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

Abstract

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 … 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