The Tube and Then Some

Since December 26th, TeriDel and I have averaged a trip to Phoenix Children's Hospital every 3 days.  That includes staying at the hospital for two weeks on separate occasions and lots of other visits through the Emergency Room.

The reason?  The tube.  A cholecystectomy tube to be more formal.  And while I don't think I can say that 5 times fast (or 1 time fast for that matter) I will say that TeriDel and I have a love hate relationship with the tube.

The whole thing started in December when Cliff started to get very itchy.  We're talking itchy to the point where he would scratch himself to the point of bleeding on his ears, ankles, elbows, chest, back, and sometimes even on the face.  The end result was that he was struggling to gain or even maintain his weight and his pleasant disposition seemed to be replaced by crankiness and discomfort.

We had tried medicine therapy with success since he was born.  But as Cliff got older the medication had less of an effect.  The second and third medication we tried didn't work which left us with only two options.  We could do something surgically (or radiologically) to intervene or we could wait until Cliff was 4 or 5 when many Alagille patients spontaneously stop being so itchy.  Cliff's change in personality really left us with no choice.  We needed to try to do something to help him.

We are so fortunate to have the team of doctors that we do! As we sat down with Dr. Miloh (Liver team doctor), Dr. Hewitt (Liver team surgeon), and Tara Keegan (Liver team RN), they laid out all of the different options available for Cliff.  The surgeries mentioned were viewed as being pretty intense and because we didn't know if doing a biliary diversion would even work, it seemed like a lot of pain to put Cliff through for an unknown gain.  So they proposed that with the help of the interventional radiology doctors they put a tube into Cliff's gallbladder to see if they could drain out some of the bile and thus, fix Cliff's itchiness.  This was viewed as a test to see if this kind of procedure would help Cliff and if it was successful we would go from there.

If you want to know the medicinal theory behind all this you can click here.

Cliff had his cholecystectomy tube put in on December 26.  From there it was a wild ride of sorts.  The tube worked (sometimes) and didn't work other times.  When it worked and Cliff got out 25 to 30 mL of bile he wasn't itchy at all.  He was happy and things were looking great.  When it didn't work, which was due to any number of different complications, the tube was rough and seemed to cause Cliff tenderness on his side and just didn't seem to offer enough benefits to outweigh the cost on Cliff.

By the time March came around Cliff was back to being very itchy and even though the tube had pretty consistently gotten out 10-15 mL a day it didn't have much improvement on Cliff's overall health.  So we decided to opt for surgery.

It was a terrifying call in some ways.  No doctors outside of Chicago or Connecticut had done this surgery before and so no one knew what to expect.  And although the surgery didn't have incredible risks associated with it, Cliff's heart condition and overall health make every surgery a big deal.

Cliff went in for surgery on March 11.  Dr. Notrica (Surgeon at Phoenix Children's) and Dr. Hewitt took out an 8-9 inch segment of Cliff's intestines and then attached that segment from the gallbladder to Cliff's abdomen ending in an ostomy.  Although Cliff's recovery had a rough start, probably in part due to the fact that they gave Cliff morphine, which makes him itchy, at a time when Cliff was already dealing with severe itchiness, by Thursday Cliff was making great strides and was discharged on Saturday.

So far the initial indicators are positive.  Cliff is getting out about 100 mL of bile a day and he seems to not be itchy anymore.  It's early.  And if we've learned anything with Cliff it is that we have to be patient and just take things as they come.  But we're encouraged.

Pictures and links will be updated soon!

From the Health Desk

Happy Update Day!

As with seemingly everything in Cliff's life we have mixed news. Here's the great news:

Cliff's heart is doing really well.

You probably know that back in March Cliff had open heart surgery out in Houston, Texas. Dr. Fraser opened up Cliff's pulmoary artery (coming from his right atrium and going out to the lungs - see the light purple diagram below) and patched it so that it would be wider and hopefully able to handle higher volume and thus reduce the pressure.

Prior to surgery, the pressure on Cliff's right side was 110% of the pressure on the left side.  For normal people you would hope to see it be somewhere around 30-50%.  The result of such a high pressure is that the heart on the right side has to work so hard that the heart wall begins to thicken and get stronger. For biceps, your muscle getting stronger and thicker is awesome. For hearts, not so awesome. The muscle thickening leads to congestive heart failure.

The goal of the surgery was to reduce the pressure on the right side.  And that happened in amazing fashion.  Cliff's post surgery pressure is now 70% of the pressure on his left.  We are grateful for the signficant change.  It is impressive, not just to us but to our doctors as well.

Long term, we still aren't sure what this new number means as it is not a permanent fix.  But our cardiologist tells us that Cliff's heart is in a much better condition now and that ususally correlates to more time.

And that's where we get to the less great news:

Cliff's liver is not doing as well.

When Cliff was still very young and we had just started treating his heart and liver issues, Cliff was placed on several medications to help with the overall itchiness that comes from his liver disorder.  They worked great for a long time, about a year and a half.  But as is normal for many kids with Alagille syndrome, the medicine lost its effectiveness.

Cliff's doctors have tried other medications but nothing has curbed Cliff's itchiness.  Thus, the next step is surgery.  And that will likely come quickly.  Quickly meaning December 26th (as in tomorrow).

The procedure is a test procedure of sorts.  In about 6 weeks we should know if the procedure will work on a more permanent basis or not.  If it looks like it will work, this could be a good step in curbing Cliff's itchiness for a while.  If it doesn't work the next step is either to try to wait it out or opt to do a liver transplant.  Oddly enough, many Alagille patients have a dramatic drop in itchiness at around their 4th birthday.  Of course, that is still more than 2 years away if it happens at all.  So we'll see.

