The human skeleton contains 206 bones, all held in place by connective tissues such as ligaments and tendons. The skeleton gives structure to the body and protects the internal organs. Bones determine our size and shape. The skeleton also works as a factory, making various blood cells in the marrow of the bones. Bones also store minerals such as calcium and phosphorus for use by the body.
The structure of bones changes as children grow. The skeleton of the fetus in the womb is made up mostly of cartilage. As pregnancy continues, bone develops, but even when the child is born, there are still areas that are a combination of bone and cartilage. At the end of long bones, such as those in the arms and legs, there are growth plates. Growth plates have a high level of activity until the child stops growing.
Survivors of childhood cancer can develop a number of complications that involve the skeleton. These can be caused by surgery, radiation, and/or chemotherapy.
If a malignant tumor extends to vital structures such as major nerves and blood vessels, then amputation of a limb may be necessary. Survivors who had only the lower parts of extremities (below the knee) amputated usually function well after some rehabilitation. Generally, amputees can ski, run, hike, and perform other physical skills very well. These survivors may experience phantom pain in the missing limb and develop problems with calluses or pain in the stump. Those who had entire limbs removed or other portions of the skeleton (such as the pelvis) removed sometimes have ongoing problems with function and pain.
I had osteosarcoma when I was 8 years old. I had a hip disarticulation (amputation of the entire leg), then chemotherapy. Because it’s sometimes uncomfortable to stand with the proper posture, I tend to slouch. This pulls my hip down and curves my spine into an S shape. It also pulls on my ribs and they draw closer together, causing pain and shortness of breath. It’s hard to sleep sometimes. This is a chronic problem for me. Some weeks are good and some weeks are really hard.
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When I had to have my leg removed when I was 16 because of osteosarcoma, I thought my life was over. I knew that I would never be able to do the things I wanted to do—especially ski. Boy, was I wrong. While I was still on chemo, one of the doctors and a couple of nurses took a group of us skiing. I went along, but figured I would never be able to do it. Well, I was wrong again. They taught us how to ski on one ski with special poles to help us balance. That trip was so important to me. I went to college, met the man of my dreams, and got married. We have two little girls, and I am very happy.
The Amputee Coalition of America (ACA) at www.amputee-coalition.org provides extensive information about organizations and resources for amputees.
Growing numbers of survivors have bone and/or joint reconstructions or rotations instead of amputation. In these procedures, bones are removed or realigned. In many cases these surgeries are very successful, and the survivor’s limb works very well. Other survivors require multiple surgeries and cope with pain, infection, and functional limitations.
I was on a national championship cheerleading team when I was diagnosed with cancer. I had an allograft and a knee replacement done when I was 16, then had chemotherapy. I do have limitations on my activity now—the knee doesn’t bend all the way and I can’t kneel or sit cross-legged. I fall occasionally. I baby that plastic knee. I want to take care of it so it won’t need to be replaced anytime soon.
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My daughter was diagnosed with osteosarcoma at age 12. They replaced 8 inches of her femur with a titanium rod and gave her an artificial knee. She ignored advice to limit her physical activity. She went right back to cheerleading and jazz dancing. She is 19 now, has full range of motion and great strength. She is on her college diving team and practices 3 hours every day. Her goal is to get into the NCAA finals so she can invite her surgeon to come and watch.
Radiation to a limb will impact growth if a child has not completed puberty. If the growth plate is involved in the field of radiation, the untreated limb will continue to grow, creating a length discrepancy in the child’s limbs. This can be particularly problematic if the affected limb is a leg. The growth plate of the untreated leg may need to be surgically altered to stop growth to help keep the legs similar lengths. In some cases, leg-lengthening procedures are performed on the treated leg. However, this is a more involved process.
Survivors who had radiation to a limb may be at risk for fractures without trauma, and fracture may occur after minor trauma that would otherwise not cause a fracture in an untreated bone. Fractures occur most often in children who received more than 4000 cGy to the bone. 1
My son was diagnosed with Ewing’s in his left distal femur at age 11. He had chemotherapy from February to April of 1994. They removed the tumor and 6 inches of his femur in May.
