Lung and Liver Surgeons

Wow, the last two days have been busy! I seriously feel like we have crammed at least a week into these two days. I am so exhausted! But, I wanted to give everyone a quick update (it's late and I'm exhausted, so please excuse any typos, etc.).

Monday

We met with the lung transplant surgeon bright and early at 7:30 am. When our CF doctor first presented Gessner's case to the transplant committee, the initial reaction was to transplant Gessner's lungs first and then, after he recovers, the liver. But apparently the lung transplant surgeon now thinks that it would be better to do both the lungs and liver at the same time. Apparently Gessner has pretty good liver functions and that if his liver disease stayed as is, he would never get a liver transplant. But, the lung transplant will put a lot of stress on his liver and the surgeon is concerned that his liver wouldn't survive the procedure and he would go into liver failure before they were able to get him a new one. This opinion is based on the level of cirrhosis in Gessner's liver and the fact that he has varicies. His opinion is that Gess's liver doesn't have enough "functional reserve" to sustain the lung transplant.

Transplanting the liver at the same times as the lungs is an even riskier procedure and does not have as good of outcomes as just a lung transplant. The procedures are difficult to do together because the systems are so different and the treatment immediately post-op is different.

The other issue that we addressed was pain medication. Gessner has had a lot of trouble with pain (in his lungs). He can't take ibuprofen (because of his bleeding issues) and can only take a bit of tylenol (because of his liver disease). He is also allergic to nearly all narcotic pain medications. Because of this, he takes IV demoral. He has needed it a lot more lately. He has worked with the pain specialist to try to find an alternative to control the pain, but nothing has worked so far. The issue is tolerance. With the long term use, Gess has built up some tolerance to the drug. The concern is that post-transplant they would not be able to control the pain and then Gess wouldn't be able to recover well. So, he needs to try to eliminate the IV demoral or at least dramatically reduce the amount he takes in order to "reset" his tolerance point. I'm not sure how this is going to affect the timing of listing or the rest of the evaluation at this point.

From the surgeon's standpoint, the things that Gess can do are 1) exercise, 2) gain weight, and 3) work with the other docs on the demoral issue.

We didn't really talk about the lung transplant surgery itself, so perhaps we will have to have another appointment with him once everything else is settled. I'm not sure about that.

After meeting with the lung transplant surgeon, I went to work and Gess went to CF clinic. He talked about the pain management issue and the CF doctor is on the same page as the transplant surgeon. They talked about the difference between palliative care and gearing up for transplant. Right now Gess's lungs seem to be okay infection-wise, so not antibiotics. Next clinic appointment is in a month.

Tuesday

This morning we met with two of the liver transplant surgeons. This appointment was supposed to be later in the week, but after Gess's last hospitalization the transplant coordinators moved the appointment up. The liver transplant doctors are not sure about the liver transplant and whether Gess needs one. They said that his liver functions are actually pretty good. What they would like to do is look into some other options to see if there is an alternative to transplant. The regular liver doctors have considered these things as well, so I am not convinced that anything will come of this, but it is worth the doctors at least having a discussion. The idea is that there may be a way to fix or at least reduce his bleeding issues, which could reduce the hemoptysis (bleeding from the lungs) and thereby delay the need for transplant.

The options that the liver transplant surgeons talked about are removing or reducing the size of his spleen, trying some different medications, and a TIPS procedure (transjugular intrahepatic portosystemic shunt). Again, we aren't sure if any of these will be viable options for Gessner, but it is something that the transplant teams will discuss.

Second appointment of the day was with the diabetes clinic. Gess's sugars have been running a bit high, so the doc thinks that getting them under better control will help Gess gain weight. So, we came up with a new plan for his insulin.

Third appointment was dentist to make sure that his mouth is infection-free. Good to go on that one.

After all of that we both got massages and then went to a movie, which was a decent end to a hectic day.

On Friday we see the lung transplant pulmonologist, Gess does his 6-minute walk test, and we meet with the pain specialist. Those are all of the transplant appointments that we have scheduled right now, so hopefully we will find out the next steps soon.

