The End

I posted the details of the events leading up to Gessner's death on my blog: http://sickness-and-health.blogspot.com/2010/11/end.html

TIPS Scheduled

Today we met with the interventional radiologist who will be performing the TIPS procedure. He gave us a lot of information. It is interesting to see how different doctors interact with patients. Some give a minimum of information and others give a lot of information. This doctor was one of the a lot of info types--which I appreciate. He explained the procedure to us and drew us a diagram. He also went over the risks in detail. That was helpful, but it was also scary. The other doctors that have spoken to us about the procedure have minimized the risks and really didn't go into detail, but Dr. P went into detail. The biggest risk for Gess is going to be bleeding. Bleeding is always a potential with type of procedure, but it is exasperated by Gess's bleeding issues. Dr. P said that the overall risk of this is under 5%, and more likely around 2%. If he does start bleeding, it will be a very bad situation and there is a chance he can die. They have never had anyone die from TIPS at this hospital, but it has happened at other hospital.

I think that this discussion really just hit home with both of us today. When we were driving home we were talking about our plans for the weekend and possibly getting away. I queried whether the timing would be good given the upcoming procedure, etc., and Gess said, "It might be my last chance to do it because I might not make it." That was hard to hear. I understand it and think about it myself, but it is really difficult to hear him vocalize it. He has always tried to portray an "invincible" attitude, so this change is hard.

Anyway...the plan is for us to see the pre-anesthesia clinic on Friday and the TIPS procedure will be on Thursday, October 21st. Because of Gessner's "difficult" situation, they are going to admit him the day before and give him blood products to try to boost his INR, etc. Then they will also keep him at least until Friday to make sure that he does okay. He will also continue on his course of antibiotics before the procedure.

Clinic Appointment

Gess had clinic today. I had to go to court so I wasn't able to go, but he gave me the blow-by-blow. Unfortunately there was some bad news. His PFTs are the lowest ever, with a predicted FEV1% of 35%. This is down by about 8% from baseline (he hasn't been able to do PFTs in awhile because of the lung bleeding, so his "baseline" could have slid down gradually).

The other bad news is that his weight is down to 112 pounds. This puts his BMI at 18.1. He has to be at least an 18 for transplant and they really want him to be much higher. So he needs to gain weight. If he can't do it on his own, they will put in a feeding tube. Apparently a feeding tube can be problematic with transplant too, so hopefully we won't have to go that route. For now it is going to be eating, eating, eating and at least 3 ensures a day.

Finally a "Real" Update!

This morning we FINALLY got to sit down with Gess's primary CF doctor and get some answers. He was present at the "big" transplant team meeting and so he knew what happened there and has been talking to the liver doctors, etc. The bottom line is that the team is recommending the TIPS procedure (Transjugular intrahepatic portosystemic shunt) and then to continue to work toward the lung transplant. The plan is to schedule the TIPS for about 3 weeks out, at the end of this current course of antibiotics. The plan sounds reasonable. So, if you just want the basics, there you go.

For the longer more detailed answer, here you go. The reason that we were getting so many different answers from the doctors about the "plan" is because there are differing opinions on the team (which is bound to happen when you get so many "experts" involved and have a case that is complicated as Gessner's is) and then also a fair amount of conclusion. Basically, the people that were talking to us probably shouldn't have been saying anything. Dr. T said that he will be the point person and filter the information to us so that hopefully we won't have to deal with the misinformation and confusion again.

The basic issue is that the liver transplant team does not think that Gess needs a liver transplant--or at least not at this point. The risk associated with doing the liver transplant at the same time as the lungs is pretty significant and it would also make it take longer to get new lungs (because of needing both the organs at the same time). The lung transplant team is worried that if they did a lung transplant, Gess's liver would fail shortly after transplant. They are worried that while his liver functions are decent, the amount of cirrhosis, plus his esophagealvaracies, he doesn't have much "functional reserve" and that the stress of the lung transplant and post-tx drugs would be too much.

Enter TIPS. One of the issues with the liver disease is that the cirrhosis causes pressure to build up in the portal vein. This portal hypertension is responsible for the esophageal bleeding thatGessner has had previously. The pressure builds up in other venous systems too, so the doctors think that it is possible that the portal hypertension is causing his lung bleeding (or at least contributing to it). This is supported by the fact that IR has went in to find bleeding twice in the last two years without being able to find any possible sources. The TIPS procedure relieves the pressure in the portal vein by diverting (via a shunt placed in the liver) blood flow away from the liver.

