Thalassemia is an inherited blood disorder characterized by abnormal hemoglobin formation, the protein in red blood cells responsible for carrying oxygen. This can lead to anemia, where the body lacks enough healthy red blood cells to deliver oxygen efficiently. Unlike other forms of anemia, increasing iron consumption can be dangerous for individuals with thalassemia. This article explores dietary recommendations for thalassemia patients to manage their condition effectively, utilizing the information provided and expanding upon it for clarity and comprehensiveness.
Understanding Thalassemia
Thalassemia is a genetic condition, meaning it's passed down from parents to their children. It's a recessive condition, requiring both parents to carry and pass on the trait for a child to have thalassemia major. When only one parent carries the gene, it results in thalassemia minor, where the individual is a carrier but may not exhibit significant symptoms.
Types of Thalassemia
There are different types of thalassemia, including thalassemia major and thalassemia minor. The severity of the condition varies depending on the specific genetic mutations inherited.
- Thalassemia Major: This is the most severe form, often requiring regular blood transfusions.
- Thalassemia Minor: Individuals with thalassemia minor are carriers of the trait and may experience mild or no symptoms of anemia.
Complications of Thalassemia
Thalassemia can lead to several complications, including:
- Anemia: Reduced hemoglobin levels lead to fatigue, weakness, and shortness of breath.
- Iron Overload: Frequent blood transfusions can cause excess iron to accumulate in the body, damaging organs like the heart and liver.
- Bone Problems: Thalassemia can affect bone marrow, leading to bone deformities and osteoporosis.
The Role of Diet in Managing Thalassemia
While medical treatments like blood transfusions and iron chelation therapy are crucial, diet plays a supportive role in managing thalassemia. The primary goal of dietary modifications is to minimize iron overload and support overall health.
Read also: The Hoxsey Diet
General Dietary Recommendations for Thalassemia Patients
The dietary needs of individuals with thalassemia can vary based on their health status, the severity of the condition, and treatment plans. Nutritional recommendations should be personalized based on the patient's health status and any complications they may be experiencing. For children, growth stage is a key consideration.
Balanced Diet
A balanced diet full of fruits and vegetables is often recommended for those with thalassemia minor. Even if you don’t have thalassemia-related anemia, eating this way will offer a range of vitamins and minerals that can help support the immune system.
Iron Intake Management
Managing iron intake is crucial for individuals with thalassemia, especially those receiving regular blood transfusions.
Avoid High-Iron Foods: Non-transfused thalassemia intermedia patients are encouraged to avoid high-iron and iron-supplemented foods. Children who have thalassemia and are transfused are still relatively anemic, so their bodies might still crave iron. Remind children to definitely avoid very high iron foods such as dried beef and other high iron beef products, even if they are craving it.
Limit Red Meat and Seafood: These contain high levels of readily absorbable “heme-iron” and increase the absorption of other forms of iron.
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Read Food Labels: On food labels, the percentage of iron in one serving of that food is usually listed. Recommended Daily Allowance of 18 mg/day. If the label says the food contains 8% of the daily recommended iron, multiply .08 by 18mg to get the mg iron from a serving of that food.
Foods to Include in Your Diet
- Plant-Based Proteins and White Meat: Eat more plant-based proteins and white meat (such as chicken and turkey).
- Non-Heme Iron Sources: Choose foods with the non-heme form of iron, which your body won’t absorb as well, including eggs, chocolate, cereals, root veggies such as potatoes and carrots, and dried fruits like raisins, peaches, plums, and figs.
- Dairy Foods: Eat dairy foods like milk, cheese, and yogurt for the calcium to prevent osteoporosis and to reduce the absorption of iron.
- Grains and Legumes: Include grains and legumes such as wheat, corn, oats, rice, beans, soy, lentils, and peas.
- Vitamin E Rich Foods: Increase foods rich in vitamin E, such as fruits (peaches and asparagus), vegetable oils (sunflower oil, soybean oil, olive, and corn oil), and nuts (peanuts, hazelnuts, almonds, and walnuts).
