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You can play a pivotal role in interpreting the diagnosis, understanding how deeply the disease affects psychosocial development, acting as liaison between family and oncologist, and?if treatment fails?supervising end-of-life care
You are, after all, the primary care pediatrician who has known the adolescent cancer patient and family since infancy. That's why you can play a pivotal role in interpreting the diagnosis, understanding how deeply the disease affects psychosocial development, acting as liaison between family and oncologist, and if treatment failssupervising end-of-life care.
Cancer is a rare disease in adolescence, but probably no other disorder has more devastating an impact. Patient, parents, siblings, peers, teachers, and other significant adults are all affected. The sick teenager disappears into the bewildering world of the high-tech tertiary care center, and the referring primary care pediatrician, who knows the patient and family best, is often shut out of the process. Busy specialists may not stay in contact, or the pediatrician may conclude that he or she has nothing to offer and bow out of the picture.
Such a break in continuous care is not in the best interests of the patient. In fact, the more the pediatrician knows about the spectrum of adolescent cancer, the diagnostic and treatment procedures that are used, and the possibilities of palliative and end-of-life care, the more he or she can contribute. Our intent in presenting the information in this article is to encourage pediatricians to stay involved. The rewardsfor the patient and the pediatricianare significant.
To begin, then, the role that primary care pediatricians can play in providing comprehensive care for the adolescent cancer patient is summarized in Table 1. But this raises an important question: How many teenagers get cancer, and what kind of cancer do they get?
Act as liaison between oncologist and family and patient
Support the family
Provide counseling for siblings
Be available at the time of death
Provide all medical care that is not provided by the oncologist
Be available around the clock to provide medical care and support when the patient is at home
In 1998 (the most recent year from which statistics are available), it is estimated that 12,400 children under 20 years of age were given a diagnosis of cancer and that 2,500 died of cancer.1 Within the overall category of childhood cancer, adolescents deserve special attention because the types of malignancy they develop and the psychosocial impact of their disease is different from the experience of younger children.
Between 1986 and 1995, the incidence of cancer was 117.3 cases for every 1 million children between 10 and 14 years old, and 202.2 cases for every 1 million between 15 and 19 years old. The most common malignancies in the 15- to 19-year-old group were Hodgkin disease (16.1%), germ cell tumors (15.2%), central nervous system (CNS) tumors (10%), non-Hodgkin lymphoma (7.6%), thyroid cancer (7.2%), malignant melanoma (7%), and acute lymphoblastic leukemia, 6.4% (see the chart below).1 Children in the 10- to 14-year range had a higher incidence of acute lymphoblastic leukemia (ALL) and CNS tumor than older adolescents but a lower incidence of Hodgkin disease, germ cell tumors, bone tumors, and thyroid cancer. Bone tumors, ALL, and non-Hodgkin lymphoma (NHL) are more common in boys, and thyroid cancer is more common in girls. Analysis of incidence by race shows that, overall, the incidence of cancer among white adolescents 15 to 19 years of age was approximately 1.5-fold higher than it was among black children their age. By tumor type, black teens have a lower incidence of Ewing's sarcoma, ALL, melanoma, germ cell tumors, and thyroid cancer.
The incidence of individual childhood cancers has increased over the past 30 yearsfrom 183 for every million children in the period 1975 to 1979 to 203.8 for every million in the period 1990 to 1995. But although the incidence of cancer in children has increased, the mortality rate has declined dramatically. The five-year survival rate for adolescents 15 to 19 years of age is approximately 77%up from 69% in the period 1975 to 1984. In 1995, 503 children ages 10 to 14 years and 691 children ages 15 to 19 years died of malignancy.1 Cancer ranks fourthafter accident, homicide, and suicideamong causes of death among adolescents.
But if the incidence of cancer among adolescents is low and the number of deaths small, the impact of the disease is ill-understood and as unique as the adolescent stage of development itself. Neither child nor adult, the adolescent cancer patient presents extraordinary challenges to the clinician.2 Attention to the developmental aspects of treatment is essential for optimum management.
