October 17, 2016
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Environmental, genetic factors may predict longevity in sickle cell disease

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A case series of individuals with sickle cell disease who lived into their ninth decade revealed potential genetic and environmental factors associated with their longevity.

The case report — published in Blood — included data from four women with sickle cell disease who have lived longer than age 80 years. Three women are American and received treatment at Thomas Jefferson University’s Sickle Cell Center. The fourth woman resided in Brazil.

Samir K. Ballas

One patient remained alive and in her 80s at the time of reporting. The remaining three patients are deceased and had lived into their 80s.

Although individuals with sickle cell disease are living longer than ever, most patients do not live beyond the age of 60 years, according to Samir K. Ballas, MD, FACP, emeritus professor of medicine and pediatrics at Thomas Jefferson University.

“In the 1990s, the average lifespan of a patient with sickle cell disease was between 40 years and 50 years,” Ballas told HemOnc Today. “In our patient population, we see a growing number of patients living well into their 50s, with some living into their 60s. In the long run, we hope that patients with sickle cell disease will approach a normal life span.”

Ballas and colleagues sought to observe common traits among patients with sickle cell disease who survive well beyond the average life span. The researchers observed that all octogenarian patients were women.

“On average, women with sickle cell disease live between 8 years and 10 years longer than men,” Ballas said.

The researchers further noted that all octogenarian patients had little history of vaso-occlusive pain crises and no history of strokes. The U.S. patients had no history of acute chest syndrome, although the Brazilian patient had a history of this condition.

Ballas and colleagues further noted that all of the octogenarian patients had low white blood cell counts, low hemoglobin levels and normal biochemical parameters. They also had no history of hydroxyurea use.

All octogenarian patients had several lifestyle factors in common, including no history of smoking; little to no history of alcohol consumption; excellent adherence to medication, medical appointments and patient referrals; and normal or below-normal BMI.

“All patients had excellent family support, which in turn may be responsible for their excellent treatment adherence,” Ballas said. “We believe that this contributed to their longevity, in addition to maintaining healthy lifestyles.”

The researchers acknowledged that their observations are anecdotal. However, Ballas asserted that physicians treating patients with sickle cell disease should consider initiating conversations about potentially actionable lifestyle factors.

“The message of our findings is to emphasize the environmental factors,” Ballas said. “These factors can be controlled or treated. We should counsel, advise and educate patients to follow the examples of these long-living individuals with sickle cell disease, all of whom achieved a life span beyond what is expected of the general population.” – by Cameron Kelsall

For more information:

Samir K. Ballas , MD, FACP, can be reached at samir.ballas@jefferson.edu.

Disclosure: The researchers report no relevant financial disclosures.

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