a·my·o·troph·ic lat·er·al scle·ro·sis
ˌāˌmīəˌträfik ˌladərəl skləˈrōsəs
noun: amyotrophic lateral sclerosis
a progressive degeneration of the motor neurons in the central nervous system, leading to progressive weakness of the skeletal muscles, including muscles for breathing
THE RESPIRATORY OUTCOME OF ALS
The hallmark signs of early respiratory muscle weakness are the inability to fully inhale and exhale, a weak cough and a reduced voice volume.
As respiratory muscle weakness advances, signs include: slow or rapid shallow breathing, inability to breathe when lying down, the need to sit upright to breathe (orthopnea), disrupted sleep, disrupted speech, frequent yawning, anxiety, restlessness, headaches, marked fatigue, daytime drowsiness, and visible signs of accessory muscle use. Late symptoms (if go untreated) include: shortness of breath/respiratory distress while sitting upright, paradoxical breathing (when the chest moves inward during inhalation instead of moving outward), excessive sleepiness, hallucinations and panic attacks.
Noninvasive (nasal/oral mask) ventilation (NIV): a ventilator (small machine) delivers room air through the nose and/or mouth and into the lungs to support breathing. NIV can be tolerated unless oral secretions become severe.
- Tracheostomy invasive ventilation (TIV): a ventilator (small machine) delivers room air through a tracheostomy ( a surgical opening in the neck) and into the lungs to support breathing. TIV is long-term breathing support.
- Comfort (hospice) care: if breathing support is no longer tolerated or desired.