Abstract
Amyotrophic lateral sclerosis (ALS) also known as Lou Gehrig’s disease is a relentlessly progressive, fatal disease. Progression of the disease results 3 - 5 years after diagnosis, often from respiratory failure. The diaphragm pacing system (DPS) is a device that stimulates the diaphragm to maximally contract so patients can breathe more effectively. It has been used in patients with neurologic injuries such as spinal cord injury and ALS. From an anesthetic perspective, both the surgery and the patient population present several unique challenges. This case series describes three patients with ALS who had the diaphragmatic pacemaker placed and the anesthetic management during those surgeries.
Highlights
Amyotrophic lateral sclerosis (ALS) known as Lou Gehrig’s disease is a relentlessly progressive, fatal disease involving both upper and lower motor neurons
Her recent pulmonary function tests (PFT) demonstrated a restrictive pattern with a forced expiratory volume in 1 second (FEV1) of 2.13 (74% predicted) and forced vital capacity (FVC) of 2.24 (62% predicted)
A forty-eight year old, wheelchair-bound female diagnosed with ALS 18 months prior to presentation was referred to general surgery for placement of a diaphragm pacing system (DPS) as treatment for respiratory insufficiency
Summary
Amyotrophic lateral sclerosis (ALS) known as Lou Gehrig’s disease is a relentlessly progressive, fatal disease involving both upper and lower motor neurons. The diaphragm pacing system (DPS) is a device that stimulates the diaphragm to maximally contract It is currently being utilized in patients with ALS to improve their ventilatory function and quality of life. This idea of aiding patients’ ventilation began in the 1950s, when phrenic nerve stimulation was used to treat patients with polio, with little success. Introduction of DPS before FVC is
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