Adolescents with cancer often exhibit vague and prolonged symptoms. To make the diagnosis and stage the malignancy, the patient submits to a barrage of tests: radiographs, computed tomography and magnetic resonance imaging scans, nuclear medicine studies, surgical biopsies, and bone marrow aspirates. Some of these procedures are invasive; all are frightening. They must take place before the final diagnosis and treatment options are discussed with the patient and family. The uncertainty of this period can be eased considerably if the primary care physician acts as liaison between the family and the oncologist, reassuring the patient and explaining what the diagnostic procedures will entail and why they are necessary.
The initial diagnosis of cancer is often met with shock, disbelief, denial, and anger: "I don't believe you. The test is wrong!" "How can this happen to me?" Given time, most adolescents adjust with minimal psychosocial problems, using a variety of coping strategies, wavering between emotion-focused and problem-focused approaches (Table 2).35
|Information seeking||How will the cancer make me feel? Will treatment take a long time?|
|Seeking help and emotional support||Can you hold my hand during the procedure?|
|Positive self statements||It doesn't hurt that bad.|
|Negative self statements||Please stop!you're hurting me.|
|Catastrophizing thoughts||The last time I had this procedure it really hurt bad.|
|Passive acceptance||I can't do anything to make the pain go away.|
|Denial and avoidance being sick.||Let's talk about the weather. I don't want to talk about|
|Minimization and distraction dentist.||I can get through this by watching TVjust like at the|
Once the diagnosis is made, treatment may include chemotherapy, radiation, and surgery. Treatment decisions are based on the histology of the cancer and the degree of metastatic disease. Most solid tumors are treated for about one year, using multimodality therapy that includes chemotherapy, surgery, and radiation; therapy for ALL may last as long as three years. Treatment of acute or chronic myelogenous leukemia and high-risk ALL may include bone marrow transplantation. These therapies, with their lengthy duration, are extremely stressful for patients to undergo and for families to support.
The physical changes caused by cancer treatmenthair loss, weight gain or loss, acne, central venous catheters, surgical scarsare particularly difficult for adolescents, whose self-image is often fragile. Teenagers may appear to be more upset about losing their hair than about losing their life. They may become depressed,6 withdrawing from friends and school and cutting themselves off from the social support of their peer group. Painful procedures, such as bone marrow aspiration and lumbar puncture, are more stressful for teenagers than for younger children, and care should be taken to make these procedures easier by using sedation or nonpharmacotherapeutic intervention such as guided imagery, hypnosis, or distraction.3
Adolescence, a time of physical, emotional, and social growth, is also a time of frustration, anxiety, and conflict. Adolescents are old enough to understand their diagnosis and its implications, but not yet old enough to react in an adult fashion. They see cancer primarily as a barrier to leading their normal life. At a time when conforming to the norms of their peer group is immensely important, having cancer makes them different.
The experience has much in common with chronic disease, in which ongoing disability or need for medical intervention separates a sick teen from her or his healthy peers. In both cases, the normal tasks of adolescence are compromised. Forced to rely on family, nurses, and medical staff at an age when they long to be independent and in control of their own life, the adolescent cancer patient may be overwhelmed by anger, anxiety, and depression.
Adolescence is also a period of rapid intellectual growth, when teens acquire cognitive skills they don't yet know how to use. Formal operational thought, characterized by introspection, abstract thinking, and logic, becomes possible. Adolescents begin to have greater insight into why they feel the way they do and why they make the decisions they do. It is the beginning of looking inward and setting goals. Abstract concepts of love, death, religion, and the meaning of life begin to occupy their thoughts.
Yet with all these new abilities, teenagers often behave as if they are still children. Adolescents may challenge their diagnosis by refusing to take the medications they know are needed to combat the disease. They may not be willing to put up with side effects of the regimen, and become noncompliant. Indeed, adherence to therapy is a major issue in adolescent cancer management and must be monitored closely.
Teenagers with cancer often use denial as a coping technique. It is not unusual, to give one example, for an adolescent patient to respond to a physician's explanations by turning over and going to sleep or turning on the TV in the middle of the discussion. This is the adolescent's way of saying he has heard enough and needs time to absorb the information before he is ready to receive any more. Denial provides a respite that relieves the strain of the disease. Moving in and out of denial is a common strategy that allows the teen to take in information at her or his own pace.
