Blogging about life in Minnesota, raising our six kids with Down syndrome while battling Breast Cancer.

Be the kind of woman that when your feet hit the floor in the morning the devil says, "Oh shit! She's up!"

Tuesday, August 16, 2016

Today is the Day!!

We've been so busy with projects around here that this event nearly escaped having a blog post attached to it. Angela has reminded me every day for the last two weeks that "August 16th is coming right up!"

You may remember that prior to her diagnosis of Abernathy Type II malformation, Angela's ammonia levels continued to climb. Twice she found her way into a coma due to hepatic encephalapthy. Normal ammonia levels are between 10-50When we finally discovered the hyperammonemia, and given she was still walking and talking at 250, we can only estimate she her ammonia level was somewhere around 300+ when she was in a coma. When we started treating her for what was suspected to be OTC deficiency, she was on a daily doses of Bupheynl, Ravicti and Lactulose.   Our goal was to keep her ammonia below 50. Anything over that was cause for hospital admission, and if higher than 60 she was admitted to the ICU and started on the rescue drugs Ammunol, and Arginine. Keeping Angela out of the hospital was a daily ordeal. She could be fine one hour, and the next we could be in an ambulance, her blood pressure bottomed out and her being admitted to the ICU. It was a very scary time in our lives.

When the portal shunt was discovered that goal of 50 was tossed out the window. Instead it was "don't bring her in unless she's having a seizure or in a coma". She was discharged with an ammonia level of 125. By that time we understood what the high ammonia levels were doing to her brain. Knowing that Angela was walking around with her brain being bathed in ammonia on a continual basis made me a panicked mess for several months. She required 24 round the clock awake care to constantly monitor her  neurological status, but we couldn't find a nurse to work the hours we needed. That meant Dean and I didn't sleep very well!

Thankfully we only had to wait a couple of months for her doctors to come up with an alternative to liver transplant, and on April 8th Angela had that life-saving surgery.

Angela has continued to do well. Her speech and fine motor skills have improved, her ability to swallow safely has returned and we're no longer using her tube. Her mood is AMAZING! (Unless she's PMS-ing, then all bets are off!) and she is just generally enjoying her new life. Last month we had a check up with her surgeon. This always involves an ammonia check, and we were thrilled with her numbers!!

The ultrasound showed the shunt functioning well with good blood flow. Also, Angela's liver had increased significantly in size and is now near normal size for her body! So pleased was her doctor by her current status that he moved things up a bit, allowing us to schedule her next procedure, and today is the day!!

This time Angela will not have that giant incision she had with her previous surgery. This time she will go into the cath lab for another  hepatic venogram. They will measure the current pressure of her liver (which is assumed to be very good right now.) Prior to her first surgery her pressure was at 14, which put her at significant risk of of vessel rupture either inside the liver or in her esophagus. At surgery they partially closed the shunt which caused the pressure to increase some, but not more than 12. Today they will see how the liver reacts when the close the shunt completely. One of three things will happen:

1) Her liver won't like the increase blood flow causing the pressure inside her liver to jump. If this happens the procedure will be stopped and we'll schedule a time for the Operating room for another partial closure like we did back in April.

2) Her liver pressure will increase some. If it hits a certain level (and I don't know what that level is) without going TOO high, they will go ahead and close the shunt, but admit her to the ICU for a couple of days while her risk of throwing a blood clot or vessel rupture is high.

3) Her liver will accept the additional blood flow without much drama, they will place a coil (similar to the ones used for heart defect repairs) allowing the shunt to clot off, and she will come home. The very same day!

Obviously we're hoping for #3, and her doctors seem confident this will be the scenario we see today. But I'm guarded, afraid to get my hopes up. Afraid that they are a bit too optimistic since Angela's body has never done what its "supposed" to do.

So, this morning she will take a shower with the special surgical scrub that has been our friend as long as Angela has been on this earth. Then we will go to the hospital and take a peek inside that pesky liver of hers and see if we can set it straight once and for all!!

If you could, please keep our girl in your prayers today.


Tamara said...

Praying for 3 today.

diane said...

Prayers for Angela coming your way. She has been through so much and hopefully and prayerfully she will come through this with flying colors. What a lovely girl.

Marci said...

Such a beautiful girl! Prayers coming your way...