Need to learn more about End Stage CF (Supporting a Friend)

[slhogan]

Hello! I'm new to this forum.

My college-age daughter is in a relationship with a young man with CF. I am trying to learn more so I can support my daughter as she supports her boyfriend.

Specifically, I am trying to find resources where I can learn more about the end-stages of CF, quality of life, planning for the end, etc. Most of the resources I am finding on the internet seem to be either Medical Journal type articles or overly upbeat "CFers can live to be more 50!" type articles, neither of which is very helpful for me.

Here's what's going on:

My daughter has been dating this 22 year old young man for about a year. When they first started dating last year, he seemed relatively healthy. I knew he had CF and he was thin and coughed a lot, but he seemed pretty energetic and overall okay. However, every time I see him (about once a month or so), he seems much worse--

-- He's had several hospital stays this past year. He was turned down for a lung transplant because he has a bacterial infection that he can't seem to get rid of. He has a port and does daily antibiotic therapy, but it's been more about 6 months and the infection isn't going away.

-- He has a feeding button, but he is still thinner and thinner each time I see him. I'll be honest-- he looks like a famine victim.

-- He has decreasing energy. These days he pretty much stays in bed all day, and sleeps fitfully off-and-on throughout the 24 hour day.

-- He now has an oxygen machine, but my daughter says that even with the machine his oxygen levels are often low and he gets confused.

-- He honestly looks like he's dying. He stayed with us over the Christmas holidays, and all the relatives were quietly asking me if the doctors have said how much longer he has (they haven't). He's so quiet now. Lethargic. My daughter had to help him stand up from the couch. He doesn't have the strength and energy to set up his own breathing machinery, so she helps him get from room to room and sets him up.


So, here's the question-- does this sound like someone who is in the end stages of CF? Or, is this normal for CF and he will still be with us for many years?

He doesn't really have any family, and he's been on his own since graduating from high school. He's given my daughter medical power of attorney which is a big responsibility for a 19 year old to have. However, there's no one else in his life but her. She loves him very much so she wants to support him; we love HER very much so we want to support her in supporting him.

Does anyone have any advice or links to websites that talk about what my daughter and I need to know at this stage of CF care?

 

[believingjesus]

This makes me so sad. I am so sorry to this young man who has to deal with his health and trying to stay healthy when he is only 22 years old. This makes me very sad. Life is so unfair and CF sucks bad. I am so glad he has the love and support from your daughter and your family. I am glad you are letting him stay there as he needs companionship and help and love. I don't know his circumstance but if his family is alive - how in the world could they abandon him? They must have no love in their heart. I am so sorry to him to have to fight to stay alive at 22. I don't know about the end stages of CF but it does not sound good. I hope your daughter and your family stay by his side and show him the love and support he should have from his own family. Life is so unfair. Maybe you and your daughter could go along with him to a doctor appointment, as he can give permission, and you could find out how you could help him or if there are any treatments or hospitalization that could help him. This is so sad. I am glad you all are in his life. If you go to church anywhere or even if you don't, you could call a church and ask them to put him on their prayer list and to earnestly pray for him. I wish him and you all the very best.

 

[cftransplant]

Yes this is end stage CF. Been there, done that.

How many TX centers did he try and get listed with? I was turned down by many centers because of an infection when I was end stage. I would not take one rejection as a get along with dying.

This kid needs a very driven person if he can not or does not have this drive or personality. I would call TX centers until someone would talk to you. Call centers like Pittsburg and Duke, ask for the lung transplant programs. Then ask for a transplant coordinator and explain the situation. He can do this if he has the drive.

I have seen many CF's without much drive (hard to have when your sick and have zip for energy), some others seem to have the life choice to cling to life with fingernails if needed. Some do not want too borrow a set of lungs.