Just as cancer itself is rare among adolescents, recurrence is uncommon, occurring in about 30% of patients.7 We focus on it here not because it is common, but because its psychosocial ramifications are so often misunderstood and mishandled. The psychodynamics of recurrent cancer differ significantly from the original experience. The risk of recurrence varies with the disease and the stage at diagnosis, but is generally greater for adolescents than for younger children. Because most malignancies are now treated with very intense chemotherapeutic and radiation protocols at the time of diagnosis, recurrent disease is more likely to be multidrug resistant and to offer less of a chance of survival. Patients with Hodgkin disease, Wilm tumor, and acute myelogenous leukemia have a good or moderate chance of survival after relapse (30% to 40%). Those with osteogenic sarcoma, ALL, Ewing sarcoma, brain tumor, non-Hodgkin lymphoma, and other soft tissue sarcoma fare very poorly after recurrence, with the chance of long-term survival less than 10% to 20%.8 With each successive relapse, the likelihood of long-term survival fades.
When cancer recurs, patients feel cheated and angry: They have endured the discomforts of treatment only to have their worst fears become reality. If they have been nonadherent with therapy, they may feel guilty. Some may want to abandon chemotherapybecause they cannot tolerate the thought of going through it again or they believe that it isn't any more likely to work a second time. Most adolescent patients with a recurrence do choose more therapy, however, because they simply do not want to die.
Patients and families need to be told clearly that the chances of cure diminish with each recurrence or progression of symptoms.9 They should be told when a therapy is experimental and offers no real chance of cure. They should be given all options, including discontinuing therapy and focusing on the quality of life in the time that remains.
Older adolescents (18 or 19 years of age) often want to make more of the decisions at the time of relapse than they did initially, because they have gained a wealth of knowledge from their prior experience. They may choose not to have additional chemotherapy, or they may want aggressive care. Younger adolescents may voice their opinion about therapy, but parents still have decision-making power.
Conflict between the adolescent and parents creates an ethical dilemma for health-care providers. In this situation, all parties may benefit from the involvement of the primary care pediatrician, who knows the family well. Issues faced by an adolescent who has an unremitting malignancy are listed in Table 3.
Changes in physical appearance
Fear of death
Fear of treatment
Feelings of being treated differently
Forced dependence on parents
Isolation (including from the community)
Loss of control
Treatment-related side effects
Regardless of the situation, teenagers always seek to be normal. They want to experience life as much as possible and, especially, to meet teenage milestones like learning to drive a car or going on a date. The chronically ill or dying teenager should be supported as far as is possible in efforts to attend school, spend time with friends, go to parties, and hold a part-time job.
Adolescents and their families today have little, if any, exposure to death. Few have attended a funeral, and even fewer have witnessed a death. Intellectually, adolescents are capable of understanding that death is universal, inevitable, and permanent, but healthy teens rarely understand death as something that can happen to them. For teenagers with recurrent cancer, that exemption from the universal human fate gradually becomes tenuous until at some point the recognition of personal death becomes a reality. Patients and families deal with this recognition in different ways. No common pattern holds true for most families.10 Most people vacillate between trying to conquer death and accepting its inevitability. Hope remains forever present, but it can shift from hope of a cure to hope of a peaceful death.
Adolescents are especially inquisitive about what will happen when they die. Their concerns are very concrete:
Understanding the impact of dying on the developmental tasks of adolescence is essential for the health-care provider. The first task that is threatened is that of gaining independence. As cancer symptoms escalate, families tend to become overprotective. Fearful of every new complaint, they watch the adolescent's every move and limit activities that may still be within the sick teenager's ability. For older teenagers, the situation is more difficult. Those who had been employed and financially independent find themselves reduced to child-like financial dependence. They may become angry and resentful, not only of the disease but of the parents who protect and support them.
The task of self-acceptance is threatened as well. Some teenagers are so afraid of what others will think of their changed appearance that they refuse to leave the house. They may reject intravenous pain medications because the pump is too conspicuous, or skip oral medications when they are with friends. As they get closer to death, some teenagers are no longer willing to visit the outpatient clinic because of what the other patients will think: "I don't want everyone to know I'm dying." They may resist meeting new peoplefor example, the hospice team.
