Noninvasive Examination of the Work of Breathing in Patients With Amyotrophic Lateral Sclerosis (ALS).

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.

Study Overview

• Status: Terminated
• Conditions: Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Neurodegenerative Disease

• Study Type: Observational
• Enrollment (Actual): 10

Contacts and Locations

Study Locations

• United States
  • Pennsylvania
    • Philadelphia, Pennsylvania, United States, 19104
      • MDA/ALS Center of Hope

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 20 years to 85 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

ALS clinic patients at MDA/ALS Center of Hope.

Description

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).

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort
ALS; Subjects having either definite or probable ALS by El Escorial Criteria.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Calories Used with and without NIPPV	Indirect calorimetry will be performed using a metabolic cart to determine caloric use over a 20 minute period without NIPPV. Following this, the test will be repeated over a 20 minute period with NIPPV.	20 minutes

Collaborators and Investigators

• Sponsor: Drexel University College of Medicine
• Collaborators: MDA/ALS Center of Hope
• Investigators: Principal Investigator: Terry Heiman-Patterson, MD, Drexel University College of Medicine

Study record dates

Study Major Dates

• Study Start: May 1, 2005
• Primary Completion (Actual): February 1, 2013
• Study Completion (Actual): February 1, 2013

Study Registration Dates

• First Submitted: July 14, 2008
• First Submitted That Met QC Criteria: July 16, 2008
• First Posted (Estimate): July 18, 2008

Study Record Updates

• Last Update Posted (Estimate): March 5, 2013
• Last Update Submitted That Met QC Criteria: March 1, 2013
• Last Verified: March 1, 2013

More Information

Terms related to this study

• Keywords: Amyotrophic Lateral Sclerosis; Motor Neuron Disease; Autonomic Nervous System; Neurodegenerative Disease; Movement Disorders; Neurodegenerative Diseases; Cerebrospinal Fluid
• Additional Relevant MeSH Terms: Pathologic Processes; Metabolic Diseases; Central Nervous System Diseases; Nervous System Diseases; Neuromuscular Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Sclerosis; Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Neurodegenerative Diseases
• Other Study ID Numbers: Internal-15860