Childhood
Cancer:
Practitioner and Family Perspectives
The
Maine Scholar
A Journal of Ideas and Public Affairs
Volume 8, Autumn 1995
Theme
Issue: Childhood
Charles M. Lyons
Gary Allegretta
Marianna Estabrooke
Introduction
Early death is no longer the inevitable outcome of childhood cancer. As progress in the medical treatment of children's cancer continues, the chances for long-term survival and cure improve. Much of this improvement is due to multi-modal treatment programs combining chemotherapy with radiation and surgery. Many children who contract cancer in childhood will survive to be cancer-free adults. One projection suggests that by the year 2000 one out of every one thousand nineteen-year-old people will be a survivor of childhood cancer.1 Many children with cancer now live with an altered identity instead of facing early death. Unfortunately, treatments are often frightening, painful, and disfiguring, and adverse side effects alter the patient's physical and emotional well-being. Enduring the treatment may be justified by improved results, but interventions to ameliorate the emotional trauma of treatment seem warranted now that the medical outlook has improved.
Each state of the disease has distinct psycho-social concerns: the uncertainty of the diagnostic phase, focused purpose of the treatment phase, relief and fear of remission or stabilization, renewed anguish of relapse and deterioration, preparation for death, and post-death adjustment of families. The experiences of individual children and their families within these stages can be expected to vary with individual characteristics of the family unit. Of these stages, the terminal-illness-and-death stage has received the most attention in research, but with the changing prognosis for children with cancer, research into the effects of the treatment phase on children who survive becomes important.
Susan Lewis and Stephen H. Armstrong were among the pioneers in this area who focused on the high degree of anxiety demonstrated by children with potentially fatal diseases. They urged that the emotional needs of these children receive as much attention as their physical illnesses.2 Elisabeth Kubler-Ross addressed the psychological aspects of potentially fatal illness in children and clearly spurred much of the later interest in the topic.3
Despite these efforts to address the topic of the experience of cancer in children, those interested in the lives of children, particularly those working in the helping professions, have little to assist them in under-standing the world of pediatric oncology from the perspective of the family and the primary medical personnel involved in the case. We offer, in this text, a physician's outlook and three such family profiles to assist in the advancement of this knowledge base.
The Practice of Pediatric
Hematology/Oncology
When a patient and family first come to the oncologist's office, the atmosphere is rich with tension. An experienced physician will estimate in one condensed moment of knowledge and intuition how the course of treatment is likely to unfold. The reaction of the child and family to the initial diagnosis is universal and is, in the judgment of both physician and patients in this study, the beginning point of treatment. A good physician will look for fear, address it, and, by telling the truth about the situation, reduce it. For example, a parent commonly wonders if his or her child will die on the day of the first hospital admission. Knowing that parents rarely express that concern, the physician can usually assure them that death is unlikely and promise to tell them if the situation changes. The physician will also be sensitive to the effects of stress upon people, will understand how this new situation creates stress, and can creatively reduce some of that stress while providing the medical care the patient needs. Most importantly, the physician is acutely aware that he/she, the patient, and the family are beginning a long-term relation-ship that must function well to provide optimal care. Each will take time to learn about others on the team and will begin to develop trust. Trust will carry this team through whatever is to come and, for this reason, the physician must endeavor to communicate clearly, always expressing the truth in a way that it can be well understood by all.
A gradual adaptation to the reality of cancer begins. The patient and family learn about the illness and its treatment and experience the first dose of chemotherapy or the first radiation treatment with the anxiety of uncertainty. They begin to see what life on therapy is like and can hope that the planned end of treatment will arrive uneventfully. In time, as medical encounters become commonplace, the family comes to accept the occurrence of cancer. We refer to this period as "maintenance. " It begins after the first one to two months of therapy, termed the "induction" phase, and may last up to two years. Emotions are generally calmer now, and the physician's role narrower. While it is easier to work in this environment, interpersonal skills remain critically important for providing complete and insightful medical care and maintaining trust. The physician must try to listen well to create a mental image of what the patient describes and to understand his or her feelings and desires. In order to foster a sense of normalcy and well-being, control should be given to the patient and family whenever possible. A family routine similar to that which existed prior to the diagnosis should be encouraged. Such stability gives comfort to children, adolescents, and parents.