The developmental task that is most difficult to achieve is a realistic orientation to the future. Looking forward is so important that many teens continue to make future plans until the day of death. They plan to go to the prom, attend high school graduation, go to college, and have a family. Older adolescents who want to feel that a part of them remains in the world they are leaving may seek to have children. They can be encouraged, in the case of male patients, to bank their sperm before starting chemotherapy.
Parents, too, need help coping with the approaching death. Some react by trying to keep the truth from the dying adolescent, although the deception seldom succeeds. Studies have shown that most terminally ill adolescents know they are dying.11 They overhear hushed conversations and are well aware of the changes in the way people treat them. In the course of their illness, most have gotten to know other oncology patients who have died.
A study by Orr and colleagues found that adolescents do not want false promises, deception, or futile therapy if death is inevitable.12 Yet parents often seek to protect their children and do not want them told the truth. The teenager often begins the process of saying good-bye to friends and family while the family is still trying to hold on to the hope of a cure. This mismatch in coping may cause a great deal of strife and struggle for the family system.
Regrettably, adolescents are also very much aware of their family's distress and often try to protect their parents by hiding their fears and concerns. They may even agree to continue therapy because their parents want them to. Health-care givers should do whatever they can to facilitate open communication between the dying adolescent and the family so that outstanding issues can be resolved and good-byes said before it is too late. The primary care pediatrician can play a very useful role in this effort.
New information regarding disease or symptom progression should be presented in a timely, direct manner. Team members should answer questions consistently and not provide false hope. Open communication allows the adolescent the freedom to express his desires: Does he want more therapy? Where does he want to die? Will he accept hospice help? What important things does he want to do before he dies? Older adolescents should be encouraged to write a living will or appoint a power of attorney to decide health-care issues for them. This may relieve the family of difficult decisions at the time of death. A social worker or other team member may assist in writing these forms or explaining them to the patient.
Like parents, siblings find acceptance of approaching death difficult. When protective parents shut siblings out from what is happening, they succeed only in magnifying the problem. Brothers and sisters who are excluded from events that are preoccupying their parents feel abandoned and angry. They may act out at school or feign an illness to get attention. Some may feel guilty, believing that they have somehow contributed to, or caused, the cancer or the death.
To prevent these consequences, siblings should be involved throughout the illness. They can have a positive function in the family, keeping everyone connected to the everyday events of normal lifeschool, play, sports, jobs. They can share in the care of their dying brother or sister, playing games or watching videos together, making memory albums, and helping the patient plan the funeral.
Classmates, peers, and significant others also can be an important part of the adolescent cancer experience. Some will abandon the patient because they cannot deal with the intensity of the situation; the death of a friend compels them to look at their own mortality. But those who have sufficient inner strength can provide the dying teen with distraction, companionship, and the reassurance that he or she will not be forgotten.
Symptom management at the end of life relies primarily on physical exam findings and the patient's subjective reports, and less on laboratory and radiologic evaluations. Table 4 lists commonly encountered end-of-life symptoms and the medications used to treat them. In the adolescent cancer patient, pain is the primary symptom. Yet in an effort to maintain independence, adolescent patients may not tell their parents or health-care team members they are hurting.
|Acetaminophen, other NSAID||10 mg/kg/dose po|
|Acetaminophen with codeine||1 mg/kg/dose of codeine po|
|Oxycodone and acetaminophen||0.05 mg/kg/dose of oxycodone po|
|Morphine sulfate||0.3 mg/kg/dose po|
|Oxycodone||0.05 mg/kg/dose po|
|Hydromorphone||0.03 mg/kg/dose po|
|Constipation||Docusate sodium||Varies with age; follow instructions on label|
|Magnesium hydroxide (as Milk of Magnesia)|
|Senna-based laxative (Senakot)|
|Fentanyl aerosols||50 µg/2 mL NS by aerosol every 24 hr|
|Nausea||Promethazine||0.25 mg/kg/dose po every 46 hr|
|Metoclopramide + diphenhydramine HCl (Benadryl)||1 mg/kg/dose po every 6 hr 1 mg/kg/dose po every 6 hr|
|Lorazepam||0.04 mg/kg/dose po every 68 hr|
|Ondansetron||0.15 mg/kg/dose po every 8 hr|
|Anxiety||Lorazepam||0.04 mg/kg/dose po every 68 hr|
|Diazepam||0.04 mg/kg/dose po every 8 hr|
|Agitation||Haloperidol||0.01 mg/kg/dose po every day|
|Chlorpromazine||0.08 mg/kg/dose po every 6 hr|
|Depression||Sertraline||25 mg/d po (age >6 yr)|
|Fluoxetine||20 mg/d po (age >6 yr)|
Caregivers should ask adolescent cancer patients if they have pain, how the pain rates on a pain scale, and if they are taking their pain medications. Ask what level of pain the patient feels is tolerable and have the patient describe the characteristics of the pain. Fear of addiction to narcotics should be addressed early; adolescents who have been taught to "Just say no" to drugs must be convinced that opiate pain medications are medically necessary. Families, too, need to have the terms addiction, tolerance, and physical dependence explained.
