I had been so prepared the day before with my pink folder and all the medical information that I had studied over that last month. (P.S. researching the Internet about a rare disease that your daughter has is emotionally exhausting). Of course I left this lovely folder in my car with all of my questions, so I had to go from memory. But we basically wanted to know if any other treatments would be helpful for her and what her future may hold. As I mentioned before, it's not that simple. We will be just going month by month here. Maybe even week by week.
First she assured us that the Alagille patients that she has had, have done really well. They weighed her and measured her and were very pleasantly surprised that she is growing well. (Yay! All those sleepless nights of feedings are worth it!) She is only in the 5th percentile on the "normal" scale but as long as she is growing, that is good.
She is now 9 lbs 7oz. and 21.3 inches.
We also asked her about worst case scenario... transplant. She reassured us that if she did have to go down that road that they have an excellent success rate with their liver transplant patients. They work with Stanford to complete their transplants. The only reason we would need to go there is if her quality of life began to be affected by either the sever itch (pruritus), if she stops growing, or if her liver starts shutting down causing other major side effects to her other organs.
She was confident that if she could make it to the age of 3 with out having to do any major procedure to her then she is more likely to live longer with her own liver.
Lastly, she had her blood taken and this by far is the worst part. Her poor little veins are so small and little missy is a fighter. Thankfully Daddy has the stomach to watch this while I hid around the corner, rocking myself and humming with my ears plugged. Here is a picture of her poor little bruise. You can also see they yellow in her skin.
Quick little medical lesson:
Your bilirubin count is a combined total of your direct bilirubin and your indirect bilirubin. This total is what they usually measure at the hospital when baby is born. So her levels were a little elevated when she was born, but unless they broke them down then they would assume that it is her indirect bilirubin that is high, which is the kind that can be cured with lights or sunshine. But direct bilirubin cannot be cured with lights, it is in the liver and indicates that there is a block, missing duct or malformed duct.
So her direct bilirubin count is 9.7. A "normal" count is 0.3. But the good news is that her previous reading a month ago was 9.2 so it has only moved up a little. Considering prior to that, her bilirubin level went from 7 to 9.7 in only two weeks. That means the medication may be working to slow down the build up. Prayer answered.
Her liver enzyme levels have gone up though. This measurement basically tells us have irritated the liver is getting with all the bilirubin in it. Her AST-Aspartate transaminase is 428 and ALT-Alanine transaminase is 325. A normal ALT 12-60.
What this means:
"Elevated liver enzymes may indicate inflammation or damage to cells in the liver. Inflamed or injured liver cells leak higher than normal amounts of certain chemicals, including liver enzymes, into the bloodstream, which can result in elevated liver enzymes on blood tests."
Her doctor doesn't seemed too concerned that these have gone up. It's expected. As long as Emmery is doing well, eating and happy then that is all that matters.
We will continue to pray:
1. That she keeps growing.
2. That she DOESN'T get that horrible itch.
3. That the medication keeps her direct bilirubin level the same or lower.
4. That her liver doesn't get mad at her. :)
5. That her Mom chills out and stops stressing.... oh and maybe gets a nap.
Emmery thanks you for all of your thoughts and prayers.. I think that is what she is trying to say in this video.
Love, Emmerys' Mom
Oh friend, I had to read through this post twice to digest it all. I can't imagine how this has consumed your thoughts over the last month and a half. I'm SO STINKING proud of you, how you have risen to the task of parenting and advocating for your daughter. She is your daughter, not your sick daughter, and you are the perfect mama for her! Thank you so much for giving us specific ways to pray for all of you. Love you to the moon!
ReplyDeleteKristen, so glad I found this blog. I have had all of you on my mind, and wanted to call and see how things were going, but didn't want to bug you. Emmery is in my prayers, as are all of you. Be strong.
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