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.