Attempted Embolization

So, Gessner has continued to bleed from his lungs and had another really large bleed on Sunday. We headed to the ER and he was admitted. The medicine team talked to the radiology team and everyone decided that it was worth another look into Gess's lungs to see if any of the bronchial arteries were abnormal. They did a bronchocospy on Tuesday to do in and explore to see if they could narrow down the area to focus on and saw that the right lower lobe seemed inflamed. So, that was the area of focus. He went down to radiology around noon and didn't get back up to the room until 5:00 pm, so he was on the table for a long time. Unfortunately they were not able to locate anything to embolize. The good news is that he went through the procedure well and there were no complications.

He isn't actively coughing up blood and the strange spasms he has in his lung are not as frequent, so he will get to come home today. The frustration is that they were unable to find the source or bleeding, so it may very well come back again in a few days. That has been the pattern--big bleed, then hospital, then discharge, home for a couple days, then big bleed again, hospital again, etc.

Beyond that, we are just continuing with the transplant evaluation appointments. He had two scheduled for today and they are held in the main hospital, so we decided to go to them. Unfortunately someone somewhere canceled them! The nutritionist was able to see us and talked to us about what he needs to do now to gain weight and then some of the stuff for post-transplant. The nutritionist wants him to eat at least 3200 calories a day and that is going to be a real challenge for him. But, he needs to put on some weight, so hopefully he will be able to do it. To be eligible for listing he needs to have a BMI of 18. He's 18.9 today. But his weight has been down low enough to put him just about 18 in recent days.

Next week we meet with the lung transplant surgeon, diabetes clinic, dentist, lung transplant clinic, and liver transplant surgeon. He will also do his 6-minute walk test. We still need to have his bone scan rescheduled and he needs to see the pain specialist. He has an appointment for the pain specialist in mid-September, but we are trying to get that consult earlier so that we can wrap up the evaluation and see where the team stands on listing.

Lung Transplant Education

We had lung transplant education today. It was basically an information session held for patients and their support teams. Gess's sister Diana joined us for the session. It was informative, but honestly it sort of freaked me out. It makes this whole process seem so much more "real" and it really hit us that this could be happening a lot sooner than either of us really expected.

Here's a brief description of what happened:

They told us about the evaluation process and the tests that are required for that. Gess has gotten several of these tests done and will get some more in the following weeks. As far as I know, he will be finished with all of the tests on September 3rd. He does have to consult with the pain doctor again and our current appointment is not until mid-September but they are going to try to get him in earlier. When all of the tests are done the transplant team (which is made up of the surgeon, social worker, pulmonologist, dietitian, etc.) meets and makes a decision on listing. The team could decide that they need to do more tests or evaluations. They could also decide that Gess is not a good candidate for some reason and decide not to list him. Or they could decide that it is time to list him and put him on the transplant list. Because Gess's situation is a bit different than the "normal" situation for transplant, the team will likely appeal to the powers that be for an exception to get him a better placement on the list. They said that we should hear about the listing decisions within 1-2 weeks after all of the tests are finished. So, conceivably he could be listed by mid-September, but it could also take longer depending on any number of circumstances.

Once he is listed, we basically wait. The average wait time at the UW is 3-4 months, but ranges anywhere from zero days to about 2 years. We need to be "on call" at all times and be able to get to the hospital within 3 hours of receiving "the call." The "call" is made when there is a likely match. This is based on a preliminary assessment of the donor's lungs, etc., so it doesn't mean that the lungs will turn out to be good, etc. The UW surgical team will have to go to the hospital were the donor is, assess the lungs, procure them, and then transport them back to the UW. At the same time, they need to make sure that Gess is healthy enough to receive the lungs. If he is having some sort of infection or fever or other issue, he might not be able to accept them. If both Gess and the donor lungs are good to go they will take him off to surgery. The surgery lasts about 6 hours for a double lung transplant, but could be more or less depending on what goes on.

They expect him to be in the ICU for a few days following the surgery, with some of that time on a ventilator and with various tubes and wires. They expect the hospital stay to be about 2 weeks but that can fair from as little as 1 week to several months depending on the circumstances.

Once he is home, he will have a pretty rigorous maintenance schedule, especially at first. He will be on a number of new medications (8-10 new ones) and will have to carefully monitor his lung function and vital signs every day. He will need someone with him 24/7 at first and will have weekly appointments. He is supposed to get a lot of exercise, but will have to be careful with arm movements because they cut the sternum during the procedure and it takes about 8 weeks for that to heal.

