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Thalassemia: The Long Road From The Bedside Through The Laboratory To The Community

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Weatherall, D. Thalassemia: the long road from the bedside through the laboratory to the community. Nat Med 16, 1112–1115 (2010). Https://doi.Org/10.1038/nm1010-1112

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Nutrition Tips For Thalassemia Patients

Imagine going about your daily routine when suddenly you feel overwhelming fatigue, weakness, and pale skin. These everyday incidents may indicate a condition called thalassemia. 

Proper nutrition plays a vital role in managing thalassemia and supporting overall health. In this article, we will explore what thalassemia is, the influence of age on thalassemia risk, and provide important dietary tips specifically tailored for thalassemia patients.

What Is Thalassemia?

According to Dr Phani Madhuri, Senior Consultant, Obstetrician and Gynaecologist, Apollo Cradle and Children's Hospital, Jayanagar, Bangalore, "Thalassemia is a hereditary condition marked by decreased or abnormal production of haemoglobin, the protein responsible for delivering oxygen in red blood cells." There are two main types of thalassemia: alpha thalassemia and beta thalassemia, each with varying degrees of severity. Thalassemia major, the most severe form, often requires regular blood transfusions to maintain healthy haemoglobin levels.

The Effect Of Age On Thalassemia Risk

According to Dr Sowmya Raghavan, Obstetrician and Gynecologist, Apollo Cradle and Children's Hospital, Chennai, "Although thalassemia impacts individuals of both genders, there is an increasing concern among women who are planning to conceive after the age of 30." As individuals with thalassemia transition into adolescence and adulthood, they may face additional challenges due to hormonal changes, increased iron overload, and the need for lifelong management. Proper nutrition becomes even more important to support overall health and reduce the risk of complications.

Also Read: World Thalassemia Day 2020: Understanding Symptoms, Types, Causes And Complications Of Thalassemia

Dietary Tips For Thalassemia Patients

As per Neha Srivastava, a Consultant and Dietitian at Ivory Hospital in Greater Noida, it is advised that thalassemia patients should avoid excessive consumption of foods rich in iron to prevent potential complications. The role of nutrition is of utmost significance in aiding the recovery of thalassemia patients and safeguarding them from adversities. Calcium, being a crucial mineral for maintaining the strength and agility of bones, holds paramount importance. 

Adequate Calories And Macronutrients

Thalassemia patients often require more calories and nutrients to support their increased energy needs and overall health. Consume a well-balanced diet that includes adequate amounts of carbohydrates, proteins, and healthy fats. Consult with an expert to determine the appropriate amounts for your specific needs.

Protein-Rich Foods

Protein is essential for the production of healthy red blood cells. Include lean meats, poultry, fish, legumes, dairy products, and plant-based protein sources in your diet.

Essential Micronutrients

Thalassemia patients may have specific nutrient deficiencies. Ensure sufficient intake of vitamins and minerals like vitamin D, folic acid, vitamin B12, and calcium. A healthcare professional can guide supplementation if necessary.

Hydration And Fluid Intake

Proper hydration is important for individuals with thalassemia to maintain healthy blood circulation. Ensure an adequate fluid intake by:

  • Drinking plenty of water throughout the day. Staying hydrated helps prevent complications and supports overall health.
  • Limiting caffeine and alcohol consumption, as they can lead to dehydration and interfere with nutrient absorption.
  • Regular Monitoring

    Regular monitoring of iron levels, overall health, and nutritional status are essential for thalassemia patients. Consult with a healthcare professional, such as a haematologist or a registered dietitian, who can provide personalised guidance and support.

    [Disclaimer: The information in this article is provided by a registered medical practitioner. However, we recommend you consult your healthcare provider for accurate diagnosis and treatment.]

