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

NCT00718003 | Terminated

• 1 other identifier: Internal-15860
• Study Type: observational
• Target: 10
• Locations: 1 country
• Sites: 1

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.

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

Outcome Measures

Primary Outcomes (1)

• 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

Study Arms (1)

ALS

Subjects having either definite or probable ALS by El Escorial Criteria.

Eligibility Criteria

• Age: 20 Years - 85 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

ALS clinic patients at MDA/ALS Center of Hope.

You may qualify if:

• 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.

You may not qualify if:

• 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).

Sponsors & Collaborators

• Drexel University College of Medicine: lead
• MDA/ALS Center of Hope: collaborator

Study Sites (1)

MDA/ALS Center of Hope

Philadelphia, Pennsylvania, 19104, United States

Location

MeSH Terms

Conditions

Amyotrophic Lateral Sclerosis; Neurodegenerative Diseases; Motor Neuron Disease; Movement Disorders

Condition Hierarchy (Ancestors)

Spinal Cord Diseases; Central Nervous System Diseases; Nervous System Diseases; TDP-43 Proteinopathies; Neuromuscular Diseases; Proteostasis Deficiencies; Metabolic Diseases; Nutritional and Metabolic Diseases

Study Officials

• Terry Heiman-Patterson, MD

  Drexel University College of Medicine

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 14, 2008
• First Posted: July 18, 2008
• Study Start: May 1, 2005
• Primary Completion: February 1, 2013
• Study Completion: February 1, 2013
• Last Updated: March 5, 2013

Record last verified: 2013-03

Locations

US (1)