At the end of the day we're just grateful to have our fun little Cliff with us!  He's awesome.

We promise to post more pictures soon!

Cliff News (on Monday)

Hi all,

We want to give you the latest in Cliff news.  So stay tuned for Monday for our latest update.

In the mean time, please enjoy this awesome picture Teri took of Cliff on his first birthday.

This was just 9 days after open heart surgery in Texas. See you on Monday!

Updates

It was at some point after midnight in the early hours of March 28 that the blog got a lot harder to update. Cliff had slept peacefully beginning at about 6 pm March 27 just like he always does. It was great because we were in Texas Children's Hospital so any time that he slept well in a bed that wasn't his you feel lucky. It's still true today. But at midnight the nurses came in to give Cliff his IV. He hated the cold water they were giving him and was awake pretty much from that point on.

They came in to wheel him up for open heart surgery at about 6 and we said goodbye to him just after 7 AM. Although the hospital was amazing at giving updates and we never went more than about an hour and a half without hearing exactly what was going on and how things were going, the day just seemed incredibly hard.

Dr. Fraser finished the surgery at about 2:30 PM but it wasn't until about 6:30 PM that we were able to see him in the Cardiovascular ICU. He was connected to a huge array of tubes, pumps, and IVs. But bit by bit he started the multi-day process of waking up and starting to heal. By Sunday, just a few days after having open heart surgery he was able to leave the ICU and go back downstairs to the 15th floor where we had stayed before surgery. By Tuesday, when I left Houston to fly home to be with the other kids, he was standing up in his crib and starting to gain back a bit of his fun personality. Cliff ended up being able to come home to Arizona about a week later - 12 days after open heart surgery.

Since then we've just enjoyed have Cliff around. So far the initial progress reports all show that as far as Cliff's heart goes, the surgery has done a better than expected job at minimizing many of Cliff's symptoms. The surgery is not a cure but more of a first step in stabilizing Cliff. Although there are many things that can affect the ultimate outcome, including many things that aren't controlled by the heart at all, the prognosis heart-wise is that Cliff could live another 20 years and it would not be surprising. We'll take it.

Looking forward, Cliff is going to have another heart surgery in October. This surgery will be to measure the growth that has hopefully occurred and do some angioplasty if needed. Other than that Cliff continues to meet with doctors about his liver, his kidneys, and now we've added a feeding therapist to help him overcome his feeding aversions.

We are so appreciative of everyone who has helped us a long the way. We know we still have a long way to go, but it's great to see little nuggets of happiness mixed in with everything else that is going on. In so many instances our family and friends have provided those moments and experiences and we are so grateful. Hopefully, we can return the favor by updating you a little more frequently on our progress.

We've made it to Texas

Today (Sunday) has been great. We took an early flight out of Phoenix. Cliff did really well. After just a few seconds of fussing due to the pressure changes Cliff decided it would be best to just sleep the rest of the way. He was zonked for most all of the flight.


Once we landed we made our way to Pappas BBQ with everyone who had just finished up at church. It was great. Teri and I now think that my suits should totally color coordinate Utah her Sunday dress and hat. Looking good Houston!
Pappas was great introduction to Houston. Teri got the brisket; I got jealous.

I got the pulled pork which was also good. I really wanted to try the chicken or ribs. Maybe next time.

All in all we're off to a good start here. Many, many thanks to all those who have helped us along the way. Tomorrow we get admitted to Texas Children's and the fun begins from there.

Open heart surgery

Here's the latest on Cliff's open heart surgery.  

It appears that things are on track from the insurance perspective to go forward with the surgery in Houston, Texas.  Surgery is set for March 28 but we still have lots of details to comb through in order to make this happen.  But we're getting closer.

Thanks for your prayers and support.  We definitely feel comforted by so many people. 

News from Texas

We're making arrangements to get Cliff in to Texas Children's Hospital in Houston to have open heart surgery.  After consulting with lots of doctors two things have become as clear as perhaps they will be in Cliff's case.  

1) Cliff's condition will lead to congestive heart failure if left untreated.  The doctors would expect to see signs of heart failure by 18 months and see heart failure at some point between 18 months and 3 years.

2) The possible options to treat Cliff are very limited. One option is to wait and hope that Cliff's pulmonary arteries get bigger, which is unlikely given that his pulmonary arteries today are the same size as when Cliff was born.  The other option to is have open heart surgery.  The surgeon will add material to Cliff's pulmonary arteries in order to increase the circumference of the arteries to allow for more blood volume to pump through which will hopefully decrease the pressure in Cliff's right side of his heart.  It also has the secondary effect of hopefully allowing for future growth of the pulmonary arteries on their own.

Because of Cliff's condition every surgery has some level of enhanced risk, but the doctors are very hopeful that this procedure can be done without too much risk to Cliff.

There are no guarantees that this will work, but as the doctors have referred to layout of Cliff's heart they have become more hopeful that he may someone who would respond positively to this procedure.  Given this outlook we've made the best choice we think is possible after much research, discussion, and prayer.

Now comes the difficult part of just trying to tie up all of the loose ends that accompany this decision.  We've been working with insurance to try to get this covered.  They have confirmed that no doctor in Arizona has ever done something like this which makes Texas Children's hospital an option for us...sort of.  Through a couple of omissions in our insurance plan documents there are a couple of overarching questions about coverage.  We are hopeful that within the next few days those issues will be resolved and we can start to plan for our trip to Texas.

We are so appreciative of everyone's help and assistance.  Big thanks if you have already purchased a cookbook!  And if you haven't, and would like to buy 1 (or 50) we would love to sell them to you!  :)  We will still need to raise some money in order to make everything work for this Texas plan and we certainly appreciate your support.