Those stupid three steps forward

We had two big appointments this week – one with urology and one with gastroenterology (the liver doctor).  First was urology…

We already knew that the renal scan showed 83% of the work being done by Lincoln’s right kidney and that Lincoln was likely to need surgery.  The urologist confirmed.  The left kidney is doing 17% of the work and 10% is the urologist’s cutoff for function that is too low to salvage the kidney.  But at 17% he thinks he can save it.

The urologist believes Lincoln has a UPJ (ureter-pelvis junction) obstruction.  Here’s a little science lesson for you…The UPJ is the spot where the ureter meets up with the kidney (the pelvis is part of the kidney, not the pelvis we’d usually think of).  When the ureter is formed it starts at the bladder and grows up to the kidney.  While it’s growing it’s not a hollow tube yet – it’s solid.  It becomes hollow later.  A UPJ obstruction happens when the hollowing out process for some reason isn’t complete.  The urologist fixes this by removing the part of the ureter that isn’t completely hollow and reconnecting it to the kidney.  The surgery has a very high success rate and according to the urologist is relatively common in his world.  The hope is for Lincoln’s left kidney to return to full function.  Lincoln goes in for surgery on February 27^th then will be in the hospital for monitoring afterwards for 2-3 days.  We’re not exactly looking forward to the surgery, but are ready for it to be done and over with so we can move on.

Then came the gastro appointment…you know what they say about 3 steps forward and what they lead to…

Every time we have a liver appointment Lincoln gets blood work beforehand.  This past week the blood work showed his liver function levels at the worst they’ve been to date.  You might remember that we were hoping for direct bilirubin levels around 4 or so.  His last test showed direct bilirubin a little above 5.  The highest he’s ever had was around 8.  This time we were above 11.  His ALT and AST (measures of liver inflammation) had doubled since last month.  We also had our first abnormal INR reading, which is a measure of blood clotting and another test of liver function.  Previously Lincoln’s liver hadn’t shown any signs of enlargement or hardening (cirrhosis) when the doctors felt it, but now it does.

So what now?  We’re back to blood work every two weeks instead of once a month.  We’ve upped Lincoln’s vitamins and liver medicine.  The doctors will keep an extra eye on the blood clotting factor, especially given his upcoming surgery.  If it gets worse they can give him a shot of vitamin K, since vitamin K is one of the vitamins Lincoln has a hard time absorbing and also is a big factor in blood clotting.  The INR reading wasn’t bad enough for the liver doctor to put a stop to the kidney surgery.  He'd like that kidney to be healthy sooner rather than later.

Now that we have signs of liver hardening and elevated bilirubin we also had to have a discussion with the doctor about new things to keep an eye out for.  He told us when to call him and when to get to the ER.  He explained that he wants the kidney fixed before the liver gets too bad in case Lincoln ends up needing a liver transplant.  It’s better to be as healthy as possible in all other aspects when going into a transplant.  Yep, fun conversation.

Going through that appointment was an odd experience.  On one hand, we’re a little bit used to medical issues and appointments so we knew how to react.  On the other hand, Lincoln was doing so well that this felt like it came out of the blue.  I knew he was looking more yellow and had seemed like he wasn’t feeling great for a few days before the blood work, so I wasn’t expecting his bilirubin to have dropped.  But I wasn’t expecting 11.  That caught me off guard.  I felt like our three steps forward was followed by four steps back. 

But Lincoln seems like he’s feeling better today, he's still eating great and has been smiling and laughing, so all we can do for now is keep hoping for the best and keep having fun with him.  Through everything he just keeps being so much fun, we couldn’t have asked for a better baby!  Hopefully the increased liver medicine dosage helps things and we can get back to our baseline levels soon.  Any good thoughts or prayers you want to send our way are appreciated!

We survived the holidays...and a renal scan!

Lincoln had his first Christmas, and it was a blast!  We didn't do any traveling on Christmas day, this was actually the first Christmas day spent in this house since I bought it.  Lincoln of course didn't really know what was going on, but he did like the lights on the Christmas tree and he liked the jumper/bouncy thing we got him.  He hasn't gotten the hang of actually jumping or bouncing yet but he likes just hanging out in it.
Lincoln got a ton of toys and cute clothes, he was spoiled!  He's still getting the hang of a few of them, but there were some instant hits with a little elephant that makes a crinkly noise and a glow worm.  It's so fun to watch him get excited to see his toys and start to figure out how to play with/try to eat them.

We did travel down to John's parents' house the weekend before Christmas, and I'm pleased to say that Lincoln handled both the 4 hour drive each way and staying in an unfamiliar house really well.  We needed to pay a little extra attention to eating because of course he was distracted with all the fun people to play with.  Nothing major though, and he met his intake goal both days we were gone.

Since Christmas we've had another weight check with the pediatrician, and Lincoln is still gaining weight since being off the tube!  He's small for his age, around the 7th percentile for weight, but he's consistently in the 7th percentile.  That means he's gaining weight at the same rate as a normal baby, and that's what we want.  His height was a tiny bit of a concern, his percentile for height dropped a little.  He can be short, that's not a huge deal, he just needs to be consistently the same level of short to show that he's growing properly.  Alagille's kids do tend to be small, but it's not because of the faulty gene, it's because they have a hard time absorbing fat and vitamins so they have a hard time getting good nutrition.  Nothing to get worked up about at this point, just something to remind us to stay diligent. 

We also had a renal scan to check on the hydronephrosis of Lincoln's left kidney.  The test took about an hour and a half from start to finish and Lincoln was a champ.  He napped for part of it and just hung out while John showed him different toys on his phone for the rest. 
To do the test they hooked him up to an IV so they could administer what they call nuclear medicine.  Basically just medicine that can be followed through his body by the scanning machine.  They monitor how long it takes for the medicine to go into his kidneys and then how long it takes to leave the kidneys.  We meet with the Urologist on the 14th to officially go over the results and discuss next steps, but we know the test showed that 83% of the work is being done by the right kidney.  This means there is a blockage in the left kidney and the last time we met with the Urologist he said if there is a blockage it would have to be surgically corrected.  He also told us the procedure is relatively common in the urology world and has a great success rate, so that's reassuring.  We're not happy about the results, but if surgery is what has to be done to get this little guy a small step towards better then that's what we'll do.

In other news, MBA classes for both John and I start on the 13th.  We'll both have class two nights a week and are pretty nervous about how this is going to go.  Luckily Lincoln's Aunt Maria and Uncle Brian are going to babysit one night a week so that will help a ton.  Having a babysitter means we'll both be able to take a class on the same night instead of having to alternate class nights.  This means we'll be able to see each other 4 evenings a week out of 7 instead of only 3.  Honestly, I seriously considered taking another semester off.  However, that would mean my loans would come due before I had my degree.  I can't stomach that thought, especially since I only have 4 classes to go until I graduate.  So the Spurriers are just going to stay energetic and positive and keep on keeping on!  Wish us luck!