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Recommendation of the American Thoracic Society on Respiratory Care in Duchenne Muscular Dystrophy

In August 2004 the American Thoracic Society adopted an official statement on the appropriate respiratory care for patients with Duchenne muscular dystrophy (DMD) [ American Journal of Respiratory and Critical Care Medicine Vol 170. pp. 456-465, (2004)].

The purpose of the statement is to educate the practitioner about new approaches and therapies that are available for the management of the respiratory complications of DMD. Although respiratory disease in DMD is its major cause of morbidity and mortality, there is inadequate awareness of its treatable nature.

The consensus statement-working group, formed in May 2001, was comprised of physicians who were experts in DMD respiratory care at institutions that handled multiple patients with DMD. The statement is a consensus guideline that represents the consensus of the 15 members of the working group. Most of the literature on DMD is results of relatively small studies with small patient numbers it was necessary to convene the panel of experts to reach a consensus.

The following two lists itemize the areas covered in physician recommendations. The web site of the Muscular Dystrophy Association has a summary of the respiratory consideration that physicians should follow in treating DMD. You can find these at: http://www.mdausa.org/research/040824respcare.html Included on the MDA web site is a link to the original publication.

1. Areas in which recommendations are made:

Routine evaluation of respiratory care

Multidisciplinary care
Respiratory care
Routine evaluation of respiratory function

End of life directives
Nutrition
Sleep evaluation
Cardiac involvement

2. Management of the disease recommendations:

Airway clearance

Manual techniques
Mechanical techniques
Mucus mobilization devices

Respiratory muscle training
Noninvasive nocturnal ventilation
Other therapies
Daytime noninvasive ventilation
Continuous invasive ventilation
Scoliosis
Corticosteroids in the management
Patient education
Long-term care issues
End of life care

In addition to the above recommendations for physicians, there are recommendations for patients. Patient education is an important part of the patient care. Education of the patient should begin as soon as the diagnosis is confirmed and should be ongoing. Its goal should be co-management of care by the patient and family in collaboration with their health care provider. Goals of the education should be:

Understand the natural history of DMD.
Recognize early signs and symptoms of pulmonary complications.
1. Visit a pediatric respiratory specialist early in the disease, between ages 4 and 6, and before confinement to a wheel chair to obtain baseline pulmonary function values.
2. Obtain guidance at this time on the potential respiratory complications and assessment of the need for therapy.
Understand and make informed choices about treatment options for airway clearance and respiratory insufficiency. Risks, benefits and quality of life issues should be discussed for the various ventilatory options, including noninvasive ventilation as well as ventilation via tracheostomy.
Provide anticipatory guidance on the assessment and management of intercurrent respiratory illness.
Understand the role of the medical devices in use and have sufficient skill to operate them effectively.
Understand and make informed decisions about the end of life care.

It is important that patient and physician work together to accomplish the above goals.