The comprehensive care of sickle cell disease

Abstract

Millions of people across the world have sickle cell disease (SCD). Although the true prevalence of SCD in Europe is not certain, London (UK) alone had an estimated 9000 people with the disorder in 1997. People affected by SCD are best managed by a multidisciplinary team of professionals who deliver comprehensive care: a model of healthcare based on interaction of medical and non-medical services with the affected persons. The components of comprehensive care include patient/parent information, genetic counselling, social services, prevention of infections, dietary advice and supplementation, psychotherapy, renal and other specialist medical care, maternal and child health, orthopaedic and general surgery, pain control, physiotherapy, dental and eye care, drug dependency services and specialist sickle cell nursing. The traditional role of haematologists remains to co-ordinate overall management and liase with other specialities as necessary. Co-operation from the affected persons is indispensable to the delivery of comprehensive care. Working in partnership with the hospital or community health service administration and voluntary agencies enhances the success of the multidisciplinary team. Holistic care improves the quality of life of people affected by SCD, and reduces the number as well as length of hospital admissions. Disease-related morbidity is reduced by early detection and treatment of chronic complications. Comprehensive care promotes awareness of SCD among affected persons who are encouraged to take greater control of their own lives, and achieves better patient management than the solo efforts of any single group of professionals. This cost-effective model of care is an option for taking haemoglobinopathy services forward in the new millennium.

Can Heterogeneity of Chronic Sickle-Cell Disease Pain Be Explained by Genomics? A Literature Review

“…In SCD, acute pain has been described as a "crisis," and it heralds tissue infarction (Okpala et al, 2002). In the past, healthcare providers assumed that pain episodes of patients with SCD correlated with healthcare utilization.…”

“…This pain is primarily due to tissue ischemia and vaso-occlusion of the microcirculation by sickled cells (Ballas, 1998). Acute pain acts as a protective agent in response to tissue injury, giving the body warning of potentially harmful agents (Ballas, & Lusardi, 2005;Bergman, 2005;Okpala et al, 2002), and disappears when the injury heals. The resolution of acute pain usually occurs in a few days to several weeks (Todd, 2005).…”

“…(Edwards, C. L. et al, 2005;Lenoci, Telfair, Cecil, & Edwards, 2002;Max et al, 2006). Pain caused by SCD can be classified as acute, chronic and mixed (Okpala et al, 2002). This pain is primarily due to tissue ischemia and vaso-occlusion of the microcirculation by sickled cells (Ballas, 1998).…”

“…Chronic pain in general is widely viewed as a multifaceted, biopsychosocial phenomenon that, as 9 mentioned above, can be associated with psychopathology (Nadel & Portadin, 1977). It is best managed using a holistic, multidisciplinary approach (Montañez & Berland, 2002;Okpala et al, 2002;Thomas, Dixon, & Milligan, 1999;Thomas, 2000;Vukmir, 2004).…”

Corneal Leucomas in a Child with Sickle Cell Disease

“…Most important acute features of SCD are: recurrent painful vaso-occlusive crisis, acute chest syndrome, acute splenic sequestration, priapism and anemia [1]. Chronic organ dysfunctions are: cerebral vasculopathy leading to stroke, leg ulcers, osteonecrosis and rethinopaty [2]. Corneal leucomas can be detected only with a formal eyes exam performed by an ophthalmologist, due to the fact that in the early stages of SCD eye's involvement is asymptomatic.…”