Home Sleep Testing in Neuromuscular Disease Patients (HSTNMD)

Landon Pediatric Foundation

Status: Completed

Conditions: Neuromuscular Disease

Treatments: Other: Evaluation for Nocturnal non invasive ventilation

Study type: Observational

Funder types: Other

Identifiers

NCT00695591

LS01

Details and patient eligibility

About

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.

Enrollment: 9 patients

Sex: All

Ages: 8 to 50 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Clinical diagnosis of neuromuscular disease
• Must have home caregivers

Exclusion criteria

• No home caregiver

Trial design

9 participants in 4 patient groups

1SevereRLD,NMD

Description:

Patients with FEV1\<40%

Treatment:

Other: Evaluation for Nocturnal non invasive ventilation

2VerySevereRLD,NMD

Description:

FEV1\<30%

Treatment:

Other: Evaluation for Nocturnal non invasive ventilation

3NINV

Description:

FEV1\<25%,on non invasive ventilation

Treatment:

Other: Evaluation for Nocturnal non invasive ventilation

ModerateRLD,NMD

Description:

Patients with FEV1 40-50% of predicted

Treatment:

Other: Evaluation for Nocturnal non invasive ventilation