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.

Tuesday, March 5, 2013


So let's get it out of the way... After realizing on-line Weight Watchers just wasn't going to cut it for me, I joined Jenny Craig. My life is so crazy right now, I really need something very structured so I don't have to make any more decisions than I already do. After one week, I lost 3.6 pounds. I had lost a few before so total loss is 7.3. Current BMI - 33.7.

Mackey had his second birthday a few weeks ago. The poor guy really gets the short end of the stick. It was a low key event at our house but he had a big celebration at daycare. He was thrilled to get presents so I don't think he minded not having a party. I will write a post strictly about Mackey soon. He is growing up so fast and I want to make sure I record some of the wonderful things he does before I forget.

Some of Seamus' symptoms seem to be changing so we spent 7 hours at CF and GI clinic on Friday and got x-rays and an ultrasound done. The concern was he might be developing ascites which is fluid build-up in the abdomen because the pressure has increased so much in the liver. The doc called us Sunday night, which is usually not a good thing, to tell us there was no sign of fluid but he has lots of trapped gas and they can't figure out why. He had some stool but isn't obstructed. He constantly looks like an 8th month pregnant woman. We also got definitive proof of the cirrhosis which up to this point they had assumed but hadn't proven. This doctor has only been following Seamus for about a year and I think he's finally starting to understand the uniqueness of the situation. He admitted that Seamus is "a mystery" and his liver (and GI) disease is not following the path of most CF patients. We had another long discussion about transplant on Friday and he still believes Seamus isn't ready but we are concerned about waiting too long because he could become too sick to be eligible.

The best part of the weekend was meeting with the Make-A-Wish volunteers. They had lots of questions for Seamus about his likes and interests and really listened to his request. He has a very specific request so we are hoping they are able to do it. He makes to go to the Activision offices where they make the Skylanders video game and create his own character called Man-atee. It's half man, half manatee. The face will be his face. He's come up with all the powers he has and whole story line. So hopefully, he could actually work with the designers to create it and incorporate it into the game, at least a version just for hem. My biggest concern is they wouldn't fully understand what he wanted and would just send us out to meet with the designers who would give us a tour and just show him how they work. I think he would be very upset if we went all the way there but he couldn't make Man-atee but it may be too much work for them.

He has a second wish, just in case, which is going to the Disney resort in Hawaii. Obviously, James and I wouldn't mind if that wish was granted but he really has his heart set on the first one so I hope they can do it or at least some comparable version of it.

So we're still waiting for culture results and more liver blood work to come back but currently Seamus is feeling good which is the most we can ask for. I'm hoping we get that huge snowstorm everyone is talking about so we can all stay home together and snuggle.

Friday, February 8, 2013

Weekly update 2/8/13

Okay, this week I did well with eating healthy and went to the gym or did something at home every day. I lost 1.2 pounds which isn't a lot but I had lost 4.2 pounds before my last post so overall I'm down 5.5 pounds so I feel good.

BMI 34.1

Friday, February 1, 2013

Weighty issues

I have always been overweight and like most overweight people, I've tried scores of different diets, some more successful than others. I've lost a few pounds and inevitably, gain it back over time, especially after having two kids. I know the statistics, how I'll be healthier and live longer if I lose weight, but of course, I think nothing will really happen to me. For the first time, I no longer have an option of taking my weight lightly (pun intended).

Yesterday, Seamus had a follow-up visit with his liver doctor. We always dance around the topic of transplant at every visit. His opinion is some day Seamus might need one but as long as the liver is still functioning and his lung function does not seem to be compromised by the ever-increasing size of his liver and spleen, then he is not ready. His lung function has actually increased lately due to three months of inhaled antiobiotics but his liver lab work is always borderline okay, some times just under the acceptable numbers and sometimes just over. Every time he gets sick, like the flu-like virus that landed him in the hospital last week, his liver has a very difficult time handling it and bouncing back. So basically we do not know if, or when, the transplant talk will change.