Decreasing the portal hypertension could potentially allow the spleen to decrease is size and the platelets to go up. That would be awesome, but it is definitely not a sure thing. The reduced venous pressure would reduce the risk of the lung transplant procedure and also some post-transplant risks. (For example, there have been patients who survived the lung transplant, only to die from esophageal varacies like Gess has had previously).

There are, of course, risks associated with the procedure. The good news is that it is a "procedure" instead of a "surgery." They do not have to cut up his abdomen, it is done byinterventional radiology. Unless something goes horribly wrong, this shouldn't affect his ability to have a liver transplant if he does need one. If he needs a liver, the TIPS will come out with the old liver. The biggest risk for Gess is encephalopathy (which is basically confusion that can be caused by advanced liver disease). This can be treated with medication, though it can also become very severe and end up in coma. Dr. T is optimistic that if Gess does have this complication it can be treated with medication because he has never had it previously, even when sick, etc.

The plan is to do the procedure in about three weeks, at the end of this current course of antibiotics. The rationale for this is to get Gess as healthy as possible before the procedure.

Then we continue with the lung transplant process. Right now the two issues are pain medication/management and nutrition. The pain medication/management issue is the biggest hurdle right now. Gess has had pretty chronic issues with pain. Right now he is experiencing what he calls "spasms." We're not exactly sure that anything is spasming, but it's the best descriptive term we have been able to come up with. Gess says that it feels like someone is stabbing him in the lung and twisting. They last for about 15 seconds and he literally doubles over in pain. No one knows what is causing them and they are sporadic in nature. The doctors don't know why this is happening and don't know how to stop it. So, he needs pain medications to deal with this. The complication is that he is allergic to most narcotics and can't take most other pain medications because of liver/bleeding issues. So he takes IV demoral, which in the past has been sanctioned by both the CF doc and the pain specialists. The issue is that the lung transplant team wants him off of all narcotics before they will list him. Still not sure how this is going to happen. We have an appointment with the pain specialist on Monday and are going to keep pushing to see if we can find some relief. He is also seeing an allergist in a couple of weeks to see if they can do something about his medication allergies.

The other issue is nutrition. Right now Gess's BMI is right around 18, which is the cut-off for transplant. But, his blood shows that his nutrient levels are very good and he doesn't have any reserves. Gess has been working on gaining weight, but it is hard when he is so sick.

They are also going to do the liver transplant evaluation work up so that if he does need a liver it will be easy to get him on the list and activated.

So...the immediate plan is to get him as healthy as possible, have the TIPS procedure in 3 weeks, and hopefully find a solution to the pain and then get him list for lungs. Whewww...

While it was good to talk to Dr. T and we feel better about the whole situation, we are both struggling to stay positive about everything. We are exhausted and so very tired of hospitals. I know that he is frustrated with not being able to work and his general downturn in health. I am fighting to stay positive and struggling with the way CF has engulfed everything in our lives. So, we take it one moment at a time and hope for some respite soon.

He's still in the hospital and we don't know when he will get to come home--not until he is starting to feel at least somewhat better. They did add a third antibiotic to his mix, so maybe that will be what he needs to start on the road to recovery.

TIPS Procedure

The transplant team had their "big" meeting on Gess today (I wish I could have seen it...apparently all of the "big" doctors were around the table with all of the others around them...sounded like quite a production). I didn't get to hear anything first hand because I had to be in court today, so I will be talking to someone tomorrow and asking some questions.

But the recommendation for now is to do a TIPS procedure to allow some of the blood to bypass the liver, thus reducing the portal hypertension. The idea is that the spleen might reduce in size and his platelets might go up some, which will help with the bleeding. With the procedure, the interventional radiologist goes in and places a stent in the liver. Because it doesn't involve abdominal surgery it shouldn't affect the transplant eligibility.

The idea is that they want to try this first because it is a lot less invasive and drastic than transplant (that's probably the understatement of the year!) and it could potentially help the bleeding, which is the main issue. Gess's lung functions are still relatively good--he's been holding steady at 43% and isn't normally on supplemental oxygen--so that if they can get the bleeding under control, he won't need a lung transplant right now.

I have so many questions and have eaten about a half of a bottle of tums today (not an exaggeration) worrying about all of this, so I am hoping that tomorrow I will be able to ask questions and feel better about everything. They are going to see about scheduling the procedure while he is inpatient, so this could be happening very soon.

Liver Doctor

Today we had an appointment with Gess's liver doctor. We got him up-to-date on all of the transplant stuff and he wants to go ahead and do the liver transplant evaluation just so that we have that out of the way. For the liver tx evaluation they schedule 3 full days of appointments. So, we will do that whenever they schedule it. This doesn't feel like "news" to us, since we have assumed that liver tx evaluation would be coming up. But it does mean more appointments.

