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.

Heart

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.

Liver

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.

Arthritis

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!

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.

It's the Little Things

Cliff has always had a thing for giraffes.  We're not really sure why.

Perhaps he likes their long necks or their keen sense of humor. Either way, he loves them.

So it was especially fitting that for Christmas Cliff received a giraffe WubbaNub.  It has proved to be Heaven sent. After Cliff's heart catheter procedure, the doctors all said that if we could get Cliff to take a pacifier it might be easier on him, especially if his future had some surgeries in it.  Cliff has never been one to like pacifiers and so we didn't really hold out much hope that he would suddenly change his mind. But apparently if you throw a giraffe on the end of a pacifier all bets are off.

Cliff took to his WubbaNub like they were long lost pals.  And it definitely helped him as he got done with the surgery for the g-tube placement.  It's actually really cute.  He and his giraffe are inseparable.

All of this made what happened this morning all the more cute. Cliff always seems to have trouble sleeping after about 2 AM. Before he got his nasal tube out he'd be up all the time fussing and crying and not letting anyone else get sleep. That seems to have subsided at least a little bit. Cliff still wakes up fairly frequently after 2 AM but now he seems to give at least some consideration to the idea of going back to sleep.

This morning was no different. Cliff woke up at varying points during the night, but after 5:30 AM he just wasn't going to go back to sleep. He wanted to have some interaction with people. But Teri was still asleep and I was tired so I figured I would lay him down next to me that way I could interact with him without having to be too energetic.  Cliff, as usual, is ok with this plan because he spends most of his day laying around anyway so if someone wants to see the world from his perspective he doesn't seem to mind.

As Cliff and I were lying there babbling back and forth to one another Cliff reached up to his mouth with both hands and grabbed his giraffe WubbaNub and pulled it out of his mouth and then moved it over to my mouth.  He didn't have the coordination to actually get the pacifier end into my mouth, and I wouldn't have accepted his generous offer even if he did have the ability to offer it, but I did get a soft giraffe rubbed on my face for a few moments.

It sounds funny, but I was very touched by the gesture. I have no idea if Cliff understands what it means to share, but I hope that he understands what it means to love and to be loved. I know Cliff loves that giraffe and I hope that what he meant by his little act was that he wanted me to share in something that gave him great joy. And although I may never know what Cliff was actually thinking, I know what I will take from the experience.

I grateful to have such a neat family. Teri and I love all of our boys. Cliff, in case you don't know our family, is our fourth child, all four of which are little boys. I am grateful for my wife, Teri, as well. And I am also grateful for Cliff.  I know Teri is too. We wish he was better. We wish he was healthy. We wish there was a clear cut path for what we could do. But even if the path is rough and a bit murky ahead, we are grateful to share in those little things that brighten our days even in the face of everything else going on. Cliff really is a special kid. I'm glad I get to love him.

Origami Owl Fundraiser

We really are blessed to have good friends in lots of places. One of our new friends is a new co-worker of mine Janina Walters. Janina is an independent distributor for Origami Owl, a jewelry website.

To help Cliff, she is offering the following deal:

1. Take a look on http://jwalters.origamiowl.com/ and let Janina know what you would like to order via email (janinawalters @ gmail.com). Please list Order for Cliff in the subject line.

2. Janina is offering some deals on the jewelry as well. You will receive a free charm for every 4 charms you buy and Janina has offered to cover your shipping costs and taxes.

3. Janina has also offered to donate any profit made on the sale to Cliff.

Thanks Janina and thanks to everyone who helps out this way.

G Tube is in

Cliff got his g-tube put in today.  Here's his last picture with his ng (nasal) tube in.

All we had to do to get him to smile was tell him that we were taking the tube out of his nose.  Well, that and make funny faces, noises, and wiggle around with his cool new stuffed elephant toy that he got from the hospital today.  And although he is pretty groggy from the anesthesia and may not know it yet, we think this will go down as a great day for him.  

For starters, he has been congested almost the entire time since the ng (nasal) tube went in.  That seems to have caused him trouble breathing and sleeping comfortably.  As a result, Teri and I have had plenty of sleep deprived moments over the past couple of months.

Even just the short while immediately after the surgery seemed telling on what kind of effect this may end up having for Cliff.  Where we used to hear Cliff's constant congestion with nearly every breath and had to wipe snot away all the time, we didn't hear him once after his surgery.  And we never had to wipe a snotty nose after surgery.  He did seem to be itchy from the morphine they gave him, but some antihistamines seemed to take that away.  