As the maintenance phase of treatment nears its end, families prepare to enter what all hope will be the final phase of the illness, which takes place after treatment has ended. This period is called "elective discontinuation of therapy." Entrance into this period signifies the physician's best guess that the patient has received enough treatment for cure. Emotions usually surface again, with intensity often as high as at diagnosis. Families feel uncertainty about cure, a loss of security as contact with the medical team lessens, and fear of persistent side effects. The physician must understand this situation and see that the issues receive attention, a task made difficult at times as other patients may require urgent medical interventions, competing for the physician's time. However, satisfaction in a job well done and hope for the future can be shared by all.
All along this path, from diagnosis to treatment to cure, several issues arise for the physician. Foremost, for many, is that of defining and understanding the social boundary between the doctor and patient. The common teaching holds that it is healthy to maintain an emotional distance - "detached concern" - from patients and their families. This position, it is argued, helps the physician make objective decisions uninfluenced by personal feelings. It also helps him or her remain emotionally healthy, avoiding the depression that can arise from over-involvement. The physician in this study prefers a different approach and consciously defines his boundary with each family. This attitude acknowledges that the physician is as human as his patients are, with feelings growing from the powefu1 experience that cancer brings. He is free to accept his feelings as they come, to share to some degree each patient's hopes, fears, sorrows, and joys. Physicians must exercise caution, however, for as they grow more attached to patients, their feelings intensify, and objectivity becomes difficult or impossible. They must then choose either to find an emotional place where they can function competently yet remain personal, or choose a closer relationship, effectively removing themselves from the role of primary caregiver and arranging for another physician to provide medical care while they continue to give emotional support.
Medical science has made great advancements in the treatment of childhood cancer over the past three decades, so that today nearly 70 percent of children who develop a malignancy become cured 4. Yet some forms of cancer remain incurable. Moreover, some children, despite developing a type of cancer for which a cure is common, still die. Presently, there is no way to predict who will not be cured. Prognostic features that permit increased therapy for poorer risk patients (standard vs. high-risk acute lymphoblastic leukemia, for instance) are understood; however, this knowledge is included in the survival statistics. There is a substantial amount of research into the biological behaviors of childhood cancer, and it appears likely that genetic factors vary with individuals as well as with tumor types, accounting for individual differences in aggressiveness of disease 5. Personal imperfection must also be faced and accepted. Even when the physician provides the best care possible, diagnoses can be inaccurate, side effects unanticipated, communication unsatisfactory, and symptom relief incomplete.
Death is something that everyone in oncology must face. People raise the subject early in conversation but tiptoe around it, avoiding talking about death explicitly. They may say, "What is it like for those poor children, with no hope, I mean . . ." or "how do you deal with the sadness every day?" Children can be helped to face death with medicines that relieve suffering; with experience, communication, and teamwork that dispel anxiety; and with a manner that perpetuates hope. In every situation, one can learn, feel, and carry on something to others. This sustains pediatric oncologists more than anyone else could ever know.
Three Families
The Kellys
It was 7:00 P.M. at the Kelly's' home on a Wednesday evening during February school vacation. The house is in a subdivision of new homes, most quite large and luxurious. Several children came down the stairs. They scooted back up except for Kerry, who waited to be introduced. She was wearing a Barney baseball cap and a 1O1-Dalmations T-shirt. She said "hello" with shyness and took off her hat. Her mother had told her that the interviewer was interested in her cancer and that, despite Kerry's preference that her mother not discuss it, she would have to allow it this time. Throughout the interview, Kerry stayed in the room, running back and forth, playing with her hat, and interacting quietly with her parents and the interviewer.