The World Health Organization approach can be used as a guide to treating pain.13 Nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen are used for mild pain; combination products, such as acetaminophen with codeine or acetaminophen with oxycodone (Percocet) are used for moderate pain. The utility of combination products is limited, however, because the dose of the narcotic cannot be increased above the daily maximum of acetaminophen or other NSAID. In addition, NSAIDs are not recommended in patients with thrombocytopenia because they cause platelet dysfunction and may prompt bleeding.
Narcotics are the mainstay of severe pain; a long-acting preparation should be used to maintain a steady bloodstream level. If the patient develops intolerable side effectsitching, constipation, somnolencefrom a narcotic, an equianalgesic dose of a different opioid should be tried. Available long-acting oral preparations include two long-acting morphine preparations (Oramorph and MS Contin); Oxycontin, which is a long-acting oxycodone preparation; and methadone. These medications are dosed two or three times a day.
In addition to oral long-acting medications, fentanyl transdermal patches can be used. The patch, which is applied every 48 to 72 hours, provides a constant blood level of drug. Two points about the fentanyl patch: First, it should not be used in an opioid-naïve patient because of the risk of overdose and, second, it may not work well in the patient with poor perfusion. This product is best used in patients with constant chronic pain, as rapid titration to control escalating pain is difficult.
Short-acting opioid preparations, including immediate- release morphine and hydrocodone, should always be available for breakthrough pain when using sustained-release products. The daily total dosage of breakthrough medication can then be used as a benchmark against which to titrate long-acting compounds. Increasing the dosage of narcotics may be required because of worsening disease or the development of tolerance, which is defined as the need for higher doses of narcotic to achieve the same level of pain control. The correct dosage of narcotic is the amount that controls the pain; there is no upper limit. Reassessment and titration are the keys to good pain control.
For patients who experience bone pain, an NSAID or corticosteroid may be helpful. Neuropathic pain is often difficult to control, but amitriptyline, gabapentin (Neurontin; unlabeled use), or corticosteroids are often successful. Large doses of narcotics may be required, especially for bone pain.
In the last 24 to 48 hours of life, patients often experience rapid and severe exacerbation of pain and other symptoms. Aggressive, rapid titration of pain medications is needed at this time; the patient may need to be hospitalized to control symptoms. If the patient becomes unable to take oral medications, subcutaneous or intravenous infusion of narcotics can be used. Oral concentrated Tensilon (soft gelatin capsule) forms that are absorbed from the oral mucosa, or specially compounded narcotic suppositories, can also be used. Voice links can allow the infusion rate of an IV pump to be changed over the phone, avoiding delay in increasing the dosage of pain medication. Side effects from opioids may include constipation, urinary retention, itching, hiccups, and fatigue, and must be dealt with as they arise.
Pain medication is not the whole story. The clinician should explore other variables that can exacerbate physical pain: depression, anxiety, and spiritual discord, for example. Pain management principles are summarized in Table 5; an extensive discussion of pain management can be found in Armstrong-Daily and Goltzer's book, Hospice Care for Children.14
Realize that pain is subjective
Use a consistent scale to rate pain
Ask what level of pain the patient can tolerate
Explore contributing factors
Use acetaminophen or other NSAID, or an NSAIDcodeine combination, for mild pain
Severe pain requires narcotics
The correct dose of morphine is the dose that controls the pain
Besides pain, dyspnea is perhaps the most distressing symptom of the dying patient, young or old, and the most difficult to control. This subjective feeling of difficulty breathing is most common in patients with pulmonary metastases, malignant pleural effusions, congestive heart failure, or pneumonia. Palliative radiation to relieve obstructive metastases or drainage of pleural fluid may alleviate symptoms; systemic narcotics are used for bronchial dilation.
