Childhood Cancer

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Childhood Cancer Survivors

The brain

The brain is the body’s main information processing center. This complex organ weighs approximately 3 pounds and is protected by membranes called meninges, a cushion of fluid, and the skull. The three main structural parts of the brain are the brain stem, the cerebellum, and the cerebrum. The brain stem connects the brain to the spinal cord. It coordinates most of the functions necessary for survival, such as breathing, heart rate, and sleep. The cerebellum controls muscles to allow smooth and coordinated movement. It also monitors posture and balance. The cerebrum controls all voluntary, or conscious, activities of the brain, including speech, language, hearing, memory, and learning.

Organ damage

The brain can be damaged by tumor growth or treatments such as radiation, surgery, and chemotherapy. The survivors most at risk for brain damage are children and teens treated for leukemia, brain tumors, and tumors of the head and neck such as rhabdomyosarcoma.

The tissues of the brain are very sensitive to radiation. The dose and location of radiation and the child’s age, sex, and individual vulnerability all play a role in how much the radiation will affect brain function. Those at highest risk are children under the age of 2. Children under the age of 5 are at very high risk, and children ages 5 to 8 are at high risk. Girls show a greater sensitivity to radiation than do boys. However, any child whose brain is irradiated may develop long-term changes in brain function. Because the damage to healthy cells depends in part on the total dose of radiation, this discussion will first deal with children who received less than 2500 centigray (cGy) of whole brain radiation.

Radiation doses below 2500 cGy

Most children who are irradiated to avoid the spread of leukemia receive either 1800 cGy or 2400 cGy to the whole brain. Children who have stem cell transplants may also receive radiation to the brain. Depending on the year children received transplants, they most commonly received 1000 to 1200 cGy of total body irradiation (TBI), usually the smaller dose.

Children who received 1200 to 1800 cGy often develop learning difficulties that may be subtle. The changes are more pronounced in those who received 2400 cGy. Greater doses of chemotherapy into the brain from intrathecal medications or high-dose systemic methotrexate may increase the effects from the radiation. Very young children (younger than 5 and particularly those younger than 2) whose brains are growing and developing are more at risk than are older children and teens. 1 , 2 Changes in the ways children or teens think, remember, and learn are called cognitive late effects.

My daughter has problems absorbing new concepts, especially math ideas. For example, we were doing multiplication—the factors of 12 (2 lots of 6, 6 lots of 2, 3 lots of 4, etc.). We used pencils for the lots and after playing with the piles of pencils for a while, I really thought she had got it. She could tell me all the factors of 12, and make the lots from the 12 pencils. Next morning I asked her, and she couldn’t remember any factors of 12! Even with the pencils in front of her.

She has good and bad days. Some days it’s like she is operating through a fog, everything is too difficult. She can’t concentrate. She just wants to be left alone to draw pictures or whatever. Mental effort is too much.

She gets very frustrated (understandable!), and in class there just isn’t the time to go over the same things day after day until she finally gets it. It is an insidious problem, because she is bright and sharp and well behaved in general, and doesn’t look like she should have a learning difficulty.

Learning difficulties usually become evident gradually, beginning a year or two after radiation and continuing to evolve many years after 1800 to 2400 cGy of radiation to the brain. Typically, problems are noted in mathematics, spatial relationships, memory, problem solving, attention span, and concentration skills. These late effects can cause changes in learning style as well as social behavior. It is important that parents and educators remain vigilant for potential learning problems to allow for quick intervention.

My daughter received 1800 rads (cGy) of cranial radiation and intrathecal methotrexate when she was 17 months old. She is now 9 years old and in third grade. She has many learning challenges, including slower processing speed, attentional difficulties, and difficulty with short-term memory and multi-step processing. She benefits from additional help in school. In the past she has needed additional drilling in phonics, math fact repetition, and refocusing on multi-step directions, as well as additional time to complete testing. It is challenging for her to maintain her focus throughout the day.

After much observation, I am convinced that these kids have a quirky organizational system. My husband and I both realize that school is a struggle for her. We have sought help through the special education system, and our daughter is now classified as traumatically brain injured. Her self-esteem is high, and she is a very bright, verbal child with a lot of strengths. We are working diligently with all of her teachers and all of the resources available to ensure that she gets the best education possible.

