World Thalassemia Day is a global healthcare event commemorated every year on May 8th to raise awareness about the condition among the local public and policymakers, apart from supporting and strengthening the morale of the victims who have battled for years with this fatal disease.
Thalassemia is an inherited blood disorder that affects your body’s ability to produce hemoglobin and healthy red blood cells. Types include alpha and beta thalassemia. Thalassemia may cause you to experience anemia-like symptoms that range from mild to severe. Treatment can consist of blood transfusions and iron chelation therapy.
Thalassemia is classified as trait, minor, intermedia and major to describe how severe the condition is. These labels represent a range where having a thalassemia trait means that you may experience mild anemia symptoms or no symptoms at all. You may not need treatment. Thalassemia major is the most serious form and usually requires regular treatment. There are two types of thalassemia — alpha thalassemia and beta thalassemia — named after defects in these chains.
Alpha thalassemia
You inherit four genes, two from each parent, that make alpha globin protein chains. When one or more genes are defective, you develop alpha thalassemia. The number of defective genes you inherit will determine whether you experience anemia symptoms and (if so) how severe they’ll be.
- One defective or missing alpha gene means that you won’t experience symptoms. Another name for this condition is alpha thalassemia minima.
- Two defective or missing alpha genes means that if you experience symptoms, they’ll likely be mild. Another name is alpha thalassemia minor.
- Three defective or missing alpha genes means that you’ll experience moderate to severe symptoms. Another name for this condition is Hemoglobin H disease.
- Four defective or missing alpha genes usually results in death. In those rare instances when a newborn survives, they’ll likely need lifelong blood transfusions. Another name for this condition is hydrops fetalis with Hemoglobin Barts.
- Beta thalassemia: You inherit two beta-globin genes, one from each parent. Your anemia symptoms and how severe your condition is depend on how many genes are defective and which part of the beta globin protein chain contains the defect.
Moderate and severe thalassemia are often diagnosed in childhood because symptoms usually appear within the first two years of your child’s life. People with thalassemia can get too much iron in their bodies. This can be due to the disease or to frequent blood transfusions. Too much iron can result in damage to the heart, liver, and glands that make and release hormones.
People with thalassemia have a higher risk of infections. This is especially true if they’ve had their spleens removed. In many cases of thalassemia, patients have their spleens removed. The spleen is an organ that helps the body fight infection. It also helps remove old or damaged blood cells.
Often, thalassemia happens along with the destruction of a large number of red blood cells. This causes the spleen to get bigger and work harder than usual.An enlarged spleen can make anemia worse. It also can reduce the life of red blood cells received in a transfusion. If your spleen grows too big, your health care professional might recommend surgery to remove it.
Thalassemia can cause the spongy tissue inside some bones, called bone marrow, to expand. That makes bones widen. It can lead to an irregular bone structure, especially in the face and skull. Expanding bone marrow also makes bones thin and brittle. That raises the chance of broken bones. Congestive heart failure and irregular heart rhythms can also be linked with severe thalassemia.
- Chelation therapy. This is the treatment to remove excess iron from your blood.
- Removing the excess iron is vital for your helth. To help rid your body of the extra iron, you might need to take an oral medication, such as deferasirox (Exjade, Jadenu) or deferiprone (Ferriprox). Another drug, deferoxamine (Desferal), is given by needle. Not The selection of these medicines must be made according to the doctor’s advice.
- Stem cell transplant. Also called a bone marrow transplant, a stem cell transplant might be an option in some cases. For children with severe thalassemia, it can eliminate the need for lifelong blood transfusions and drugs to control iron overload.
But unfortunately, apart from blood transfusion, no other life-saving medicines are being provided to thalassemia patients in the Kashmir Valley and nor do these patients yet have access to bone marrow transplant facilities.
While in almost all other states of the country, as well as in the Jammu division, such medicines are being provided free of cost at the government level. In the Kashmir Valley, around 100 thalassemia patients are registered, with more than 50 of them at the Children’s Hospital in Srinagar and over 30 at GMC Srinagar. Among these, approximately 70% are children.
There is an urgent need for government institutions to focus their attention on this humanitarian and serious issue. Thalassemia patients in the Kashmir Valley must be provided with life-saving medicines, and a bone marrow transplant centre should be established as soon as possible, as it is critically needed. This would help these patients live a relatively better life and allow them to move forward with hope for the future.
These steps are not just necessary—they are urgent. With timely intervention and support, patients with thalassemia can live longer, healthier, and more fulfilling lives. Let us unite to give these young warriors a fighting chance and restore their hope in a brighter future.
(Author is president Hemophilia society kashmir)
