Clinical trial NCT00718003: Noninvasive Examination of the Work of Breathing in Patients With Amyotrophic Lateral Sclerosis (ALS).
Official title: Noninvasive Examination of the Work of Breathing in Patients With Amyotrophic Lateral Sclerosis (ALS).
Study type: OBSERVATIONAL
Overall status: TERMINATED
Conditions: Amyotrophic Lateral Sclerosis
Neurodegenerative Disease
Motor Neuron Disease
Keywords: Amyotrophic Lateral Sclerosis
Cerebrospinal Fluid
Neurodegenerative Disease
Motor Neuron Disease
Autonomic Nervous System
Neurodegenerative Diseases
Movement Disorders
Lead sponsor: Drexel University
Sponsor class: OTHER
Collaborators: name: MDA/ALS Center of Hope
First posted: 2008-07-18
Enrollment: 10
Sex: ALL
Minimum age (years): 20
Maximum age (years): 85
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: CASE_ONLY
Time perspective: CROSS_SECTIONAL
Sampling method: NON_PROBABILITY_SAMPLE
Study population: ALS clinic patients at MDA/ALS Center of Hope.

Brief summary: The literature to date indicates that noninvasive positive pressure ventilation (NIPPV) provides effective noninvasive ventilator support, prolongs survival, and improves quality of life (QOL) in Amyotrophic Lateral Sclerosis (ALS) patients. It is generally recommended to patients when their pulmonary function testing demonstrates a drop to 50% forced vital capacity (FVC). One result of using NIPPV may be a reduction in the work of the breathing which would lead to decreased caloric needs. However, the work of breathing and the effects of noninvasive ventilation on caloric use have not been studied in patients with ALS. This is extremely important since there may be a reduction in the caloric needs when ALS patients are placed on NIPPV and if the caloric intake is not adjusted, overfeeding can occur. Overfeeding with too many calories can lead to an increase in carbon dioxide which would actually worsen the respiratory failure.

The overall aim of this project is to evaluate how many calories are used by ALS patients while at rest, when placed on NIPPV, and when breathing against a resistance. This will be accomplished using a metabolic cart during these activities. At present, the metabolic cart is routinely used in ALS patients at the time of feeding tube placement to calculate caloric needs. Using the cart to calculate the caloric expenditure on and off the ventilator will aid in calculating the work of breathing and the effects of NIPPV on work of breathing.


Eligibility criteria: Inclusion Criteria:

* Patients with probable or definite ALS by El Escorial Criteria between ages 20-85 will be included for evaluation. They must have progression of disease over the preceding 6 months and duration of disease less then 5 years.

Exclusion Criteria:

* Patients who do not meet clinical criteria of the diagnosis including those with pure upper motor neuron syndromes (PLS) or pure lower motor syndromes (SMA, Multifocal conduction block, motor neuropathies).

Primary outcomes:
measure: Calories Used with and without NIPPV
timeFrame: 20 minutes
Secondary outcomes:
Other outcomes:
Locations:
city: Philadelphia
country: United States
facility: MDA/ALS Center of Hope
state: Pennsylvania
Study officials:
affiliation: Drexel University College of Medicine
name: Terry Heiman-Patterson, MD
role: PRINCIPAL_INVESTIGATOR
References: