Husband with CF, declining health,lungs functioning at less than 20%,what can be done

[Shelby Delacruz]

Hi, my name is Shelby, my husband's name is Brandon. He has CF, the mutation F508del and P. aerugonisa, which knocked him off of Keck Hospital's transplant list earlier this year. In mid-july we were told he has 6 months to a year left to live, and then yesterday the docs came in and said "time is running out". He's been in the hospital for over a month now, some days hes good, most days he's bad. He's almost always short of breath, when he stands or moves much, it becomes severe. He's been coughing up blood a lot more than usual. They had him on heparin and then they took him off, he almost filled a cup with pure blood, but hasn't had any since then, until today there was a small amount.

We've both been stressed due to family issues, and I think it's really taking a toll on his health. He was doing great before some of these family issues popped up, and now it's like a rapid decline. He's getting benadryl and morphine every 4 hours, along with ativan for anxiety due to being on the bi-pap. They lowered his treatments from every 4 hours to every 6 hours even though we feel like they should be increased, not decreased...his doctors act like they're doing all they can but I feel that they aren't. I've been on this forum for less than an hour reading and I see so many different things for people with his mutation that help them, that hasn't even been told to him.

He hates being on the bi-pap but his Co2 levels are rising up to the 70's because he's just not able to get enough of that Co2 out with his breathing. His constant morphine is also a huge factor in it, but the doctors aren't making any point to stop him from receiving so much, or offering an alternative pain medicine for the soreness from his new port. It's so frustrating to sit here and be in this research hospital thinking they're are better doctors and hospitals that could make him better but they're out of our reach!

Another thing I want to know is if we should even be at this hospital.. it is a research hospital, and there are grad students always wanting to come in and observe him, touch his port, give him IV's and things that they don't really know how to do yet. We had a nurse in training or whatever do a blood gas on my husband...and it took everything I had not to start causing a scene because this trainee had no idea what he was doing! He couldn't find the arterial vein, and just kept digging around in my husband's arm, CLEARLY hurting him until my husband finally said to just stop and don't touch him again.

I just really need some suggestions on what can be done to preserve his health, better his health, keep him alive for as long as we can...what hospitals in CA are best for CF, and how to find decent housing for disabled young adults (he's in a wheelchair)?

 

[Kyle O'Neill]

Im 26 with a 20% lung function, I too have Pseudomonas....I too was kicked off a transplant List... But I went to another Hospital that was near me. And now I'm apart of that program, going through their work up phase. Maybe that would be a possibility too... Try another Transplant Center...

 

[jaydub75]

I have a page on Facebook and it would be a whole lot easier if you joined and I could answer those questions I know both of you guys have. Its called lung transplant (single and double) the profile pic is a set of blue lungs look forward to talking to you two soon.

 

[Kyle O'Neill]

I didn't have any questions. I was suggesting that person try another Transplant Center because they were kicked off of their List... That happened to be May 6th this year. But now i'm working my way back on the list by going to another Transplant Center.

 

[Shelby Delacruz]

Wow! That's great to hear Kyle, we're happy for you. This sounds like the best viable option for us as well, I really don't like this research center. Too many noobs poking around without knowing a thing about CF.

I'll add that FB page as well, Jaydub.

 

[lexiesmommy]

Shelby,
I am not sure why he was kicked off list because he has pseudo.?? Most all CF lungs by the time they get to transplant all have it. I do and I qualify at both centers where i was evaluated. Anyhow, you asked about CF centers. I spent my first 40 years in southern california. I will say USC for southern and Stanford for Northern is two top transplant centers. There are many transplant centers in CA. I went to CF center at USC and loved it. Having said that, his docs need to be on phone calling other centers to see who will take him. You may have to stay behind them and even start making calls yourself. Be agressive!! Is he in Pulmonary Rehab? That is extremely important as they want to make sure he will be strong enough the make it through surgery and recovery. What CF center are you at? Wishing the best for your husband!
Christina 46pwcf

 

[Shelby Delacruz]

We are currently at USC, and they've knocked him off their list permanently. His doctors aren't trying to find anywhere else to take him, they're basically just doing the same ole same ole. While I love the staff here, our general nurses, the food and nutrition people, we have to find somewhere that will take him. We both like USC in regards that it's nicely kept, and it's a pleasant place to have to stay for a few months at a time, but it's just not delivering in the CF department for Brandon. The full name for his alcalegene is Pseudomonas aeruginosa.

