Sickle cell drug reduces risk of dying
Bermudians who suffer from the painful symptoms of sickle cell anaemia could soon be taking a drug that lowers the risk of dying from the inherited disease, researchers have claimed.
The drug hydroxyurea was found to cut the nine-year mortality rate of adult sickle cell patients by 40 percent compared to those not taking the drug, according to a study published in the Journal of the American Medical Association on Tuesday.
"Most people who take this medication will feel better - we now know they will live longer," said study author Martin Steinberg of Boston Medical Center.
Steinberg said only a minority of sickle cell anaemia patients take hydroxyurea, which is commonly used to reduce the frequency and severity of sickle cell attacks that cause pain in the bones, joints, and abdomen, along with other symptoms such as fever and breathing problems.
At least one Bermudian woman has been taking hydroxyurea for six years, and swears by it.
Gwen Smith said the drug has been available to patients here for about six to seven years.
"I feel so much better now, previously I had nine to eleven crisis (attacks) a year, but now I average two to three and they and they are less severe," said Ms. Smith.
"Hydroxyurea was like it was custom made for me - my problem was that my body was not not producing strong haemoglobin cells and hydroxyurea help produce strong fetal haemoglobin.
"When I first started taking hydroxyurea, children under 12 and patients with mild attacks were not eligible for (it), now it is available at younger ages.
"I suggest that if this drug is recommended by a sickle cell patient's doctor that they take it without hesitation."
Suspicions had been raised that hydroxyurea could increase the risks of leukaemia and infections, but an editorial in the journal said the drug's benefits may override such concerns.
"With this new finding that hydroxyurea reduces mortality in patients with sickle cell disease, the present cautious and conservative approach to hydroxyurea therapy will need to be reassessed," wrote Dr. Debra Weiner of Children's Hospital in Boston in an accompanying editorial in the journal.
Sickle cell anaemia, which is primarily found in those of African, Latino and Mediterranean ancestry, causes an abnormality in red blood cells, which form into a curved "sickle" shape that gives the disease its name. There is no cure, though more sufferers are surviving into adulthood.
The disease occurs in one of every 500 African-American births and one in every 1,000 to 1,400 Hispanic births.
The study of 233 adults also found that sufferers who had more frequent attacks or more acute pain also had higher mortality rates.
"We conclude that underlying disease severity remains critical to determining the prognosis of adult (patients), but hydroxyurea may mitigate disease severity," the study said.
With files fromAP
