A 10 year old boy presents to the pediatric oncology ward with epistaxis for 2 hours and hematemesis. He complains of nausea, constipation, severe hip pain and headache. He has a history of recurrent Stage IV neuroblastoma, initially diagnosed 2 years ago, treated with chemotherapy and a bone marrow transplant. He received chemotherapy again when his cancer recurred six months ago. Initially his tumor responded, but eventually it progressed and includes bone marrow involvement. He has started experimental chemotherapy at the request of his parents, with his assent. He has multiple metastatic bone lesions, most pronounced on his head and right hip. He has required increasing doses of pain medications. He has been attending school a few hours a day, but this week he has been increasingly tired with increased pallor. This morning, he woke up with epistaxis that would not stop with pressure. About 1 hour later he started vomiting bright red blood. He is currently taking 8 mg of hydromorphone (Dilaudid) orally every 6 hours around the clock for pain. He rates his current pain level as a 6 on a scale of 10.
Exam: VS T 37, P 120, RR 18, BP 100/50. His skin is pale and dry with multiple bruises. He has multiple lumps on his scalp. Dried blood, blood clots and some oozing blood is noted in both nares. Subconjunctival hemorrhages and pallor are noted. His neck is supple with no lymphadenopathy. Heart regular rate without murmurs. Lungs are clear. A central venous catheter is present in his left anterior chest. His abdomen is soft with no hepatosplenomegaly. There is mild tenderness. Exam of his extremities is significant for bruising and pallor. He has moderate tenderness over his back, hips and pelvis.
Lab: WBC 2.4, 70% neutrophils, Hgb 6.2, Hct 19.6, platelet count 3,000.
Hospital Course: He receives packed RBC transfusions to correct his anemia (which may also improve his stamina) and platelet transfusions which stop his epistaxis. He is started on MS Contin (slow release morphine) 60 mg PO q12 hrs with morphine 15 mg PO for breakthrough pain as needed which controls his pain well. His physician discusses the future use of IV morphine on a patient controlled analgesia (PCA) pump. He is started on Senokot (senna) to prevent constipation. Since he seems to improve, or at least remain stable, with the experimental chemotherapy, this is continued.
Palliative care is a broad philosophy of total compassionate care for children when their disease no longer responds to curative treatment. The goal is to give the best quality of life by preventing and relieving suffering. Palliative care affirms life and recognizes death as a normal process. It does not hasten death, nor does it postpone it. The objectives are to prevent or relieve physical symptoms, maintain activity and independence for as long as comfortably possible, alleviate psychological distress, and support those who are bereaved.
Interventions are defined as "palliative" by their therapeutic intent (e.g., comfort) rather than the type of intervention (e.g., radiation, medication, surgery). So that unrealistic expectations are not encouraged, parents and staff must be clear that the purpose is to make the child more comfortable, not to cure the child. Many of the interventions in palliative care focus on symptom management.
To minimize fatigue, prioritize the child's activities, prevent sleep disruptions, and provide rest periods. If they are anemic, consider transfusion depending on their potential for improved quality of life.
For respiratory difficulty, an early objective should be to improve their respiratory effort, and later focus on alleviating anxiety due to respiratory changes and shortness of breath. Other measures include: 1) oral-pharyngeal suctioning as needed, 2) opioids for dyspnea or cough, 3) cough suppressants (dextromethorphan) for dry, nonproductive cough and expectorants (guaifenesin) for wet, productive cough secondary to infection, 4) supplemental oxygen may be needed, 5) anticholinergic medications for increased secretions or dyspnea related to congestion, 6) bronchodilators for dyspnea, wheezing or congestion, 7) diuretics for pulmonary edema, 8) anxiolytic medications for dyspnea with anxiety, 9) aerosolized morphine for dyspnea. A pleural effusion or pneumothorax may require invasive procedures to increase comfort, but this must be weighed against the discomfort of the procedure
For anorexia, treat the contributing factors (nausea, vomiting, pain, constipation). Consider medications to increase appetite, such as prednisone, Marinol (dronabinol) or Megace (megestrol). Benefits to quality of life must be weighed when considering aggressive nutritional support (TPN, enteral feedings).
Nausea and vomiting can be reduced by using antiemetics such as Zofran (ondansetron), Kytril (granisetron) or Anzemet (dolasetron). Consider changing the opioid medication to one which causes less nausea.
Start a laxative regimen to prevent constipation if opioid therapy is used. Treat constipation promptly when it occurs. Mineral oil eases passage of stool by decreasing water absorption, softens stool and lubricates the intestines. If no fecal impaction or bowel obstruction exist, use stimulant laxatives such as Senokot (senna). Avoid suppositories in neutropenic or thrombocytopenic patient. Add fruits, vegetables, and fiber to the diet, and encourage fluids and activity.
