Table 4. [Treatment of Manifestations in Individuals with HMERF]. - GeneReviews®

Manifestation/Concern	Treatment	Considerations/Other
Distal leg weakness	Ankle-foot orthoses	To optimize independent ambulation
Other mobility aids such as canes, walkers, or wheelchairs	May be required later in disease course
Inactivity	Exercises & activities suggested by PT consultation	To prevent continued loss of physical function
Nocturnal hypoventilation	Noninvasive ventilation w/BiPAP or CPAP
Respiratory failure	Mechanical ventilatory support as needed
↑ susceptibility to respiratory tract infections	Influenza vaccination
Gradually progressive nature of this disease	OT & social services support

Manifestation/Concern

Treatment

Considerations/Other

Distal leg weakness

Ankle-foot orthoses

To optimize independent ambulation

Other mobility aids such as canes, walkers, or wheelchairs

May be required later in disease course

Inactivity

Exercises & activities suggested by PT consultation

To prevent continued loss of physical function

Nocturnal hypoventilation

Noninvasive ventilation w/BiPAP or CPAP

Respiratory failure

Mechanical ventilatory support as needed

↑ susceptibility to respiratory tract infections

Influenza vaccination

Gradually progressive nature of this disease

OT & social services support

From: Hereditary Myopathy with Early Respiratory Failure

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