Cf transplant cepacia 64 14 years out

 

[welshwitch]

No advice, but sending hugs your way <3

 

[slhogan]

How many TX centers did he try and get listed with? ... This kid needs a very driven person if he can not or does not have this drive or personality. I would call TX centers until someone would talk to you. Call centers like Pittsburg and Duke, ask for the lung transplant programs. Then ask for a transplant coordinator and explain the situation. He can do this if he has the drive. I have seen many CF's without much drive (hard to have when your sick and have zip for energy), some others seem to have the life choice to cling to life with fingernails if needed. Some do not want too borrow a set of lungs.

He does have family but it's not intact, and he was shuffled from relative to relative a lot as a child. As an adult, he doesn't really have a relationship with any of them.

No he doesn't have a strong drive. He's pretty passive about the whole thing. Actually, he struggles with depression, but he had to get off his antidepressants 6 months ago when he got on his current antibiotic regimen. Living on his own and struggling to keep afloat on SSI and medicaid alone, I think he hasn't been as proactive about his health as he perhaps could have been, although I'm sure he's doing the best he can. My daughter, however, is one of the most strong-willed, stubborn people you'll ever meet, so she's trying to wade through all this and crash-course learn what he needs.

As I said in my original post, he looked just terrible when he was at our house for a couple days at Christmas. A few hours after I made that post, he went to the ER and was admitted into the ICU-- his first time there. He seems to be doing better now, but he has a fever and is in full-body pain. I have no idea how long he'll be in the hospital.

He was turned down for a transplant by Methodist Hospital in Texas. At first, it was turned down because he has a mycobacterial infection (NTM, I think). After 6 months of IV antibiotics, they said he was clear of the infection but he still didn't qualify because they said he was too weak and thin to handle the surgery (he's just over 100 pounds at 5'10"). However, the fact that today he's in the ICU for fever tells me that perhaps his infection has returned. I don't know.

Is it possible that other transplant centers may still take him even if Methodist rejected him? Does that involve us flying him out of state to other hospitals for an evaluation?

Are there any studies that show how long CFers tend to live after reaching the point they need constant oxygen? I'm trying to get a feel for what we're dealing with here.

 

[believingjesus]

I don't know anything about transplants but like the person who responded above that has had a transplant, I would call the University of Pittsburgh Medical Center in Pittsburgh PA. I know they will do transplants for people who have b. Cepacia which most hospitals reject in the nation which is another story I could gripe about as it is so unethical and unfair. U could call there and ask for their cystic fibrosis clinic and ask to speak to the head nurse and explain how bad he is. I don't know anything about Medicaid but maybe they would pay to move him close to wait for a transplant considering his dire situation. I would call them tomorrow and ask. I'm glad he has your family in his corner and a mom like you who is willing to help him. I'm sure he is too bad off to do any of this for himself.

 

[Gammaw]

I really want to thank you and honor you for your willingness to love, support, and assist this young man. Sometimes people want to run the other way to avoid the potential pain of loving someone through their challenges. You, your daughter, and your family are obviously exceptional people. Many of us have come to understand that the way we respond to challenges is the stuff that enriches our lives in ways we never imagined..
All that aside, your daughter, the fighter, needs to be at his doctor appointments with him asking all the questions possible. Find out his mutations and post it here for potentially helpful information - with his permission of course. Fine out his detailed staus - lung function, infections, med therapies, and help him accomplish those things in a day to make sure he's not missing things or too tired to do it. Learn how to help him clean and maintain his equipment so he's not using expired or dirty nebulizers (they must be sterilized and last only last 6 months), and his chest therapy, port etc are all in working order. Or better yet, in his circumstances see if his insurance will give you a visiting or home nurse to help check it all out and show you what to so. If not at home, get one at the CF Center to educate your daughter and retrain him to make sure.
If I were he, unsupported by family, trying to navigate my medical care on my own, I would be more prone to tire rapidly and begin to slip from being overwhelmed and feeling that few people really cared. It's simply harder to maintain in those circumstances. Having all of you could be very motivating! He needs you. Regardless of what happens.
If your daughter gets to meet his CF Team there should be a nurse, pulmonologist, respiratory therapist, and social worker or psychologist available as well that could be helpful. It's not unusual though to have to push for that help. So feel free to insist on what you need. You will also need to see if his insurance will allow him treatment options outside of Texas - sometimes there are restrictions but a simple call should confirm. Perhaps he already has done that.
As mentioned, Pittsburgh has been know to approve transplants for those with active infections. He may not realize that.
This forum is a great place for HIM to come and seek out others who have been through it as well as you and your daughter. See if you can get him here. There are thousands of us willing to help or even just listen.
Bless you and your daughter.
I'm calling out to those who lurk or otherwise know of ways to help this family help your fellow CFer. I have not had to personally deal with this degree of debilitation, so my knowledge is somewhat limited. But so many on this forum know so much ore about it and the resources available......