Foods and Drinks to Limit or Avoid
- Iron-Fortified Foods: Avoid consuming iron-fortified cereal.
- Red Meat and Seafood: Avoid red meat and seafood; these contain high levels of readily absorbable “heme-iron” and increase the absorption of other forms of iron.
- Vitamin C-Rich Foods with Iron: Avoid foods high in vitamin C or eat them separately from iron-rich foods (because vitamin C increases iron absorption), such as citrus fruits, tomatoes, and bell peppers.
- Cast Iron Cookware: Do not cook with cast iron cookware (e.g. a wok) because iron from the cookware can transfer onto the food.
Beverages
- Tea and Coffee: Drink tea and coffee, the compounds in coffee and tea (tannins) are known to decrease iron absorption. Non-transfused thalassemia intermedia patients are encouraged to drink tea with meals, which decreases iron absorption.
- Avoid Alcohol: Additionally, patients should avoid alcohol consumption due its properties in exacerbating oxidative stress caused by the excess iron and worsens liver damage, particularly in patients with hepatitis B or C.
Specific Dietary Considerations
Thalassemia Minor
If you have thalassemia minor, you may wish to monitor the amount of iron-rich foods you consume, but you’ll want to discuss this with a doctor. Individuals with thalassemia minor may need to limit the amount of iron in their diet. Healthcare professionals typically recommend a nutritious diet rich in fruits and vegetables and low in fats for people with the condition.
Transfused Thalassemia Patients
For transfused thalassemia patients undergoing iron chelation therapy, an iron-restricted diet is unnecessary and could negatively impact their quality of life.
Other Considerations
- Smoking: Finally, smoking is not recommended due to its common association with osteoporosis in thalassemia patients. Smoking and the use of recreational drugs are also discouraged for those with thalassemia minor as these can harm bone development, decrease your overall physical health, and negatively impact your mental health.
- Lactose Intolerance, Vegetarian Diets: For thalassemia patients who also have pregnancy, diabetes, lactose Intolerance, or vegetarian diets and prescribed medications such as oral chelators or bisphosphonates; these exceptional cases shall require specialized dietary counseling.
The Importance of Supplements
It’s always important to talk with a doctor or, better yet, a registered dietitian nutritionist (RDN) before adding any supplements to your diet. Too much of certain vitamins and minerals may cause health complications. Depending on your diet and current vitamin levels in your body, some supplements that may be recommended include calcium, vitamin D, folic acid, vitamin C, and zinc.
Multivitamins
A doctor may recommend a multivitamin, especially for young children with growth concerns. However, some multivitamins include a high amount of iron and may not be appropriate for those with thalassemia, so it’s important to discuss the best multivitamin with your child’s pediatrician or RDN.
Read also: Weight Loss with Low-FODMAP
Vitamin D
If the patient’s 25-hydroxyvitamin D level is below 20 ng/mL, Vitamin D supplementation (50,000 IU weekly) is recommended until levels stabilize. Patients with thalassaemia should not take additional calcium or vitamin D unless prescribed by their medical practitioner.
Folic Acid
Patients with thalassaemia who remain untransfused or are on low transfusion regimens have increased folate consumption and may develop a relative folate deficiency. Supplements (1mg/day) may be given if this occurs. Patients on high transfusion regimens rarely develop this condition, and usually have no need for supplements.
Vitamin C
Iron overload causes vitamin C to be oxidised at an increased rate, leading to vitamin C deficiency in some patients. Vitamin C may increase the ‘chelatable iron’ available in the body, thus increasing the efficacy of chelation with desferrioxamine. However, there is currently no evidence supporting the use of vitamin C supplements in patients on deferiprone, deferasirox or combination treatment. Indeed, vitamin C ingestion may increase iron absorption from the gut, labile iron and hence iron toxicity. Therefore, supplements should only be considered for patients on desferrioxamine (see Chapter on Iron Overload). Some drugs, such as aspirin and throat lozenges, as well as certain ‘health foods’, may contain vitamin C and should be avoided. A diet rich in fresh fruits, including citrus fruits and vegetables, is recommended.
