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20 October 2016

Researchers have found that proper management of mildly symptomatic sickle cell disease can have an impact on lifespan.

Some people with sickle cell disease have limited access to appropriate care, say Dr Samir Ballas of Thomas Jefferson University in Philadelphia, USA, and colleagues in Blood.


The team analysed four case studies of women with milder forms of sickle cell disease who have "far surpassed" the U.S. median of 47 years old for women with the disease, with one currently at 86 years of age.

Compliance with treatment was determined by observations carried out by health care providers. All had "desirable" disease states, according to the study team. Hence, none were eligible for treatment with hydroxyurea, the only FDA-approved treatment for adults with the disease.

Instead the women had standard treatment including hydration, vaccination (such as flu), and blood transfusion and analgesics, as needed. They were all encouraged to attend regular follow-up visits, not to smoke, watch their weight, and keep up a support system if possible.

Among these women, their adherence to medication and strong family support appeared to be linked to their long lives.

Dr Ballas says: "For those with mild forms of sickle cell disease, these women show that lifestyle modifications may improve disease outcomes. Though they had dissimilar ancestries (two African American, one Italian American, and one African Brazilian), all led healthy lives bolstered by long-term family support.

"All of the women were non-smokers who consumed little to no alcohol and maintained a normal body mass index. This was coupled with a strong compliance to their treatment regimens and excellent family support at home."

However, the women did experience some disease-related complications, such as acute chest syndrome - fever, cough, excruciating pain, and shortness of breath

Ballas, S. K. et al. Case series of octogenarians with sickle cell disease. Blood 4 October 2016 doi: 10.1182/blood-2016-05-715946



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