Another Doctor Visit - More Questions

[tripete]

At my doctors appointment last week it was commented again that from their perspective there was "no discernible weakness."

Yet my Hands feel like they barely function, I cannot coordinate q-tips, typing is clumsy and I don't hit keys hard enough, my wife opens bottles etc, and using a screwdriver or other activities causes cramping and locking up of my hands, and then they will ache for days. Driving more than half an hour cause fore arm and hand cramps and locking.

When they ask me to do a leg lunge I can barely get back up with my right leg(my opposite leg cramped), and could not get back up with my left. He noted visible muscle wasting in left thigh, calf and foot.

My breathing has dropped from 136% of expected to 91% sitting up and from 73% to 71% lying down. I know from others post that these numbers are not horrible. I can tell you though that any activity (towel drying, carrying in groceries, bending over to pick something up etc) makes me feel like I have no breath left in me, and I loose my voice.

I asked as my onset was diaphragm or other breathing muscles what this meant for me longevity wise. I was told that there is a lot that can be done to help with breathing and they have ordered the bipap for me. We will look at the diaphragm pacemaker as my numbers lower.

How do I understand the "no discernible weakness" comments given that I cant do most of what I once did? And how does that apply to breathing? Why does lifting something effect the breathing so much?

 

[4tloml]

I wonder if the answer could be that "discernible weakness" is a relative term. ALS doctors see patients at various stages of progression, the majority of whom have limb onset and do not begin progression as athletes. I won't bore with details of my husband's diagnosis (as I already have in earlier posts), but he is a large, extremely strong guy who used to lift and throw shot put. His first neurologist diagnosed him with bulbar onset in spring of 2013, but it took till fall of 2014 for the ALS specialist to diagnose him, partly, I believe, because his legs and arms remained relatively (key word) strong. Signs even showed up in his EMG/NC in 2013, but doc said it didn't look "quite like" ALS. Even in November 2014, with obvious atrophy, including on chest xray and in his hands, it took 2 other neurologists to push the diagnosis with this "specialist." I guess at twice his size and still much stronger than him, my husband just doesn't look the part to him. It's all relative.

Use your bipap--it will allow your diaphragm some rest and help you feel stronger in general. Do breath stacking to keep your lungs expanded, and if you have any trouble with coughing, clearing your chest/throat, insist on a cough assist. It has made a huge difference for my husband. Hours of coughing each day and night are exhausting physically and emotionally--he doesn't do that any more between the cough assist and daytime bipap.

Hope bipap gets to you soon and you get feeling stronger.

 

[gooseberry]

With respiratory onset, your limbs will not necessarily show clinical weakness although you may feel it. This happened with my husband. He would say his legs were heavy, fatigued feeling. Even now with his fvc tanking he gets 5/5 for his legs and one arm and he can't walk more than a couple steps with a walker and his arm is very hard to use. The arm with 2/5 for strength is mostly frozen in the shoulder and his grip is shot. His breathing, limits everything. His whole body tires because all his energy is used up simply trying to breath of air. He is qualifying for a power chair, even with his strength numbers, because he can't stand or walk because of his breathing.

 

[Clearwater AL]

Pete, I am not ALS but PLS and my breathing difficulties have been attributed to neuromuscular as my lungs are clear, no COPD no asthma. As I was told the stomach muscles and the truncal muscles assist the diaphragm during exertion. So, the lifting thing comes into play. I sleep nearly sitting up now.