• • • • •

I had 2400 rads of radiation when I was 15. I don’t think it changed my intellectual functioning at all. I finished high school, college, and attended graduate school. I’m now a physician’s assistant.

The growth of the skull (cranium) of young children who receive 2400 cGy of cranial radiation may slow, leaving the child with a smaller head than he would otherwise have had. The areas on either side of the eyes may develop a slightly pinched look. This late effect develops in approximately 3 out of 10 children. Children who were given 1800 cGy of cranial radiation may also develop this late effect, although to a lesser degree. Children under the age of 5 when treated are most at risk.

Parents of children who had 2400 cGy of cranial radiation, and in some cases as low as 1800 cGy, sometimes report that the child’s affect (i.e., emotions shown on the face) has changed. Rather than a face that reflects what she is thinking and feeling, her face appears expressionless. This can affect making and keeping friends, because facial expressions and other body language are a big part of effective communication. Other parents notice reduced curiosity and interest. Although not much research has been done on these late effects, they are included here because they occur in some survivors and can affect the way those survivors deal with the world.

I asked my son’s therapist if she thought anti-depressives might help because I thought my son was depressed. The therapist evaluated him and said he wasn’t depressed, but his affect was so flat it just seemed that way. She said when a person doesn’t show emotions in their face or tone of voice, whoever is talking to them thinks they don’t feel anything. She told me that they still feel as much, but their ways to express it have been altered.

• • • • •

I’ve never heard anyone talk about this, but my daughter’s personality changed big time after her treatment for relapsed ALL (acute lymphoblastic leukemia) that included radiation. She was always a very exuberant and verbal child. She talked early and was just full of it. She used to laugh and joke all the time. Now she’s quiet and never jokes. I don’t think she understands jokes anymore due to cognitive problems from the radiation. It’s very hard for me. I don’t think she remembers who she used to be, but I do.

Slow processing speeds can impact overall decision making and the ability to make good judgment calls. The amount of information a survivor has available to make decisions may be lessened, because the process of considering options might be slow. Again, this is not a universal late effect, but it affects a significant percentage of survivors who received cranial radiation. 3

My daughter is very vulnerable because she believes whatever anyone tells her. The kids with behavior problems seek her out because she will just go along with whatever mischief they dream up. Even when we discuss it later and she explains that she knows she wasn’t supposed to do those things, she always says, “They really wanted me to go along, so I did.” It’s scary.

Children who received cranial radiation doses of 1800 or 2400 cGy are at risk for problems with hormone production, puberty, and growth. Children who were younger than age 8 when they received radiation are at highest risk. There is also a small risk that the thyroid might not produce enough thyroxin. This risk increases if spinal radiation was also received. It is important to remember that the risk continues throughout life, and in some children, the effects do not appear until a decade or two after treatment has ended. These issues are covered in depth in Chapter 9 .

Children, especially girls, who received radiation to the brain at a young age are at risk for becoming overweight. The exact reason why some children become overweight after radiation is unclear. This is discussed in Chapter 17 .

A rare effect from radiation to the brain that occurs during treatment and may be progressive is leukoencephalopathy. Children who develop this disorder may have lasting problems with balance (ataxia), difficulty swallowing (dysphagia), or speech problems (dysarthria). Other symptoms are seizures, blindness, and coma. Leukoencephalopathy usually occurs in children or teens who relapsed and received cranial radiation plus high total doses of intravenous and/or intrathecal methotrexate.

Radiation doses above 2500 cGy

In the past, many children or teens with brain tumors received 3500 cGy to the whole brain, with a boost of up to 5540 cGy to the tumor bed (i.e., the place where the tumor originated). Others received high-dose radiation only to the tumor itself. Currently, lower doses of radiation are used. Specific disabilities may partly depend on which area of the brain received the highest dose of radiation. However, in general, the higher the dose and the younger the age, the more dramatic the effect on brain functioning.

Higher doses of radiation cause slower brain processing speeds and greater drops in IQ scores. The location of the tumor also influences the type and severity of learning disabilities that may develop. For example, children with temporal lobe tumors may have problems with memory. Learning may also be affected by medications used to treat seizures, or by surgical complications, hydrocephalus, vision problems, and hearing loss.

