Heart problems are common complications of sickle cell anaemia (SCA), and they can lead to severe illness symptoms and even death.

A new study published in the journal Blood Advances is the first to show that hydroxyurea therapy can improve or even reverse cardiac problems in SCA patients. The findings, according to the researchers, highlight the importance of early screening for sickle cell-related cardiac problems, even in people who aren’t experiencing any symptoms.

People with sickle cell illness frequently experience heart problems. Among these include heart enlargement and a reduced ability to relax heart muscles, a condition known as diastolic dysfunction, which can lead to heart disease, heart failure, and death. Long-term hydroxyurea treatment, on the other hand, appears to improve and even reverse some of these abnormalities, according to the researchers.

“Early treatment with hydroxyurea should be offered to all children with sickle cell anemia starting at 9 months of age, regardless of disease severity,” said lead researcher Dr Arushi Dhar.

Close cardiac monitoring of children with sickle cell anemia, especially asymptomatic kids, should begin as soon as possible, according to her.

“At our institution, all children with sickle cell anemia are referred for baseline cardiac evaluation starting at 5 years of age,” Dhar said.

In children with the condition, abnormalities such as enlargement or thickening of one chamber of the heart and excessive blood pressure affecting the lungs are prevalent.

In the United States, sickle cell anaemia is the most prevalent genetic red blood cell condition. According to the US Centers for Disease Control and Prevention, it affects around 100,000 Americans, including one in every 365 Black people and one in every 16,300 Hispanic Americans.

Red blood cells, which are normally spherical, become crescent or sickle-shaped in those individuals and then break apart, clump together, and attach to the blood channel walls. This reduces blood flow, which can result in cardiac problems, extreme discomfort, joint and organ damage, and stroke.

Hydroxyurea, one of the drugs licenced to treat the illness by the U. S. Food and Drug Administration, inhibits the development of sickled cells. However, the precise mechanism by which it operates is unknown. According to the Sickle Cell Disease Association of America, the medicine can help patients experience fewer pain crises and potentially live longer.

However, this is the first study to demonstrate that hydroxyurea can also cure heart damage associated with sickle cell disease, according to Dhar.

Her team gathered data on 100 children and adolescents with sickle cell disease for the study (aged 3 to 22). Half had hypertrophy or thickness of the left ventricle of their hearts. Additionally, some developed a form of hypertension (pulmonary hypertension) that affects the lungs and right side of the heart.

Sixty subjects were on hydroxyurea. The study discovered that 25 patients who had been on the medicine for less than a year were considerably more likely to have left ventricle enlargement. The researchers highlighted that patients using hydroxyurea had improved heart outcomes over the long term.

Patients who took the medicine for a longer period of time were less likely to develop an enlarged or thickened left ventricle. The researchers observed that among 34 patients who initially had an enlarged left ventricle, 18 had a normal one at their most recent visit.

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

According to him, the statistics indicate that the anti-sickling effect of hydroxyurea is more essential than the advantage of simply increasing the oxygen-carrying capacity of blood.

However, he added, additional research is necessary.

“Studying cardiac remodeling retrospectively with no uniform starting point for hydroxyurea and a modest number of patients has many limitations,” Hsu said.

“I see this paper as a call for additional rigorous studies on this topic of preventing cardiac complications starting in the pediatric age.”

The findings were published in the journal Blood Advances.