Kerry was three years and nine months old at the time of the interview, the youngest of five children who ranged in age from seven to fifteen years old. Since Mrs. Kelly has not been employed outside the home, Kerry has not been in day care, but she is enrolled in preschool for the coming fall. Mr. Kelly's work requires traveling a great deal, so Mrs. Kelly left her job thirteen years ago and has not worked outside the home since. When the cancer was first diagnosed and Kerry was hospitalized for three weeks, Mr. Kelly took time off from work to stay home and care for the other children. Kerry's oncologist described her parents as a very solid couple, saying, "You would want them on a jury if you wanted a fair
trial."
Her mother described Kerry as a bubbly, happy child, a joy and a pleasure. She said that Kerry changed from a baby to a little girl during her initial hospital stay in the summer of 1994. She said she noticed a difference that has not gone away. "She has grown up too fast. She's not a baby anymore. She went into the hospital a baby and came out all grown up." Kerry seems to have developed normally in every way. She rides a bike, ice skates, and roller skates. Her mother reported that she takes Kerry everywhere: to the store, library, and mall. She does not, however, take her to the other children's schools because of germs. This is the only concession she has made to the fear of illness.
At age two and a hall, Kerry began having low-grade fevers for a period of several weeks. They would go away and then return in a few days. During this time, she scraped her knee, and it didn't seem to be healing. Suspecting an infection, her mother took her to the pediatrician to check out the fevers. The doctor felt it was just a virus and sent her home. A few days later, Kerry awoke from a nap and was unable to balance, bumping into the wall as she walked down the hall. The mother contacted the doctor again and took her to see him. In searching for a cause, a blood test was done which revealed the presence of leukemia. "Before I got in the door from the return trip from the pediatrician, he was calling me to say to prepare for the worst. He thought she had leukemia. He told me to pack a bag and return to the hospital, prepared to stay. We were diagnosed that night, June 21, 1994. The balance problem was a symptom of anemia as a result of the leukemia.
Complications characterized her initial treatment. She could not have a central line initially because her brother's chicken pox made her ineligible for surgery. She had a PICC line (temporary IV) which became infected, so they had to return to the hospital. By then the incubation time for the chicken pox had expired and her physician inserted the central line. Kerry, listening to the conversation, offered to show the interviewer her line. It is a semi-permanent line into the chest allowing for the administration of medication into the blood vessels without needles. A second port allows for blood testing without the use of needles. IVs can be hooked up to that, as well, when she has to be hospitalized. Every week she has blood drawn to determine the dosage for chemotherapy. For a while, the central line did not work, so she had to go into the hospital each week to have blood drawn from her arm. Her father noted that without the line the cure might seem worse than the disease. The complications with it are also significant. A blood clot developed at one point and Kerry had to be hospitalized again to have it dissolved. Since then, she has had to take a blood thinner to prevent further clots. Chemotherapy is administered in many ways: by mouth, through the line, and through injections. Kerry's last injection is scheduled to be given soon after the interview. After that, trips to the physician's office will slow down to once every three weeks. Her mother expressed concern over not having that weekly chance to talk to the staff about her questions. Although they are available by phone, she believes that face-to-face contact is important in feeling cared for and protected.
Kerry's mother reflected on the beginning phase of treatment. After the first three-week induction period, a series of chemotherapy spinal injections were administered. After each one, Kerry's fever would spike and she would have to return for a forty-eight-hour hospital stay to determine if an infection had invaded her body. Further, during the early months of treatment, the central line was not reliable.
The chemotherapy Kerry received did not leave her feeling particularly ill. However, bone pain was not uncommon for her. Her father noted that, in dealing with cancer in children, problems in the treatment process are very complex. Each takes several days to diagnose and rectify. The amount of psychic energy absorbed in the process appears enormous. As Kerry's father stated, "They say that kids are resilient and I think it is true. It is tough on the child, but it is tougher on the parents. She doesn't know that this is different than what other three-year-old children have to do.
Kerry's father mentioned her "diabetic problem." Her mother explained that when Kerry receives a certain steroid, her blood sugars respond poorly and she needs monitoring and sometimes insulin. Now when she administers the steroids she also monitors her blood and gives her insulin, if needed. The blood monitor requires that she do finger pricks to get a sample and then give Kerry some insulin; a few hours later, she repeats the finger prick Kerry will take steroids until treatment is ended in July 1996.
