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COMMENTARY |
a Departments of Anesthesiology
b Pediatrics
c Psychiatry, Yale University School of Medicine, Yale New Haven Children's Hospital, New Haven, Connecticut
Abbreviations: SCD, sickle cell disease • CAM, complementary and alternative medicine
Palliative care is both a philosophy of care and an organized system for delivering care. The goals of palliative care are to prevent and relieve suffering and support the best possible quality of life for patients and their families.1 Contrarily to common belief, palliative care is not synonymous with hospice care and should be introduced early in the disease process, possibly even at the time of diagnosis. Indeed, palliative care, with its emphasis on symptom management and quality of life, nurtures a sense of well-being and is an important coping principle in the care of children with life-threatening illnesses.2
Although many strides have been made in the management of children with sickle cell disease (SCD), the life expectancy of these patients is still significantly decreased compared with the general population. Indeed, a recent study reported that half of all patients with sickle cell anemia only survive into their 40s.3 The chronicity of illness in SCD is punctuated by painful episodes, end-organ disease with the development of chronic sequelae, and fear of sudden death. Infection, acute chest syndrome, stroke, and multiorgan failure continue to be the main causes of death.4,5 As such, one can assume that the principles of palliative care are relevant and should be applied to patients with SCD. Current literature on pediatric palliative care, however, does not consistently list SCD as an illness that is appropriate for palliative care, and children with SCD are only occasionally included in palliative care programs once the onset of complicating sequelae has occurred.
In contrast to the palliative care philosophy in which partnership between patients and health care providers is visible, the sickle cell experience is noted for adversarial relationships with health care providers.6 Oftentimes, these patients feel that the medical community is less than compassionate and is, at best, tolerant.2 Patients with SCD may experience emotional marginalization, amplification in depressive moods, and expressed helplessness and hopelessness.7 Deficiencies in school readiness have been noted in children with SCD that do not seem to be caused by the underlying medical pathology. These biopsychosocial factors can culminate in older adolescents as social anxiety and manifest as anger and low expectations; feelings of disfranchisement will result in limited productivity in the adult workforce with resultant dwindling expectations from medical and lay communities.8,9 Indeed, attitudes of the caregiver toward patients with SCD have been reported to contribute to the undertreatment of sickle cell–related pain.10 Medical providers are often reported to be apprehensive of the treatment requests of patients with SCD.11 This apprehension stems from misinterpretation of opioid requests by these patients as drug-seeking behavior. For example, a recent survey indicated that 53% of emergency department physicians and 23% of hematologists think that >20% of patients with SCD are addicts.12 These beliefs and perceptions about SCD-related pain and the presumed prevalence of addiction in this patient population must be addressed with objectivity if clinical care is to be changed substantively.13
We strongly suggest that palliative care for patients with SCD should be introduced in early childhood. Using palliative care philosophies in the management of patients with SCD would change the public and medical purview of this population's plight and is likely to have a tremendous impact on the health care system as well as patients and their families. Indeed, it is predicted that utilization of emergency department services alone would plummet if caregivers fostered better psychosocial adjustment and acceptance of this population's dilemma.14
An integrative medicine approach to palliative care is proposed for improving the health and well-being of patients with SCD. Integrative medicine is "the practice of medicine that reaffirms the importance of the relationship between practitioner and patient, focuses on the whole person, is informed by evidence, and makes use of all appropriate therapeutic approaches, health care professionals and disciplines to achieve optimal health and healing."15 The integrative approach of palliative care emphasizes pain management and attention to psychological, social, and spiritual issues. We submit that the 4 principles that serve as the foundation for palliative care could be aptly applied in the care of patients with SCD. These principles are autonomy of the patient, beneficence, nonmaleficence, and justice.13,16 Transfer of the integrated palliative philosophy and practice to the care of pediatric patients with SCD would change the medical culture and improve this patient population's life experience. Indeed, from the patient's perspective, a sense of well-being would likely develop. Expressions of care, respect for patients' problems, and the development of a therapeutic alliance with these patients would maximize patient and, ultimately, physician satisfaction.16
It is well established that undertreatment of pain compromises patient autonomy and that good pain control can help preserve autonomy.17 Excellent access to treatment can be better assured by formal pain management education of the health care providers to impact attitudes and practices.18 Disease sequelae or coexisting disease place constraints on the pharmacopoeia available to treat pain in this population. For example, the triptans have a >75% success rate in the management of migraine headaches, yet this drug class is relatively contraindicated for those patients with SCD and a history of migraine headaches. Use of second-line multidrug therapy of nonsteroidal antiinflammatory drugs and opioids typically results in <50% satisfaction for migraine management.19 Tolerance to opioids and analgesic rebound from chronic nonsteroidal antiinflammatory drugs are potential complications for the sickle cell migraineur.20 Thus, we also submit that in addition to traditional allopathic therapies, complementary and alternative medicine (CAM) therapies have to be used as adjuncts for pain and symptom management. These therapies include massage, healing-touch therapies, acupuncture, and hypnosis. Hypnosis has shown promise as an effective adjunctive therapy in reducing vaso-occlusive crisis frequency and intensity.21 If the CAM model of palliative care was applied to the sickle cell population throughout the continuum of care, then preemptive care may result in a reduction of the severity of the disease. As of October 2006, the National Institutes of Health Clinical Center was recruiting patients 18 years and older with SCD and a history of pain for trials in hypnosis as a randomized, controlled, single-crossover, single-blinded pilot study. This investigation will examine whether hypnosis can reduce the frequency and intensity of pain in patients with SCD.22 Acupuncture has been used successfully in pain management when conventional therapies have failed in the treatment of vaso-occlusive crisis in hemoglobin SS/SC patients.23 Results of a University of Florida study suggested that body work (ie, both massage therapy and progressive muscle relaxation with guided imagery) show promise as adjunctive interventions for reducing acute and chronic SCD-related pain.24 The illness management of SCD is most befitting for the palliative care model with CAM adjunctive therapies.
In conclusion, the American Academy of Pediatrics recommends the development of policies and standards that promote the welfare of infants and children living with life-threatening or terminal conditions and their families, with the goal of providing equitable and effective support via an integrated model of palliative care in which the components of palliative care are offered at diagnosis and continued throughout the course of illness regardless of whether the outcome ends in cure or death.25 Failure to involve patients with SCD in decision-making about their care undermines their self-reliance and self-knowledge. It also reduces capacity for self-management and weakens the very fabric of health care.26 We strongly urge the medical establishment and our colleagues to adopt and implement the palliative care approach to children who suffer from SCD.
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FOOTNOTES |
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Address correspondence to Brenda McClain, MD, FAAP, Yale University School of Medicine, Director of Pediatric Pain Management Services, Yale New Haven Children's Hospital, 333 Cedar St, TMP-3, New Haven, CT 06520-8051. E-mail: brenda.mcclain{at}yale.edu
The authors have indicated they have no financial relationships relevant to this article to disclose.
Opinions expressed in these commentaries are those of the authors and not necessarily those of the American Academy of Pediatrics or its Committees.
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