THALAVITAL - first global rehabilitation program for Thalassemia and hemoglobin disorders
Improvement in the treatment of ß-Thalassemia, especially in chelation therapy have fortunately led to prolonged survival. Many patients with Thalassemia major and most patients with Thalassemia intermedia reach adulthood.
Typically these patients develop diseases like osteoporosis and cardiac failure normally only seen in old patients about 30 years earlier. These complications usually involve several organ systems, stressing the need for a comprehensive care by internal medicine and physical therapy.
Cardiac complications are the commonest cause of death in patients with Thalassemia. Cardiac arrhythmias can occur at any stage of the disease. This stresses the need for cardiac monitoring during exercise in the rehabilitation. Cardiac dysfunction is a frequent complication of iron overload. It has to be assessed with echocardiography and T2-cardiac MRI-scanning.
Fortunately it is reversible with intensive (if necessary intravenous or combined subcutaneous and oral) chelation therapy. The intensive chelation therapy can be done during rehabilitation. Thalassemia patients with cardiac dysfunction can effectively be treated by aerobic endurance training. Individual capacity has to be defined by a stress ECG and by spiroergometry before and during the course of rehabilitation.
Patients with Thalassemia intermedia frequently (23%) develop pulmonary hypertension. It is important to measure the degree of pulmonary hypertension by echocardiography before starting aerobic training.
Endocrine complications include short statue, hypogonadotropic hypogonadism, Diabetes mellitus, hypothyroidism and hypoparathyroidism. These complications have to be treated by an internist during the Rehabilitation.
The iron level of the liver is routinely assessed by T2 MRI of the liver. Iron overload may lead to liver cirrhosis which may cause several complications, e.g. hepatic encephalopathy, ascites and oesophageal varices. Older patients with Thalassemia may have acquired hepatitis C infection which may also cause liver cirrhosis.
More than 50 % of the Thalassemia patients develop osteoporosis. Typically, osteoporosis in Thalassemia patients becomes clinically apparent at an age of 30-40 years. Osteoporosis has to be treated with medication and a special rehabiltation program. Complications of osteoporosis include low back pain, vertebral fractures and other fractures e.g. hip fractures. Long term deferrioxamine therapy may cause vertebral disc disease which can be diagnosed with MRI of the spine. Treatment is done in department of neurosurgery/spine centre. Patients with Thalassemia intermedia often develop extramedullary hematopoetic masses which may cause problems by compression of nerve roots or other structures.
THALAVITAL rehabilitation program
For every patient, an individual Thalassemia rehabilitation program will be developed according to his personal needs, depending on the specific complications and his physical capacity.
Standard rehabilitation time is 6 weeks. Including personal therapy targets.
Each patient gets personally involved in the success of his rehabilitation program.