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Respiratory weakness is only recently being acknowledged as a symptom of CMT so there is still a lot that we as patients need to learn regarding standard of care so that we (and our loved ones) can be our best advocates. Having CMT doesn’t necessarily put you in the “high risk” category for COVID-19, but if you have a weak diaphragm and reduced lung capacity please keep reading for answers to the most frequently asked questions.

On March 20, 2020, HNF teamed up with Dr. John Bach of Rutgers Medical School to answer some of our community’s most vital questions regarding pulmonary health and the specific risks and precautions pertaining to COVID-19. 

SHOULD I BE CONCERNED ABOUT THE EFFECTS OF COVID-19 DUE TO MY CMT/HNPP DIAGNOSIS? 

If you have respiratory issues, yes, you may be considered “high risk” and you should take proper precautions such as isolation, frequent hand washing (for a min. 20 seconds), disinfecting surfaces, staying at least 6-9 feet away from anyone who may have been exposed to COVID-19. It can be spread by someone who is asymptomatic. The incubation period is from 2-14 days.

Studies show that the COVID-19 virus remains: 

In the air for up to 3 hours

On cardboard for up to 24 hours

On plastic and stainless steel for 2-3 days

WHAT ARE THE CLASSIC SYMPTOMS OF COVID-19?

Fever (101°F)

Muscle aches

Shortness of breath

Cough

WHAT ARE THE DANGERS OF COVID-19 FOR PEOPLE WITH NEUROMUSCULAR DISEASE?

The biggest danger is that if you are intubated, as you recover from the pneumonia the doctor will try to wean you from the ventilator. If the doctor feels it is not possible to extubate you quickly to CPAP or BiPAP (possibly with inadequate spans), it can be fatal. Let your family members know that they should NOT immediately accept a trach tube, but contact/use Dr. Bach’s procedures for extubation for neuromuscular patients. (see webinar at about 57:00)  

Webinar link: https://www.hnf-cure.org/covid19

IS IT POSSIBLE FOR PEOPLE WITH CMT TO HAVE DIAPHRAGM PROBLEMS WITHOUT KNOWING IT? 

It may be possible, yes. Things that you can ask yourself to determine if you have a weak diaphragm include: 

Do you sleep mainly on your side because you can’t breathe well when you’re on your back (orthopnea)?

Are you unable to take a deep breath and hold it for 20 seconds?

Do you have fatigue, sleepiness during the day?

Do you have morning headaches?

If you answered yes to these questions, you may consider visiting a pulmonologist (preferably one that understands neuromuscular disease – see Dr. Bach’s list of centers

Dr. Bach does not recommend a sleep study. Patients with a CMT diagnosis and limited lung capacity should be approved for a Respiratory Assist Device (RAD) by insurance –  here is a link to the Medicare guidance on treating with RAD. 

WHAT SHOULD I DO IF I’M SHORT OF BREATH AND PUT ON OXYGEN?

Dr. Bach suggests you let the HCP know that you have a neuromuscular disease that weakens your diaphragm and limits your ability to expel CO2, so they’ll need to be prepared to intubate you if you stop breathing. “No O2 without ventilation” is the rule.

IS IT SAFE TO RETURN TO WORK NEXT WEEK? 

Dr. Bach advocates isolation and social distancing for everyone at this time. The more quickly we stop the spread of COVID-19, the more quickly we can all go back to our normal lives.

WHAT EXERCISES CAN I DO TO INCREASE MY LUNG CAPACITY?

Lung volume recruitment exercises include breath stacking, and “frog breathing” or Glossopharyngeal Breathing. This will keep your lungs functioning at their best capacity.

CAN COVID-19 CAUSE FURTHER PROGRESSION OF CMT?As with any virus, CMT patients often take longer to get over it completely and they often do experience progression of muscle weakness.

HOW DO I USE COUGHASSIST?

The CoughAssist settings are always the ones that give the highest flows. That is almost always 50 to 60 cm H2O in and out to full chest expansion then full chest retraction. It is not complicated! Do it manually to determine times if you want to use automatic, but manual is often better.

WHAT EXERCISES CAN I DO TO INCREASE MY LUNG CAPACITY?

Lung volume recruitment exercises include breath stacking, and “frog breathing” or Glossopharyngeal Breathing. This will keep your lungs functioning at their best capacity.

CAN COVID-19 CAUSE FURTHER PROGRESSION OF CMT?

As with any virus, CMT patients often take longer to get over it completely and they often do experience progression of muscle weakness.

HOW DO I USE COUGHASSIST?

The CoughAssist settings are always the ones that give the highest flows. That is almost always 50 to 60 cm H2O in and out to full chest expansion then full chest retraction. It is not complicated! Do it manually to determine times if you want to use automatic, but manual is often better.

WHAT FOUR TOOLS DOES DR. BACH RECOMMEND ALL CLINICS WITH NEUROMUSCULAR PATIENTS HAVE? 

  1. Peak Flow Meter (like this) to measure cough strength
  2. Oximeter (like this) to measure oxygen saturation in the blood
  3. CO2 monitor (capnogram)
  4. Spirometer (like this)

Dr. Bach’s instructions for use of Cough Assist with pneumo-thorax (collapsed lung) are at the 1:32 mark in the video.

Watch full webinar: www.hnf-cure.org/covid19