"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.