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Monday, October 6, 2014

Heart & Liver & Arthritis Update

If you haven't seen Cliff lately here's a funny photo of him that our sister Tricia took.  Don't be fooled by the scowl.  Cliff is a happy, playful kid.  He's also has a sense of humor.  Thus, when he was asked to smile for a picture this was the result.

Here's an update on our fun loving kid.


It is hard to believe that Cliff had open heart surgery almost a year and a half ago. Due to the number of procedures Cliff has undergone since heart surgery it makes that year and a half seem a long ways away.

By and large Cliff seems so much better than he was at that time. He has had so much more energy and his growth has accelerated. In his heart checkups the doctors describe his heart as doing great for him, although it is still high for a normal person. He has another follow-up with the cardiologist before the end of the year and we expect him to continue with visits every 6 months for the ongoing future.


Cliff's liver has been all over the place in the last year.  After having the partial biliary diversion we described earlier in March, Cliff's liver numbers have been decent. Cliff has been a lot less itchy which is the primary symptom we are seeking to avoid.  It hasn't been perfect.  We've discovered that little illnesses, like colds, dramatically reduce the effectiveness of this procedure for a few days.  But on the whole, Cliff's overall comfort has been great.  Now if we could just keep him from ripping off the bile bags.  He just thinks it's funny to rip off the bag and make messes.  I guess that comes with being two.


In what is a new development, Cliff was diagnosed with juvenile arthritis this summer.  It all started when he fell and hit his arm.  He seemed like he was a bit slow to heal from that and acted like he was stiff.  After a few weeks had passed Cliff's arm was so stiff that he started holding it like it was broken.  We thought he had fallen again so we took him in for a few x-rays, and ultimately an MRI. All these revealed no breaks in his bones.  But the orthopedic surgeon reviewing the MRI ordered blood tests and told us to consult with a rheumatologist.

Apparently arthritis is normally diagnosed over time period and several visits to a rheumatologist.  Cliff's was diagnosed in about 3 minutes. He did some manipulations of all of Cliff's swollen joints, including his wrists, fingers, shoulders, elbows, knees, ankles, neck, and probably even his hips, and told us that Cliff needed some help. Although Cliff's liver condition gives him a bit more risk when it comes to medicine, he had gotten to the point where he didn't want to stand up in his crib because he was too stiff. It was hard to see him like that. But it made the decision to go on medication simple.

Cliff now takes Enbrel once a week and it has made all the difference. No word yet on if he'll grow up to be a multimillionaire golfer, but we'll take the playful happy kid we know.

The one downside to Enbrel is that Cliff has become so mobile and is feeling so good that he climbs up and jumps off of everything. He's become hard to keep track of even just inside of our house.

But in the end we're happy to have him. He is such a neat kid. He brothers love him and he loves them. He brings a wonderful spirit into our home. We consider ourselves lucky to have the fun family that we do!

Tuesday, March 18, 2014

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!

Huge Thanks

We are so grateful for all the wonderful people who have helped us, are are helping us through this experience.

Our families have been great support systems for us and have given so much of their time and their talents to benefit Cliff.

We also have so many good friends who have given way more than we could have possibly imagined, sometimes a moment's notice.

To everyone we offer a heartfelt thanks! We really, truly could not do this without you.

An Inspiring Thought

Here's one quote that inspires us.
"There is no growth in the comfort zone; there is no comfort in the growth zone."
If I could figure out who to attribute this to I would, but the Internet isn't quite sure who said it.

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