Cliff definitely has some uphill battles ahead but this is at least one of them that he appears to have cleared successfully.  Now we'll see what tomorrow brings.  

About Cliff

We welcomed Cliff into this world in spring of 2012.  Like all newborns, he was adorable.  But we noticed early on that Cliff seemed to be suffering from a stubborn case of jaundice.  Unfortunately, it turned out to be a symptom of something far more serious.

After two weeks of severe jaundice our pediatrician sent Cliff to the hospital, where a parade of doctors and various –ologists came in to check on our little guy.  One hospital visit followed another and, over time, we began meeting with a whole host of specialists.  The conclusion, confirmed by genetic testing, is that Cliff has a variant of Alagille Syndrome that has only been seen once before.

For Cliff, Alagille Syndrome means health complications.  Cliff’s liver is not able to route the majority of the bile produced there to his intestines to digest food and his kidneys are unable to filter out enough acid from his blood.  Cliff also has a condition called pulmonary stenosis where the arteries leading from his heart to his lungs are too small which results in his heart having to work harder in order to do its job.  Cliff takes multiple medications each day to assist with many of these problems.  In addition, Cliff struggles to gain weight which is a problem for many children with Alagille Syndrome.  His doctors have put Cliff on a specialized formula and given him a nasogastric feeding tube for night feedings.

All this has kicked off what has become our new normal.  Cliff heads into Phoenix Children’s Hospital, among other places, on a regular basis to check-up with his doctors, including his hepatologist/gastroenterologist, nephrologist, cardiologist, nutritionist, and pediatrician.  We are very grateful for such a talented and helpful group of doctors who have been gracious in their help and explanations as we’ve gone through this experience with our little boy.

At this moment the future is still unclear.  There are some benchmarks that Cliff is doing great on and there are some that are more worrisome.  We enjoy each and every moment we have with our little guy and hope that we continue to have him here in our lives.  But in the meantime, we do our best to take care of him and put the rest into the loving hands of our caring Father in Heaven.

A Minor Roadblock

A few weeks ago Cliff's cardiac surgeon did a catheter of Cliff's heart where we found out Cliff's pulmonary stenosis was getting worse.  His pulmonary arteries are still the size that they were when he was born even though he is now 9 months old.  The effect is like attaching 3 garden hoses to a fire hydrant.  The water flows but under incredibly high pressure.  Ultimately that pressure makes Cliff's condition unsustainable long term without some kind of intervention.

With future surgeries on the near horizon our doctors have all recommended putting in the g-tube so that Clif would have an easier time getting nutrition after surgery.  It seemed like it took Cliff a week or two to bounce back after the heart catheter.  Perhaps some of that was because having the ng-tube in his throat was causing some difficulty.  But it also seemed as though Cliff had a rough time with congestion

That seems to be our biggest concern at the immediate moment. Cliff is just so congested that we worry about him not being able to even undergo the g-tube procedure on Monday.  With Teri having just had bronchitis last week we didn't want to take any extra risks tonight so Teri took Cliff down to the Phoenix Children's Urgent Care to get him looked at because his breathing and wheezing has gotten worse. The trouble is that it is very difficult to determine if Cliff is having trouble because of the complications from his heart or if it is just chest congestion.  Cliff is headed for the cardiologist tomorrow so hopefully they will be able to give us a bit more insight.  And hopefully this congestion goes away sufficiently that Cliff can get his g-tube in on Monday.  Here's to hope.

Cookbooks have arrived

We are very excited to announce that the cookbooks have arrived.  If you'd like to get a copy please give TeriDel or Tad a call or email us at babycliffnotes@gmail.com.  We'll post pictures once we get them.

Thank you for your support of Cliff.  It means a lot to us.  

Preparing for another hospital visit

Cliff is headed back to Phoenix Children's Hospital next week to have a G-tube put in. This will hopefully alleviate a bit of the congestion he has had with the ng-tube in place and make life a little easier on him. I'm sure he'll appreciate not being tethered as he learns to crawl.

The surgery itself is normally out-patient but with Cliff's heart condition they plan to keep him overnight to watch and see how he does. Hopefully the procedure will go fine and we'll be back home the next day.

We'll keep you posted.

Cliff is here

Welcome to Baby Cliff's online home. We hope that you'll come back regularly for updates on our little guy. And hopefully we'll have lots of updates to share.