About ALS
noun: amyotrophic lateral sclerosis
a·my·o·troph·ic lat·er·al scle·ro·sis
ˌāˌmīəˌträfik ˌladərəl skləˈrōsəs
a degenerative disease of the motor neurons in adults causing weakness of the affected skeletal muscles, including the respiratory muscles
THE RESPIRATORY OUTCOME OF ALS
The hallmark signs of initial respiratory impairment are a weak cough, the inability to fully inhale and exhale, and a reduced voice volume.
ALS ultimately results in respiratory failure due to the progression of respiratory muscle weakness. Respiratory failure may be prevented and treated with use of noninvasive or invasive breathing support. People with ALS have life choices and need to indicate their wishes in their Advance Directives.
*** Education on life choices is necessary
for making informed choices and fulfilling
patient wishes. ***
LIFE CHOICES
Noninvasive (nasal/oral) ventilation (NIV): positive pressure air is delivered from a ventilator (small, portable machine) through the nose and/or mouth and into the lungs to support breathing. NIV can be tolerated unless oral secretions become severe. Thus, NIV is temporary, short term treatment.
Tracheostomy invasive ventilation (TIV): positive pressure air is delivered from a ventilator (small, portable machine) through a tracheostomy (surgical opening in the neck) and into the lungs to support breathing. TIV supports breathing beyond respiratory failure, despite severe oral secretions. Use of TIV can be long term. Progressive disability does not cause death. ALS patients have the right to stop TIV, if desired.
Comfort (hospice) care: indicated if NIV is no longer tolerated due to severe oral secretions and tracheostomy invasive ventilation is not desired.
The Mechanics of Respiration
ALS AFFECTS THE MUSCLES FOR BREATHING, CALLED THE “VENTILATORY PUMP.”
Respiratory failure will occur unless prevented and treated with use of noninvasive (short-term) breathing support or invasive (long-term) breathing support for maintaining adequate ventilation.
The diaphragm is the principal muscle of inspiration for breathing in. Breathing out is ordinarily a passive process.