That's where my weight issue comes in. I am the same blood type as Seamus but James is not. That means that I am a candidate to be a living donor for Seamus and I always assumed that would be no problem. Yesterday I found out that one of the biggest donor requirements is weight and I am too fat. It never really occurred to me that the fat is not just hanging off the outside of your body but your organs actually get a layer of fat as well and in order to have a healthy liver, your BMI should be around 23-25. That means I would have to lose more than 45 pounds to be an acceptable donor.

Since his liver could take a turn for the worse very quickly, I really need to be ready at any time. I could not lose 45 pounds immediately if he needs a new liver quickly. He could be listed for a cadaver liver but there is a shortage of organs and you never know the outcome of that.

So I have to take this weight loss thing seriously, extremely seriously, for the first time in my life. It's not a matter of just looking good in my clothes but it could literally save my son's life. I am going to post my progress here for everyone to see. Maybe that will shame me into keeping on track. I could use your support and advice.

2/1/13 - BMI 34.3

Friday, January 11, 2013

Catching Up

So the last two years in a nutshell:

 Mackey is almost two and is totally awesome; crazy, and defiant, and full of piss and vinegar. On days when he's throwing temper tantrums and saying nothing but "No, No, No", I may not think he's exactly awesome but to hang with this family you have to be tough and not take anyone's shit. I think he's ready for the challenge.

I am so glad we made the choice we did. Seamus is such a good big brother and the love they share is so special. Mackey absolutely adores his big brother, Bubba, and tries to emulate him in every way. He loves to sneak into the medicine drawer and grab empty syringes and "take his medicine" and we've caught him on a few occasions puffing on dirty nebulizer cups. He is completely obsessed with Dora the Explorer and does a mean Dora dance. Drawing is his other passion, much like his brother.

Mackey spent the first year at home being taken care of by Ga, my mom, who lives with us. After we caught him standing inside the dishwasher a few times and doing laps around the house, we realized he was too much for her to handle. He now goes to daycare and is doing great. His caregivers speak Spanish to him most of the time and he's picked up a lot. When he first started becoming vocal, we thought he was speaking lots of gibberish but then we realized he was speaking Spanish!

Seamus is doing well at his new school. He attends a performing arts school and has drama, art, music, or dance every day. Last year, he skipped a grade so he's now in the second grade. The only downsize is the large class size but that's the same in any public school. The nurse there is great and treats him like her own.

His health has been a concern. He has been hospitalized quite a few times in the last two years (now up to 33 admissions.) Some were for lung exacerbations but most were for GI or liver issues. He was diagnosed with CF liver disease in December 2011 and it has progressed much faster than anyone expected. His liver and spleen have become very enlarged and are in danger of rupturing if he accidentally falls or gets hit. He is not supposed to do any contact sports but he is still allowed to take karate for now. He loves it and is up to a yellow belt but he won't be able to continue much further because he can't spar.

He has cirrhosis and portal hypertension which means his liver has a lot of scar tissue that doesn't allow the blood to flow through. Instead the blood flows back into the stomach and esophagus and the pressure builds up so the blood vessels can burst at any time. He has had some bleeding so they went in and did a banding procedure to cut off the affected blood vessels. Normally, after a few weeks the band sloughs off and heals but in his case it didn't heal and instead formed an ulcer. One night, he woke up vomiting about two cups of blood from the ulcer and had to be rushed to the hospital. It eventually healed but the concern is the next time he needs to be banded, which is definitely a "when" not an "if", it could happen again.

He's starting to question things and some stuff does seem to bother him, but overall he still has a positive outlook. He is starting to have some trouble concentrating in school. We don't know if that's because the work is getting harder, if it's related to the liver disease, or if he's having sleep issues which are common to CF. He is going in for a sleep study soon.

Tonight, bedtime was a circus and James said something about how we aren't a "normal" family. Seamus asked "Is that because of my CF?" That's the first time he ever said anything like that. We told him of course that had nothing to do with it. There's really no such thing as "normal" and if there was, what fun would that be?

Thursday, January 10, 2013

Still Here

It's been two years but I'm still here. Here's some photos from our trip to Florida last Spring.

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