 

[donin]

SLHogan - I am so sad for this young man. I am sure you are also concerned about your daughter. It sounds like she is attempting to take on a very big responsibility that she may or may not be able to shoulder. What you're dealing with here is very grave. I am sorry. I am impressed by you and your daughter.

My son is 26 and was just rejected from Mass General because of NTM - M. Abscessus (his strain is resistant to a class of drugs called macrolides). We are going to Duke next week and Pittsburgh after that. We had an initial appointment at Columbia but were very, very unimpressed. Since he has been rejected from MGH we will revisit the Columbia option. I am encouraged by the comments from cftransplant (thank you). When my son got the rejection from MGH he was devastated. He did not eat or even leave the room for four days. He has since rallied - but that is hard to do when you can't breathe, are in terrible pain, have GI distress, and are watching your potential drain away. It is no wonder your daughter's friend feels defeated. With this disease every day you're Sisyphus rolling the stone back up the mountain. What presents as a lack of drive may be exhaustion.

Being the primary caretaker for a person pursuing transplant and then post-transplant is a full time job. We had to supply the centers with detailed plans for pre and post transplant care. Patients are routinely rejected because they do not have adequate psychosocial support. It seems that it would be important to really explore how much you/your daughter can take on. Of course I don't know whether or not you're in a position to fly him to Pittsburgh or elsewhere, but it's not something he could do alone.

There should be a social worker on his CF care team. This is the person that he and your daughter need to be in contact with. The social worker will also help with figuring out whether or not it really makes sense for a 19 year old to try to manage this. If the pursuit of transplant is not realistic then the hard conversation about hospice needs to happen. I think this is important to the 22 year old and to your daughter.

 

[believingjesus]

Everyone that has responded knows way more than I do about transplants, etc. so I would follow their advice but I wanted to tell you if he is still in the hospital you should ask his doctors there if they can call the University of Pittsburgh Medical Center transplant team and set that up for you and get all the ins and outs of that. Since he is on Medicaid and disability, I do not know how they can deny him to get the help he needs even if it is across the country. I would not wait till tomorrow like I stated earlier (I was thinking this is Sunday). I would ask the hospital to get it set up for you today. I would also ask - like was stated earlier if there is a nurse or someone who could come to your house and show you or your daughter how to take care of him. Maybe Medicaid would approve for someone to assist him until he is able to - if he is able to. It is a lot for a person with CF to take care of their self and without help from anyone else and with him being so gravely ill, I know it will be a full-time job for whoever is doing it. What I don't understand is that hospitals can just reject people but do not redirect them to another place who could potentially help them.!! Maybe they did but it took some digging to find out about the University of Pittsburgh in case we ever need them. I wish you all the best. -

 

[peter]

If you Google Texas Medicaid this is a quote printed in one article from August Texas law makers' deliberations. "Texas leads the nation in uninsured residents, with 6.4 million Texans, or nearly a quarter of the population, lacking health care coverage". Standards very, so looking elsewhere has strong precedent with the better centers. Best of luck. You yourself must have some pretty terrific parents. God Bless you for your evangelism.

 

[Jane]

Your daughter's friend is so blessed to have your support. End stage CF is so overwhelming for the patient and the caregivers. It sounds like he is depressed and defeated which makes sense. Unfortunately he may not have the stamina and will to fight. There are other options for antidepressants that wont interfere with antibiotics. I agree with Gammaw and donin about seeking out a social worker from the clinic. They will help with finding resources for care, insurance and support. You all can't do it alone. Please let us know what you find out. Best wishes.