Note that respiratory depression does not occur with narcotic medications unless the patient has chronic lung disease; indeed, oxygenation may improve with a narcotic. Morphine or fentanyl aerosols have also been used with good effect on dyspnea at the end of life15 (morphine may cause bronchospasm, however). Bronchodilators and oxygen may also ameliorate symptoms. Non-pharmacotherapeutic interventions such as fanning the patient, opening a window, or instituting guided imagery may be helpful. Benzodiazepines can control agitation and anxiety associated with hypoxia and dyspnea.
Depression can be very difficult to diagnose in the face of terminal illness, as typical symptomsexcessive tiredness, constipation, weight loss, altered sleep patterns, persistent sadness, or depressed moodmay reflect progression of the disease. Nevertheless, clinical depression may be present and responsive to antidepressant medication.
Last, we caution that these guidelines and general principles of care offer a stepping stone to a greater understanding of the medical needs of adolescent cancer patients. For a detailed explanation of management of the dying patient, however, readers should consult textbooks of palliative care, including Armstrong-Dailey and Goltzer's Hospice Care for Children14 and Doyle, Hanks, and MacDonald's Oxford Textbook of Palliative Medicine.16 (For additional information on this topic from Contemporary Pediatrics, see "How you can help meet the needs of dying children" in the April 2002 issue.)
In our experience as oncologists, most adolescents prefer to die at home. Hospice is, perhaps, the best way to provide optimal control of symptoms and allow the adolescent to be at home. But the hospice approach is not acceptable to all families because, first, it requires them to accept impending death and, second, it may not provide supportive services, such as total parenteral nutrition, that families desire.
The services of a home care agency are an alternativea means of providing terminal care at home. The pros and cons of hospice and home care are compared in Table 6. In either setting, the managing physician must be available to provide continuity of care around the clock or must designate colleagues who are comfortable with end-of-life care to provide coverage. Although the pediatric oncologist often manages end-of-life care, the primary care provider can also assume this role.
|On the positive side||On the negative side|
Services of chaplain and social worker
Expertise with dying
Pays for medications and durable medical equipment
Visits from a registered nurse available around the clock
Minimum 13 months of bereavement services
Other services and staff available: physical therapy, occupational therapy, speech therapy, nurses' aides
|Home care||Medical care provided by physicians and nurses May provide home total parenteral nutrition and blood products Physical therapy, occupational therapy, speech therapy, and nurses' aides available Family does not have to openly accept impending death Not as intrusive as hospice care||No chaplain services Services of a social worker may or may not be available Family pays for medications and durable medical equipment that is not covered by health insurance No bereavement services Emergencies require a visit to the physician's office or a hospital emergency room Visiting registered nurses may be less familiar and comfortable with end-of-life issues|
Hospice is a philosophical approach to end-of-life care that provides emotional, psychologic, spiritual, and medical support for the patient and family. The focus is on providing the best quality of life possible for the patient's remaining days. A hospice referral can be made by anyone involved in the dying patient's care: oncologist, primary care pediatrician, or family. The patient's health-care insurer may dictate which local hospice can be used so that services are covered.
The hospice team includes nurses experienced in end-of-life care, a social worker, chaplain, and medical director. Each hospice patient must have a managing physicianideally, the oncologist or a primary care physician with whom the patient is most comfortable. In some situations, the hospice medical director assumes the role of managing physician. The pediatrician or managing physician prescribes medications to manage end-of-life symptoms.
The hospice nurse visits the patient at home and provides physical assessments, arranges for durable medical equipment and oxygen when needed, and supplies anticipatory guidance about signs and symptoms of impending death. She teaches family members ways to manage pain, constipation, and dyspnea. Hospice nurses are also experienced in discussing issues related to death and dying. Nursing visits can occur as infrequently as every other week or as often as several times a day, depending on the patients' condition. Most bedside nursing is done by family members, friends, and hospice volunteers; the hospice does not generally provide around-the-clock nursing care.