Her mother said that "actually now it doesn't seem that bad," despite the administration of drugs, trips to the doctors, and monitoring Kerry. She stated, "You just fit it into the routine. It becomes part of your routine. You just do what you have to do." She added, "When I was first introduced to this, I felt overwhelmed. I really did. But you gradually get into the swing of things. You just do it."
Kerry's father noted that when the diagnosis was first explained the doctor seemed to be telling them only what they needed to know that moment, thinking they would not be able to absorb much more. Now that the next phase is beginning and the doctor knows them better, Mr. Kelly expects him to tell them more of what will happen next. Mr. Kelly thinks that when the treatment phase is over, they will have to wait two to five years before they know if Kerry is cured.
Kerry's mother said it gets easier. The five days on steroids now 'fly by," whereas before it was hard. "She would be in pain and cranky for the five days, plus her diabetes problem while on the steroids was a big factor."
When Kerry is hospitalized, her mother goes with her. At first that was hard because of the four other children at home and Mr. Kelly's frequent travels. She reported that the two older ones each took one of the younger children and pitched in. The summer vacation was good in that less transportation was required, yet hard because the kids were not occupied with school routines. "I consider the family just about back to normal. I mean, it will never be like it was before, but it's pretty close to it."
Kerry's treatment is expected to continue for another year. After that, assuming a continued state of remission, treatment will end, but monitoring will continue for another five years.
Leah, Barbara, and Grace
Leah, the mother of two-year-old Grace, the patient, was interviewed in her apartment on the upper floor of her parents' home. She had lived there with her daughter for a period of time before moving out when she was married in the fall. She, her husband, and daughter had recently moved back into the apartment. The apartment was neat and comfortable. Grace's room was at the front. It was bright, even on this rainy interview day, and filled with toys. A huge Big Bird sat on a chest, and other characters from "Sesame Street" were scattered about the room.
Over the couch was a picture of Grace taken at her mother's wedding just before Grace became ill from a brain tumor. She was dressed in a fancy, little-girl dress. Her long, red hair and rosy cheeks presented the picture of health. Even during the interview, with the evident signs of her recent surgery, she was an attractive little gift with a winning smile. She was not shy with the interviewer and played nearby as her mother talked. After a while, her grandmother Barbara joined us, and Grace met her with a smile and a hug. At one point, when her mother was called to the phone, Grace showed off her Big Bird and other toys, talking in a quiet voice. She seemed calm and confident for a little girl who has been through so much. Her grandmother said that Grace was worried when she first saw the interviewer, but once she could see she was not going to be touched by her, she relaxed.
At the time of the interview, Leah was twenty years old, slim, with long brown hair. She was soft-spoken and willing to share the family's experience. She had kept a journal of all of the procedures that Grace had, beginning with the diagnosis. Information was neatly written in a spiral notebook, with each date near the left margin and the occurrence for that day listed. She referred to it to show the interviewer how much had happened in a short time, but she didn't need it to jog her memory-every event was there.
Leah described Grace before the illness as a very independent, active toddler. She had begun to talk and walked well. There was no indication of any problem, and it is still a shock to Leah to realize what has happened.
In early November 1994, Grace became wobbly when she walked, was sleeping much more than usual, and vomiting. Her eyes hurt and she couldn't see. Leah took her to her doctor, who diagnosed and treated an ear infection. After the infection cleared up, Grace was still not behaving normally, so her mother took her back to the doctor. The doctor said to wait and watch. However, Leah was concerned, so she took her to the emergency room of the local hospital for another examination. The emergency room doctor did not feel that she was very ill; but in a day or two, Grace had trouble seeing. This frightened her, and she became hysterical. Leah took her back to the hospital and a CAT scan was done, which revealed a massive tumor. The diagnosis was made from the scan on 11 November 1994.
