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Becker Muscular Dystrophy Follow-up

  • Author: Benjamin R Mandac, MD, MD; Chief Editor: Stephen Kishner, MD, MHA  more...
 
Updated: Sep 03, 2015
 

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[12]
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Deterrence

See the list below:

  • 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

See the list below:

  • 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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Contributor Information and Disclosures
Author

Benjamin R Mandac, MD, MD Chief Physical Medicine and Rehabilitation, Medical Director Pediatric Rehabilitation Kaiser Permanente at Santa Clara

Benjamin R Mandac, MD, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Physical Medicine and Rehabilitation

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Kat Kolaski, MD Assistant Professor, Departments of Orthopedic Surgery and Pediatrics, Wake Forest University School of Medicine

Kat Kolaski, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Physical Medicine and Rehabilitation

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kishner, MD, MHA Professor of Clinical Medicine, Physical Medicine and Rehabilitation Residency Program Director, Louisiana State University School of Medicine in New Orleans

Stephen Kishner, MD, MHA is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Elizabeth A Moberg-Wolff, MD Medical Director, Pediatric Rehabilitation Medicine Associates

Elizabeth A Moberg-Wolff, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Physical Medicine and Rehabilitation

Disclosure: Nothing to disclose.

References
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