Thalassemia is a life-threatening, inherited blood disease affecting a person's ability to produce hemoglobin. Thalassemia is the most common single gene disorder in the world and is endemic in areas of the world affected by malaria. Children with thalassemia need monthly transfusions from the first year of life for the rest of their lives. These blood transfusions are life saving but, over years, result in iron accumulation in the vital organs of the body. As a result, after age two, patients require medicine called deferoxamine to remove transfused iron before it causes damage to the liver and heart. A medical pump is needed to administer this drug in controlled amounts over a 12 hour period each day.
In higher income countries, children with thalassemia grow up with access to all the treatments they need to ensure they will grow to healthy adulthood. Through bone marrow transplant some patients can even be cured of thalassemia. In developing countries such as Sri Lanka the treatment costs of medical pumps, necessary drug amounts, and travel costs to and from the nearest clinic are more than many families can afford. Hemoglobal is trying to change this troubling situation for the children of Sri Lanka -- where the decades-long civil war, the devastating 2004 tsunami, and harsh poverty have combined to threaten the lives and health of children with thalassemia.
What kind of medical equipment is needed to treat thalassemia?
are needed to administer the drug that helps save children's lives. The battery operated pump is usually used over a 12 hour period each day of the patient's life, slowly releasing the drug. For many families in developing countries these pumps are too expensive and they cannot get access to the life-saving drug as a result. For other families they may be lucky enough to own a pump, but as thalassemia is a genetic disease there is often more than one child per family with thalassemia. Under these circumstances children often share one pump between two.
Average price: $200 - $350 CAD for locally made or commercial brand pumps
is a clinical machine that measures patient blood levels. These levels are essential for evaluating patient progress and if they are receiving necessary amounts of their prescribed drug. In the few Sri Lankan clinics where hemoglobinometers have been purchased, bloodwork can be assessed quickly and accurately. In those clinics that do not have hemoglobinometers patients may need to wait up to 2-3 days in the clinic to receive their blood results. One hemoglobinometer in each clinic can save over a thousand days in hospital waiting time for these families and bed space for the already overcrowded hospitals.
Average price: approx. $2,500 CAD
is used to separate plasma from the different types of blood cells. It is an essential medical device used in the diagnosis of thalassemia.
Average price: approx. $TBD
is currently the most popular drug used to reduce iron build-up in a thalassemia patient's blood and internal organs. The key to survival in thalassemia is the prevention of iron overload. Because the life-saving medicine is a huge expense, it is in short supply in developing countries, especially so in Sri Lanka's northern and eastern provinces where hospitals are used by the army, and children with thalassemia are a lower priority. Sadly, the limited amounts of deferoxamine, as provided by the health care system, are often not enough to save a child. Families with more than one child with thalassemia are often hardest hit by this shortage. An assured regular supply of deferoxamine can ensure that a child will survive.
Average cost for an assured 5 year supply of deferoxamine for a child under the age of 5: $5,000 CAD
Average cost for an assured 5 year supply of deferoxamine for a child over the age of 5: $10,000 CAD
What other treatments are needed for patients with thalassemia?
: Families of children with thalassemia face unique challenges. The intensive treatment regimen, travel times to and from home, and often a sense of guilt or shame associated with a genetic disease cause a higher prevalence of divorce and stress within families of children with thalassemia. Hemoglobal has funded the salary of a counsellor/social worker in the National Clinic in Kurunegala to address some of these challenges. One of our goals is to place a counsellor in each large clinic in Sri Lanka to help educate people about thalassemia and destigmatize this disease.
Cost of an annual salary for a Social Worker/Genetic Counsellor in Sri Lanka: approximately $800-$1000 CAD.
: Many Sri Lankans with thalassemia live in rural areas far from the nearest clinic. Monthly travel to and from the clinics where they will receive their life-saving transfusions and medical supplies is a major burden on many rural families. Parents are responsible to bring in their children to clinic and thus the travel fares are double and the parent cannot make an income for the days spent in clinic. Poverty is one of the greatest problems facing the effective treatment of thalassemia.