Pediatric Palliative Care: A Novel Approach to Children With Sickle Cell Disease

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COMMENTARY

Pediatric Palliative Care: A Novel Approach to

Children With Sickle Cell Disease

Brenda C. McClain, MD, FAAPa,b_{, Zeev N. Kain, MD, FAAP}a,b,c

Departments ofa_{Anesthesiology,}b_{Pediatrics, and}c_{Psychiatry, Yale University School of Medicine, Yale New Haven Children’s Hospital, New Haven, Connecticut}

The authors have indicated they have no ﬁnancial relationships relevant to this article to disclose.

P

ALLIATIVE CAREis 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 cop-ing principle in the care of children with life-threatencop-ing illnesses.2

Although many strides have been made in the man-agement of children with sickle cell disease (SCD), the life expectancy of these patients is still significantly de-creased 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 syn-drome, 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 pe-diatric 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 adver-sarial relationships with health care providers.6

Often-times, 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 help-lessness and hopehelp-lessness.7_{Deficiencies in school}

readi-ness 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 ad-olescents as social anxiety and manifest as anger and low expectations; feelings of disfranchisement will result in limited productivity in the adult workforce with result-ant dwindling expectations from medical and lay com-munities.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

appre-hension stems from misinterpretation of opioid requests by these patients as drug-seeking behavior. For example, a recent survey indicated that 53% of emergency depart-ment 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 pre-sumed prevalence of addiction in this patient population must be addressed with objectivity if clinical care is to be changed substantively.13

Abbreviations:SCD, sickle cell disease; CAM, complementary and alternative medicine

Opinions expressed in these commentaries are those of the authors and not necessarily those of the American Academy of Pediatrics or its Committees.

www.pediatrics.org/cgi/doi/10.1542/peds.2006-3580 doi:10.1542/peds.2006-3580

Accepted for publication Dec 12, 2006

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@yale.edu

612 McCLAIN, KAIN

at Viet Nam:AAP Sponsored on August 29, 2020

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We strongly suggest that palliative care for patients with SCD should be introduced in early childhood. Using palliative care philosophies in the management of pa-tients 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 dilem-ma.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 rela-tionship 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}

pal-liative 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 pa-tients with SCD. These principles are autonomy of the patient, beneficence, nonmaleficence, and justice.13,16

Transfer of the integrated palliative philosophy and prac-tice 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, physi-cian satisfaction.16

It is well established that undertreatment of pain compromises patient autonomy and that good pain con-trol can help preserve autonomy.17 _{Excellent access to}

treatment can be better assured by formal pain manage-ment 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 mi-graine headaches, yet this drug class is relatively contra-indicated for those patients with SCD and a history of migraine headaches. Use of second-line multidrug ther-apy of nonsteroidal antiinflammatory drugs and opioids typically results in⬍50% satisfaction for migraine man-agement.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 allo-pathic therapies, complementary and alternative medi-cine (CAM) therapies have to be used as adjuncts for pain and symptom management. These therapies

in-clude massage, healing-touch therapies, acupuncture, and hypnosis. Hypnosis has shown promise as an effec-tive adjunceffec-tive 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 Octo-ber 2006, the National Institutes of Health Clinical Cen-ter was recruiting patients 18 years and older with SCD and a history of pain for trials in hypnosis as a random-ized, controlled, single-crossover, single-blinded pilot study. This investigation will examine whether hypnosis can reduce the frequency and intensity of pain in pa-tients with SCD.22_{Acupuncture has been used}

success-fully 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 mas-sage therapy and progressive muscle relaxation with guided imagery) show promise as adjunctive interven-tions 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 effec-tive support via an integrated model of palliaeffec-tive 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 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.

REFERENCES

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UNION DISRUPTS PLAN TO SEND AILING WORKERS TO INDIA FOR CHEAPER MEDICAL CARE

“Carl Garrett, a 60-year-old North Carolina resident, was packing his bags to fly to New Delhi and check into the plush Indraprastha Apollo Hospital to have his gall bladder removed and the painful muscles in his left shoulder repaired. Mr Garrett was to be a test case, the first company-sponsored worker in the United States to receive medical treatment in low-cost India. But instead of making the 20-hour flight, Mr Garrett was grounded by a stormy debate between his employer, which saw the benefits of using the less expensive hospitals in India, and his union, which raised questions about the quality of overseas health care and the issue of medical liability should anything go wrong. ‘I was looking forward to the adventure of being treated in India,’ Mr Garrett said the other day. ‘But my company dropped the ball.’ The union, the United Steelworkers, stepped in after it heard about Mr Garrett’s plans, saying it deplored a ‘shocking new approach’ of sending workers to low-cost countries as a way to cut health care costs. Its officials insisted that Mr Garrett be offered a health care option within the United States.”

Rai S.New York Times. October 11, 2006 Noted by JFL, MD

614 McCLAIN, KAIN

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DOI: 10.1542/peds.2006-3580

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Brenda C. McClain and Zeev N. Kain

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Pediatric Palliative Care: A Novel Approach to Children With Sickle Cell

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DOI: 10.1542/peds.2006-3580

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Pediatrics

Brenda C. McClain and Zeev N. Kain

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Pediatric Palliative Care: A Novel Approach to Children With Sickle Cell

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