ltd17
Member
- Joined
- Jul 24, 2017
- Messages
- 20
- Reason
- Loved one DX
- Diagnosis
- 07/2017
- Country
- US
- State
- MN
- City
- Rochester
Dear PALS, I've had an earlier thread regarding my mother, who's diagnosis is still uncertain. Both Laurie and Karen were very helpful. A short intro:
My mother is a very complex case and has shortness of breath, orthopnea, loss of strength in upper arms and some atrophy of the deltoid muscles. The symptoms with breathing started in April 2016 and have progressed since. EMG'S ( 2, about 6-7 months apart) showed only the cervical region match the El Escorial criteria. Both ALS, MG or MG and Cervical radiculopathy (without sensory symptoms or pain) are the different options the neurologists are considering.
At the moment she is being treated for MG, (since August 2017) but they think she could have both ALS AND MG.
A few weeks ago, while tapering the prednisone, 24 hours after the lowered dosage, she felt the dyspnea getting worse and the strength in upper arms became even worse.
She was admitted into the hospital 17 days ago for severe shortness of breath and raised PCO2 levels in her arterial blood gas. She was barely able to complete sentences and ended up staying 2 weeks.
The doctors concluded that it could be either the progression of the ALS, or a myasthenic crisis. Because it was a bit sudden after the lowering of prednisone it was considered a myasthenic crisis ( Myasthenic crisis is a complication of myasthenia gravis characterized by worsening of muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation.)
She was treated with Immunoglobulin (IVIG) for 5 days, and she has been placed on NIV (Philips trilogy) for during the night, as they noticed she had carbon dioxide retention.
The breathing is going better now, she is not short of breath anymore, but it's not like she can run a marathon and walking up and down stairs still makes her a bit tired. Before IVIG she could not finish 1 sentence and was short of breath almost non stop, even after a toilet break. Her forced vital capacity varied the whole time, from 15% when she was very short of breath, to 22% to 33% and 27%. Not great but definitely improved I would say. It's just weird how it fluctuates. Her cough strength increased from 90 to 270 after 13 days. They also started with Air stacking.
But the strength of the upper arms has not been restored. In a few weeks a new EMG will be made. I wonder maybe it this could be due to the atrophy. Loss of muscle means of course loss of strength.
I am wondering if there are any PALS that had IVIG and if so, if it helped them with anything? I would expect it not to help in case of ALS as I always understood ''you only go downhill' aka it's not reversible. Also, do you fluctuate in strength? And shortness of breath?
Laurie btw, thank you for recommending the BiPap, she is sleeping much better now and we are happy she has it. They decided to start her on BiPAp just in case it is ALS and progression.
My mother is a very complex case and has shortness of breath, orthopnea, loss of strength in upper arms and some atrophy of the deltoid muscles. The symptoms with breathing started in April 2016 and have progressed since. EMG'S ( 2, about 6-7 months apart) showed only the cervical region match the El Escorial criteria. Both ALS, MG or MG and Cervical radiculopathy (without sensory symptoms or pain) are the different options the neurologists are considering.
At the moment she is being treated for MG, (since August 2017) but they think she could have both ALS AND MG.
A few weeks ago, while tapering the prednisone, 24 hours after the lowered dosage, she felt the dyspnea getting worse and the strength in upper arms became even worse.
She was admitted into the hospital 17 days ago for severe shortness of breath and raised PCO2 levels in her arterial blood gas. She was barely able to complete sentences and ended up staying 2 weeks.
The doctors concluded that it could be either the progression of the ALS, or a myasthenic crisis. Because it was a bit sudden after the lowering of prednisone it was considered a myasthenic crisis ( Myasthenic crisis is a complication of myasthenia gravis characterized by worsening of muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation.)
She was treated with Immunoglobulin (IVIG) for 5 days, and she has been placed on NIV (Philips trilogy) for during the night, as they noticed she had carbon dioxide retention.
The breathing is going better now, she is not short of breath anymore, but it's not like she can run a marathon and walking up and down stairs still makes her a bit tired. Before IVIG she could not finish 1 sentence and was short of breath almost non stop, even after a toilet break. Her forced vital capacity varied the whole time, from 15% when she was very short of breath, to 22% to 33% and 27%. Not great but definitely improved I would say. It's just weird how it fluctuates. Her cough strength increased from 90 to 270 after 13 days. They also started with Air stacking.
But the strength of the upper arms has not been restored. In a few weeks a new EMG will be made. I wonder maybe it this could be due to the atrophy. Loss of muscle means of course loss of strength.
I am wondering if there are any PALS that had IVIG and if so, if it helped them with anything? I would expect it not to help in case of ALS as I always understood ''you only go downhill' aka it's not reversible. Also, do you fluctuate in strength? And shortness of breath?
Laurie btw, thank you for recommending the BiPap, she is sleeping much better now and we are happy she has it. They decided to start her on BiPAp just in case it is ALS and progression.
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