Medical Management of FCMD

Fukuyama congenital muscular dystrophy (FCMD) is a type of muscular dystrophy that is present at birth and affects the Japanese population almost exclusively. The condition’s primary symptoms are progressive weakness in skeletal muscles and impaired brain development.

There currently is no cure for FCMD, but medical management can slow the disease’s progression and improve patients’ quality of life.

Physiotherapy

Physiotherapy, including stretching exercises, can help promote mobility and prevent contractures (limited movement of joints).

Adaptive devices

Adaptive devices such as long leg braces can improve standing posture. Wheelchairs can increase mobility.

Surgery

Spinal fusion surgery can preserve breathing function when scoliosis (abnormal curvature of the spine) is present. It also can help improve sitting balance.

Respiratory care

Respiratory function should be assessed regularly in individuals with advanced FCMD and who are older than 10.

Non-invasive ventilation can relieve respiratory distress, especially at night.

FCMD patients frequently develop respiratory tract infections, which must be treated immediately. These infections are the most common cause of hospital admissions and death in FCMD patients.

Diet

Caloric intake and nutritional status should be assessed regularly. A feeding tube (gastrostomy) may be necessary to ensure adequate caloric intake.

Heart function

Heart function should be monitored routinely by chest X-ray and echocardiography (ultrasound of the heart) in individuals older than 10.

Acid reflux

FCMD patients may develop gastroesophageal reflux disease (GERD), which can be treated surgically or with medication.

Seizures

Patients who experience seizures should be clinically evaluated every three months. An electroencephalogram (a procedure that measures the electrical activity in the brain) should be performed every six months. Anticonvulsant medications can help control seizures.

 

Last updated: Aug. 21, 2019

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