There is nothing on the schedule for the immediate future. We are mostly just waiting to hear what the team has to say after their meeting next week.

Waiting

We had the last of the scheduled transplant evaluation appointments on Friday. We met with the transplant pulmonologist and Gess also did his 6-minute walk test.

Now we wait for the transplant team to meet, hash everything out, and come up with a game plan. This meeting is supposed to take place on September 15th, so hopefully we will hear something shortly after then and have an idea of what is going on. One thing that everyone seems to agree on is that Gess's case is tricky. So, we wait.

Gess had a pretty rough weekend. He made the switch from IV pain medication to oral pain medication and that really made him loopy. He also had quite a bit of vomiting and generally did not feel well. He is coughing up some really gross mucus and coughed up a little blood on Friday. Hoping that this turns around soon.

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.

"Vacation" in Colorado

We came to Pueblo, Colorado to visit family last week. It is nice to visit with everyone. We even got to go to our niece's 6th birthday party. Unfortunately, Gess ended up in the hospital on Sunday morning. He had some bleeding from his lung and a lot of lung pain. We went to the ER in Pueblo and he was admitted. Turns out his oxygen levels were really low too (probably caused in part by the altitude and in part because he is just to that point). Things have went fairly smoothly, especially considering the fact that we are at a non-CF center hospital. He has been here for 3 nights so far and I think that they will keep him one more night because they are going to give him a blood transfusion today.

We are getting home oxygen set up and also a portable concentrator for him to use on the airplane. I hope that we won't have big issues with all of this, especially with insurance, etc.

It has been great to have family around and to have them help out. I think that the reality of Gess's health has been hard on them (understandably so), but it has also been really good for them to learn about the procedures, etc. I feel good about the situation and think that this will help make things easier in the future. It has been good for relationships, etc.

So...hopefully we will be out of here soon and able to get back to Seattle.

On the transplant front...all of the appointments for the lung evaluation are set up. We start next Monday and it will be a whirlwind of appointments for about a month.

Squeaking along

The squeaky wheel gets the oil...so I am squeaking away! We still haven't heard back from the scheduler, so today I called the transplant coordinator to check in. The coordinator said that she talked to the scheduler today about Gess, so that work is being done and we should hear soon. So, hopefully in the next couple of days we will get a call from the scheduler (or I'll squeak some more!).

On a health update...Gess is still not feeling well. He is sleeping a lot and not doing much else. He has lost so much weight, we are really worried about that. He weighed in at 114 the other day (fully dressed PLUS a lot of edema, which likely added at least a couple of pounds in water). This is definitely not good and it will definitely be on our list of issues to discuss at clinic in a couple of weeks. He will need to get his weight up for transplant and we're not sure how that is going to happen.

His mood is pretty low. Hopefully he will start to feel better soon and the mood will improve.

We are supposed to be heading to Colorado this week to visit his family, but I am really concerned about his ability to travel. I know that he does not want to hear it, but I'm not sure he is healthy enough and am afraid that the travel will end up making things worse. I want him to be able to see his family, but I am worried. So...we'll see how it goes.

Rough few days

Gess is having a rough time. He feels sick and is not getting better. He is frustrated. I am listening to his labored breathing right now and wish that there was something that I could do.

We still haven't heard back from the scheduler--I am going to try to contact someone else on the team to see if we can get this ball moving.

Waiting and Health Update

Maybe the scheduler is preparing us for the frustration and helplessness of "the wait" or maybe she is flaky or maybe Gess keeps missing her calls...I don't know, but we are waiting to actually connect with the scheduler to set up the first round of appointment. Did I mention that waiting sucks?

Gess also took a trip to the ER this week. He was not feeling well and in typical Gessner-fashion debated it over and over. He finally called the pulmonary fellow on call and explained what was going on (shortness of breath, pain in his lung, increased cough, increased sputum, etc.) and *surprise* the doctor told him to come into the ER. I had taken an ambien already (I have major sleeping issues) and so wasn't in a good position to take him, so he opted to go by himself. He said that the ER was crazy busy and he waited in the waiting room for over 4 hours. The chest xray showed a spot (or lesion) on his right lung. They think that the spot is a bacteria infection (aka pseudomonas) but it could also be another fungal infection. Gess's main CF doctor recommended that he try to treat this infection at home because of the germ exposure at the hospital. So, after about 18 hours he came home with orders for IV meropenem, inhaled TOBI, and Demoral.

As for me--quite honestly I am exhausted in a way that I cannot fully explain. The exhaustion is beyond physical and can only be described as total. Not sure the best way to recover from this, so for now I will just continue to plug along.

