normal cells. dactinomycin - a cytotoxic drug used to treat Wilms tumor, usually in combination with other drugs; also known as actinomycin D dialysis (hemodialysis) - a technique in which a machine removes waste products or poisons from the blood of people whose kidneys can no longer perform this function differential leukocyte count - a test to determine the proportions of different kinds of white cells in the blood doxorubicin - a cytotoxic drug used to treat Wilms tumor, usually in combination with other drugs echocardiogram - a painless test in which ultrasound waves are used to monitor the action of the heart as it beats favorable histology - describing a microscopic appearance associated with a better chance of cure general anesthesia - injection or inhalation of substances which produce total unconsciousness so that surgery can be performed without pain hereditary - passed from parents to their children through inherited genes

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histology - study of the structure of tissues using special staining techniques and microscopes laparotomy - an operation by which the abdomen is surgically opened so that abdominal organs can be examined; used in diagnosis of disease and in determining the spread of a cancerous growth magnetic resonance imaging (MRI) - a procedure in which a powerful magnetic field is used to align molecules in the body so that very detailed pictures can be taken of internal organs. The pictures are taken in cross-section and then assembled by a computer to create images on film. malignant - describing a tumor that invades and destroys the tissue it has grown from, and can spread to and attack other sites in the body metanephric blastema - the excretory organ of the fetus which later develops into the kidneys metastases - malignant cells that break off from the main tumor, are carried to other parts of the body, and begin to grow there metastasis - the spread of malignant cells to other parts of the body nephrectomy - surgical removal of a kidney partial nephrectomy - surgical removal of part of the kidney radical nephrectomy - surgical removal of the entire kidney, plus surrounding fat and adjacent adrenal glands nephroblastoma - also known as Wilms tumor; a malignant tumor of the kidney that affects children nephrogenic rests - remnants of metanephric blastema found in the kidneys of a child, and thought to develop into Wilms tumor lesions oncologist - a doctor who treats cancer. A medical oncologist uses drugs; a surgical oncologist uses surgery; a radiation oncologist uses radiation; a pediatric oncologist treats children. oncology - the study and practice of treating tumors palpation - using the hands and fingertips to examine a part of the body pathologist - someone who studies disease processes with the aim of understanding their nature and causes. A pathologist examines tissues both with and without a microscope. patient-controlled analgesia (PCA) - a method of self-medication in which a patient prompts a computer to administer pain-relieving medicines through an intravenous (I.V.) line. platelets - cells in the blood which are responsible for clotting platelet count - a test to determine how many platelets are present in the blood radiotherapy - the use of radiation (e.g., X-rays) to kill abnormal cells radiographs - images produced on film by X-rays relapse - a return of disease symptoms after apparent recovery, or the worsening of a recovering patient’s condition renal artery - one of two large blood vessels carrying blood from the heart to the kidneys renal vein - one of the blood vessels through which blood leaves the kidneys to return to circulation sarcoma - cancer of connective tissue in any area of the body; can occur in muscle, fat, bone, blood, and various other tissues sonic transducer - a hand-held device used in an ultrasound sporadic - describing a disease that occurs only occasionally staging - a system by which doctors use physical features of a tumor to determine the risk of local or distant recurrence and to develop a treatment plan synergistic - referring to drugs which are more potent when used in combination

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tumor - a mass of rapidly growing, abnormal, out-of-control cells bilateral tumor - a tumor affecting both kidneys metachronous bilateral tumor - a tumor occurring first in one kidney and later in the other primary tumor - a tumor at its original site residual tumor - a tumor which remains after treatment or after a part of treatment is accomplished synchronous bilateral tumor - a tumor occurring in both kidneys from the outset tumor bed - location from which the tumor was removed unilateral tumor - a tumor affecting a single kidney Wilms tumor - a malignant tumor of the kidney that affects children; also called a nephroblastoma ultrasound - a painless test that uses sound waves to create pictures of organs and other structures inside the body. Frequently used to determine whether a mass in the abdomen is fluid-filled or solid. unfavorable histology - describing a microscopic appearance associated with a lesser chance of cure ureter - a tube connecting the filters in the kidney to the bladder vincristine - a cytotoxic drug used to treat Wilms tumor, usually in combination with other drugs

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JOINING THE KIDNEY CANCER ASSOCIATION We hope this booklet has answered some of your questions about Wilms tumor. You can obtain additional information about the disease from a variety of publications and organizations, some of which have been mentioned here. In addition to information, you will need the support of family members, friends, and the medical community in your battle against Wilms tumor. The Kidney Cancer Association is a patient and physician-supported organization that provides information, sponsors research on kidney cancer, and acts as an advocate on behalf of patients. We are here to help. Join the KCA and get immediate benefits: = Physician referrals = The Association’s quarterly newsletter, Kidney Cancer News = Contact with other Wilms tumor families

Donations are tax deductible. KCA has been recognized by the U.S. Internal Revenue Service as a tax-exempt charitable organization. IRS number EIN 36-3719712. To join, or to contact us for additional information: Kidney Cancer Association 1234 Sherman Avenue, Suite 203 Evanston, IL 60202 Telephone: (847) 332-1051 (800) 850-9132 e-mail: office@kidneycancer.org Web site:www.kidneycancer.org

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Profile for Kidney Cancer Association

Wilms Tumor: What Now?  

Published by the Kidney Cancer Association, the world's oldest and most respected kidney cancer charity, this book is a guide for the parent...

Wilms Tumor: What Now?  

Published by the Kidney Cancer Association, the world's oldest and most respected kidney cancer charity, this book is a guide for the parent...

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