More hospital time

Gess is back in the hospital...I think that this is the fourth time in four weeks, but who's counting? More bleeding from his lungs. It seems like the bleeding is getting worse. On Monday he ended up with about 6 cups total (which is 3 pints)! The docs gave him 2 unit of blood and admitted him to the hospital. The painful spasms in his right lung continue and no one seems to have any idea what is going on.

They are continuing to do transplant evaluation stuff, so at least we are getting that done. They did a bunch more blood work (taking 15 vials!) and more urine. They were also able to do the right heart cath today. Apparently the doctors want to do a endoscopy too, but I am not sure if they are going to be able to do it while he is inpatient.

Gess's sister Diana is in town and she has been doing a lot while he is in the hospital. She and Gess conspired to have her come into town for a week and send me away for a little break. I went to Portland for a few days and was able to get some rest and relaxation, which was very nice. And while I was gone, Gessner and Diana did a lot of cleaning since they know that having a messy house stresses me out. This was all supposed to be a surprise (I really do have a sweet husband), but their dad let the cat out of the bag. So, it wasn't a surprise, but still a wonderful gift.

Tomorrow we are supposed to have an appointment with the liver doctor but since Gess is inpatient, I don't think that we are going to make it. We also have lung transplant education, which I think that we are going to go to, inpatient or not.

He missed his DEXA scan (again!) and diabetes appointment because he was inpatient, so we will have to schedule that.

Updates

More drama on the CF front. On Saturday Gess coughed up 3 cups of blood. He is certain about the amount because he happened to have an empty pint glass next to him. Definitely not good. The pulmonary team on call was headed by the director of the CF center and she told Gess that he needed to come in. The good thing was that she called the ER ahead of time and that paved the way to get him admitted to the ICU. The issue was whether or not to call interventional radiology (IR) in to see if they could locate the source of the bleeding. To be blunt, IR wants nothing to do with Gessner so it was no surprise that they didn't want to go in. The CF doctor did talk to them, but they decided not to go in this time. The recommendation was to press on with the transplant evaluation as that is the only real option.

The medicine team that followed Gess this time (and last) was great. They got a few of his tests done, so we are on track evaluation-wise. They ran some blood work, did a CT of his sinuses, an echocardiogram, and an esphogram.

I did have one freak out when the tx coordinator told me that "there is no way they are going to list him while he is on pain meds." In following up, I think that this was a vast overstatement. The pain management is a big hurdle because of his extensive allergies, etc., but the pain specialist was reassuring that this will not be an exclusionary issue. We need to see the pain specialist for (another consult) but they don't have any appointment soon, but they are going to work on trying to get us in sooner.

What's up for next week: DEXA scan, diabetes clinic, hepatology clinic, and lung transplant education class. Gessner's sister Diana and her daughter Hannah are going to come for a week and give me a break. This was intended to be a surprise, but Gess's dad let the cat out of the bag. It stressed me out a bit because my house is a disaster, etc. But...I am trying to let it go and try to take this as a very nice gesture from my husband and his sister. I am going to go out of town for a few days without many plans...just chill, sleep, and try to relax. I believe that the plan is for Diana to clean the house some--which will be awesome, though the whole thought of someone seeing my mess causes a slight (or not so slight) panic. I'll get back in time for some work obligations and the transplant education class.

To follow: nutritionist, heart cath, social worker, lung transplant surgeon, lung transplant clinic, pain clinic, and liver transplant clinic. If we can get squeezed in for the pain clinic, all of this will be done on September 3th. Yikes!

Home and more hospital

So, we finally made it home from Colorado--thank goodness! It was great seeing family and hanging out, but it was not fun to be in a "foreign" hospital. The moral of the trip is that Gess cannot go to places with high altitude without supplemental oxygen and if we fly, he'll need oxygen.

The same day we got home from Colorado, Gess was admitted to our local hospital. He had quite a bit of bleeding from his lungs, which lead to the admission. I am worried about the increase in bleeding. It seems to be happening more frequently and the bleeds seem to be getting "bigger" (meaning more volume). The good news is that he feels like his exacerbation is getting better, so maybe less infection will lead to less bleeding. Let's keep our fingers crossed!

On the transplant front, yesterday (Monday) was the first day of testing. He got poked for a TB test and has to return tomorrow for the nurse to read the result. He also got paperwork and instructions for a bunch of blood work and a 24-hour urine test.

On Thursday he will have a bone density (DEXA) scan and also has an appointment with the diabetes clinic.