For diarrhea, stop all laxatives, avoid milk products, fats and protein. Anti-diarrheal medications such as Imodium (loperamide) and oral electrolyte solutions can be administered if tolerated.
For febrile patients, administer antibiotics for infection, and antipyretics such as acetaminophen, ibuprofen, other NSAIDS or indomethacin (use with caution if the patient is thrombocytopenic).
For insomnia, maintain normal sleeping and waking routines and discourage daytime naps if they are awake at night. Sedative/hypnotics such as Ativan (lorazepam) can be given as needed (watch for interaction with pain medications), but are seldom necessary in the pediatric oncology patient. Avoid corticosteroids at bedtime because of the stimulating effect. Initiate interventions to deal with fears, dreams or nightmares.
Pain control is often the greatest challenge in palliative care. Medication should be used as needed in adequate doses on an appropriate schedule to relieve the pain. Multiple agents are available, ranging from acetaminophen to opiates, which can be given by several different routes. A major challenge is to give sufficient medication to relieve the pain while maintaining as much alertness as the child and family wish. Concerns must be allayed about addiction and the amount of medication sometimes required to eliminate the pain. The amount of medication required is whatever it takes to eliminate the pain.
For optimal pain control, administer pain medications around the clock rather than on an as needed basis. Choose the least traumatic and simplest route of administration. Include co-analgesic medications (acetaminophen, antidepressants, anticonvulsants, NSAIDS) as needed. Establish an alternative plan to be used if the pain increases or if oral medications become less effective. With the start of opiate analgesics, initiate measures to prevent constipation (a common side effect). Use non-pharmacologic pain reduction measures (massage, distraction, music, relaxation) when appropriate
The decision to stop monitoring labs and to stop transfusing blood products when blood counts are low is a difficult one. Routine blood testing is usually not performed in palliative care and the use of transfusions is based on clinical symptoms. Treatment of bleeding is directly related to the child's comfort and the degree of unpleasantness witnessed by the family. Increased bruising and petechiae do not produce discomfort, but severely bleeding gums, uncontrolled epistaxis, or severe GI bleeding can cause discomfort and increase anxiety. Red blood cell transfusions may be given if the child is very tired from anemia and wants to remain active, or has headaches related to anemia, but anemia is not painful and transfusions may not improve the child's activity level. There may come a time when transfusions prolong the dying process and not the living.
1. True/False: There is no "ceiling" on the amount of pain medication that can be used in palliative care.
2. True/False: Transfusions are not appropriate for terminally ill patients.
3. True/False: NSAIDS and acetaminophen can potentiate the action of opioids
4. True/False: The amount of pain medication required is whatever it takes to eliminate the pain.
5. True/False: Although the physical suffering related to a child's dying may not be totally eliminated, there is no reason for the child to be in pain.
1. American Academy of Pediatrics, Committee on Bioethics and Committee on Hospital Care: Palliative care for children, Pediatrics 2000;106:351-357.
2. Armstrong-Dailey A, Goltzer SZ (eds). Hospice Care for Children. 1993, New York: Oxford University Press.
3. Fochtman D. Chapter 17 - Palliative Care. In: Baggott CR, Kelly KP, Fochtman D, et al (eds). Nursing Care of the Child and Adolescent with Cancer, third edition. 2001, Philadelphia: W.B. Saunders, pp. 400-425.
4. Foley GV, Whittam EH. Care of the child dying of cancer, Part I. CA - A Cancer Journal for Clinicians 1990;40:327-354.
5. Foley GV, Whittam EH. Care of the child dying of cancer, Part II. CA - A Cancer Journal for Clinicians 1991;41:52-60.
6. Hellsten MB, Hockenberry-Eaton M, Lamb D, et al. End-of-Life Care for Children. 2000, Austin, TX: The Texas Cancer Council.
7. Masera G, Spinetta JJ, Kankovic M, et al. Guidelines for assistance to terminally ill children with cancer: A report of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology. Medical and Pediatric Oncology 1999:32:44-48.
8. Nitschke R, Meyer WH, Sexauer CL, et al. Care of terminally ill children with cancer. Medical and Pediatric Oncology 2000;34:268-270.
9. Wolfe J, Grier HE, Klar N, et al. Symptoms and suffering at the end of life in children with cancer. New Engl J Med 2000;342:326-333.
10. World Health Organization & International Association for the Study of Pain: Cancer Pain Relief and Palliative Care in Children. 1998, Geneva: World Health Organization.
Answers to questions
1.True, 2.False, 3.True, 4.True, 5.True