Vitamin E
Vitamin E requirement is high in thalassaemia. Treating staff should recommend a regular intake of vegetable oils as part of a balanced diet. However, the effectiveness and safety of vitamin E supplementation in thalassaemia major has not been formally assessed and it is not possible to give recommendations about its use at this time.
Calcium
Calcium supplementation is advised if dietary intake is inadequate. Many factors in thalassaemia promote calcium depletion. A diet containing adequate calcium (e.g. milk, cheese, dairy products and kale) is always recommended. However, nephrolithiasis is seen in some adults with thalassaemia major, and calcium supplements should not be given unless there is a clear indication, instead a low oxalate diet should be considered.
Zinc
Zinc deficiency may occur during chelation, depending on the chelator, dose and duration. Zinc supplementation requires close monitoring.
Lifestyle Recommendations
In addition to dietary changes, lifestyle adjustments can significantly improve the quality of life for individuals with thalassemia.
- Exercise Regularly: Exercise regularly as recommended by a doctor. In general, physical activity must always be encouraged in patients with a chronic disease. Patients with thalassaemia should have a quality of life and range of life experiences as much like those of others as possible. There is no reason to prevent patients from engaging in physical activity to the limits of what they are capable of and interested in doing, unless there is a precise secondary medical condition. Moderate physical activity is beneficial, if is matched to the clinical condition and its treatment.
- Stay Up-to-Date on Vaccinations: Stay up to date on your vaccinations and take steps to avoid infections. Vaccines are a great way to prevent many serious infections. Children and adults with thalassemia should get all recommended vaccinations, including a flu vaccination.
- Mental and Emotional Well-being: Talk about your life experiences with family and friends. Join a support group (online or in person) for people affected by thalassemia. Having warm, supportive relationships is an important part of life. Romantic relationships can also offer a source of support.
- Genetic Counseling: You may also wish to consider undergoing genetic testing with romantic partners before deciding to conceive a child so that you can be more knowledgeable about the risks of having a child with thalassemia.
Medical Management
- Regular Monitoring: Serum ferritin is evaluated in adolescents. Iron overloaded individuals receive a liver biopsy. Early cardiac evaluation with Holter monitoring and stress ECHO cardiogram is done in individuals with significant hemosiderosis.
- Iron Chelation Therapy: Desferrioxamine is instituted early in the development of hemosiderosis. To help keep the iron stores from building up too fast, a medication called Desferal will be used in conjunction with a low iron diet.
Living a Normal Life with Thalassemia
If the disease is fully compensated by ideal treatment, an individual with thalassaemia major can enjoy a near-normal lifestyle and experience regular physical and emotional development from childhood to adulthood, including parenthood. Treating staff should promote such a progression by trying to reduce as far as possible the degree to which the disease interferes with the patient’s personal and social life. Where the disease cannot be fully compensated with proper transfusional schemes, the obstacles to a normal lifestyle should be taken into account with a realistic but positive approach, based on informing and encouraging the patient, and reviewing limitations on time and treatment schedule.
Practical Considerations
- Treatment Schedules: Manage treatment and monitoring schedules so as to minimise any unnecessary impact on normal daily activity.
- Confidentiality: The patient should have the right to decide if, when and with whom to talk about the disease. Staff should assure confidentiality of patient identity and data in all circumstances, trying to be compliant with local, International laws and rules on privacy, if not against patient’s rights. Help parents to be aware of disease-related issues early on in the patient’s life.
- School: If the patient’s haemoglobin levels are maintained close to the values recommended in this book, no relevant interference with academic performance should be seen. Where the haemoglobin level is allowed to fall too low, the patient may have difficulty in school. Although normal transfusion and follow up schedules many require a number of absences, these should not be to the extent where the patients’ school performance is negatively affected.