I’m 28 and was diagnosed with an astrocytoma when I was 15. This brain tumor was surgically removed, and I had radiation treatments for several months to follow. Some of the details are a little fuzzy still. I graduated high school with my class and enrolled in college. I started as a theater/dance major but was unable physically and mentally to keep up with the pace. So I changed my major to science. I graduated in 1994, and you couldn’t get me out of there fast enough! I was experiencing what I now know to be long-term effects. It was very hard keeping up in class. I wasn’t aware until after the fact that I had real symptoms and that there were laws to protect me. I just thought I wasn’t smart enough to excel.

Children or teens with brain tumors who get very high-dose radiation to the brain can have multiple and life-altering late effects. Brain tumor survivors can develop seizure disorders, gait and balance problems, hand/eye coordination problems, personality changes, and learning disabilities. Radiation to the pituitary and hypothalamus can cause problems with growth, puberty, and fertility (see Chapter 9 ). Vision problems, cataracts, and diminished hearing can also develop after radiation (see Chapter 10 ). All of the above late effects from high doses of radiation to the brain can range from mild to severe.

I’m 22 now and had an astrocytoma when I was 10. I had surgery, chemotherapy, and radiation to my head and spine. They ended up removing some of my vertebrae because I had so much tension from scoliosis and kyphosis. I get around in a wheelchair now. The radiation affected my ovaries and I’ll never be able to have kids. I’m also pretty short (4’6”) and skinny, but I try to eat nutritious food. I go to a healing center and exercise to try to keep a strong upper body. One of my legs is pretty bent, so the massage therapist works on that.

With the use of proton beam radiation, there is hope that the risks to healthy brain tissue will be minimized.

Chemotherapy

Chemotherapy used to treat leukemia and some sarcomas can also cause learning disabilities that are sometimes subtle. Intrathecal methotrexate and high-dose methotrexate with leucovorin rescue can cause learning disabilities, though usually much milder than those caused by radiation. 4 Therapy for acute lymphoblastic leukemia sometimes includes triple intrathecals (methotrexate, hydrocortisone, and ARA-C) and has been associated with learning disabilities similar to those seen with lower doses of radiation. Very young children (younger than age 5 and particularly younger than 2) whose brains are growing and developing are more at risk from chemotherapy to the brain than are older children and teens.

My son was on a high-dose methotrexate protocol and developed learning disabilities. He was having problems with concentration, organization, and mathematics. At that time, medical personnel were not aware of the potential for learning problems as a result of treatment. He really didn’t get any help until high school when the learning specialist on the staff tested him. She decided to treat him like a head injury patient. She taught him to concentrate for longer periods of time. His schedule was redesigned so that it was in 15-minute blocks. She taught him numerous tricks like word associations to improve his short-term memory. She was the first person who really got through to him, even though he has had tutors before. She was the first to say, “Yes, you have a problem, and this is what I can do to help.” It worked. He graduated from a well-known university and is doing well in the investment field.

• • • • •

My son was on a high-dose methotrexate protocol from age 3 to 6. He entered preschool while on therapy, and we enrolled him in a private kindergarten because of the small class size. He transitioned to public school in second grade with no problems. He is now 18, an A student who excels in math. He has had no after effects from his years of treatment.

Surgery

Surgery to the brain can cause a host of late effects. The body system and amount of damage depend on the part of the brain that was operated on, the amount of healthy tissue that was removed, and complications after surgery.

My daughter Sarah had two surgeries to the posterior fossa near the brain stem. All side effects from the first surgery resolved. But after the second she had balance and vision problems and she also lost the ability to write with her right hand. She learned to write with her left hand, but her printing in earlier years was beautiful and the messy printing really bothered her. Being left-handed caused some output problems at school because she just wasn’t as quick as before.

As with all the late effects described in this book, these cognitive late effects are not all-or-nothing phenomena. You may have none of these late effects, a few, or many. These lists of possible problems are not meant to fit you into a category, but rather to cover all of the possibilities so that problems, if they develop, can be identified and treated early to give the best possible outcomes.

Signs and symptoms

Brain damage from cranial radiation was first recognized in the late 1970s because survivors were having difficulty in school. Some young survivors were easily distracted and had trouble learning. This spawned many studies of neurocognitive changes from treatment.

