Ftd/als expertise, please!

[goldie_12]

This post is for those who have experienced (or are currently experiencing) caring for someone with FTD/ALS. I am hoping to maybe gain some insight and direction from those of you who have been down this road before. I'll give you a quick rundown of our situation: After exhibiting increasingly odd behaviors for a few years, my bright, hard working HB was diagnosed (2 yrs ago) at the age of 49 with FTD. Last summer he was also diagnosed with ALS. We have four children ranging from ages 10-19.

I am becoming increasingly frustrated whenever we leave the ALS clinic as I rarely seem to leave with any answers. The drs/specialists all give me the standard answer that "everyone is different" and "everyone progresses differently". I certainly understand that and realize they don't have a crystal ball. I just wish they could give me some sort of direction or even a general idea. Personally, I think that they truly don't know how to help us because I don't think they've seen many patients who've had FTD along with the ALS.

So realizing that "everyone progresses differently" and that your experience will not be my experience, could you please help me to know what kind of things I MIGHT expect? For instance, my husband seems to be getting worse by the week--cognitively and physically. At his last appt he had lost 10 lbs. (in a 3 month period). He has lost another 3 lbs in the last couple of weeks. The swallowing is becoming an issue. He ends up spitting out his food several times during a meal because he can't get it down. The doctor mentioned getting him a PEG tube but I have some reservations about it. 1) Did your LO with dementia ever need a PEG tube and how did they handle it? With the FTD, I worry that he won't leave it alone and will pull it out, and 2) a few years ago we both signed legal documents stating that we would not want to be put on feeding tubes, etc. to prolong life if ever in that situation. Does anyone know what the legalities are of discontinuing a PEG tube once it's placed? Both his respiratory and leg strength are very good (upper body strength is poor), so I am having a difficult time making this decision. I would love to know about your experiences and would also welcome any advice you could give me.

Other things I am wondering about:
1) Did your LO with FTD/ALS become incontinent? Is there a possibility this won't happen?
2) Did your LO have to go into LTC? If so, at one point do you know it's time?
3) Did the disease seem to progress at a steady rate or were there times that it seemed to plateau?
3) Have any of you that have gone through this with a LO have young children at the time? Any suggestions on how to keep this from being such a traumatic experience?

I apologize for such a long post and for asking so many questions. I'm just feeling so lost and don't know where to turn, as I don't feel like we don't fit in any particular category. If any of you have answers to any of the questions, I would love to hear them. Thank you so much!

 

[affected]

WARNING - PALS if you are not sure that you want to read this, DON'T.

Hi Goldie,
Firstly I'm sorry but it's the bitterest of truths that no one can tell you what/how progression to expect.

I can talk directly to you from my experience, but already there are many differences to yours, so it's only my experience. My husband had bulbar onset ALS with FTD. He was rapid progression and was gone 11 months after diagnosis, he never had any plateaus but some PALS certainly do have this.
So I can't say that your husband will be anything like this as mine had breathing progression from early months after diagnosis and leg issues came too quickly as well.

FTD also is as dissimilar as the ALS sadly. It is a degeneration of the frontotemporal lobe and so it depends on just how much degeneration is happening and specifically what parts of the lobe are affected as to how much/what effects will be seen by the CALS. My husband mostly displayed symptoms of paranoia (esp towards me), anger/rage, poor decision making, inability to follow anything through. Otherwise he appeared to be a terribly positive man who was 'fighting' the disease, and seemed quite intelligent.

Around 50% of all PALS will experience some degeneration but many of these would not fall into a category that anyone would be comfortable calling FTD, however in technical terms it is because there is degeneration. Tricky hey? It was once called Frontotemporal Dementia but they now have replaced Dementia with Degeneration as there has been so much more research and understanding of this in the past 5 years or so.

My husband had behavioural issues and in the last 2 months it progressed to language issues which only those very close to him would have known because he could not truly speak by then.

He elected for the PEG. Never were his issues the kind that would have caused him to attempt to pull the tubing out. However, he had been well on the path to starvation before this and was constantly told he needed the PEG and would say he will know when he needs it and he will decide. He was emaciated (around 54 kg) and dehydrated by the time he suddenly wanted it, and he never adjusted to it enough to begin putting weight on. He would refuse enough feeds to give him the calories he needed and continued losing weight. (around 48 kg when he died)

However, for me it was a godsend because I could keep him hydrated and believe me a dehydrated person with FTD will behave far worse because of the dehydration, and I could get the meds into him that were so important particularly when he reached the active dying stage. I do know however that we could have used morphine patches, but he suddenly went into actively dying on the morning of Easter Friday and was in no fit condition to get to a hospital so suddenly getting that palliative action taken would have been a nightmare. I had the PEG and could just give him his meds under phone instructions from palliative care with ease.