- Home: Splenectomised patients should be warned about the risk of having pets at home, due to the possibility of bites and this increased risk of septicaemia (Capnocytophaga canimorsus-associated). Patients with active viral hepatitis or other viral infections should take general measures to minimize or prevent the risk of transmission to the family.
- Work: In general, it is important for patients to have a positive attitude towards their ability to work. Well-treated patients generally do not face difficulties in performing work as a direct result of their disease.
- Sexual and Reproductive Health: Differences in appearance (facial features, height, and skin colour) may affect self-confidence and participation in social life. The general improvement in the health of patients with thalassaemia means it is now possible for them to have children spontaneously or by induction of pregnancy. Treating staff should help the patient and his/her partner to achieve a balanced position.
- Routine Health Care: There is no reason for patients with thalassaemia to skip or delay standard recommended vaccinations. Patients who are untransfused, under-transfused or who begin transfusion at a later stage in the disease may have some malformations of the facial bones due to marrow expansion. Orthodontic care may be successful in improving masticatory function and/or correcting unaesthetic dental appearances.
- Travelling: Travel carries a degree of risk, which increases if the patient cannot receive expert local treatment. If a patient is travelling to a remote country, it is vital that adequate travel insurance is obtained so that if serious complications develop, s/he can be flown home immediately, with provision of any necessary medical assistance. Travel plans should be coordinated with the patient’s transfusion schedule, in order to avoid receiving transfusions elsewhere, particularly if visiting areas where blood supplies carry a high risk of infection. Splenectomised patient should always travel with antibiotics, to assure prompt medication in case of fever, sepsis or animal bites.
The Importance of a Nutrient-Dense Diet
One of the most common questions nutritionists are asked is, ‘What should I be eating?’ In many ways, the diet for individuals with thalassemia is no different than for anyone else: the key is balance. However the needs for certain nutrients are much higher in thalassemia. Therefore, nutrient density is very important! This means every calorie must count. Rather than worrying about which specific foods are ‘good’ for you, or which foods are ‘bad’, it’s better to focus on choosing a variety of foods that are packed with vitamins, minerals, fiber and other nutrients.
Colorful Foods
Healthy foods we eat come in a variety of colors: kale (green), carrots (yellow), beets (red), red cabbage (purple). Each food derives its color from the rich concentration of antioxidants in its skin and flesh. The variety of colors come from a diverse range of anthocyanins (plant pigments) and antioxidants (for example, carrots and sweet potatoes are orange because of the antioxidant, beta-carotene).
MyPlate Guidelines
If you are not familiar with the ‘My Plate’ campaign from the USDA, you should take a look at it. It is a simple: set-up of a small plate which is divided into portions, roughly ¼ for protein, ¼ for grain/carbohydrates and ½ of the plate set aside for fruits & vegetables. If we ate all our meals this way, we would be consuming many more vitamins and minerals in our diet.
Getting Nutrients from Food
The best way to get all of our nutrients is through our food. Most nutrients are best absorbed when they come from our food. Food also contains much-needed fiber as well as other substances (phytochemicals, flavonoids) that are important for your health. When healthy foods are replaced by foods with poor nutrient density (e.g. empty calories) + dietary supplements, you miss out on all of the benefits of food.
Healthy Lifestyle Choices
Making healthy choices doesn't have to be complicated - simply begin by making one change in your daily routine, such as making sure to have one colorful vegetable every day. A healthy lifestyle is important for everyone. For people with thalassemia, it is especially important to know that a healthy lifestyle means “managing the disorder”. Although some people with thalassemia may have trouble participating in vigorous forms of exercise, many people with thalassemia can participate in moderate physical activities including biking, running, and walking. If a person with thalassemia has problems with their joints, there are many kinds of low-impact activities to choose from including yoga, swimming, or water aerobics.
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