They replaced the femur with donor bone, but neither end grafted. The donor bone broke several times, and the surgeons tried several creative methods to fix it. However, they ended up just putting a rod in. The growth plate was damaged in the surgeries, so that leg stopped growing. He was on crutches much longer than any doctor had hinted at, which got very depressing and upsetting for him.
Since then, he has had to have surgery once to shorten his “unaffected” leg. He still has a lot of bone pain, and a limp that gets more pronounced each day. He dislikes walking because it causes him discomfort, and seldom attempts to run. All in all, though, I think he is thankful that he got to keep his leg.
Some children and teens with cancer get their entire spines irradiated. Others have a portion of the spine treated. Scoliosis, a sideways curvature of the spine, is caused by radiation to only one side of the spine (down the midline of the body). When the untreated part continues to grow, the spine curves like a bow. This was a common side effect of children treated in the 1970s for Wilms tumor, neuroblastoma, or rhabdomyosarcoma. However, spinal radiation delivered since the 1980s has included the entire spine in the field of radiation, as well as the side of the body affected by the tumor, thereby decreasing the risk or degree of scoliosis. These children have a shorter trunk, but better spinal alignment. The damage is more pronounced for children treated when younger than age 6 or during the growth spurt of puberty. A survivor whose whole spine was radiated also may develop scoliosis; this is thought to occur because damage to the muscles and soft tissue on the irradiated side of the body pulls the spine out of alignment.
My 9-year-old daughter Terri had a laminectomy to allow space for the tumor, which takes up more than the width of a normal spinal cord. I think the removal of the vertebrae contributes somewhat to the scoliosis. The other causative factors could be radiotherapy to the cervical/thoracic area and paralysis in her left arm.
Terri cannot bend her left arm at the elbow or raise it up from the shoulder, although she does still have movement in her wrist and fingers; so the arm does get some exercise but never anything too weighty. Her spine seems to be gradually curving more and more as time goes on, probably due to the heavy usage of the right arm. Her muscles in her right arm are almost as big as mine.
Other factors that may increase the risk of developing scoliosis are changes to the spine from tumor, osteoporosis, and fusion of parts of the spine.
A rare side effect of radiation to portions of the spine is kyphosis, where the upper spine curves outward, giving a hunchback appearance. This can occur with scoliosis after radiation to portions of the spine. Severe kyphosis and/or scoliosis can affect the functioning of other organs, such as lung volume.
I had high-dose mantle radiation in 1972. I developed kyphosis, which has become much more noticeable in the last 10 years. It feels like I have thrown out my back. I also have fibromyalgia all along my spine. The muscles and tissue in my upper back and neck are atrophied, making it hard for me to stand up straight. When I was at the doctor’s recently, he called in a medical student and said, “Look at this woman’s back. This is the reason we don’t give high doses of radiation to kids anymore.”
If radiation to the whole spine is needed, current protocols attempt to spare adjacent tissues to decrease the risk of scoliosis and kyphosis. Better staging of solid tumors has also decreased the number of children and teens who require radiation.
Radiation to the whole spine can stop or slow the growth of the spine. A short trunk (measured from the top of the head to the rump) occurs most often in brain tumor survivors whose entire spines were radiated with more than 3500 cGy. Total body radiation given prior to stem cell transplantation (i.e., bone marrow, stem cell, or cord blood) can also affect the growth of the spine, as well as growth of other bones exposed to radiation.
My daughter was born with neuroblastoma in 1991 and relapsed 3 months later. The second tumor was very large and sat on top of her kidney. After the big chemotherapy blitz, the tumor stopped growing. But in the several years it was in there, it eroded two of her vertebrae. She had to have part of her spine rebuilt. The section of the spine the surgeon worked on was fused and is supported by metal rods. It’s inflexible and doesn’t grow. From her mid-back to her waist is rigid, but she can still bend over at the hips.
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Terri had spinal surgery and spinal radiation in her thoracic and cervical regions. She is still growing taller and at a reasonable rate, although she is well and truly below the lowest percentile for her age group. We’ve been very lucky that this hasn’t really affected her too much.
Radiation to any bones can cause slowed or stopped growth, depending on the age of the child and the dose of radiation. For instance, radiation to the orbits of the eyes in survivors who had retinoblastoma or rhabdomyosarcoma usually stops bone growth of the treated orbit. Hodgkin lymphoma survivors who had high-dose radiation often have underdeveloped ribs, collarbones, shoulders, and pelvises.