First Step: Financial Interview

So, after the initial call for the transplant coordinator tell us that Gess was referred for transplant evaluation, we were contacted by the Financial Services Specialist. This appears to be the first "official" step for us. I was able to call and do this portion of the process. Basically the Financial Services Specialist contacted our insurance company to make sure that we have sufficient coverage for the lung transplant and the aftercare. The lung transplant costs $500,000 (for the lungs themselves and the actual transplantation and also for the pre- and post- hospital/tests). This is a rough estimate and can change dramatically if there are any complications or we do go the lung/liver transplant route.

This was the first time that the reality of lifetime benefit caps on insurance policies hit home. Our policy has a $2 million limit. So far, he has used $570,000. If all goes smoothly, we should be fine. But if there are complications, we could reach that amount. Plus, after a couple of years of post-transplant medications, we could certain get over that amount. Thankfully, one of the provisions of the Health Care Reform Act that goes into effect immediately (which in congressional terms means after 6 months) is the ban on lifetime benefit caps. So...we shouldn't have to worry about that at least for now, which is a big relief for me.

There are also significant prescription costs for post-transplant medications. The estimate given to me was $6000 per month for the first 3 months and then $2000 per month for the rest of his life. Our insurance will cover significant portions of this, so we shouldn't have a problem, but it is pretty daunting to think about the cost.

Also, these numbers don't include other expenses or taken into account the fact that Gess will be out of work for awhile. He's overall hope is to work as long as possible before the transplant and his employer is being really good about supporting that, then go on short-term, then long-term disability. We hope it will work out that way, but it is of course possible that it won't go as planned and he won't be able to work. So, we have to consider that and also the effect it can have on my job. Whewww....so SCARY!

The Financial Services Specialist sent us some forms to sign and we returned them out. So, step one is done. Apparently the Financial Services Specialist is going to notify the team that we are a "go" on the financial side. Now we wait to be contacted by the team to schedule the next step.

Introduction

This is a blog about our journey through the transplant evaluation process. I am Lisa and am married to Gessner. Gessner has cystic fibrosis and just started the transplant evaluation process. This blog is to keep our family and friends updated about the process and also to educate others about cystic fibrosis and transplant. Here's a little about us:

Gessner was diagnosed with cystic fibrosis when he was three months old. He has one older sister who does not have CF, but she is a carrier of the gene that causes CF. Cystic Fibrosis is a genetic disease caused by mutations of the CTFR gene. The result of these mutations is that the body produces a thick, sticky mucus that clogs the lungs and other organs (particularly the pancreas). Beginning at an early age, Gess started to take pancreatic enzymes to help his body absorb food. He also did daily treatments that include chest physical therapy (CPT) to break up the mucus. As a teenager, Gess developed portal hypertension as the result of mucus build-up in his liver ducts, result in cirrhosis of the liver and an enlarged spleen. He also developed CF-related diabetes (CFRD) and is insulin-dependent.

Gess has frequent lung infections that often require IV antibiotics to fight the bacteria pseudomonas aeruginosa, which he chronically cultures in his lungs. The liver disease also has the effect of suppressing his immune system. His enlarged spleen causes his blood clotting factors to be low as well. He has also experienced frequent episodes of bleeding from the lungs (hempotysis) and has had multiple bronchial artery embolizations (BAEs) to stop the bleeding.

The hempotysis can be very dangerous and the multiple BAEs have damaged his lungs. This, along with lost lung function due to the progression of the disease have lead to the decision to consider transplant. Gess's situation is particularly difficult because of his liver disease. By traditional transplant evaluation measures his lungs are not "sick" enough for him to receive a transplant. Additionally, his liver is not sick enough for transplant. But, because of the difficulties that his case presents, his CF doctor at the University of Washington Medical Center decided to present his case to the lung transplant committee. Their initial decision was to refer Gess for a lung transplant evaluation and to consider a staged approach to the transplant process. Under that approach, the lung transplant would happen first and then after he recovered from that, he could get listed and have the liver transplant. We honestly have concerns about this approach and will be talking to the team about it. The lung team will also consult with the liver transplant team and get their input.

According to the United Network for Organ Sharing (UNOS), only 37 dual liver/lung transplants have been done in the United States. So we are in pretty uncharted territory. We are doing all the research that we can on our end and will be actively involved with the team during the process.

In the meantime, Gess continues to work as the Offshore Initiatives Project Manager for an online travel company.

Gess met Lisa while in college and they have been married for 11 years. They have a cute, 9-year old beagle named Beauty.