Inadequate blood transfusion and improper iron chelation can lead to major cardiac complications. Consistently low levels of haemoglobin can cause the heart to beat fast to compensate for the inadequate oxygenation. This could cause enlargement of the heart. Iron overload, the consequence of regular transfusions, also affects the heart due to deposition of iron. This can lead to irregular heart-beat and decreased pumping ability. If not well-chelated and well-transfused, patients show cardiac complications towards the end of the second decade of their lives.
No compromises should be made with respect to the blood transfusion and iron chelation therapy. At the same time, for early detection of cardiac problems it is necessary to get tests done for Chest X Ray, Electro Cardiogram, Echo Cardiogram, Cardiac MRI on an annual basis. It would be also advisable to have an physical exercise schedule with the advice of the doctor.
Inadequate iron chelation can lead to deposition of iron on the endocrine tissues causing serious complications. This can lead to alterations of the growth hormaone axis, delayed puberty and hypothyroidism .Between the second to third decade replacement therapies may be required for various endocrine complications.
No compromises should be made with respect to blood transfusion and iron chelation therapy. Periodic height and weight measurements should be taken for detection of growth retardation. Annual consultation should ne started after three years of transfusion or as advised by the doctor.
Grossly inadequate blood transfusions lead to deformities of the skull and face due to increase in the bone mass as a result of a hyperactive bone marrow. Spontaneous fractures are commen with poorly transfused patients. Even patients with adequate blood transfusions and iron chelation show bone complications in the form of osteoporosis.
Periodic tests should be carried out for checking the bone density to ensure that the onset of osteoporosis is detected at an early stage. Treatment as advised by doctors, should commence.
Inadequate blood transfusions can lead to release of abnormal red cells, removal of which is the responsibility of the spleen. The constant bombardment of red cells leads to hyperactivity by the spleen. This hyperactivity may get carried to the extent of destruction of even healthy cells. Frequency of transfusions may increase due to early destruction of healthy red cells. At an advanced stage, this hypersplenism is visibly evident in the form of a bulge.
Once the blood requirement goes up it may be necessary to have the spleen removed. The procedure is called splenectomy. On being advised splenectomy, it is necessary to protect the body from infections. The child needs to be given Pneumococeal/Hib vaccines 4 weeks prior to the splenectomy and Penicillin prophylaxis life-long post the splenectomy.
Psychosocial support
Detection and management of the disorder can put the individual and family to tremendous strain and trauma. New demands may be placed on the roles and relationships of the patient, his siblings and parents. Patients and families with psychosocial problems should be immediately detected. Support should be provided for smoother assimilation in society by organizing the smooth running of existing treatment and promoting the development of parents’ associations.
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