I was 13 years old when I was diagnosed with Hodgkin’s disease stage IIIA. I was treated with 9 weeks of radiation, no chemotherapy. I was a pretty big kid then—5’5” tall and 108 pounds. I only grew another inch after treatment, so I’m much shorter than I would have been (my dad’s 6 feet tall). All of the bones that received radiation stopped growing—my ribs, spine, collarbones. My chest and shoulders are the same size as they were when I was 13.
Exostoses (also called osteochondromas) are outgrowths of the bone that are sometimes seen in children who were treated with radiation. Young children who receive total body irradiation (TBI) can develop osteochondromas as they begin puberty. 2 An x-ray is needed to confirm the diagnosis. These growths often do not require intervention. However, in a small number of cases, removal is necessary due to location and discomfort.
Slipped capital femoral epiphysis (SCFES) is displacement of the capital femoral epiphysis and is associated with radiation to the femoral head. It is often seen at the beginning of the pubertal growth spurt. New evidence is beginning to emerge that children who receive TBI and growth hormone replacement may be at even higher risk of SCFES. If a survivor whose hip was irradiated has hip pain, immediate attention is required. 3
Some childhood cancer survivors experience a thinning of their bones (low bone density). This makes it more likely that they will break bones or develop osteoporosis, a disease of the skeleton that results from too little new bone formation or too much loss of bone tissue. This late effect is not well understood. It is known that survivors who took high doses of steroids (e.g., prednisone, dexamethasone), received high-dose methotrexate, had cranial radiation, had high-dose radiation to bones, or have low growth hormone seem to be most at risk. Young women who have an early menopause and men with low testosterone production also have a higher risk of developing osteoporosis.
My daughter Melissa developed severe osteoporosis during her treatment. The process might have accelerated because she was in bed for almost a year, doing no weight-bearing exercise, and taking steroids. When she came home from the hospital, she seemed to be getting a bit better so we let her ride her bike. She fell and started screaming. It turns out she had seven fractured vertebrae. She was given pain medications and was on an IV (intravenous) drug in the hospital 3 days a month to treat the osteoporosis. Now she’s on Fosamax ® and her bone density has improved. She’s playing soccer this year.
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Coley was treated from ages 2½ to 3 with carboplatin, vincristine, and 5-fluorouracil, and daily injections of GCSF [granulocyte colony-stimulating factor]. When she broke her leg two times, they said the chemotherapy more than likely made her bones brittle.
Taylor hurt his ankle from a jump off a fence. We thought it was a sprain, but they x-rayed it and they found he had bad osteoporosis. His nurse said, “He has bones like Swiss cheese.” His case nurse and I asked the doctors to start regular bone density scans to see if it improves. If it doesn’t, maybe they can help with some drugs. So now he will get yearly bone density scans.
Osteonecrosis (ON) is a condition caused by the death of the small blood vessels that nourish the bones. Other names for this condition are avascular necrosis (AVN) and ischemic necrosis. It is usually caused by radiation to bones and/or use of high-dose steroids (e.g., prednisone or dexamethasone).
I was an athlete who played three sports prior to my AML (acute myelogenous leukemia) diagnosis and bone marrow transplant. The steroids saved my life but destroyed my bones. I have had surgery on my shoulders, hips, and knee joints. My shoulders and hips are much better, but my knees are still painful, and I have trouble getting around. The surgeon is hoping that soon he will be able to use live bone in experimental surgery to help me.
Osteonecrosis is generally seen early rather than late—often within the first year off therapy. Adolescent girls are especially susceptible. Magnetic resonance imaging (MRI) is the best tool for diagnosis. As bone deterioration progresses, the bone may become weak and may eventually collapse. The course of the disease is variable. Some survivors have osteonecrosis for years with only minor problems with pain or movement, while others require surgery soon after diagnosis. Osteonecrosis can be very painful and sometimes leads to osteoarthritis. The website for the ON/AVN Support Group International is at www.avnsupport.org .
