The news

We have recently discovered that our new baby girl Emmery is having some liver function problems.This is the reason we are beginning this blog. This will be an outlet for sharing information to family and friends who are praying and also a way to connect with others whose children may have some of the similar health problems.

The history:

Emmery was born on April 15th, 6lbs 6oz and 19 inches long, happy and healthy. Upon leaving the hospital they did notice that she was slightly jaundiced. They said she should be fine as long as she keeps eating well. Our pediatrician agreed at her first newborn appointment.

Fast forward 6 weeks. . .

Emmery was still slightly yellow and we became concerned. I took her to her doctor to check her bilirubin levels. By the time I arrived home from her appointment the doctor called and informed us that Emmery's labs indicated that her direct bilirubin level was sky high and we needed to head to OHSU to be admitted. They believed that she may have biliary atresia, which basically is a block in her liver duct, and they would have to perform surgery. I will spare you the emotional details for now (I'm sure I will provide a sappy emotional break down post soon), but you can imagine the response we had.
We headed up to the hospital and stayed for two nights while they performed blood tests, x rays, ultrasounds, ect. The GI doctor met with us to discuss what they were finding. Based on the ultrasound she believed that in fact she did not have biliary atresia but possibly a very rare syndrome called Alagille Syndrome. .
They believe this to be her diagnosis because she has a mutation to her vertebrae called "butterfly vertebrae". It doesn't cause any problems for her, but just a slight deformity that was caught on her x ray. Because of this they began to explain what Alagille's is.
Alagille's can effect the heart, liver and kidneys and cause many complications.
They believe that her liver bile ducts are malformed. Unfortunately with Alagille's there is not a surgery or "cure" for this condition. This syndrome is a genetic mutation that she would have been born with and can be hereditary. The next step was to have her heart and kidneys checked Thankfully she currently just has a slight heart murmur and her kidneys are great. This is a huge blessing.

So what did this mean for her?
Well if you google "Alagille Syndrome" you will see that there isn't much that can be done. This syndrome is so rare that our family doctor even had to look it up. Dornbecher's children hospital currently only cares for 5 patients with Alagille syndrome.

We basically have to watch and wait to see what her liver does. What is happening, in basic terms, is her liver ducts are unable to clear out the bilirubin properly, which causes a back up. The bilirubin just keeps being re-absorbed into the liver then into the blood causing her to look jaundiced. The liver becomes irritated with the bilirubin backing up and can cause liver failure. We don't know how long this will take or if it will happen. So we watch and wait. She can begin to have side effects such as severe itching and failure to grow. The itching is caused by the bilirubin being built up and can be very severe. If this gets really bad they can give her medication.. She also will be on the smaller side. Because bile helps absorb fatty acids, she can become vitamin deficient and unable to gain weight at a normal rate. So it is important she eats a lot. She will always be smaller than the average girl. We are still awaiting the official results from the genetic test to confirm Alagille. We should have the results by the end of the month.

What happens if her liver is going to fail?
Basically the only cure is a liver transplant. She would only need this if she begins to have serious side effects and her liver begins to enlarge and harden. Thankfully the liver is a magnificent organ and she could even use a live donor because the it can grow if removed from someone else.


So what are you doing now?
I feed her like a maniac. My goal is to keep this girl growing! We also supplement her with a formula called Progestamil. She also takes a multi vitamin to help with her vitamin deficiency. Lastly, she is taking a medication called Ursodiol to help break down the bile and keep the itching away.

For now we just deal with the fact that she is smaller than average and she looks like she had a bad spray tan.


We just had an appointment today and will have updated lab results tomorrow. This will tell us if her Ursodiol medication has helped break down the bile. For now she is actually doing really well and gaining weight! They were very impressed with her growth rate.
Next, we go back to Dornbecher's in two months to have her checked out again. We also will follow up with the cardiologist to make sure her heart is doing well.

If you are the praying kind..
We are praying that her bilirubin gets moving through her liver and that she does not have to suffer from that awful itching. We are also praying she continues to grow at a good rate. God has been so good to us through this whole ordeal. She has been blessed with amazing doctors and is doing the best she can do considering the circumstances. We know He is in control and has a great purpose for her life. He has created her and loves her even more than we do. He has great plans and we are trusting in Him.

Proverbs 3:5-6

Trust in the Lord with all your heart
    and lean not on your own understanding;
 in all your ways submit to him,
    and he will make your paths straight.

My favorite song right now is "Strangely Dim" by Francesca Battistelli. The chorus is:

"But when I fix my eyes on all that You are
Then every doubt I feel
Deep in my heart
Grows strangely dim
All my worries fade
And fall to the ground
Cause when I seek Your face
And don't look around
Any place I'm in
Grows strangely dim"

 

 

 

So that is a quick run down on what is going on with our baby girl. I will be posting updates and how we are managing through this.... it's a whole whirlwind of emotions people!

Thank you for your prayers,
Emmery's Mom