 

[Gammaw]

I really wonder about his family. Their relationship may not be salvageable. It may even be toxic. But I wonder whether a psychologist or social worker might help explore that. Given his age, it might simply be a case where teenage angst was unmanageable and they parted at odds to let the parties mature. Or you may have a case of parent or siblings who were too afraid or confounded to deal with his illness. There are so many possibilities. One of the best things for him may be rekindling the support or connections. So often people regret their mismanaged relationships. But that's absolutely for a professional to facilitate, not you or your daughter. Has he given any indication of wanting to contact them?

 

[ladybird]

Thank you for your support of this young man. He really needs an advocate and I agree with the other posters that what presents as lack of drive is probably exhaustion. He is so young and deserves every chance at life and a transplant. I would also encourage you to contact Beth Sufian at the CF Foundation legal hotline, should you have issues obtaining medical coverage for any of his needs. Perhaps you could consider looking into a nursing home, as it sounds like he needs round the clock care. Also contact the Cystic Fibrosis Foundation for support, they should be able to provide you with some kind of patient advocate. I will keep you in my prayers.

 

[slhogan]

Thank you for all your good advice. It's very overwhelming to read through all this (and all the websites I am reading right now). I can't believe how much work goes into keeping the body functioning. It's no wonder he has been so overwhelmed with it all.

I'm very sorry to share that this afternoon he was intubated and put on a ventilator. I'm afraid my inquiry may be too late. My daughter is by his side in the ICU. His family has been notified.

Is there any chance he'll recover from this?

 

[Gammaw]

I'm so sorry to hear this. You and your daughter gave him a gift of love and caring when he had no one else. Bless you. The best person to ask about his chances of survival are his doctors. No one else knows his condition so intimately. I do so hope his family can respond warmly. I'm sending up prayers for his recovery and comfort and peace for you and your daughter.

 

[believingjesus]

I am so sorry to hear this. It makes me want to cry. This young man deserved so much more than what life gave him. I hope somehow he can pull through. I appreciate that u sought out help for him. Please continue to let us know if he is okay. I hope he can be seen by the doctors in Pittsburgh somehow.

 

[believingjesus]

I hope he is going to pull through. I don't know why some things happen. Shame on his mother and father. I don't care what situation they are in, if they are alive then shame on them. They failed him. What goes around, comes around. I am glad your daughter and your family showed him love. God bless your daughter and your family and him most of all.

 

[slhogan]

Back to my original post that began this thread.... I'm still looking for CF literature that deals with end-stage CF. As I posted earlier, it seems like everything I find online falls into two categories: 1) Overly-upbeat/positive patient information about how to avoid end-stage; 2) Medical journals that I only halfway understand.

I'm looking for patient/family information that gives information and support about the end-stage of CF, such as what DD's boyfriend is in right now. There's a lot to think about and consider, and we're just wading through this with the help of the ICU nurses, trying to figure it all out.

 

[believingjesus]

All I know is to fight CF. I don't have any other knowledge about the end other than to fight. Did you ask his doctors if it is a possibility to get ahold of Pittsburgh? That would be my only advice at this point other than hospice. He can't fight for himself, he has to have someone else do that unless they have told you all there is no hope. I wouldn't always listen to that either as they r wrong so many times. All I know as a mom is that we will fight it. If u want help like hospice, the hospital can maybe set that up for you.

 

[Aboveallislove]

Mom,
There is so much I want to say, much about how wonderful a daughter you must have raised and what an incredible young man he must be to have earned her love, but I want to try to help first with your specific question. Ask the nurse or social worker privately to get a copy of Cystic Fibrosis "A Guide for Patient and Family" from the CF Center at the hospital. They typically give those out to new patients. The book is by David Orenstein and has a chapter and Cystic Fibrosis and Adult and Dying and Cystic Fibrosis. I think that is what you want. The book also has details on all you are seeing now. I concur with all of the other advise.