My daughter had leukemia and then relapsed. She has seven collapsed vertebrae in her back and a hip joint that has avascular necrosis. They think these problems were caused by steroids and having her nutrition compromised for so long when she was hospitalized. She’s in a lot of pain, so she takes pain medication daily. We give her extra calcium and encourage exercise. She played soccer last year. We’ve taught her to ride a bike, and we hike. She also takes Fosamax ® . She went to physical therapy for a long time and has a good range of motion and can even bend over. But when she gets tired, she limps.
My 13-year-old daughter Lisa had a BMT to cure her AML. She required huge doses of steroids for over 8 months to treat graft-versus-host disease. She started having knee pain when the steroids first began, but we had no choice but to continue with them. The avascular necrosis was confirmed soon after she was weaned off the steroids. Both femurs and tibias at the knee joint are affected, and her femurs also have what appear to be hairline fractures in them, although they don’t cause her any pain now. Her knees swell periodically, and we ice them and wrap them in Ace ® bandages. It’s painful for her to walk, but she still tried out for and made the basketball team, although she could only play for a few minutes per game because of the pain. Things got worse and she needed to use a wheelchair for a few months until the pain settled down. Now she’s walking again, but with the elastic bandages. It’s a hard thing because she was a great athlete before. She lettered in track and was the second highest scorer in basketball. Now she can’t run, jump, do PE. They did an MRI recently, and it hasn’t worsened and hasn’t improved.
Osteoarthritis is a type of degenerative joint disease. It is characterized by pain with activity that subsides when resting. Survivors who had radiation to joints are at risk. People who have late effects that increase stress on the joints may also develop osteoarthritis. Helpful information about arthritis can be found at the Arthritis Foundation’s website— www.arthritis.org .
I’ve walked on crutches for 20 years because my leg was amputated. I have arthritis in the knee from all those years of pounding on it. I ski with a group of amputees, and one time I sprained my knee. When I went to see the orthopedist, it turned out to be the fellow who had amputated my leg many years earlier. He was thrilled to see me. He gave me knee exercises to do to keep the knee strong. But he also told me he’d need to scope it in a couple of years to see how bad the arthritis is. He warned that I would eventually need a knee replacement. I’m trying to delay that as long as possible, hoping the technology continues to improve. I’d like the first replacement joint to last the rest of my life.
Signs and symptoms of leg length discrepancy include the following:
Signs and symptoms of fractures include the following:
Loss of function
Signs and symptoms of spinal late effects include the following:
Uneven shoulder height
Back or hip pain
Signs and symptoms of osteonecrosis, osteoarthritis, and SCFES include the following:
Pain in affected joint
I was diagnosed with acute lymphoblastic leukemia when I was 11 years old. I relapsed twice, then had a bone marrow transplant in 1988. Then I relapsed after the transplant. I was then put on a very experimental protocol at the National Institutes of Health, which cured me. I was on treatment for most of 14 years, and the majority of that time I was taking steroids.
I have a big problem with osteonecrosis. Both hips went first, but only one hurt enough to treat. I had a surgery done on it called a core decompression. It relieved the pressure and the core grew into the bone. I was only 22 years old and wanted to buy time before I had the joint replaced.
A couple of years later I started having problems with my shoulders, and they rapidly got worse than my hips. I have a high tolerance for pain, so by the time I went in, both shoulders had collapsed. I had the left one replaced and that relieved the pain. The right one stopped hurting so I put off the surgery. I can use it but don’t have full range of motion, and I can’t do any heavy lifting.
Signs and symptoms of osteochondromas include the following:
Hard lump on any boney surface
Possible pain (depending on location)
Osteoporosis usually has no signs or symptoms, and bone density changes might not show up on regular x-rays unless the affected bone gets broken or infected. Advanced osteoporosis can cause the bones in your spine to compress, causing shorter height. It can also cause a hump on the upper part of the back.
Survivors with amputations need an annual examination of the stump that includes a discussion about whether a prosthesis could be useful. Range of motion and function are evaluated. Survivors who had a limb salvage procedure should have both treated and untreated limbs measured every year (without clothes on to get accurate measurements), usually by an orthopedic surgeon. A baseline and then annual x-rays are needed until growth is complete to assess the growth plate. Survivors should also have a discussion with their healthcare provider if they have any back pain, limb pain, limping, or changes in muscle mass.
A scoliosis check needs to be done every year if you are at risk. Your back will be examined while you bend over with your fingers touching your toes and your knees straight.
