Neurodegenerative-Neuromuscular Diseases and Airway Clearance Therapy

 

Advances in medicine have been responsible for longer overall survival in patients with neurodegenerative-neuromuscular diseases. According to a recent report in RTMagazine, an increase in the number of patients also means an increase in emergency room (ER) activity due to respiratory complications.

Caregivers and even some nurses may not be aware that patients with diseases such as Duchenne muscular dystrophy, ALS, or those with spinal cord injuries may need assistance with airway clearance.

These patients are generally too weak to breath deeply or cough. That encourages mucus to collect in a patient’s airways leading to severe infection and unnecessary visits to the ER.

Dr. Jeremy Orr, pulmonary specialist in neuromuscular respiratory weakness at UC San Diego, suggests that the medical profession should be more proactive and apply airway therapy upon the first sign of a weak cough.

Muscle weakness often results in patients who rely on sending out signals rather than express their discomfort verbally. The congestion may be building up but its severity may be overlooked by their physicians. More often clinicians and care givers expect and react to respiratory problems when caring for people with the more common diseases such as asthma or COPD.

Dr. Orr’s Protocol

To help hospital staff identify patients in need of respiratory assistance as early as possible, Dr. Orr developed a program specifically for respiratory therapists. He also provided recommendations to manage the therapy.

Devices That Aid Respiration

An MI-E machine is one of several that are able to prevent infections. The machine functions by slowly inflating the lungs. At that point it changes to a negative pressure thus producing rapid exhalation. The device acts as a natural cough and draws up any accumulated mucus or fluids from the airways.

MI-E therapy is most often delivered by using a face mask. The technician must be certain that the mask fits properly in order to avoid leaks which cause poor lung expansion.

Philips Respironics CoughAssist T70 is used to clear secretions from central airways. It has an additional feature that captures data that can be used by healthcare providers to design other effective therapies.

Another note of caution pertains to the settings for airway pressure that must not be set too low, or proper airway clearance will not be achieved. Patients are advised to bring their machine to their physician to ensure proper settings.

Expert Opinions

Studies have shown the benefits of using both cough assist devices and respiratory physiotherapy for patients in ICU on ventilators. Using these two devices together proved to be twice as effective as physiotherapy by itself.

The studies also show that the risk of pneumonia generally associated with ventilators is reduced by using the MI-E machine.

Other Cough Assist Devices

A multi-therapeutic portable device called VOCSN manufactured by Ventec Life systems combines five therapies. The therapies are: suction, cough, oxygen, nebulization (mist inhaled into the lungs) and ventilation. Patients can change therapies by simply pressing a button.

VOCSN, which is covered by Medicare, was recently approved by the FDA.

About Vest Therapy

Vest therapy, or high-frequency chest wall oscillation (HFCWO, thins secretions through the use of air pressure. The pressure creates pulses on the chest wall. If the patient’s cough is then sufficiently strengthened by the HFCWO, the fluid will be removed by coughing. If not, the fluid will be transported out of the airways by the cough device.

Insurance plans may not cover HFCWO. However, studies have shown that HFCWO has not only reduced the frequency of inpatient admissions and length of stay but also the cost of inpatient care for various neuromuscular diseases.

In summary, HFCWO therapy is helpful to patients who just need assistance with their natural cough whereas CoughAssist benefits patients who have either a weak cough or it is nonexistent.

Dr. Orr gave advice to clinicians saying that it is important to select the proper interface such as the mouthpiece, trach adapter or mask. For instance, HFCWO requires that the vest fit properly and appropriate pressure is applied for optimum results.

The devices may be intimidating, especially for patients who must rely on their caregivers for these decisions.

Dr. Orr suggests that hospital administrators request that the manufacturers of these devices set up training sessions for hospital staff.

Cough assist devices are bringing much needed relief to many patients who have neurodegenerative-neuromuscular diseases.


What are your thoughts about cough assist devices? Share your stories, thoughts, and hopes with the Patient Worthy community!

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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