That same day, Grace was rushed to Maine Medical Center, and staff there put in an external shunt to drain off fluid from the tumor. On 14 November, the brain surgeon did a biopsy and removed about 40 percent of the tumor. A permanent shunt was implanted that drains the fluid through a tube in her nose to her stomach. It is still in place at this writing, but at certain points it has not worked, requiring the reinsertion of an external shunt and further surgery to put in another permanent shunt In the first month, Grace underwent seven brain surgeries of one type or another. Unexpected complications seemed to become constant for her.
Grace's oncologist noted that the family was "devastated" by the diagnosis of pineoblastoma, since they had to be told that there are no known survivors treated with standard therapy. On 8 December, chemotherapy was begun, and the family found hope that it might be the answer for Grace. After several weeks of chemotherapy, a 75 percent reduction in her tumor size was recorded. Her oncologist noted that this degree of response was essentially unheard of prior to this case, and it raised hopes that a cure may be possible.
The next part of the treatment will entail a trip to Duke University, where a surgeon will remove some of Grace's bone marrow, give her massive doses of chemotherapy to knock out the remaining tumor and other cancer cells that may be present, and then re-implant her bone marrow to restore her immune system. Using her own marrow eliminates problems of infection and rejection associated with donor-marrow use. The hospital stay will last a month or more. If successful, it will be the end of the cancer and the treatment. She will be monitored for a time, but no further therapy is anticipated.
Leah was asked what "firsts" were related to Grace's illness. She said that she had learned to administer medication through the central line, flush the lines to keep them open, use a feeding tube, and do other medical tasks. "I never imagined I'd be doing this stuff" She said that being in the hospital and caring for Grace in these ways has made her think about becoming a nurse. Talking to other children a little older than Grace who have had similar procedures helped Leah know more about what Grace is feeling.
Leah has a strong support system in her mother and her husband. Barbara said that watching Leah deal with all of this has been hard, especially because Barbara had been through a bout with cancer with her son. Against the odds, he had recovered, and this gave them reason to hope for Grace when things looked bad. She stressed that she told Leah that she has to try to be cheerful even when it's an effort-that the mood of those around her made a difference to Grace. Barbara accompanied Leah and Grace to the hospital on the weekly trips. Faith and holding onto the little bit of hope that exists is essential to get through the process.
Grace's scarred, shaved head, the shunt line going into her nose, and her lopsided face from a partial paralysis left by her tumor surgery are all testimony to what she has gone through and what still faces her. For her family, the fallibility of the original prognosis has provided the foundation for their hope for the future. Hope, for them, is the only alternative to despair.
Roger, Joyce, and Kelly
Joyce and Roger were interviewed at their home. At the time of the interview, each was forty-seven years old. Kelly, their daughter, was thirteen. She was diagnosed with leukemia ten years ago, and, at this point, she has been free of cancer since her treatments ended in January of 1988. Roger and Joyce were asked to recall Kelly before she had cancer. Joyce said that Kelly had been a quiet and cuddly child since birth. At age throe, she was happy to sit and play with a toy or look at a book for long periods of time. Compared to her older sister and younger brother, Kelly was quiet and calm. She slept well at an early age. People were drawn to her because she was 'quiet, cuddly, lovable.' She was delayed developmentally, especially in motor development, and walked at seventeen months. At the time of the diagnosis, the family consisted of six-year-old Mary, four-year-old Kelly, and two-year-old Tom.
For several months before the diagnosis, Kelly had been more and more tired and listless. On a couple of family outings she had needed to be carried or wheeled in a stroller because she was just too tired to walk for very long. She began to complain of joint pains when playing. She was always slim but was getting thinner. She was restless at night and feverish. Joyce took Kelly to the doctor because of the combination of symptoms. Joyce vacillated between thinking that there may be something wrong and wondering if she was imagining it. The symptoms were vague: fatigue, listlessness, loss of appetite, and joint pain. Her pediatrician was concerned with her condition and sent the family to a colleague at Boston Floating Hospital. It seemed that she might have had some type of juvenile arthritis. She returned home and began taking twenty aspirin a day to treat the presumed arthritis. When, after several weeks, there was no improvement, they returned to see a specialist who had been away during the first consultation. She immediately diagnosed leukemia. They went to the hospital for a transfusion the next day.