Hospice nurses communicate at least once a week with the managing physician, keeping him or her up to date on the patient's condition and requesting medication orders that are determined to be necessary. The social worker assists the family with financial matters and funeral planning and provides grief counseling. The chaplain addresses spiritual concerns. If the family has a strong church affiliation, the chaplain may play less of a role. The chaplain may also assist with funeral planning, provide counseling, perform the funeral if the family chooses, and offer bereavement counseling after death. The hospice medical director meets twice monthly with the team to review the case and ensure that high-quality care is being provided.
State Medicaid programs and most third-party insurers offer a hospice benefit at a per diem rate. The current Medicaid reimbursement rate is $105 a day, which covers nursing and social work visits, chaplain services, medications, durable medical equipment, and oxygen. Hospice services usually also cover hospitalization for symptom management, respite, and terminal care. At death, members of the hospice team arrive at the patient's home to pronounce the patient dead, comfort family members, and assist with telephone calls. The benefits of hospice for the adolescent patient are shown in Table 7.
Advocate for the patient's needs
Empower the teenager through shared decision making
Facilitate communication with family members
Help with a living will, funeral planning, and distribution of personal effects
Support independence and autonomy
When the patient is expected to die at home, the county coroner should be notified in advance and at the time of death. A hospice team member or the physician may make this contact. Doing so makes it unnecessary for police to get involved when death occurs. Death may be pronounced by the physician at the patient's home, by the hospice nurse with physician coverage, or at a nearby medical facility to which the body has been transported. When death is pronounced at home, the funeral director sends a vehicle to transport the body. Autopsy arrangements should be made in advance; the funeral director may be willing to transport the body to the hospital for the autopsy.
The family's acceptance of hospice is often delayed until late in the course of the adolescent's illness. Both parents and child may resist because they believe that to accept hospice care is to give up and lose hope. This is regrettable because entering hospice when only days of life remain deprives patients and families of many of the benefits that such a program can offer. Health-care providers can facilitate hospice acceptance by explaining that hospice represents a shift in the focus of care from concerns about the quantity of life to an emphasis on its quality. Accepting hospice does not mean abandoning hope but moving from hope of a cure to hope of days free from pain and distress. The pronouncement "There's nothing more we can do" should be avoided. Hospice can be presented as an alternative to futile therapy and a way for the patient to avoid unpleasant associations of the hospital and the emergency room.
Adolescent patients should be encouraged to see hospice as a way to control symptoms so that normal activities can be continued for the longest possible time. At a time when parents are often overprotective, the team can advocate for normalcy. They can assist the patient in staying in school and spending time with friends. They can help the teenager stay in control by offering choices: whether medication will be delivered by mouth or IV or transdermal patch, whether to be visited by a nurse at home or go to the clinic or the emergency department for procedures.
The dying adolescent often wants to talk about death, and sometimes this conversation goes more easily with a friend or a hospice team member than with a parent. Some teenagers attempt to control their situation by planning the funeral down to the last detail: What they will be dressed in, who will speak, what songs will be played. They may want to create mementoes for their loved ones in the form of videotapes, letters, or poems. They may be very specific about distributing their possessions, making a will with precise instructions about who should get what. They may want friends invited to the home to say good-bye. They may welcome the opportunity to talk over with the hospice chaplain existential questions that often trouble dying patients.
Adolescent cancer patients are a special group. No longer children but not yet physically or psychologically adults, they strive to maintain normalcy and independence, to continue their dreams of the future. With proper assistance, their illness and even their death can be a meaningful experience to all involved. A hospice or home care team experienced in dealing with adolescents can meet the special needs of the dying adolescent. The primary care pediatrician who desires to be part of the team can make a priceless contribution to the family's well-being.
The American Academy of Pediatrics and the International Society of Pediatric Oncology have issued statements on the elements of terminal care in pediatric cancer patients,17,18 and all those who treat adolescents with cancer should attempt to meet those requirements. Care of the dying is time-consuming and stressful, but the rewards include a profound relationship with the family and the satisfaction of a vital job done well.
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