Becker Muscular Dystrophy Follow-up

Updated: Mar 17, 2021
  • Author: Benjamin R Mandac, MD; Chief Editor: Stephen Kishner, MD, MHA  more...
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Follow-up

Further Outpatient Care

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  • Rehabilitation management coordinates the administration of appropriate therapeutic modalities.

    • The physiatrist initiates and coordinates diagnostic studies, because muscular dystrophy may not have been diagnosed in these patients prior to the initial visit.

    • Routine health care issues include the recommendation of yearly influenza vaccinations, as well as the administration of pneumococcal vaccine.

    • Given the progressive nature of the BMD, anticipatory guidance is ongoing. The physiatrist provides recommendations for classroom accommodations and activity during a patient's school years. Work-related concerns during the patient's adult years of employment are primary issues, with work modification scenarios and the use of assistive devices being prominent concerns.

  • Cardiopulmonary evaluations include pulmonary function and electrocardiographic testing.

    • Pulmonary evaluations are important in tracking the progression of muscular weakness affecting ventilation.

    • A simple clinical test with a spirometer may be employed to measure maximal expiratory volume during routine clinic visits.

    • A pattern of falling maximal expiratory volumes over time may indicates the need for formal pulmonary function to determine the need for ventilatory support.

    • Nighttime ventilatory support with a mask or nasal bilevel positive airway pressure is commonly used if a rising pCO2 is noted. The results from sleep studies also may suggest progressive difficulty in nighttime ventilation.

    • Cardiovascular health supervision guidelines are available [14]

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Deterrence

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  • Prenatal diagnosis is available for a woman with an at-risk pregnancy who has been identified as having a family history of muscular dystrophy. Identification of dystrophin gene exon deletions in a male fetus points to the risk of a child with muscular dystrophy. Couples may elect to terminate the pregnancy if the fetus is affected.

  • Carrier status may be determined in the mother and siblings of a proband.

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Complications

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  • Progressive disability

  • Dilated cardiomyopathy

  • Respiratory symptoms, with a potential need for ventilatory support

  • Joint contractures

  • Scoliosis

  • Dysphagia

  • Functional constipation

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Prognosis

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  • See Mortality/Morbidity.

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Patient Education

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  • Well in advance of the projected necessity, discuss the decision, with the occurrence of pulmonary failure, to place a tracheostomy tube for positive-pressure ventilation, as well as for airway and secretion management. Inform the patient, family members, and/or caregivers of the implications of placing the patient on a ventilator. Early education can help the patient and his/her family to determine advanced directives.

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