Signs and symptoms of damage from radiation and/or chemotherapy include problems with the following:

  • Handwriting

  • Spelling

  • Reading or reading comprehension

  • Understanding math concepts, remembering math facts, comprehending math symbols, sequencing, and working with columns and graphs

  • Remembering and copying shapes

  • Using calculators or computers

  • Learning to ride a bike or tie shoes

  • Auditory or visual language processing: trouble with vocabulary, blending sounds, and syntax

  • Attention deficits: becoming either inattentive, hyperactive, or both

  • Short-term memory and information retrieval

  • Social maturity and social skills

    I had cranial radiation when I was a child and have some problems remembering things. Occasionally I have trouble figuring out in a conversation what point the speaker is trying to make. But I’m an adult now and feel very socially competent. I’ve discovered who I am. So I don’t take these little glitches too personally.

  • Understanding facial expressions or gestures

  • Understanding deceit, cunning, or manipulation

  • Planning and organizational skills

  • Showing emotions on the face

Cognitive problems usually develop within a year or two of radiation and progress over time. So if your child used to color within the lines and draw proportional figures but gradually loses these abilities, the radiation and/or chemotherapy are probably the culprits. The effect on individual children is quite variable. Some children have no late effects, some develop very subtle disabilities, and others develop life-altering problems.

My daughter had 1800 cGy of radiation when she was 3 years old. During preschool and kindergarten, she had the most beautiful handwriting. She was also a great artist and could draw using perspective at an early age. By the time she was 7, however, her handwriting started to deteriorate and she had trouble writing in a straight line—her sentences tending to slope up. She reverted to drawing stick figures. She started pressing really hard on the paper when drawing or writing, and the process became very laborious. She now mostly uses a word processor for writing projects, and we are negotiating with the school for her to use a laptop (they call it assistive technology) in school. She has problems with math and has social difficulties because she can’t really follow conversations well. Her neuropsychological scores range from the 5th to the 95th percentile.

• • • • •

My daughter had 1800 rads of cranial radiation when she was almost 5 years old. The possible side effects listed on the consent form included a drop in IQ. We were concerned, but our doctor said, “There are no options now; if the late effects develop, you’ll deal with them then.” Three years later, she was tested for the gifted and talented program in our school district. I bawled when the letter came in asking for permission to place her in the gifted school. Clutching the letter in my hand, I drove to the clinic to hug the doctor one more time.

You should suspect learning difficulties if any of the following learning changes occur:

  • Your child was an A student prior to cancer, and she is now working just as hard and getting Cs.

  • Your child takes 3 hours to do homework that used to take 1 hour.

  • Your child reads a story and then has trouble explaining the plot.

  • Your child frequently comes home frustrated from school, saying he just doesn’t understand things as well as the other kids.

  • Your child’s teacher complains that she “just doesn’t pay attention” or “just needs to work harder.”

  • Your child says he doesn’t like school.

If any of the above situations are occurring, take action to begin the evaluation process before your child’s self-esteem plummets. It is often hard to take this first step because some children affected by radiation and/or chemotherapy can often reason well and think clearly and may be above average academically in several areas. They may fall behind their classmates, however, on tasks that require fast processing skills, short-term memory, sequential operations, and organizational ability (especially visual). Once identified, these differences can be addressed in school through extra help with memory enhancement, eliminating timed tests, improving organizational skills, and providing extra help in mathematics, spelling, reading, writing, and speech. Early intervention can make a huge difference.

When my daughter stopped reading aloud in second grade, we thought it was because her younger sister had surpassed her in reading ability. But the neuropsychologist told us that it was because she couldn’t hold that many balls in the air at once. Reading aloud requires juggling several skills at the same time: read the words, hold them in your brain, form the words, use your mouth. She couldn’t do that many things simultaneously. However, silently, she can read and comprehend at the fifth grade level. When we got the report back, I showed her and told her it was really good news. She said, “Why?” And I said, “Because you don’t have to read aloud. The majority of the time, adults read silently, and you’re great at that. So just forget reading aloud. You don’t have to do it anymore.” She got a big smile on her face.

• • • • •

I was diagnosed with leukemia in 1976 when I was 5 years old. I had 2400 rads of cranial radiation and while still in grade school was told I had a perceptually impaired learning disability. I had difficulty copying from the board. I have trouble following a line straight across and then going to the next line down. When I read, I use a piece of paper under each line, then move to the next. On the board, it just all got jumbled up in my mind.