    Image Credit: Freepik


    An Overview Of Thalassemia Intermedia

    Causes, Treatments, and More

    Hero Images/Creative RF/Getty Images

    Medically reviewed by Gagandeep Brar, MD

    Thalassemia is a group of blood disorders affecting hemoglobin, a protein that's a component of red blood cells (RBC). People who inherit thalassemia are unable to produce hemoglobin normally, leading to anemia (low RBC count) and other complications.

    Hero Images / Creative RF / Getty Images

    Thalassemia can be broken into three categories:

  • Trait: Person carries the gene for mutation but does not have the disease. This is sometimes called thalassemia minor.

  • Intermedia: Person with thalassemia somewhere between trait and major.

  • Major: Person with thalassemia who requires lifelong RBC transfusions.

  • Diagnosis

    Although most of the time thalassemia major will be identified on the newborn screen, people with thalassemia intermedia may not be identified until years later. These people are generally identified during the process of a routine complete blood count (CBC). The CBC will reveal a mild to moderate anemia with very small red blood cells. This can easily be confused with iron deficiency anemia.

    Diagnosis is confirmed by a hemoglobin profile (also called electrophoresis). In beta thalassemia intermedia and trait, this testing reveals an elevation in hemoglobin A2 (a second form of adult hemoglobin) and sometimes F (fetal). Alpha thalassemia intermedia is generally called hemoglobin H disease, as this is the predominant hemoglobin seen on the profile.

    Complications

    Complications of thalassemia intermedia include:

  • Iron overload

  • Osteoporosis: Weakening of the bones

  • Extramedullary hematopoiesis: This refers to enlargement of spleen, liver and/or bone marrow to increase red blood cell production. The bones most notably affected are in the skull—forehead and cheekbones.

  • Hypogonadism: Reduced maturation of sexual organs. This may prevent puberty from occurring naturally.

  • Gallstones

  • Blood clots

  • Causes

    There are two reasons why people with thalassemia intermedia develop iron overload.

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  • Repeated red blood transfusions: Even though children with thalassemia intermedia generally don't require transfusions every 3 to 4 weeks like children with thalassemia major, they still may require several blood transfusions every year. Each red blood transfusion received is like an intravenous (IV) dose of iron. The body does not have a great way to remove this iron from the body. So over time, these repeated transfusions may result in the development of iron overload, although generally later in life (adulthood) than people with thalassemia major (childhood).

  • Increased absorption of iron from food: The body recognizes that the bone marrow is not doing a good job producing hemoglobin and red blood cells. Hepcidin is a protein that blocks the absorption of iron. In thalassemia, hepcidin levels are low, allowing more iron to be absorbed than is needed. It is recommended that people with thalassemia intermedia follow a low-iron diet.

  • Treatments
  • Routine medical care: Not every person with thalassemia intermedia will require treatment, but it is important to continue close medical care to monitor for complications.

  • Transfusions: People with thalassemia intermedia may require transfusions, but generally not as frequently as people with thalassemia major until adulthood. Transfusion may be needed in times of increased growth and development (puberty), illnesses (particularly those with fever), pregnancy, or in preparation for surgery.

  • Folic acid: Some physicians may recommend folic acid daily to support RBC production.

  • Splenectomy: In thalassemia, the spleen may enlarge (splenomegaly) in an effort to improve RBC production. This is often ineffective and may worsen anemia and/or increase transfusion need. In these circumstances, splenectomy may be considered.

  • Hydroxyurea: Hydroxyurea is a daily medication taken by mouth in an effort to increase hemoglobin, thereby reducing the need for RBC transfusions.

  • Low-iron diet: Because people with thalassemia intermedia are at risk to develop iron overload from increased absorption of iron in the diet, a low-iron diet may be recommended. Some studies have shown that tea decreases the absorption of iron and may be recommended with meals.

  • Chelation: This treatment uses a medication that binds to iron, which is then eliminated from the body via urination or defecation.

  • Learning you have thalassemia intermedia can be shocking as you may not have had any symptoms. Be sure to follow up with your physician as scheduled so you can be monitored for potential complications.






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