Friday, September 24, 2010

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 esophageal varacies, 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 that Gessner 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 by interventional 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.

Wednesday, September 22, 2010

Rough day

Today was a rough day. I'm not sure that I have ever seen Gess in so much pain. It was so hard to watch him in that much pain and not be able to help him. He's always said that he has never cried from physical pain, but I saw tears today and I see his spirit breaking. I worry that he doesn't have much fight left in him. I don't know how he could in light of the unrelenting pain and the utter inability of the doctors to figure out anything to help him. I hate the feeling of helplessness and it's killing me to watch him like this. For now, it's one moment at a time and I just hope and pray that he can get some relief soon.

Wednesday, September 8, 2010


Confession: I am completely exhausted. Physically and (even more so) emotionally/mentally. I know that this isn't any big shocker, but man, am I feeling it today! It feels like it has been non-stop craziness and drama for the last couple of years. I think that part of why it is so hard is that there isn't a real end in sight. This whole transplant process scares the crap out of me. I am scared about the wait and the surgery and the recovery. I am scared that it won't work or he won't make it. The statistics aren't very encouraging. In fact, they depress me. I hear success stories and am tempted to be hopeful, but hope scares me at this point. I think that most of all I am scared that I am not going to make it through this process. It feels selfish to say that since I am just the witness, but there is something in me that is afraid that it will be too much. I know that I need to be strong for Gess and my focus needs to be on helping him through this, but I'm scared.

P.S. Just to make things complicated, we have a separate transplant blog. I will likely post some transplant stuff here, but for more frequent updates visit the blog at