These things were a big problem in grade school, but less and less as I continued my education. I now have a master’s degree in social work. It was a big help learning about my legal rights. I took my SATs timed and only completed half of the items. When I took the GREs (tests to enter graduate school) untimed, it was so much better. They allotted me 4 hours, and I only took 3. I was relaxed, not worried about the time, and thus able to concentrate more. I did quite well. I am still a poor speller, but that’s what spell check is for! I also get words mixed up, for instance, recently I wrote “not for prophet” instead of “not for profit.”

It is also important to remember that higher cognitive functioning often remains intact; it is just getting the information in (“processing”) that is impaired. Children who were gifted usually remain so; children with average abilities retain them. Their performance may be slower; they may require extra instruction in memory enhancement and organizational skills, but they can still achieve to their potential. There are thousands of survivors in their late teens and 20s who are successfully attending high school or college, or are pursuing professional careers.

For me, the most difficult part of each semester of graduate school has been exam time. The normal stresses of the competitive academic program are compounded by my feelings of being different and even some ostracism for getting certain testing accommodations that aren’t available to other students. A few students have thought that I get an unfair advantage or even questioned my acceptance into the program if I can’t keep up with the rigors of time-pressured tests and crowded testing environments. At times, all my emotions about having cancer resurface when I have had to talk about my learning disability with other students. I have opted to take advantage of confidential services as much as possible; however, some students notice that I’m not in the same exam room with them. I feel like I have lost some friends after telling them about my learning disability, yet those difficult experiences make me truly grateful for the friends who have understood.

Addressing these issues with the schools can be tough because these disabilities are very different from those the schools are most familiar with. It usually takes a lot of time and effort to get the best and most appropriate education for survivors with cognitive problems. Older survivors need to learn how to advocate for themselves when they go to college or enter the workforce. These issues are covered in detail in Chapter 4 .

My Katie (then 2½) received radiation two times a day for 3 days in 1993 prior to a bone marrow transplant for juvenile chronic myelogenous leukemia. My husband and I were told that as a result, Katie would be sterile, be forever at risk for secondary cancers, lose some IQ, develop cataracts, not reach her original expected height, and have a whole host of shorter-term problems.

I remember sitting in the waiting room thinking, they’re zapping away my baby’s babies. They’re zapping away her laughter, her insight, her joy, her very being.

That was 6 years ago, and today Katie is alive and my joy. What did the radiation do, at least what I can tell right now? She has problems with reading and with math. She sometimes has problems making logical jumps from one thought to another. For reading and math, we work with the school and have her on an intensive program. So far, she is reading at grade level and is slightly below grade level for math.

She is shorter than her classmates and has had the cataract in her right eye removed. We live with these late effects, and to tell you the truth, they’re not all that bad. At least we knew about them, were prepared, and knew how to approach the school system. I really feel for parents of learning-disabled children who struggle for years before they find there is help available.

But all this long gobbledygook aside, I want to let you know what radiation didn’t do. It didn’t take the soul and spirit of my child. She is a lovely, lively, happy child. I don’t know if she’ll ever work in a research lab, but she certainly has all the expectations for a full life just like the majority of her peers. She tells me she wants to be a vet who specializes in horses. I have no reason to think that can’t happen. She is inquisitive, humorous, affectionate, and very social. I think she’ll make a great vet. She might need some extra help in chemistry classes, but heck, so did I.

Additional signs and symptoms associated with radiation to the brain (for brain tumors, relapsed leukemia, or bone marrow transplant following relapse) are:

  • Problems with balance and coordination.

    The damage from Matt’s brain tumor affected his balance, coordination, and walking. By the time he was 3, he had outgrown the double stroller we used to tote him and all of his medical gear in. We went right to a pediatric wheelchair, even though he is a part-time user. It is great for festivals, a walk outside when the neighborhood kids want to push, travel by bus to/from school, etc. I can’t even tell you how the steering and maneuvering compares to a stroller! He uses his walker or a forearm cane for short distance walking. We do use a wagon outdoors under direct supervision, which he loves, and an occasional push on a trike. We’re trying to find a tricycle that he can pedal.

  • Impaired growth.

  • Altered fertility with higher doses of radiation.

  • Early or delayed puberty.

  • Problems making and keeping friends.

  • Second cancers.

