New Hope for Sickle Cell AnemiaTreatment

New Hope for Sickle Cell Anemia Treatment. the shape of normal red blood cells are round. But people with sickle cell disease (sickle cell anemia) of red blood cells have a crescent-shaped. Blood disorders sickle cell disease is indeed incurable, but now there is new hope for treatment.

During this treatment given to patients with sickle cell anemia aims to control and relieve symptoms. Sickle cell patients are advised not to be too tired or doing heavy activity so that the amount of oxygen in the blood decreases. It also avoids damage to red blood cell hemoglobin (Hb) did not go down.

The study conducted by researchers from the University of Michigan Health System in America and University of Tsukuba in Japan found that when the expression of TR2 and TR4 protein increases, the level of fetal hemoglobin produced in mice with sicle cell also increased more than 2-fold and reduced organ damage .

New Hope for Sickle Cell Anemia Treatment

The researchers believe this concept is the first study that aims to target specific proteins to prevent the disease. The study has been published in the Proceedings of the National Academy of Sciences.

Experiments increased TR2 and TR4 protein is expected to trigger the production of red blood cells sickle cell anemia patients, thus increasing the expectations of potential new treatments to prevent organ damage sufferer.

Dr. Andrew D Campbell, a pediatrician and director of the Pediatric Comprehensive Sickle Cell Program at the UM Cancer Center, said most patients with sickle cell anemia is diagnosed early in childhood when normal adult hemoglobin replaces fetal hemoglobin.

"But the severity of the disease can be very different and is associated most strongly with the existing level of fetal hemoglobin in red blood cells," said Campbell as reported from MedicalNewsToday, Thursday (03/11/2011).

Currently the drug hydroxyurea was approved by the FDA (the U.S. drug regulatory agency) and is known to increase fetal hemoglobin levels in patients with sickle cell anemia and a large number of patients respond to treatment. But long-term effects of hydroxyurea has not been known clearly, especially in children.

Dr. Campbell and colleagues describe the use of laboratory mice. They found a way to increase fetal hemoglobin levels by modulating the expression of TR2 and TR4.

Dr. James Douglas Engel, professor and chair of UM's Cell and Development Biology Department says that the average fetal hemoglobin was 7.6 percent in mice with sickle cell anemia. Whereas in mice that carried the expression of TR2 and TR4 modulation of fetal hemoglobin has an average 18.6 percent.

Sickle cell anemia is improved and the number of red blood cells increased in mice with sickle cell anemia who do TR2 and TR4 expression modulation.

The researchers say that in mice with sickle cell anemia which is a human model, which is modulated by TR2 and TR4 expression HBF increased from 7.6 percent of total hemoglobin 18.6 percent.

Accompanied by an increase in hematocrit from 23 percent to 34 percent and reduction in reticulocytes from 61 percent to 18 percent. And showed a significant reduction in hemolysis.

Researchers also describe how the complications of the disease has been reduced in mice with sickle cell anemia. Moreover, in mice by modulating the expression of TR2 and TR4 decreased hepatosplenomegaly, liver parenchyma necrosis, and inflammation.

Dr. Engel said more study is needed to determine whether such an approach would also work well in humans. Clinical trials are needed to determine the effect of such therapy in humans.

Sickle cell disease or sickle cell anemia is a blood disorder in which patients inherit mutations in the hemoglobin gene that causes changes in shape and effectiveness of red blood cells.

Red blood cells should be round and soft, being rigid and crescent-shaped (crescent). So that the crescent shape of red blood cells can not pass through small blood vessels.

Problems began to develop the disease within the first few weeks after birth, when fetal hemoglobin is replaced by the adult hemoglobin. The disease has affected hundreds of thousands of people around the world, who have experienced this disease with a lifetime of debilitating pain. The disease causes chronic organ damage and the age of sufferers are getting shorter.

Some babies are born with fetal hemoglobin with a higher level, indicating a less severe complications and live longer. So by increasing fetal hemoglobin is one way to treat the disease.