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Sickle Cell Disease Cases Are Increasing

Sherry Webb is given an injection for pain relief by a nurse at the Sickle Cell Center in Truman Medical Center, Wednesday, March 7, 2007 in Kansas City, Mo. Pain management is vital in the care of patients suffering from Sickle Cell disease.
Sherry Webb is given an injection for pain relief by a nurse at the Sickle Cell Center in Truman Medical Center, Wednesday, March 7, 2007 in Kansas City, Mo. Pain management is vital in the care of patients suffering from Sickle Cell disease.

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Joe DeCapua
Basic health interventions may significantly reduce deaths among young children with sickle cell anemia. The illness causes the body to produce sickle or disc shaped red blood cells making it difficult for them to transport oxygen from the lungs. The number of newborns with the inherited blood disease is increasing, especially in sub-Saharan Africa.


A new study in PLOS Medicine says by 2050 over 400-thousand babies will be born every year with sickle cell anemia. That’s an increase of about 100-thousand per year. Most of those births will occur in Nigeria, Democratic Republic of Congo and India. The three countries accounted for 75 percent of sickle cell newborns in 2010.

Dr. Frederic Piel led the research by the University of Oxford, Imperial College and the KEMRI/Wellcome Trust Research Program in Kenya.

“It’s a genetic disorder and if you inherit one copy of the gene from one of your parents, you don’t have any symptoms and you’re called a carrier or a heterozygote individual. If you inherit two copies from your parents, then you have sickle cell anemia, which is quite severe and lethal in countries where there is no treatment available,” he said.

While most of the sickle cell cases currently are concentrated in a few countries, Piel said there is a growing global burden.

It was initially limited to malaria endemic areas, but because of population movement it’s now common in many other parts of the world, if not all – in particular in the United States or in the United kingdom, where they have put in place newborn screening programs for this disorder. So this is clearly a global burden and it’s going to increase and populations are still moving from countries where the prevalence of this disorder is high to countries where the prevalence is lower because of the globalization context.”

He said that it’s very difficult for developing countries to deal with this health issue on their own. However, many of those countries have been working to the reduce mortality rate for children under age five. This includes better pre and post natal care and improved nutrition. It’s one of the Millennium Development Goals and gives sickle cell newborns a better chance of survival.

“In terms of sickle cell anemia it means that in newborns, who would previously have died at the early stage of their life undiagnosed, can now be diagnosed because they are surviving that five year age limit. And then they can start presenting symptoms or kind of clinical complications associated with sickle cell anemia, for which simple interventions can reduce the long-term burden,” he said.

Some of the simple interventions after diagnosis, he said, include penicillin prophylaxis and basic vaccinations, which can prevent infections and long term complications. Better access to basic health care services is also necessary.

Couples planning to have children can also be screened for the disease using a simple blood test.

“The first step is kind of screening parents and then trying to provide them with genetic counseling and define which pregnancies are at risk and which ones aren’t. And in developing countries there are some new technologies, which can help [in] selecting which fetuses or which eggs might be unaffected. But this is really costly and still kind of new technology. So I don’t this is a solution at the moment for low and middle income countries. I think education and screening are key steps in the short term to try to reduce this burden,” said Piel.

The U.S. Institutes of Health describe sickle cells at stiff and sticky, blocking blood flow to limbs and organs. The result can be acute or chronic pain, damage to organs and an increased risk of infection, among other things.

The NIH reports treatments – including fluids, drugs and oxygen therapy -- can relieve symptoms and complications and reduce pain. A cure may be possible in a small number of cases through blood and bone marrow stem cell transplants. However, this is not something readily available or affordable in developing countries.

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