We had no children at home and I will be forever grateful for that, I am sorry I can't help you much from my experience on that one.

My husband did not become incontinent at all to answer that one.
My husband died here at home alone with me tending him over 4 days of actively dying and he knew he was dying and he was totally at peace (the only time he was at peace!) and we always wanted him to die here. It's not the right choice for everyone, but discussions now with hospice or a palliative care team will allow you to get your plans in place and clear now so you know what to do when you get to that point.
Not all PALS even go into active dying, some simply go suddenly when you think you have a lot of time left.
On the Easter Friday I gave him half PEG feeds at the regular times, on the Saturday it was obvious what was happening and I ceased giving him any nutrition. Even if you have a PEG placed, you can still elect (or the PALS can) to not use it.

Trauma to children - I am the kind of person that believes that honestly talking it through is the least traumatic. Tell them a bit, let them digest and ask questions and encourage them to ask and to share their feelings. Death is part of life whether we want it or not, and maybe helping them to see this rather than feel all is so unfair will help them in the long run.

Some people think that with no PEG the person will die a horrible death of starvation but this is not necessarily true. Many PALS choose no PEG and pass peacefully and I've known it.

Are you blending his food at all so that he has a better chance of swallowing it? This is really important. My husband again resisted this for a long time then he was amazed at the difference it made and very quickly we had moved to complete puree.

My husband had a PEG and his journey could not have been less peaceful anyway. He fell often and had serious injuries that made both our lives very difficult especially because he insisted on attempting to be mobile and walking without any assistance and falling again.

My heart goes out to you. As if ALS isn't enough, the addition of FTD is beyond cruel. Explain to your children that his brain is wasting the same as they see his upper body wasting to help them know that it isn't him and it isn't them, it's the monster eating him from every angle it can.

For my husband the end started with an aspiration pneumonia 3 weeks before he died. This really took a huge toll on his body and all the fevers and antibiotics left him riddled with fungal infections. I believe that the treatment they gave to try and stop the fungal stuff was the final straw for him as he ended with sudden severe diarrhoea and within 2 days was actively dying. I don't blame anyone, it was the way it went and he was in misery and cried a lot for the last couple of months. But this is part of being so hard to say what will happen for your husband, there are too many variables. If my husband had stopped trying to eat and not had that aspiration (it took 1.5 hours to clear his throat), would he have lived longer and died a worse death or a better death? I've never played the 'what if' game on any of it. The disease has many ways to take our PALS down, and if you skip around one way it will get them another.

Too many with FTD die from head injuries falling, and I know one that stole an apple from the fridge and choked. I don't want to scare you, but I do need to try and make it clear how little we can tell what path any PALS will take, let alone one with FTD in the mix.

 

[Atsugi]

Goldie, Tillie gives good advice. I'll just add 2¢. We did hospice at home, and put the hospital bed in our main (TV) room, so that my wife was always involved with the family.

Courts have held that it is allowed to remove or withdraw life support. The cause of death is recorded as the disease, not the action of removing support. Families have been known to disagree, however, so a Living Will may be needed to enforce your LO's desires. An estate attorney helped my wife (with ALS) to complete legal papers by watching her blink. My wife refused all tubes, including PEG's and water, but accepted a catheter toward the end, so it was easier to urinate.

Children are adaptable. They will follow your lead. If you cry and babble, so will the 10-year-old. But if you accept the duties of attending to your HB, so will the children. My kids, 13 and 15, crushed meds, fed mom, rubbed feet, massaged legs, and transferred mom using the Hoyer Lift to the restroom. We even trained them to operate the air, oxygen, and cough assist machines. After it was all over, I was really proud that they really pitched in to make mom comfortable.

Of course, the most important thing kids can do is simply be there, watch TV in front of your LO and, by so doing, make sure that your LO doesn't feel alone.

 

[affected]

So glad you are back Mike and I was really hoping you would add to this concerning the way you involved your children with Krissy and how it will have helped them move on afterwards knowing they were totally involved.

 

[Snowflake]

Goldie, you might want to post your question on the ftd forum. There are quite a few people there who have dealt with ftd mnd.

