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Addressing gaps in care for patients with thalassemia

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Dr Sachin Suresh Jadhav, Group HOD – Haematology and Bone Marrow Transplant, HCG Cancer Hospital explains that thalassemia is a curable condition that can be managed effectively with blood transfusions and chelation therapy

Vaccines are an excellent strategy to protect against a wide range of dangerous illnesses. All recommended immunisations, including a flu shot, should be obtained by children and adults with thalassemia.

Childhood memories are core events that every child cherishes. But children with thalassemia spend most of the times in hospitals and blood transfusions. With such memories filled with games, sports and fun, children with thalassemia need not be robbed of this opportunity to build fond memories if they get the right care.

Thalassemia, a non-cancerous disease, is an inherited blood illness that occurs when the body does not produce enough haemoglobin, a protein that is essential for the formation of red blood cells. When there is insufficient haemoglobin, the body red blood cells do not function effectively and live for shorter period, resulting in fewer healthy red blood cells in the bloodstream.

Red blood cells transport oxygen to all of the body’s cells which is important to nourish the cells and help them function normally. When there aren’t enough healthy red blood cells, not enough oxygen is supplied to the rest of the body. This makes a person anaemic, a condition that makes a person feel weary, weak, or short of breath. Anaemia can be minor or severe in patients with thalassemia.

The effects of thalassemia on the body

As thalassemia causes the body to produce fewer red blood cells, where the patient may have symptoms of a low blood count. This may make them feel weak and fatigued. They may also experience:

  • Shortness of breath
  • Dizziness
  • Faster heart rate
  • Headaches
  • Leg cramps
  • Pale skin
  • Difficulty concentrating

Infection

Patients with thalassemia are more likely to develop infections. There are more chances of infection especially if the spleen is removed.

Bone deformities

The bone marrow, the dark spongy component in the center of bones produces the blood cells. Because the bone marrow is working harder than usual, it may grow. This causes the bones to expand, perhaps stretching them and making them thinner and more brittle.

Enlarged spleen

The spleen aids the body in fighting infections and filtering out undesirable materials such as old or broken blood cells. Thalassemia is frequently accompanied by a substantial number of red blood cells being destroyed. The spleen enlarges and works harder than usual as a result.

Challenges thalassemia patients face

No safe blood for transfusion – Sometimes, patients with thalassemia are at the risk of contracting hepatitis infection which is transmitted through blood.

High iron levels leading to organ damage – Patients, who often undergo blood transfusions are at the risk of iron poisoning. Red blood cells contain excess iron, and the iron from all the transfusions can accumulate in the body over time. When it accumulates, iron gets concentrated in the organs, including the heart, liver, and brain. It is making it difficult for these organs to function effectively. Every time a person receives a blood transfusion, their risk of developing the condition of “alloimmunization” increases. Alloimmunization occurs when a person’s body reacts to blood from a transfusion as it is hazardous to their immune system and attempts to eliminate it.

Fatality due to high iron content (Chelators) — Chelation may be used in thalassemia patients to remove the excess iron from the body. Chelation, while an effective therapy, can result in side effects like gastric issues, some amount of renal damage because of dehydration or in severe cases kidney failure.

Patients opt for transplant at a very late stage – The earlier the bone marrow transplant is done, the better the body can adapt to the new marrow. Very often, patients are too late in opting for a transplant. After the age of 7 years, the risk will be on a higher side, leading to an increase in the cost of the transplant.

Management of thalassemia

Thalassemia is a curable condition that can be managed effectively with blood transfusions and chelation therapy. Other healthy choices a person with thalassemia should consider include taking vaccines, eating healthy and nutritious food, exercising daily, and maintaining healthy and positive relationships.

Vaccines are an excellent strategy to protect against a wide range of dangerous illnesses. All recommended immunisations, including a flu shot, should be obtained by children and adults with thalassemia.

A diet rich in fruits and vegetables, and low in fat is best for supplying our bodies the vital nutrients they require. Foods heavy in iron may need to be limited for a person with thalassemia. Meat, seafood, and some plants like spinach contain iron. Other foods, such as cereal and orange juice may include additional iron. Individuals with thalassemia should consult their doctor about whether they should limit the quantity of iron in their diet.

Exercise contributes to better health outcomes. Although some patients with thalassemia may find it difficult to engage in intense exercise, many may engage in moderate physical activity such as biking, running and walking.

Coping with thalassemia can be challenging. It is advised to never hesitate to ask for help from a friend or a family member. For medical advice and guidance, it is best to talk to health experts.

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