 

[erock77]

Shelby this sounds odd to me as well. I just looked on CFF's care center list in CA and Keck is listed, so you must have a CF doc overseeing him. Honestly I think most CF centers are teaching hospitals, it's pretty common. But nothing should get done without approval from a CF doc.
I've never heard of aeruginosa being a problem for transplant, most everyone with CF gets this early on in life. I've had it since I was 2. There must be another reason, I would push the issue with the CF doc as to why and if other centers might pose a better chance.
Btw, A list of CF centers can be found here: http://www.cff.org/LivingWithCF/Car...tion-accreditedCareCenters/index.cfm?state=CA

 

[Shelby Delacruz]

Shelby this sounds odd to me as well. I just looked on CFF's care center list in CA and Keck is listed, so you must have a CF doc overseeing him. Honestly I think most CF centers are teaching hospitals, it's pretty common. But nothing should get done without approval from a CF doc.
I've never heard of aeruginosa being a problem for transplant, most everyone with CF gets this early on in life. I've had it since I was 2. There must be another reason, I would push the issue with the CF doc as to why and if other centers might pose a better chance.
Btw, A list of CF centers can be found here: http://www.cff.org/LivingWithCF/Car...tion-accreditedCareCenters/index.cfm?state=CA

Alrighty thanks a bunch !

 

[Shelby Delacruz]

I just got a print out of all the bugs my husband has. They read as follows: Pseudomonas Aeruginosa, Multi-drug resistant Pseudomonas aeruginosa+, Burkholderia Species, Stenotrophomonas maltophilia, MSSA, MRSA and H. Influenzae

How to treat/get rid of these? I feel like they're what's making his health so crappy right now. But the docs are acting like it's just his health is declining and nothing can be done, or treated. It's frustrating.

 

[erock77]

I've heard of transplants being denied due to Burkholderia Cepacia, but that was over 10 years ago. I don't know why cultures pose an issue. I'm sorry Shelby, I wish you guys good luck.

 

[bcl0328]

be careful with MRSA, my brother died from it.

 

[longtimer]

Ok
The reason he is not being considered is probably because of his Burkolderia not his Pseudo aeruginosa. I suggest

1. there are centers who will transplant patients with MRSA and cepacia. I suggest you doctor shop and ask UC San Diego, UCSF and Stanford if they will take a CF transplant patient with cepacia. I would google the center and then call the nurse case manager directly. UCSF will especially try to do patients on BIPAP and vents if their nutrition is good-frequently patients that other centers have turned down
2. Is he on maximum therapy? Someone in his status I would have on therapy vest 60 minutes four times a day, albuterol four times a day, hypersal twice a day, pulmozyme twice a day. Is he on Cayston or TOBI. I use these in addition to iv antibiotics when sicker. Have they tried Linezolid to treat the MRSA? Septra to treat the cepacia? These are just suggestions.
3. Did he have an echo of the heart? Sometimes my patients respond to a trial of diurectics and some people will treat pulmonary hypertension if it is there.
4. Pulmonary rehab is important. Do they have an exercise bike in the room?
5. Morphine can decrease your drive to breath as can benadryl. Is he on antidepressants? Paxil or Zoloft work wonders
6. Heparin for hemoptysis does not sound right. He should be on Vitamin K 10 mg by mouth every day
7. UNC Chapel Hill will transplant cepacia.
8. Sounds like you need to be your own advocate.
9. When you call around, I would call the transplant center directly an not the CF Center to start. They will give you the info about the cepacia.
10. good luck

 

[Shelby Delacruz]

Ok
The reason he is not being considered is probably because of his Burkolderia not his Pseudo aeruginosa. I suggest

1. there are centers who will transplant patients with MRSA and cepacia. I suggest you doctor shop and ask UC San Diego, UCSF and Stanford if they will take a CF transplant patient with cepacia. I would google the center and then call the nurse case manager directly. UCSF will especially try to do patients on BIPAP and vents if their nutrition is good-frequently patients that other centers have turned down
2. Is he on maximum therapy? Someone in his status I would have on therapy vest 60 minutes four times a day, albuterol four times a day, hypersal twice a day, pulmozyme twice a day. Is he on Cayston or TOBI. I use these in addition to iv antibiotics when sicker. Have they tried Linezolid to treat the MRSA? Septra to treat the cepacia? These are just suggestions.
3. Did he have an echo of the heart? Sometimes my patients respond to a trial of diurectics and some people will treat pulmonary hypertension if it is there.
4. Pulmonary rehab is important. Do they have an exercise bike in the room?
5. Morphine can decrease your drive to breath as can benadryl. Is he on antidepressants? Paxil or Zoloft work wonders
6. Heparin for hemoptysis does not sound right. He should be on Vitamin K 10 mg by mouth every day
7. UNC Chapel Hill will transplant cepacia.
8. Sounds like you need to be your own advocate.
9. When you call around, I would call the transplant center directly an not the CF Center to start. They will give you the info about the cepacia.
10. good luck

Wow thanks, great info. Will be bringing all of this up, thanks again!

