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mattb

Member
Joined
Sep 7, 2013
Messages
14
Reason
Loved one DX
Diagnosis
09/2013
Country
US
State
NC
City
Charlotte
It has been a while since I have posted so I wanted to relay a chain of events that may help others in a similar situation or heading toward a similar situation.

My father was diagnosed with ALS in September 2013 at 71 years old. His symptom onset was a year prior with overall weakness, weightloss and drop foot. It wasn't until he was hospitalized for shortness of breath that we went through two weeks of neurological tests in a university hospital and received the diagnosis of ALS. He was discharged with a BiPAP prescription and a referral to the local ALS clinic. Dad got a G-Tube in October and by the end of December he was 95% dependent on his BiPAP machine. He could still walk, had full use of his arms and hands but was forced to make a decision to get a trach and go on full time invasive ventilation or go into hospice care and be sedated while he suffocated to death. We went to the best voice and swallowing ENT in the state and he recommended the Blom trach tube which would reduce the risk of VAP and give him speaking capability through an interchangeable speaking cannula. He said the surgery would be routine and he would be out of the hospital within a week.:roll

After surgery my dad got pneumonia and seemed to take a few steps backward in muscle weakness when coming out of anesthesia. He ended up in the ICU for a month (which is the only place in most hospitals equipped to care for vent patients), then he was discharged, and ended up back in the ER the same day for two more weeks in the ICU before he was discharged to an LTAC for three more weeks to learn how to handle his ventilator, trach and cough assist. Since then my dad has gone a year and two months with no respiratory issues. His trilogy 100 coupled with Phillips cough assist machine and Blom subglottic suction trach have given my dad another 14 months of life to prepare for his end of life as watched his voluntary motor skills deteriorate.

Today my father is 95% paralyzed, incontinent and communicates through a roller ball mouse on an on-screen keyboard. He still has some use of his fingers, but is losing that too. His care has become a great burden on us and him. He has decided to go into Hospice House next weekend after his wife and daughter visit. At that time he will be sedated and terminally weaned from his ventilator. We were never sure when this day would come but we knew it was in our future and my dad still does not want to die, but he does not want to be a burden or become locked-in. So here we are.

Was it worth it, I am not sure, I hope he feels that it was. A few things that were very useful along the way (which we should have gotten sooner) were obviously his powerchair with lift feature, Rhoho seat and back cushion, wheelchair mini van, ceiling lift over bed, Aquatec reclining shower commode, care.com aids, quiet portable battery operated suction machine, iPad and Tobii. Not to mention the wonderful neurologist and staff at Carolinas Neuromuscular ALS clinic. My dad moved from Louisiana (where he lived with my sister) to North Carolina to live with me 10 months ago. The difference between the Louisiana and NC ALS clinic was dramatic. If you have ALS and you are not getting care or attention find a new clinic or doctor. Also of note: he took 3,4 DAP on a compassionate use drug trial because he has the LEMS antibody. It was of no benefit and possibly speed up his ALS progression. He gets botox injections in his saliva glands every 3 months which were very helpful with secretion management. My dad also took Riluzole and Neudexta along with an antidepressant. Neudexta works with emotion control, but I am not sure it is worth the price if insurance isn't covering it. Riluzole is of minimal value and high cost. Toward the end the neurologist prescribed Provigil for increased wakefulness or less tiredness. This was a wonderful drug that did give dad less tiredness. We only took it as needed, but I wish he would have had it all along.

This disease has been a sad, slow surrender, not much of a fight. We are thankful for the time we had and the things we learned. If I can help anyone by relaying our experience I would be happy to help. I work for a medical technology company and studied relentlessly to give my dad the best possible end of life journey.

Best wishes to anyone traveling on this slow surrender journey. Our hope is built on nothing less than Jesus' blood and righteousness;
 
Thank you Matt. I copied it and will forward to my family. I am considering a vent because I am almost breathless, and I am still pretty much self sufficient.
 
Thank you for this helpful info. Sorry so many are faced with this decision. When my dad gets to that point I don't know how anyone will handle it. Currently breathing is the main issue but at the second
Clinic they noted weakness in one foot, he's choking more and his voice is getting weaker although he's still mobile and independent. At times I can't even believe there could be a disease that takes so much.
 
