OMAN: Kuwaiti experts joined Omani, Bahraini and international doctors to discuss the treatment and management of the genetic blood disorder sickle cell disease in Oman last weekend. The speaker tour, sponsored by health leader Novartis Oncology, aimed to deliver up-todate clinical data and optimal practice in the treatment of the disease. According to Professor Adekunle Adekile, Head of Pediatric Hematology Unit, Mubarak Al-Kabeer Hospital, and Chairman, Department of Pediatrics, Kuwait University, Kuwait, who presented information on the disease registry in Kuwait, sickle cell disease is one of the most common genetic diseases in the world, especially in areas affected both currently and previously by malaria, as the sickle cell trait tended to provide protection against malaria infection.
Professor Adekile estimates that prevalence of the sickle cell trait is around 3 to 6 percent, with the disease affecting up to 1 percent – a significant number considering that it applies only to the local population. Recent implementation of a registry in Kuwait with the approval of the Ministry of Health has allowed local doctors to estimate that up to 600 patients suffer from the genetic disease, with almost 300 patients already registered.
“Children born with sickle cell disease tend to have two significant problems – anemia, which in itself comes with problems, and abnormal shaping of red blood cells, which tend to block small blood vessels, cause recurrent pain, and can affect any part of the body,” added Prof Adekile. Sickle cell disease is named after the abnormal sickleshape that red blood cells take in affected individuals. “In addition to the two major problems mentioned, there are other complications that must be addressed. Patients are very prone to bacterial infections and major organs, particularly the spleen, begin to malfunction. As a result, many children that don’t have access to specialized medical centres die in their first few years of life due to infection,” Prof Adekile warned.
Ischaemic stroke is one of the most devastating complications that affects children and, to a lesser extent, adults with sickle cell disease. Approximately 24 percent of patients experience stroke before the age of 45, and the risk is highest in the first decade of life. Early diagnosis is crucial to the management of the disease, with local doctors stressing the importance of newborn screening and familial education seminars to help prevent or mitigate complications.
“The registry is incredibly important because we need to compare regional data to the rest of the world. It is also useful for planning purposes and formulating appropriate and uniform management protocols.It would be even more helpful if there was a combined Gulf registry where we could pool data and increase quality of care across the entire region,” added Prof Adekile. The general public tends to be more informed of the blood disorder thalassemia, and more awareness is required for sufferers of sickle cell disease.
“Contrary to thalassemia, patients with sickle cell disease are not recognizable in their developmental appearances and diagnosis, though easy, occurs via screening,” said Dr.AndroullaEleftheriou, Head of the International Thalassemia Foundation, which also covers sickle cell disease, and who spoke at the Oman congress. “We very much look forward to providing information that policy makers and the medical community can use to help implement policies to assist in the management of these blood disorders,” she added.
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