Home Sleep Testing in Neuromuscular Disease Patients
Condition: Neuromuscular Disease
MeSH terms: Neuromuscular Diseases
Study type: Observational

Summary: 
      Lay Summary Patients with severe neuromuscular develop hypoventilation, which leads to
      elevated carbon dioxide levels. Measuring only oxygen saturation levels with pulse oximetry
      may be inadequate. End tidal carbon dioxide levels or arterial blood gases should be measured
      periodically, depending on the clinical condition of the patient. A thorough review of
      systems will help define any problems. Patients who are hypoventilating often have elevated
      carbon dioxide levels at night and complain of a morning headache, restlessness or
      nightmares, and poor quality sleep. This may cause daytime sleepiness. Insufficient
      respiration with hypoxia may occur later, especially if the lung is damaged by chronic
      aspiration.

      We propose to evaluate the use of the Nonin LifeSense monitor in home evaluation of
      respiration, oxygen level, heart rate, and carbon dioxide level and to develop interpretation
      of the results that will lead to appropriate interventions for apnea, and insufficient
      respiration.

      Relevance to MDA Fewer than one per cent of the Muscular Dystrophy Association have
      pulmonologists as co-directors.Late referral of progressive restrictive lung disease leads to
      invasive support of respiratory failure. Early initiation of non invasive ventilation
      techniques requires patience on the part of the caregiver and exploration of mask interfaces
      and ventilation techniques. In addition, the development of new therapies, currently
      manifested through enhanced diagnostic accuracy, will require new signal for initiation and
      in the assessment of success or failure.

      Aims Aim 1. To assess the utility of a small portable device (LifeSense Monitor Nonin Medical
      Inc. Plymouth Minnesota) with extended recording capabilty to provide accurate diagnosis of
      hypoventilation.

      Aim 2. To provide an easily interpretable report defining sleep hypoxemia, hypercapnea, and
      apnea.

      Aim 3. To promote early evaluation and treatment of the respiratory problems in centers that
      do not have pumonologists as these are essential to prognosis, whether of survival or of
      quality of life, in neuromuscular diseases.
    


Intervention name(s): Evaluation for Nocturnal non invasive ventilation
Intervention type(s): Other
Intervention description(s): Patients will be referred for evaluation for NNIV is indicated in patients with diurnal Pa,CO2 >6.0 Pa; and 2) nocturnal Sa,O2 <88% for 5 consecutive min. End tidal CO2 representing equivalent values will be referred for NNIV
Observational model: Case-Only
Time perspective: Prospective
Sampling method: Non-Probability Sample
Study population: 
        Study Population Description Patients from the Neuromuscular Disease Clinic at Ventura
        County Medical Center

        Non-Probability Sample: invitation to volunteer

        Neuromuscular Disease
      
Keywords: Home sleep testing neuromuscular disease; Neuromuscular disease patients with FEV1<50% of predicted