A multidisciplinary approach is necessary for the management of FRDA patients (1).
Evaluation of the patient following an initial diagnosis is important to establish the extent of the disease. e.g. -
- neurological assessment
- speech and swallowing assessment
- ECG and echocardiogram for evidence of cardiomyopathy
- random blood glucose concentration for evidence of diabetes mellitus
- ophthalmologic assessment if any ophthalmologic symptoms are present
- hearing assessment (1)
Currently there are no approved treatments that can alter the course of underlying condition in majority of progressive ataxias. The following antioxidant agents have been considered potential treatments for slowing the progression of FRDA
- Idebenone is the most studied pharmacological agent in FRDA
- coenzyme Q10 and vitamin E
- iron chelator (2)
Treatment of FRDA focuses on supportive measures:
- prostheses, walking aids, wheelchairs, and physical therapy as necessary to aid in carrying out daily tasks
- occupational therapy
- for spasticity - physical therapy including stretching programs, standing frame and splints, medications (baclofen and botulinum toxin)
- for scoliosis and foot deformity - operative and/or non-operative orthopaedic interventions,
- speech and language therapy
- dysphagia - dietary modification, nasogastric or gastrostomy feeding (for the late stages of disease)
- cardiac conditions - anti-arrhythmic agents, anti-cardiac failure medication, anti-coagulants, and pacemaker/ implantable cardioverter defibrillator insertion
- diabetes - diet and, if necessary, oral hypoglycemic agents or insulin
- hearing aids, microphone, and receiver as needed
- psychological (counseling and/or pharmacologic) support for patients and family (1)
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