My son is 8 years old and has been a medulloblastoma survivor for half his life. He has many late effects from the chemo, radiation, and surgery: hearing aids, glasses, Pullups ® at night, inability to walk alone, very poor balance, and slow speech. He lost all abilities after surgery. He was unable to walk, move purposefully, or eat for several months. He is working hard and making slow but steady progress. He told me one day that he just wanted to run like the other kids. I get teary-eyed hearing that. I am so very grateful for the technology that has saved his life, but he doesn’t understand what happened. I remember a lot of events and dates now as “before the tumor” or “after the tumor.” He is such a trouper, though. He does not see himself as handicapped. He is usually a happy guy, always cracking a joke, and seldom complains.

Children who had radiation to the head may also experience permanent hair loss or thinning hair, dental problems, hearing loss, and cataracts. These late effects and the ones listed above are covered in other chapters in this book.

I had neuroblastoma when I was 9 months old and leukemia when I was 3 years old, more than 30 years ago. I’ve had surgery, radiation to the flank and cranium (I don’t know the doses), and chemotherapy. I’m more than a foot shorter than my brothers, have had glasses since I was 3, have cataracts in both eyes, and am infertile. My hair is thin all over my head and my muscle tone is weak. My baby molars didn’t fall out until I was 30. I’ve paid a big price for my survival, but I’ve also had a pretty good life. My emotional well-being is good, and I’ve achieved many of my goals.

Seizure disorders are another lasting effect that can develop in the brain after surgery, radiation, or chemotherapy. They occur most commonly during treatment, although they sometimes begin many years after therapy. Seizures are caused by electrical disruptions in the brain. There are many types, ranging from mild partial seizures, in which the child does not lose consciousness, to generalized seizures involving convulsions and loss of consciousness. Signs and symptoms of seizures include the following:

  • Staring into space

  • Not hearing people talking

  • Glassy eyes

  • Auras (i.e., an abnormal smell, taste, abdominal sensation, or emotion that precedes a seizure)

  • Stiff body

  • Smacking lips and mumbled words

  • Convulsions

  • Jerking or twitching in parts of the body

Ways to screen for and treat all of the above late effects are covered in the next part of this chapter.

Screening and detection

Any child at risk for cognitive problems should have neuropsychological testing done as soon as possible after diagnosis. This can happen after treatment starts, when the child starts feeling better, or after treatment ends. The first test is called a baseline.

Neuropsychological tests are done by Ph.D. psychologists who specialize in evaluating how children learn and think. These tests usually take 4 to 6 hours, and may be done over 2 days for younger children or those who are easily fatigued. All of that time is spent with the child, and the parents are interviewed separately. The psychologist gives a series of general tests appropriate for the child’s age level, and then another series of more and more specific subtests based on the results of the general ones. Pediatric psychologists usually make the testing fun for children, and children need to know this ahead of time so they don’t worry.

The baseline testing is used as a yardstick to measure future changes in brain functioning. Many institutions do baseline tests, then repeat them every 2 to 3 years until adulthood. Parents and older survivors use the information from these tests to advocate for the most appropriate education. Getting and paying for neuropsychological tests and advocating for the best education are covered in Chapter 4 .

I was treated in the 1970s and had cranial radiation and lots of intrathecal methotrexate. I have difficulty remembering how to spell, and there is a lot of information I simply don’t seem to be able to retain, like addition and multiplication facts. I also have some other memory problems like forgetting directions to familiar places. I forget what exit to take and frequently get lost. I keep notes in my glove box with written directions to help me find my way around. I didn’t initially realize it was treatment-related until another survivor friend and I were driving around one day. She had 2400 rads of cranial radiation when she was 2. And she was making the same mistakes navigating as I do. And she got just as stressed about it. She and I are a bad combination in a car!

Survivors at risk for long-term effects from treatment to the brain need extensive, periodic evaluations throughout their lives. These should include an educational analysis every year while in school, yearly dental exams, yearly evaluations of puberty and growth, yearly eye and hearing examinations, education about second cancers, and a discussion about any problems that have developed. See the tables at the end of Chapter 6 , for lists of recommended follow-up tests based on specific treatments.

Our son, now age 10½, was recently diagnosed with what is called a nonverbal learning disability. It is amazing since he is the best speller, best reader, and best at math in his class. We found out because we are in a long-term effects study at Stanford. He went through a week of tests and when we had the conference, I was shocked by their findings because he has always done so well in school. He is even in the gifted program.

His particular condition will usually show up in junior high or high school when they do more conceptual thinking. After speaking to the bone marrow transplant (BMT) department last week, they confirmed that this is a common side effect of all of the spinal chemo he had (he relapsed in his CNS twice) and the radiation related to the BMT. The psychiatrists were very supportive and gave lots of information for the school district and teachers. It was still a shock.

