Tuesday, June 4, 2013


Let's start with my progression. BMI is now 31.9. It's coming down slowly but surely and with the last few months we've had, I'm not going to beat myself up about it. I've lost 17 pounds total.

In April and early May, James spent 11 days in the hospital with a terrible lung infection. It caused empyema which is a build of infected fluid in and around the lung which caused it to collapse. He thought he had bruised or broken a rib and didn't get it looked at right away. It had progressed very far by the time he got to the hospital and he needed surgery to clear out all the pus from his chest cavity. He is finally feeling better now.

Not to be outdone by his father, Seamus had to make a hospital appearance as well. Seamus had been having a hard time with his nightly feeds for weeks and on May 19th he vomited again but this time there was a fair amount of blood. I contacted his GI doc who said he should get an endoscopy soon to see if he had more varices that needed banding but it wasn't an emergency. The next day he went to school and vomited again with a lot more blood. We headed straight to the ER.

He was admitted and an endoscopy was done the next day. They kept telling us they were sure it was just a tear in his esphogus from all the vomiting and if he had more varices, he wouldn't be acting so happy. I told them "You don't know Seamus".

After the procedure, Dr K asked us to talk in another room which is never a good sign. Usually the docs talk to the parents in the waiting room right in front of everybody. He told us Seamus had two grade 3 varices which they banded and another grade 2. Grade 3 are very serious. It means the cirrhosis has progressed and the portal hypertension has gotten worse causing the pressure to build up. The blood vessels were so big they nearly filled up the whole esphophagus.

While he was under, they changed his feeding tube from a g-tube that goes into his stomach to a g-j tube so the feeds could bypass the stomach and go directly into the intestines. This is to help his nutrition level because currently he is severely malnourished, partly because of his cirrhosis and partly because with his chronic constipation, food is not being absorbed. Immediately after the surgery, he vomited and dislodged the new g-j tube so it was unusable.

Two days later, they put him under again to replace the g-j tube and things went fine until a few hours later when he started feeling ill and spiked a fever of 105. They started him immediately on antibiotics which was good because he ended up having sepsis. During the procedure with all the manipulation of his gut, bacteria crossed over into his blood causing an infection. Sepsis is very dangerous, especially in people who are weak and have low immune systems. They, and we, were very worried about him.

During that time, we were in discussion with his doctors about how to proceed with his care. At this point, he has them perplexed. He was only diagnosed with liver disease a year and a half ago and it's progressed faster than anyone could imagine. It's definitely time to start talking about transplant. We saw the medical director of the CFF in clinic a month ago and he told us we should probably talk with Dr Michael Narcowicz in Denver because he is the top GI/liver doctor in the CFworld. We brought this up to Dr K after the procedure and he agreed it would be a good idea. Besides just discussing tranplant options, he would like Dr N's advice on improving Seamus' nutrition (which is necessary for Seamus to recover from a transplant) and any other ideas on handing his chronic constipation. And on top of everything else, we can't forget about his lungs. Although his lung function is still very good, his x-rays do show CF lung progression.

So that's where things stand. He is now doing 18 hour feeds everyday as well as 1.5 hour of medicines through his tube so he is basically constantly attached to his pump. He is in good spirits although he has "the worries" often and is seeing a psychologist to help him feel better. We are all looking forward to going to Denver next week but I am very nervous because I think our life may be changed completely after meeting with Dr Narcowicz. I'm trying not to think about or make any plans for the future because things may all change.

Our wonderful friends set up a fundraising site to help us with the travel and medical costs and it raised $3000 in just a few days. I can't thank them enough, that money will help us out tremendously.

Here's a cute video of the kids in the sprinkler this weeked. After I stopped taping, Mackey really got into it and we couldn't pull him away.Sprinkler fun!

I'll post again after we go to Denver.


Rick said...

Thanks Jules. You are awesome. Love & hugs to all.

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megs said...

Hi, I'm a mum to 2 boys in the UK eldest with CF. Have been reading your blog, and praying for Seamus. How is he doing? In fact I hope you're all doing ok.