A tough decision. God be with you. I wish you peace.
Vincent
 
I was once where you are. Time passes quickly and what seems like a slow progression can speed up quickly once milestones are crossed like being able to stand, toilet yourself, speak or write legibly.

If you ever come to a crossroads and need some perspective on what we did, I am happy to share our experiences. We came to just about every crossroad this disease brings and explored every option that medical technology and money could offer.

God bless you are your dad.

--Matt
 
Which unfortunately is just about zero
 
This is the part of the disease that is the worst for us. Tim seems to have had trunk onset then legs and arms, but so far although he is 100% dependant on his Trilogy, he is able to use the sip & puff and not a trach. I have noticed that the weakness is now creeping up his neck, and swallowing in any position other that bolt upright is nearly impossible. He still speaks and sings in a band if you can imagine, but I fear the day that he can no longer swallow at all, or that he loses the muscles in his mouth. We have discussed it, and a trach is something neither of us want, but I am afraid that when that time comes it won't be that simple. His ALS is so atypical that no one can tell us what the end will look like. Tim is just so vital, never seems to tire, seems so in tune with all the beauty around him, enjoys visiting with people, and well has a lust for life. He is as sharp as a tack, and touches everyone that comes in contact with him, and not a sign of any depression at all. He is such a blessing to me, and we have had such a short period of time together (5 Years) I just have to trust that God will give us both wisdom.
Paulette
 
We have discussed it, and a trach is something neither of us want, but I am afraid that when that time comes it won't be that simple.
Paulette

It seldom is that simple even if you have "decided" not to vent. You simply can't know how you will feel about it until you are facing the choice. I think that nearly everyone says "no vent" early on. Information and experience with being disabled can make you rethink or confirm your decision. Thankfully, it isn't written in stone no matter how often you say it!
 
I can still walk and type, and feed myself, but run out of breath with the least exertion. I have a bipap beside the bed and another by the recliner in front of tv, where I spend most of my day. It's getting worse fast. I have a battery pack for the bipap, but it's heavy. Is a vent compressor any lighter, or the same?
 
Graybeard, my husband is using the Trilogy at night, and it's set up for sip and puff, which he's resisting but probably shouldn't. Anyway, it is the ventilator that he will be hooked up to when he needs a trach. It attaches to the back of his PWC and is VERY portable. Seriously, if you have good quality of life, why not vent? I understand if your body is failing and life is miserable, but while the brain AND body function, and the diaphram is the problem, use the vent and enjoy life. Just a wife's humble opinion...
 
There is no comfort or dignity in this disease. If i had to decide for mom again, I would have have said, "HELL NO!" when they asked if I wanted them to do trach and vent conidering her condition. Countries that prohibit the use of vents in ALS patients are more compassionate than they will ever realize...

Ok...maybe hell no was a bit harsh. I would have tried to talk mom out of it...
 
but was forced to make a decision to get a trach and go on full time invasive ventilation or go into hospice care and be sedated while he suffocated to death.

We were faced with that and chose trach, but to what end? It is just going to end the same way. Except we all have had lives twisted. For what? A few more months of breathing? What mom has been through is not life by any means...
 
My husband Chris had a completely pain-free, peaceful and fear-free death without ventilation, and yes he passed from respiratory failure.

That doesn't mean nobody should vent, but I don't believe it's at all right to say they will suffocate to death, it's over-dramatising.
 
I go to the same clinic and they are wonderful, we may have seen each other, God Bless

Janie
 
I am sorry that you feel that way. Caring for a trach patient is no walk in the park, but it can give a person who wants it another, 3 months, 6 months or 2+ years depending on progression and the persons will to live or desire to die. A family should not have to make the decision for a PALS needing invasive ventilation or withdrawl. If the PALS is at all indifferent about the idea of extending life by extraordinary means, they should probably not do it. The disease effects the frontal cortex of the brain which controls "executive function" and promotes a general indifference. These are the patients that I believe get locked in because the cannot see beyond right now and lack empathy for others. They will never decide to let go which requires executive capacity. That's my take on it.
 
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