drug could stop heart disease linked to sickle cell anemia | Health Info

By Steven Reinberg Health Day Journalist

FRIDAY, September 17, 2021 (HealthDay News) – Treating sickle cell anemia with the drug hydroxyurea may also reverse associated heart abnormalities, a new study suggests.

Heart problems are common in people with sickle cell disease. Among them are an enlarged heart and an impaired ability to relax the heart muscles, a condition called diastolic dysfunction which can lead to heart disease, heart failure and death. However, long-term treatment with hydroxyurea appears to improve and even reverse some of these abnormalities, the researchers say.

“Early treatment with hydroxyurea should be offered to all children with sickle cell disease from 9 months of age, regardless of the severity of the disease,” said lead researcher Dr Arushi Dhar. She is a pediatric cardiologist at Cohen Children’s Medical Center and Zucker School of Medicine at Hofstra / Northwell in Hempstead, NY

Close cardiac monitoring of children with sickle cell anemia, including asymptomatic patients, should start early, she said.

“At our facility, all children with sickle cell anemia are referred for a baseline cardiac assessment from age 5,” said Dhar.

Abnormalities, such as enlargement or thickening of a chamber of the heart and high blood pressure affecting the lungs, are common in children with the disease.

Sickle cell anemia is the most common inherited disease of red blood cells in the United States. It affects approximately 100,000 Americans, including one in 365 blacks and one in 16 Hispanic Americans, according to the United States Centers for Disease Control and Prevention.

In these people, the red blood cells, which are usually round, take the shape of a crescent or a sickle, then separate, clump together and stick to the walls of the blood vessels. This impairs blood circulation, which can lead to heart complications as well as severe pain, joint and organ damage, and stroke.

Hydroxyurea, one of the drugs approved by the United States Food and Drug Administration to treat the disease, prevents the formation of sickle cells. But exactly how it works is not understood. The drug can help patients have fewer pain attacks and even live longer, according to the Sickle Cell Disease Association of America.

But this is the first study to show that hydroxyurea can also reverse sickle cell heart damage, Dhar said.

For the study, his team collected data on 100 young people with sickle cell disease (aged 3 to 22). Half had an enlarged or thickened left ventricle of their heart. Some also had a type of high blood pressure (pulmonary hypertension) that affects the lungs and the right side of the heart.

Sixty participants were taking hydroxyurea. Twenty-five people who had been taking the drug for less than a year were significantly more likely to have an enlarged left ventricle, according to the study. Long-term cardiac outcomes were better in patients taking hydroxyurea, the researchers added.

The longer patients took the drug, the less likely they were to have an enlarged or thickened left ventricle. Of the 34 patients who had an enlarged left ventricle on their first visit, 18 had a normal ventricle on their last visit, the researchers reported.

According to Dr. Lewis Hsu, chief medical officer of the Sickle Cell Disease Association of America, “These intriguing results suggest that hydroxyurea has benefits for another organ system in sickle cell disease.”

He said statistics suggest that the anti-sickle cell impact of hydroxyurea is greater than its benefit from simply increasing the oxygen carrying capacity of the blood.

But, he added, more study is needed.

“The retrospective study of cardiac remodeling without a uniform starting point for hydroxyurea and a modest number of patients has many limitations,” Hsu said. “I see this article as a call for further rigorous studies on this topic of preventing heart complications from pediatric age.”

The results were published online September 16 in the journal Blood advances.

SOURCES: Arushi Dhar, MD, pediatric cardiologist, Cohen Children’s Medical Center, Zucker School of Medicine at Hofstra / Northwell, Hempstead, NY; Lewis Hsu, MD, pediatric hematologist and chief medical officer, Sickle Cell Disease Association of America, Hanover, Maryland; Blood advances, Sep 16, 2021, online

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