If your child had radiation to his or her spine, several tests should be done to check for spinal abnormalities. Your child should have height checked while both standing and sitting, and the results should be plotted on a chart. Your child’s healthcare provider should examine your child’s spine every 3 months during puberty until growth is complete, and every year thereafter. A spine x-ray should be obtained for a baseline before puberty, then as needed.
If you had more than 4000 cGy radiation to any bones, they are at risk for fractures. Go to your healthcare provider if you have pain, swelling, or bruising in the areas that were irradiated. You may be at risk if:
You went into an early menopause (periods stop).
You are a stem cell transplant survivor who took high doses of steroids.
You have any ovarian or testicular dysfunction.
You are growth hormone deficient.
You have a family history of osteoporosis.
Discuss your risk with your healthcare provider to see whether you need bone density studies. X-rays or computed tomography (CT) scans may be done to assess the amount of damage.
A painful joint should be evaluated by your healthcare provider. You may need an x-ray or CT scan to check for osteoarthritis and osteonecrosis.
Medical management of amputations includes a visual inspection and checks for range of motion and muscle contractures. You should have your prosthesis checked (if you use one) and have a discussion with your healthcare provider about any advancements in technology.
I wore a prosthesis for years because I was told by my parents, doctors, and physical therapists that I had to wear it. I think they wanted me to fit in with the other kids. But because I had such a high amputation, it was uncomfortable and painful.
Finally, when I was 15, I just refused to wear it and I started using crutches. But I felt like I let them down and felt guilty about it for years.
A doctor I met 10 years later told me if he had a high amputation he wouldn’t wear a prosthesis. No one had ever told me that and it was so wonderful to hear. No one had ever said it was okay to use crutches or whatever was most comfortable for me. It was a relief just to hear one person give me permission to feel comfortable.
If you had limb salvage surgery or radiation and one limb is now longer than the other, discuss the treatment plan with your healthcare provider. Small differences in length of less than 2 centimeters (cm) usually don’t require any treatment. Differences of 2 to 6 cm are treated with a shoe lift or surgery to stop the growth of the other limb. If the discrepancy is greater than 6 cm, other surgical steps may be necessary. These include shortening the untreated limb or lengthening the treated leg to restore a comfortable gait.
If you have an endoprosthesis, you need to take antibiotics prior to dental work to prevent possible infection that could spread to the prosthesis.
If you have any scoliosis or kyphosis, you should be referred to an orthopedic specialist with experience treating survivors of childhood cancer. If the curvature is noted during a period of rapid growth, such as puberty, do not delay seeing the specialist, because the curvature can increase rapidly during a growth spurt. Long-term survivors who have scoliosis or kyphosis often have back pain. The following may help make the pain more manageable:
Using a brace
I have severe pain from my kyphosis, fibromyalgia, and weakened back. I have tried over 15 different pain medications, including anti-depressants. You name it, I’ve tried it. They don’t help much. I went to physical therapy and that helped quite a bit. I also did something called Pilates, which helped make the muscles that I still have a bit stronger.
If the curve in the back progresses (worsens) beyond 30 degrees (or curves 20 degrees with rapid progression), wearing a brace may be necessary. Curves greater than 40 degrees may require surgery. There are no standards for treatment of scoliosis after tumor surgery or radiation; the decision to use a brace or operate is individualized to the survivor’s specific circumstances.
The scoliosis was small, only around 12 to 15 degrees, and not sufficient to treat; however, the kyphosis was considerably larger and ended up being nearly 60 degrees. For that, I wore a Milwaukee brace 23 hours a day and did physical therapy exercises at home for over a year, then continued exercises and wore the brace at night for almost another year. No surgery for it. When I saw our pediatric orthopedist here after more than 10 years as a follow-up dropout, the curve had progressed to 69 degrees, which is more than 10 degrees worse than pre-bracing; however, as that was when I was entering the growth spurt, I feel it was worth it. At this point, it’s not a problem cosmetically, and the pain is manageable with exercise.
If you have osteoporosis, your yearly examination should include information that stresses the importance of:
Weight-bearing exercise such as walking or running.
A diet rich in calcium. This includes dairy products, shellfish, leafy green vegetables, and tofu. Your healthcare provider might also recommend taking supplemental calcium with added vitamin D.
