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Thalassemia and Life-Style

March 8, 2014

Red Blood Cells14: General Health Care and Lifestyle in Thalassaemia 

Routine Health Care

Dental care
Thalassaemics who are untransfused, undertransfused, or begin transfusion at a later stage in the disease may have some malformations of the facial bones due to marrow expansion. This can affect growth of the teeth and cause malocclusion. Orthodontic care may be successful in improving masticatory function and/or correcting unaesthetic dental appearances.

Nutrition

General
Patients with thalassaemia do not have dietary requirements much different from other children and adolescents, unless they have complications. In general, a restrictive diet is easy to be prescribed but difficult to be maintained on long term. In thalassaemia, the patient already has a heavy treatment schedule and it is counterproductive to be burdened with further restrictions without the likelihood of clear benefit.

During growth, a normal energy intake, with normal fat and sugar content is recommended. During adolescence and adult life, a diet low in highly refined carbohydrates (sugar, soft drinks, snacks) may be useful in preventing or delaying the onset of impaired glucose tolerance or diabetes.

Iron
Increased iron absorption from intestinal tract is characteristic of thalassaemia (see Chapters 5 and 12). The amount depends on the haemoglobin level. Drinking a glass of black tea with meals reduces iron absorption from food, particularly in thalassaemia intermedia [DeAlarcon 1979]. There is no evidence that iron poor diets are useful in thalassaemia major however. Only foods very rich in iron (such as liver and some ‘health drinks’ or health vitamin cocktails) should be avoided.

Patients with thalassaemia should never be given iron supplements. Many baby foods, breakfast cereals and multivitamin preparations contain added iron, along with other vitamin supplements. The patient should make a habit of reading labels carefully.

Calcium
Many factors in thalassaemia promote calcium depletion. A diet containing adequate calcium (e.g. milk, cheese, dairy products, spinach and kale) is recommended. However, nephrocalcinosis is seen in some adults with thalassaemia major, and calcium supplements should not be given unless there is a clear indication. If nephrolithiasis is present, a low oxalate diet should be considered. Vitamin D may also be required to stabilize the calcium balance, particularly if hypoparathyroidism is present. Careful monitoring is required if supplements are used, however, to prevent toxicity.

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 (1 mg/day) may be given if this occurs. Thalassaemics 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, causing vitamin C deficiency
in some patients. Vitamin C may increase the ‘chelatable iron’, thus increasing the chelation efficacy with desferrioxamine. On the other hand, vitamin C ingestion increases labile iron and hence iron toxicity. Vitamin C also increases iron absorption from the gut. Therefore, supplements should not be taken except when combined with desferrioxamine treatment (see Chapter 5).

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. The doctor should recommend a regular intake of vegetable oils as part of the 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.

Zinc
Zinc deficiency may occur during chelation. Zinc supplementation must be given under close monitoring.

Substance abuse

Alcohol
Alcohol consumption should be discouraged in thalassaemic patients. Alcohol potentiates the oxidative damage of iron and aggravates the effect of HBV and HCV on liver tissue. If these three factors are all present, the probabilities of developing cirrhosis and hepatocarcinoma are significantly raised. Excessive alcohol consumption also results in decreased bone formation and is a risk factor for osteoporosis. Alcoholic drinks may have unexpected interactions with medications.

Smoking
Cigarette smoking may directly affect bone remodelling and is associated with osteoporosis.

Drug abuse
In many countries, drug abuse is common among adolescents and young adults. For an individual with a chronic disease, drug abuse can be a serious threat to a condition that is already endangered, by upsetting the delicate balance of factors affecting physical and mental health. This is the most realistic attitude and the doctor should help the patient to maintain this position. There is a danger that drug abuse may be seen as a compensatory way to be popular among peers or to fit their behaviour. Feelings of dependence, difference, and anxiety can motivate young people with thalassaemia to seek “normality” through an abuse habit. A frank discussion of these issues may help the patient gain insight about these pressures.

Recreational activities

Physical Activity
In general, physical activity must always be encouraged in patients with a chronic disease. Patients with thalassaemia should have a quality of life and life experiences as much like those of others as possible. There is no reason to prevent them 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.

Conditions to which the doctor must pay special attention include:

 Splenomegaly: the more enlarged the spleen, the more prudent the doctor must be in recommending avoidance of those sports and physical activities with significant risk of abdominal trauma.
 Heart disease: moderate physical activity is beneficial, if is matched to the clinical condition and its treatment
 Osteoporosis or back pain in adults may limit physical activity. Osteoporosis carries an increased fracture risk with contact sports and these should be avoided if osteoporosis is present.

Driving

No special attention is needed. In some countries, the presence of diabetes mellitus requires special checks and limitations.

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