OACRS Best Practice - Communication and Practice Regarding Ventilation Options for Youth with Duchenne Muscular Dystrophy (DMD)

Last modified by Support on 2012/03/01 11:09

www.oacrs.ca

Contact(s)/Date(s)

Eric Ferguson McMaster Children’s Hospital – Ferguson@hhsc.ca, 905-521-2100 ext 74479 (Marilyn Wright, Teresa Carter, Cindy Van Halderen, Reny Vaughn, Marg Otter)

Goal

To ensure all clients with DMD will have information about options for ventilation support across the lifespan. This includes preparing youth with DMD and their families to respond to the progressive nature of the condition and make decisions that are informed and meet their own values and wishes knowing that these choices may offer extended life expectancy as well as improved quality of life.

Measurement of Change (Assessment or Outcome Tools)

Not applicable.

Supporting Information

  1. Evidence Based Research “Recent advances in the respiratory care of the DMD patient have improved the outlook for these patients, and may caregivers have changed from a traditional non-interventional approach to a more aggressive, supportive approach. Despite the availability of new technologies to assist patients with DMD, many families do not receive sufficient information regarding their options in diagnosis and management of respiratory insufficiency. “ American Thoracic Society, 2004.  There are long-term health benefits (respiratory, cardiac, psychosocial) to providing ventilation support.  A survey of OACRS centres and families of youth with DMD in Ontario indicated parents and youth have many resources at their disposal but they receive information about ventilation supports in an inconsistent manner.
  2. Clinical Expertise - There is an ethical obligation to provide youth with DMD and their families with information regarding ventilation options to ensure they make informed decisions.
  3. Family/Client Values Information from surveys indicates families want more information and they want it earlier than they are receiving it currently. They like to receive information from a variety of sources including professionals, reading materials, web sites, and other families.

Intervention Protocols or Process Tools

  • Provide general information about respiratory function soon after diagnosis. This can be done in the context of explaining the progression of muscle weakness that includes cardiac and respiratory muscles.
  • There is variability among families as to when they would like to receive in-depth information. There is no ideal time. The best approach would be to ask families individually when they would like to have an in-depth discussion about options for ventilation support. Given the link between loss of ambulation and decline in respiratory function, this might be a good time to suggest a discussion.
  • Youth with DMD should be included in discussions to facilitate their input in decision-making.
  • Options for families who indicate they do not want to discuss the issue include providing regular brief information at each visit, offering written information, or suggesting separate meetings with parents before discussing options with the young person.
  • Good communication between Children’s Treatment Centres and neuromuscular clinics is essential to ensure appropriate support with difficult decisions.

References

American Thoracic Society. Respiratory care of the patient with Duchenne Muscular Dystrophy. Am J Respir Crit Care Med 2004;170:456-465.

Benditt JO & Boitano L. Respiratory support of individuals with Duchenne Muscular Dystrophy. Phys Med Rehabil Clin N Am. 2005;16:1125-1139.

Simonds AK. Recent advances in respiratory care for neuromuscular disease. Chest. 2006;130:1879-1886.

Simonds AK. Respiratory support for the severely handicapped child with neuromuscular disease: Ethics and practicality. Semin Respir Crit Care Med 2007;28:342-354.

Other

Full report being written.

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Created by OACRS Best Practice Committee on 2010/01/15 10:14