A single mutation in the gene that produces hemoglobin, the protein that carries oxygen to the tissues of the body, causes the sickle cell anemia affecting 3 million people worldwide. These individuals suffer intense pain crises caused by the changing shape of a group of red blood cells,: these cells become elongated and sticky, and clog the capillaries used to transport them. As a result, the oxygen is not delivered to the tissues, causing pain, damage to the organs, skin ulcers and in some cases leading to blindness, or even life-threatening complications like acute chest syndrome and cardiac arrest.
All these complications are well known by Umut Gürkan, a young Turkish biomedical engineer who is trying to develop a technology to alleviate the suffering of these patients and to better understand their condition. Sickle cell anemia is the most common type of inherited blood disease. Furthermore, according to Gürkan, it affects areas with lower resources disproportionally. 'In Africa 800 babies are born with this disease every day, and more than half of them die during childhood due to the lack of early diagnosis and treatment," says the young man, who is currently an associate professor at Case Western Reserve University (USA).
The duration, intensity and frequency of the painful episodes sickle cell anemia patients suffer vary per individual and are very difficult to predict using current methods. These crises can occur more than six or seven times a year and can last for a few hours or up to two weeks. Normally those who suffer these episodes need to be hospitalized and treated with supplementary oxygen and a medicine called hydroxyurea which will reduces blockage in the blood vessels. According to Gurkan, while this medicine has been effective in reducing the frequency of these episodes in approximately half of the patients with sickle cell anemia, there is still no treatment capable of eliminating them completely.
In fact, the main complication for doctors is that it is difficult to anticipate when these painful episodes will occur or when are they will remit. Conventional blood tests do not provide useful information in this regard since they measure the number of blood cells and their volume but do not provide data regarding the shape of blood cells nor whether they have become stickier. The doctors can only confirm that the treatment is effective once the pain begins to subside, and cannot determine when the next acute episode may occur.