The parents reported differing reactions to the diagnosis. Joyce felt a sense of relief that at last they knew what was wrong. Roger was shocked that something so major could be causing such vague symptoms. Kelly was hospitalized for six days to be hydrated and given a blood transfusion. By then, she was in bad shape-unable to walk and crying all the time. Kelly and Joyce returned home for Christmas. Roger reported that, for him, there was a period when the diagnosis lacked reality. He accepted it, but the reality of how big a change it would mean to their lives was hard to grasp. He was anxious to get the treatment going and get on with life. A lack of knowledge of what he was getting into kept him from appreciating the magnitude of what was going to happen. Joyce suggested that, like all big news, it became real little by little. The shock of it protected them from understanding all of it at once. Once Kelly had her treatment and transfusion and began to take prednisone, she was "like a new person.' She began to eat well, which she hadn't done for a while, since she had energy from the transfusion.
Traveling to Boston was stressful for the family. The doctors there told them that they had colleagues in Portland who could give the same treatment. They transferred her to Portland for convenience and with the hope that a network of parents might be available if they were involved with a more local doctor.
Treatment involved IVs, intra-muscular injections, and pills. Later, in Portland, she had radiation to her head for nine days in a row. This meant daily trips to Portland, and Joyce's mother came to stay with them while the radiation treatments were given. She accompanied Kelly and Joyce to Portland and back for those days. For the first three months, Kelly was just about knocked out. She didn't eat well and began to "look like someone who was starving". Some patients said that the food tasted like aluminum foil, so how much would any of us eat?" Kelly was hospitalized to get IV nourishment for three weeks in April. As they predicted, she began to feel better by the first of May. Her hair started to grow back. Spring was arriving, and she seemed to be experiencing a rebirth, too.
Kelly was on treatments for two years. They ended in January of 1988, and now she has blood tests once a year. She has grown physically; delayed or stunted growth from radiation had been a risk. She is now 5'7" at age thirteen. She seems to be progressing just as expected before the treatment. Many things we see in Kelly may be related to the cancer treatment, but they seem to be what Kelly would have been anyway. She has always been slow in physical, social, and emotional development. One of the predicted effects of the radiation was that she would have a hard time making connections. Integrating non-verbal cues was expected to be hard for Kelly, and it is. This shows up in social situations and reading comprehension. She is overly reactive to the normal interactions of peer relationships, sometimes taking offense where none is intended. Kelly, however, is persistent in the face of difficulty in doing school assignments.
During the stage of active treatment, Joyce was not working outside the home. She said that the hardest part for her was that she missed Roger. When she was at the hospital with Kelly, they would meet for a few minutes to share a pizza. Roger was busy taking care of the other two children and they were both very tired from stress and lack of sleep. Having to attend to the other two children kept things in perspective. Joyce said that, at one point, her mother-in-law took the three children so she and Roger could have some time alone for a trip to Arizona. Later, the mother-in-law admitted that it had been very scary for her to have that responsibility. Joyce remembered with much appreciation the gift of that week with Roger. Joyce also said that she felt she had learned to accept help during the treatment period. Receiving help is always difficult, and she felt that learning to let other people enjoy giving was a good thing for her. Specific offers of assistance were especially helpful. Roger said that people would often not say anything for fear of saying the wrong thing, and that was hard on him. He hoped that people would just say, "I want you to know that I'm thinking about you." That was enough to make him feel less alone.
Kelly's oncologist remembers her family as "the epitome of emotional expression and health." Their influence on him was so strong that he refers to them as his "teachers" regarding what families must feel as they travel this difficult road.
Summary
The stories presented here represent a minuscule amount of what actually transpired in each case, yet they offer a perspective of pediatric oncology usually reserved solely for the members of the treatment team. Those of us who have never experienced this disease, but who interact daily with children and their families who have, can learn from the lessons they teach us.
Notes