Myasthenia Gravis Support Group
Myasthenia gravis (MG) is a neuromuscular disease leading to fluctuating muscle weakness and fatiguability. The hallmark of myasthenia gravis is muscle weakness that increases during periods of activity and improves after periods of rest. Although myasthenia gravis may affect any voluntary muscle, muscles that control eye and eyelid movement, facial expression, and...
I recently had a sleep study--the results not yet in--and of course had to fill out the usual questionnaire for obstructive sleep apnea which I found did not address MG very well, so included an explanation which the technician said he found helpful. He also said he didn't know much about MG and was going home to read. In case anyone else would like to modify it or use it for your study, I will submit it here and link it in the links group. The links group can be reached by clicking on its member group at the bottom right hand corner of the main discussion page.
"Since MG is rare and the reasons for apnea slightly different than the usual obstructive sleep apnea, the questionnaire doesn't reflect the primary concerns of the MG patient. Although there may be an obstructive component to breathing problems caused by upper airway fatigue, it is primarily diaphragm fatigue that is of concern and the unfamiliarity with the disease may cause misunderstanding when the patient presents with respiratory distress.
The main problem with the questionnaire is that since by definition and nature MG waxes and wanes, most often unpredictably, almost every answer could be "it depends." So I have answered as best I could, but it might be more helpful to explain the concerns:
Personally, at my best, I do not have breathing problems. Then we try to decrease the medication. I am not at my best. The question I have regarding sleep study is: Does diaphragm fatigue contribute to apnea or other interrupted sleep patterns resulting in generalized weakness and fatigue I am experiencing, and would some breathing assistance help with those problems and allow further tapering of risky medications without interfering in daily activities and quality of life.
When people with MG have respiratory distress, it is primarily due to diaphragm fatigue and not perfusion abnormality, so the oxygen concentration does not fall until late in the process, long after carbon dioxide build up, and just shortly preceding complete respiratory collapse. Many of those with MG who have more severe problems sleep with BiPap or modified CPAP to assist the diaphragm and some require continuous assisted breathing during exacerbations. Non invasive assistance may be all that is needed to avoid intubation and avert crisis."
"Since MG is rare and the reasons for apnea slightly different than the usual obstructive sleep apnea, the questionnaire doesn't reflect the primary concerns of the MG patient. Although there may be an obstructive component to breathing problems caused by upper airway fatigue, it is primarily diaphragm fatigue that is of concern and the unfamiliarity with the disease may cause misunderstanding when the patient presents with respiratory distress.
The main problem with the questionnaire is that since by definition and nature MG waxes and wanes, most often unpredictably, almost every answer could be "it depends." So I have answered as best I could, but it might be more helpful to explain the concerns:
Personally, at my best, I do not have breathing problems. Then we try to decrease the medication. I am not at my best. The question I have regarding sleep study is: Does diaphragm fatigue contribute to apnea or other interrupted sleep patterns resulting in generalized weakness and fatigue I am experiencing, and would some breathing assistance help with those problems and allow further tapering of risky medications without interfering in daily activities and quality of life.
When people with MG have respiratory distress, it is primarily due to diaphragm fatigue and not perfusion abnormality, so the oxygen concentration does not fall until late in the process, long after carbon dioxide build up, and just shortly preceding complete respiratory collapse. Many of those with MG who have more severe problems sleep with BiPap or modified CPAP to assist the diaphragm and some require continuous assisted breathing during exacerbations. Non invasive assistance may be all that is needed to avoid intubation and avert crisis."
At first tried CPAP, I was unable to breath against the flow. BiPAP has changed my life. Once you get used to it, you will find yourself more rested when you wake. I use nose pillows, instead of mask. My pulmonologist added 2% supplemental oxygen a year later, mostly as a precaution I think. When we travel I always take Bipap but not the oxygen.