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.
- 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.
- 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).