About Blood Disorders

Hemoglobal® is interested in helping children with all blood diseases. At present, we focus our efforts in three blood disorders: thalassemia, acute lymphoblastic leukemia, and haemophilia.

Thalassemia: This inherited hemoglobin disorder, the disease on which Hemoglobal® first focused its attention so many years ago in Sri Lanka, is the most common anemia throughout Asia. The two cornerstones of treatment for thalassemia are red blood cell transfusions and iron-chelating drugs (which remove the iron that inevitably accumulates from long-term blood transfusions). Ideally, and in most higher income countries, blood is tested for viruses and for elements that may result in transfusion reactions. Blood is also matched (which is called phenotyping) to the blood group of the child who is to receive the blood. As well, in most higher income countries, iron-chelating therapy is monitored precisely to avoid drug toxicities. For children undergoing this life-long treatment, multidisciplinary comprehensive supportive care programs can address the needs of children and adolescents.

In many countries in Asia, for children affected with thalassemia, the standards of care set in higher income countries are sadly lacking. For example, in Kolkata, most patients are transfused without adequate testing for viruses. Because facilities for phenotyping are also inadequate, mismatched blood may cause problems. Most patients cannot afford treatment with iron chelating drugs, and for those who do receive these drugs (usually in doses which are inadequate for the prevention of complications), monitoring for safety and effectiveness is inadequate. Bone marrow transplantation, which offers a potential cure for some patients, cannot be afforded by majority of the children of India.

Acute lymphoblastic leukemia (ALL) is a cancer of the white blood cells known as lymphoblasts, which in ALL are overproduced in the bone marrow, inhibit the production of normal cells, and spread to other organs. ALL is most common in childhood, with a peak incidence at ages two to five years. Complications include fever, increased risk of infection, and an increased tendency to bleed. In higher income countries, ALL is treated with years of specific chemotherapy, the safety and effectiveness of which is monitored with specialized laboratory support. Complications such as infection and fever are evaluated and treated with antibiotics. Patient support networks along with rehabilitation services are available. Cure is achieved in over 80% of affected children.

But in Kolkata, for example, one in three children with ALL do not undergo the specialized adequate analysis of the bone marrow necessary for appropriate treatment to be provided. Appropriate chemotherapy is provided to only about half of eligible patients. Inadequate laboratory support often results in delayed evaluation of complications, and many essential antibiotics are not available. Patient support is inadequate, and overall, children — who could be saved in another country with added resources — die after a markedly compromised quality of life.

Haemophilia is an inherited blood disease which impair the body’s ability to control blood clotting, or the stoppage of bleeding once a blood vessel is broken. When a blood vessel is injured, a temporary scab forms but the coagulation factors that are normally necessary to maintain the blood clot are not produced. A haemophiliac does not bleed more intensely than someone else, but may bleed for a much longer time: even a minor injury may result in blood loss lasting days or weeks and in areas such as the brain or inside joints, this can be fatal or permanently debilitating. In Kolkata, managing children with hemophilia, as in those with thalassemia and leukemia, is also substantially halted by financial constraints. Fewer than 1% of these children receive what is known as “prophylactic treatment” with Factor VIII/IX (the clotting protein(s) which they cannot produce); in higher income countries, this is often used to prevent complication of joint bleeds, stiffness and immobility. In general, in Kolkata, bleeds are managed with plasma preparations which increase the risk of transfusion-transmitted infections such as hepatitis B and hepatitis C.