 

[Shelby Delacruz]

Ok
The reason he is not being considered is probably because of his Burkolderia not his Pseudo aeruginosa. I suggest

1. there are centers who will transplant patients with MRSA and cepacia. I suggest you doctor shop and ask UC San Diego, UCSF and Stanford if they will take a CF transplant patient with cepacia. I would google the center and then call the nurse case manager directly. UCSF will especially try to do patients on BIPAP and vents if their nutrition is good-frequently patients that other centers have turned down
2. Is he on maximum therapy? Someone in his status I would have on therapy vest 60 minutes four times a day, albuterol four times a day, hypersal twice a day, pulmozyme twice a day. Is he on Cayston or TOBI. I use these in addition to iv antibiotics when sicker. Have they tried Linezolid to treat the MRSA? Septra to treat the cepacia? These are just suggestions.
3. Did he have an echo of the heart? Sometimes my patients respond to a trial of diurectics and some people will treat pulmonary hypertension if it is there.
4. Pulmonary rehab is important. Do they have an exercise bike in the room?
5. Morphine can decrease your drive to breath as can benadryl. Is he on antidepressants? Paxil or Zoloft work wonders
6. Heparin for hemoptysis does not sound right. He should be on Vitamin K 10 mg by mouth every day
7. UNC Chapel Hill will transplant cepacia.
8. Sounds like you need to be your own advocate.
9. When you call around, I would call the transplant center directly an not the CF Center to start. They will give you the info about the cepacia.
10. good luck

We just talked to ID, infectious disease, and the lady we spoke with gave me completely different info than the doctors did a week ago. She said he doesn't have the cepacia, even though the docs said he tested positive for it, he has the aeruginosa and some other alcalegenes. Some Xydos... I can't remember the rest. He's still growing them, but I'm going to see if we can get another culture and I can have the exact results and I'll post what I get.

 

[Shelby Delacruz]

We just talked to ID, infectious disease, and the lady we spoke with gave me completely different info than the doctors did a week ago. She said he doesn't have the cepacia, even though the docs said he tested positive for it, he has the aeruginosa and some other alcalegenes. Some Xydos... I can't remember the rest. He's still growing them, but I'm going to see if we can get another culture and I can have the exact results and I'll post what I get.

I just spoke to the docs again, the alcaligene that has him frozen on the transplant list is called Xylosoxidans ssp. It's resistant to every antibiotic given to him, Amikacin, Cefepime, Ceftazidime, Cipro, Colistin, Gentamicin, Imipenem, Meropenem, Minocycline, Piperacillin, Ticarcillin, Tigecycline, Tobi, Trimethoprim

 

[JustDucky]

My guess is that the Burkholderia probably was more of a factor than the pseudomonas as far as denial of transplant goes. Not many centers take cepacia patients into their tx program, but there are a few, you might want to look into them ASAP. Currently, I do not know the names of the centers, I think UPenn might be one of them, maybe Duke....others will chime in here with the correct centers.
Check out other centers, especially if you aren't happy with his current care.
Keeping you both in my thoughts and prayers

Jenn 40 wCF

 

[CrisDopher]

In addition to echoing the thoughts of others here, I am wondering why he's on morphine? When I got my port, all I needed was Tylenol for a week. There shouldn't be that much pain. A patient being on morphine is a huge red flag to me. Another red flag is "teaching hospital". No more trainees, not at this point. Either he gets treatment from trained and experienced doctors and nurses, or you find a new place to go!

My FEV1 right now is 24%, but I'm still getting around. I'm in transplant territory and have been accepted as a patient at my center, though not listed yet. Even if I were listed, the Cepacia and MRSA would knock me off the list until they could be eradicated. Pseudamonas is - honestly - rarely eradicated in us cystics and most transplant centers don't care about it much. However, they need to know a) your husband would survive the procedure and b) present infections (colonized in throat and sinuses) would not immediately overwhelm and destroy new lungs. It's a very difficult road to walk, I know, and I am hoping for the very best for you both.

 

[maryiris]

Like the others, I too have pseudo and not a problem - the difference could very well be that the pseudo is resistant to the available meds.

 

[azdesertrat]

You have the option of saying 'NO STUDENTS!' I did. I was a 'Science Project' for plenty of students in my time & now I refuse. It is your right!
If his lung capacity is 20% I think its time to consider a transplant. I was at arounf 20% when I was listed.
By the time I got new lungs I was at 13%. I think this is his only alternative. I know all about the pain meds; I have chronic pain issues even post-trans.
Transplant is not fun, but it is absolutely do-able! I would have been dead by Aug '05 without it. Please keep me advised.
If there is anything I can do to help, please don't hesitate to ask. I will be more than happy to share my experiences or just be a shulder to cry on.
This is no fun; I know, I've lived through it.
Best of luck, you're in our prayers, 'Pat'.