Adequate vitamin D. Some dairy products are fortified with vitamin D and the body makes some on its own when exposed to sunlight, but many survivors require supplementation.
I was told by my gynecologist that I should stay on the calcium tablets, and I was put on a nasal spray to help with the bone loss from the osteoporosis. However, I have severe allergies and the nasal spray gave me too many problems, so I am back to only taking the calcium tablets again. It becomes more of a problem because I am lactose intolerant and can’t have milk or ice cream. I can eat yogurt and frozen yogurt. I tolerate pizza, lasagna, and macaroni and cheese in small portions.
Treatment for osteonecrosis may include:
Range of motion exercises.
Surgery to remove the inner layer of bone (core decompression). Sometimes a piece of healthy bone is put in when the inner layer is removed.
Surgery to remove a wedge of bone (osteotomy) to reduce the weight bearing of the bone with osteonecrosis.
Pain and/or anti-inflammatory medication.
Tim began having problems with his ankles at the end of his year of intense relapse therapy. He would have intense, intermittent pain. When the first MRI was done, it showed necrosis in the ankles, but nowhere else. The next year, the MRI showed necrosis still in the ankles and some involvement in the shins and knees, but none in the hips.
This fall, the orthopedic doctor hoped things might be better after being off the steroids for so long, but in fact, it looks like it may be increasing. The edema has gotten quite a bit worse. So we are watching it. Tim has not had further pain. He is on extra vitamin D and calcium supplements.
The ankle is not a good candidate for joint replacement. If Tim’s necrosis gets worse, they will switch him to braces that include bracing along the front of the leg and, I think, the knee. The idea is to distribute some of the weight to the knee from the ankle. I would have them do MRIs of the knee before we did that.
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My son Ben was diagnosed with acute lymphoblastic leukemia when he was 17. He was given painkillers pretty early in treatment for leg pain and headaches. Then, he was hospitalized for almost a month due to sepsis, and he was encouraged (and needed) to take more. As a caregiver and parent, I was totally unprepared when he became addicted to painkillers. I missed some pretty obvious warning signs and had no idea how serious a problem this can be until Ben nearly died from an overdose. Now I know that many things combined to increase Ben’s likelihood of addiction—a possible genetic predisposition, access provided by legitimate physical pain, the emotional trauma of such a serious diagnosis, and he was at an age when so many people experiment with drugs.
By the time the avascular necrosis (AVN) was diagnosed, we were aware of his addiction and closely monitored the use of the painkillers. He had four surgeries at the end of treatment on his shoulders and hips, so of course had to continue to take painkillers, but with careful monitoring. After his last surgery, he went to residential rehab for a month. This spring, Ben is doing a “grand rounds” on his painkiller addiction at the hospital where he was treated. He’s doing great now, but it was a process.
If you get a fracture in irradiated bone, it most likely will occur where the bone was biopsied or at the site of tumor in the bone. These types of fractures may need surgery to insert a device to keep the bone aligned. Because the surrounding tissues were also irradiated, this presents a surgical challenge. Make sure this surgery is done by an orthopedic surgeon with experience operating on irradiated bone and tissues.
Osteochondromas are generally noted first by the survivor or a family member. Once the diagnosis is confirmed by x-ray, management will be determined by the symptoms. If there is pain or if the osteochrodroma is affecting other bone growth or joint function, a referral to an orthopedist is recommended.
Table of ContentsAll Guides
- 1. Survivorship
- 2. Emotions
- 3. Relationships
- 4. Navigating the System
- 5. Staying Healthy
- 6. Diseases
- 7. Fatigue
- 8. Brain and Nerves
- 9. Hormone-Producing Glands
- 10. Eyes and Ears
- 11. Head and Neck
- 12. Heart and Blood Vessels
- 13. Lungs
- 14. Kidneys, Bladder, and Genitals
- 15. Liver, Stomach, and Intestines
- 16. Immune System
- 17. Muscles and Bones
- 18. Skin, Breasts, and Hair
- 19. Second Cancers
- 20. Homage
- Appendix A. Survivor Sketches
- Appendix B. Resources
- Appendix C. References
- Appendix D. About the Authors
- Appendix E. Childhood Cancer Guides (TM)