Respiratory Onset ALS

[smacish]

After finding the love of my life 5 years ago and just planning our retirement traveling with our toy hauler all thru out Canada and US my partner was diagnosed wish ALS on December 18,2015. Not only are we in full shock but we found out today that he has the worse type "Respiratory Onset ALS". I just would love to find out more if you all would be so kind and pardon my lack of knowledge.

I have noticed Dan (my love) over the last year really out of breath, he has lost 60 lbs. is slouched over as from what I understand his muscles around his chest have so deteriorated that is why he is so slouched over.

As of now he has no problem breathing.eating - has some slight issue with his right foot and can only walk for short distances because of the lack of oxygen.

We are awaiting for a bi-pap machine and the respitologist has also ordered a nebulizer and possibly oxygen.

We went the other day to the Centre here (GFStrong) and had meeting with social worker, nurse, physiotherapist and dietician....all were very positive as they said he was doing good and his strength is still strong as well as no problems talking or eating. Then today we go to the respitologist and he basically has given Dan a death sentence in the next week, moth, six months. He just sat down and told Dan to basically get his affairs in order. Not a hand shake or anything positive,

My poor man had, what we found out later, a panic attack but he got super hot, flustered stood up, passed out and super low blood pressure. They called 911 and was brought to hospital where all ecg and blood tests looked good.

I guess really no-one is really giving us an answer. Some of the docs say up to 2 years and this one basically says anytime.

I'd love to know more about respitory onset. All I know is that it happens in only 3% of ALS patients.

I can't see him dying so quickly...as he still eats, talks, walks ...it's his damn breathing. Any ideas or suggestions of who to see or what to do next would be so wonderful.

Thanks for listening - Sue

 

[Atsugi]

Sue, sorry to hear you're frustrated. We're all really well aware how difficult it is, sometimes, to get a straight answer.

You just never know. Some people keep track of breathing statistics to watch for the downward trend, but to my knowledge, that doesn't work, either.

My wife was told 2-5 years, as we all are, but she lived only 9 months from first symptom. Now, that sounds really bad--and it is--but the flip side is she had a very dignified, painless, and calm passing, surrounded by loved ones holding her hands.

I don't think any prediction is any good. But I will give advice on how to live those remaining days, regardless how many there are. Take life day-by-day. Wake up each morning, see what his condition is, then plan the day for maximum enjoyment with minimal stress. Just do this over and over, and you won't regret it.

 

[Diane H]

He doesn't need oxygen. His lungs can absorb the normal amount of oxygen from the air. What is lacking is the ability to breathe deeply enough to get the air into his lungs. Oxygen is NOT the answer and can be dangerous to anyone who is retaining CO2, which is common in ALS. Your respiratory doctor is not qualified to care for an ALS patient if he doesn't recognize that! What is needed is mechanical help to push air into his lungs. That is what BiPAP will do. With BiPAP and a knowledgeable physician to adjust the settings on the machine as needed over time, he has much more time than that doctor said. Since his swallowing is good, he is not at high risk for aspiration pneumonia, a common cause of death. Just keep watch on that and get a swallow study if he begins coughing when he eats. At that point, a feeding tube should be considered. Being new to all this, a feeding tube probably sounds extreme to you, but it is pretty basic in ALS and not at all difficult to use.

Weight loss is typical in ALS, usually from loss of muscle, but in Dan's case, it is as much, if not more, due to the large amount of calories being burned by the effort of breathing. It is important to get plenty of calories in -- no time to worry about carbs and fats! A high calorie diet is needed.

Don't sell that toy hauler yet! It is a great way to get a wheelchair into an RV, and with a BiPAP or eventually perhaps a ventilator, your traveling remains possible! For a crash course in living with ALS, just click on my name and then on the link to visit my blog. I have been on BiPAP for about 6 years and then on a vent the last 11.

 

[gooseberry]

My husband was diagnosed as respiratory onset. He began riluzole the day after diagnosis. He plateaued to small progression for a year. He has just gone thru a period of rapid decline but is semi stable again. May will be 2 yrs since diagnosis

 

[smacish]

Hi Gooseberry: Thanks so much for posting. This has given us hope. He was given 3-6 months after last check up but after being on the Bipap and being on Riluzole (started a week after diagnosis) - he is like a new man. He still walks, talks, eats and swallows but his walking is slowing and he does have curved back because of lack of muscle. But it just doesn't seem that it could be 3-6 months the way he is going. It sounds to me that your husband may have been given the same sort of diagnosis but he has overcome the odds and being now nearly 2 years...that is what we are hoping.

I pray and hope for the best for you and please keep me posted and glad to hear he is semi stable again

Sue

 

[smacish]

Thanks Diane H - for the positive feedback. We haven't sold the toy hauler and truck yet. We have it parked in our place down in Birch Bay WA and go down there nearly every weekend. The BiPap has made a world of difference and right now he is like a new man. We just hope and pray it does not decline quickly. Thanks again for posting.

 

[lgelb]

The last ms I have seen on survival in a series of respiratory onset pts is old-- 2006 (studied pts from 1990-2005)-- and even in that one, the conclusion is:

The mean survival time of respiratory onset ALS if NIPPV [BiPAP] was tolerated was 36.4 months and if NIPPV was not offered or tolerated was 21.5 months.

Nutrition, as Diane points out, is critical as well, and that wasn't measured in this particular study.

Let me know if you need any help tweaking the BiPAP at any time for therapy or comfort.

Best,
Laurie

 

[BillH]

Sue, so sorry to hear resp onset. My Mom was too. You didn't mention any talks about a PEG (unless I missed it). Mom got hers pretty soon after her official diagnosis and it was a great help as I remember it. My sis tells me she didn't like the BIPAP though and used it far less than she should have, which may have contributed to her fast progression. She wasn't a big fan of wheelchairs, and in fact didn't get to the place of having a power chair...

Do all you can to enjoy the time you have together. Take advantage of the recommendations of the docs (PEG, BIPAP, etc) sooner rather than later. And >use< them (sorry Mom). They really will make a positive difference in both of you lives.

God bless,
Bill

 

[smacish]

Laurie - thanks so much!!! He is actually finding the Bi-Pap the best thing ever and we have a program here in BC that come and help the patient with setting it up, the correct settings etc. and have phoned every other day. They came over the other day with different masks to suite his needs and his appetite is amazing! It sorta pisses me off, he can eat anything and everything and not gain weight...while I have gained 20 lbs matching what he is eating.

Bill - so sorry for the loss of your mom, I'm sorry she couldn't take the bi-pap well...it to me is the most amazing thing. Dan is not in a wheelchair and at times I see nothing wrong with him and then when he walks I see him hunched over and he can only walk so far. This is the hardest and worse disease out there-we just keep dealing with the day at hand and not look at the future (unless there is a cure)

 

[gooseberry]

Smacish, that has been our experience. My pals limbs have been relatively strong but he cant move much because he is so short of breath. They always say you are doing so well, you are so strong but he cant breathe much without his bipap. My pals is hunched over and has lost 2 inches in height. His head drops to the chest and he sits in a semirecli ed positIOn. This is how he is most comfy,