FTD Support Forum

 

[Edward E]

Hello to all, and thanks Tillie for sharing your story,
My wife, 47 yo, has been diagnosed w FTD and Upper motor neuron disease. We have a boy, 13 and a girl 11. By force of circumstance they have had to become caregivers. They have done an amazing job w this. They show caring and compassion. They do feel the stress and sometimes get impatient. So far my wife has not displayed the emotional roller coaster that seems to be part of FTD. She has also not shown any lower motor neuron involvement yet. We still do not have a complete diagnosis yet. Falling is very bad and brings up horrible memories for me. Gina fell down our steps last year and spent a month in a hospital. She is getting to the point where steps are going to be too much. I do not know what else to share except we will pray for you and your family.

 

[patgayle]

About 1-2 years before my husband was diagnosed with ALS there were behavior changes I of course had no idea what was going on and it wasn't all the time, but sudden outbursts of unreasonable anger started. After about a year or so after diagnosis I realized that these continuing changes were most likely FTD. For me it was the most difficult part of the disease, it is very hard to accept verbal abuse when you are trying your best to make their life as good as possible. My husband refused all mechanical aids, so there was never an issue of pulling out tubes etc. Because he had experienced such severe pain from muscle cramps he was on morphine and severe constipation was a huge issue, I would rather incontinence any day and I was his main caregiver. In the last year all his food was blended and he managed to eat up to the day he passed. We did have to use a suction machine as his ability to swallow his own saliva became very difficult. As I live out in the country in a 3rd world country our experiences would be very different in some ways but at the same time all we caregivers share many similar experiences. Although we have no children, I know that friends whose children have gone through this experience made sure that they went to a professional to talk about it all. He passed in his sleep, my prayers were answered. Tillie gave you lots of information and there are many here who will listen and not judge, use the forum it will help you.

 

[goldie_12]

I guess I need to accept the fact that there is no crystal ball and that there's no way of knowing how this will progress. And it sounds like I have some difficult decisions to make. Thank you for your support and kind words.

 

[affected]

goldie, coming here and talking it through will allow us to support you.

It was a life saver for me.

 

[gooseberry]

I spoke to a psychiatrist who works with over 300 als patients and their families. She told me that memory changes, confusion, even some temperament changes to a point are a normal part of this disease and common. Its the more extreme cases that cross over to ftd and recording instances to take to appointments really help.

 

[poppies]

Hi Goldie. So sorry you are having to face this awful disease. My husband has not been diagnosed with FTD but I know about it because he has at times exhibited some of the symptoms. In our case fortunately it appears that his symptoms were more as a result of severe depression. Tillie is a mine of information. I also found the times that my husband displayed FTD symptoms the absolute worst, it is when I felt that I had already lost him. We have two teenage daughters. They were 13 nearly 15 at the time of diagnosis and are now 15 and going on 17. They are both handling the situation very differently, so in your case I would be very aware of how each one is coping. For example, the younger one talks, cries and shows emotion. She appeared to be coping very well for the first two years, but now I realise that she is really suffering inside. She is feeling disconnected from everyone, even her teenage friends, as she says that she just can't relate to the things that they are concerned about - they all seem so trivial to her. She is even losing interest in her sport which has always been her passion. I wish she would go and see the counsellor at school or at the hospice, but she refuses. I talk to her daily, about everyday things, lighthearted things and serious things, trying my best to continually keep the channels of communication open so that she can come out the other side of the bad patch she is experiencing. My older daughter is handling it in a completely different way. She does not talk about it. She listens but always says she is fine. She distanced herself from her dad for a while, I wonder if it was a way of protecting herself from the pain. Now she will greet him and chat to him as she comes and goes but there is still a distance there that was not there before. She also refuses to see a counsellor. Our girls have always been exceptionally close to their dad so at first it made me incredibly sad that there was a distance developing. I have had to make peace with it though. They all love one another immensely but each one, my husband included, is handling it in the way that they are comfortable with. He does not want them to help him physically. I think he worries that it will be a burden on them and on another level it embarrasses him that he needs this level of care. I think it would be therapeutic for the girls to be able to be more involved with his care, but he will not allow it. Sorry for the long message, I guess I just wanted to give you some insight into our situation as we have children, so you can see that there are unfortunately know 'right' ways of handling things. Read up as much as you can, come here for support and try not to be scared to trust your own intuition about what is best for your family.