Medical management

Because treatment that affects the brain can cause a wide constellation of medical late effects, medical management includes a thorough evaluation and referral to appropriate specialists. An important component of medical management is a clear discussion of the risks for specific late effects to the brain and nerves. These should occur at each follow-up visit, as some of the late effects do not arise until years after treatment and are, in some cases, progressive.

My daughter Melissa was diagnosed with low-risk ALL when she was 3 years old. She relapsed in the CNS 18 months into treatment. She was then given 2400 rads (cGy) of cranial radiation as well as spinal radiation. Her CNS problems really started when she had a seizure that lasted 90 minutes. We have used every seizure drug available, with limited success. They are partial complex with some generalization. She tends to get them at night when she’s tired. We need to see the neurologist frequently.

Medical care should include referrals not only to medical specialists, but also to professionals who can help address any psychological, social, or educational issues that arise. Many institutions have educational liaisons who help parents and survivors understand the laws governing appropriate education. Sometimes these specialists travel to the school to attend Individualized Education Program (IEP) meetings. Some institutions have transition specialists who work with survivors as they shift from pediatric medical care to adult care. These specialists can also help survivors with educational and vocational planning.

In the early 1970s, at age 3, I had 2400 rads of cranial radiation. I always had to struggle and work harder in school than anyone else to get good grades. When I took the LSATs to get into law school, I was very disappointed in my performance. I went back to the hospital where I was treated and was stunned to discover that my learning difficulties were caused by my treatment for leukemia. I had my first educational assessment and it showed strong aptitudes in vocabulary, comprehension, and verbal reasoning. But I had poor short-term sequential memory and processing speeds. I hired an educational consultant, worked on my areas of weakness, and applied to take the LSATs under untimed conditions. My scores were much improved, and I will graduate from law school this year.

Recent research has examined the role of cognitive remediation in helping survivors overcome learning problems caused by treatment. This therapy teaches methods to improve memory, attention, and math skills. Children learn strategies that help them keep on task and lessen attention drift. They also learn ways to organize both their thoughts and work habits and practice ways to retain information.

Although children and teens may develop attention problems after treatment, these are not the type usually diagnosed as attention deficit hyperactivity disorder (ADHD). Some researchers, however, are using medications effective for that disorder to treat survivors with attention problems, and these children are showing improved attentional skills. Parents and medical professionals need to do a careful risk/benefit analysis for each child to determine if using these medications is appropriate.

Medical management of seizure disorders starts with a thorough evaluation from a pediatric or adult neurologist. Many medications are available to treat seizure disorders, and sometimes the survivor will need trials with different drugs to find out which ones control the seizures the best with the least number of side effects. Parents should ask the treating physician about added effects from these medications on the child’s already impaired thought processes.

If these methods do not help and the seizures interfere with daily life, surgery may be recommended. An excellent resource for understanding seizures and treatment options is Seizures and Epilepsy in Childhood: A Guide for Parents , Third Edition , by John Freeman, M.D.; Eileen Vining, M.D.; and Diana Pillas.

My son has partial complex seizures. At the beginning, he had occasional staring spells. We then witnessed a big seizure, and he was immediately put on the first of many medications. We have tried every medicine known to man as single therapy. Last year we tried double therapy, but it didn’t eliminate the seizures. Depending on which medication he is on, the seizures have been shorter or longer, milder or more frequent, but never eliminated. The emotional behavior side effects from the drugs have been hard to cope with. He gets very argumentative and has trouble controlling his impulses. Most seizure meds also make him sleepy, which causes more problems with school. He was on a continuous EEG (electroencephalography) for several days at a hospital to evaluate whether he was a candidate for surgery, and he was not.

Some clinics have support groups for long-term survivors where they can share experiences with their peers. Some follow-up clinics link survivors going off to college or into the workplace with mentors who are several years ahead of them in the process. Mentors can provide a lifeline of advice, support, and friendship. Medical management should address all aspects of life: social, educational, vocational, and medical.

Because no one can know it all, survivors, their families, and their physicians must become lifelong learners about the newest research and treatments. Although this is a challenge, it also provides hope that some late effects that cannot be treated today may be able to be addressed in the future.

Chapter 8. Brain and NervesNerves

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