Very Frustrated Please Give Insight

[CRR10]

Hi everybody, I posted a few months back and I am just severely frustrated. Long story short, I have been to Beth Israel, John Hopkins, and at the advice of forum members Mass General. I have a severe problem because my chest has collapsed and back is very weak which all started with severe stiffness and than weakness. I have seen 3 ALS specialists who have essentially diagnosed me with "unknown" MND. I am only a 28 year old male, and feel myself declining in the same areas on a daily basis. Doctors will not diagnose as ALS because of several clean EMG, now I know this is a good thing but the damage my body has sustained is very severe. I just do not know where to go from here. My breathing is getting worse on a daily basis, and the BIPAP is just not cutting it. Currently my supine FVC is at 50%, and I am struggling for every breath on a daily basis. My FVC has declined from nearly 100% in the supine position to 50% since January and I get no solid explanation about what is going on with my body. I clearly explained to the doctors that I want to be vented, and have the financial resources to do so, regardless of insurance requirements. The doctors want to monitor me at my next appointment at Mass Gen which is in 3 months. I feel like no matter what clinic I go to they want to wait until I am on death's door to truly address my breathing concerns. I have honestly had enough of the medical profession and their attitude towards MND/ALS etc. It seems to me what these doctors look at us like we are the walking dead while their glad to treat a vent dependent c1-c2 quad and do not even act like they have a terminal disease at all. Also, I am tired of hearing the what if you vent and it progresses, "do you want to live like that." First of all, they cannot even properly diagnose me, and how about we worry about the current problem. I am very sorry for ranting to this board but something must be done for those who suffer from MND. Does anybody have any suggestions? I am very close to traveling overseas and getting vented because regardless of what happens to the rest of me, I want to live as long as possible. I do understand the risks associated with venting, but 24 hour Bipap at near the highest settings is no picnic within itself. They seem to have the opinion not to vent until your around 25% FVC. I know my body and feel like I would be near dead at that point. Furthermore, they give me a 3 month follow up appointment when I have declined nearly 10% per month since January. I know venting is a personal choice, but I would rather live with MND than die a suffocating death from it. I truly feel bad for all members of this board, and wish I could offer wisdom to some that frequent it. Thanks in advance for any input.

 

[Uptown]

My FVC has also declined from over 100 to as low as 19 supine since December. The bipap made things worse. That feeling of gasping for air is very difficult to say the least. That feeling of not waking up the next day as you go to sleep is also hard to live with every day. Have you thought of finding different docs? Do you see a neuropulmonologist? My EMG's have all indicated nerve denervation and increasingly more intense fasiculations. My strength is pretty decent even without chest, shoulder, intercostal and back muscles. I was bounced around between about 20 doctors for a couple of years more because the docs wanted a diagnosis. I finally just scaled back and fin a doctor to see to deal with a specific need. Go to a neuropulmonologist and tell them you can't breathe. If tat doesn't work, your only choices a re different docs or the ER.

 

[CRR10]

Thank you for your kind reply and giving me something forward to look to. I have very good strength in all my extremities as it has been only in my chest and back for the last 12 months. I started noticing sympthoms as far back as 3 years, such as intense stiffness in the center of my chest and back and a slight bit of weakness.I have been an avid weight lifter for many years, otherwise I would not have realized the problem. Last September I had an attack of stiffness, and my chest and back have been gradually getting weaker and weaker. I just hope that it stays where it is. It is very scary having torso onsent, but I have read that most people retain good strength in their extremities for a much longer period of time. Are you currently on a ventilator? If so, how has it worked for you? I agree in the sense that the diagnosis doesnt matter at this point, but rather treating my specific problem. I am honestly tired of getting EMG'S, and want my sympthoms addressed. I have tried to explain to family and friends that it doesnt matter if disease "unknown" is doing this to me, but rather, I will be dead shortly if I cannot breathe. It seems like family just does not understand what is going on, while I struggle to make it through the day. I agree that Bipap is not enough, and may indeed make me feel even weaker. Do you have trouble holding your torso up, and if so how do you deal with it?

Kind Regards,

 

[HelenL]

I think going to a pulmonary doc to address the breathing makes sense. A PALS I know got a special setup he requested from our pulmonary doc (we go to the same clinic). You have to have enough strength in your mouth to make a tight seal on a tube attached to oxygen... he "sips" on the air tube which is in front of his face on the wheelchair. He uses a bipap at night I think.