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!"

Wednesday, August 17, 2016

Some good news with a little drama

Angela pretending to watch tv, but really trying to think of more things to talk about to keep the  male nurse in her room longer.

Well, yesterday was pretty interesting. Apparently there was an option #4 that nobody had taken into consideration!

Angela was in the cath lab MUCH longer than she was the last time she had a hepatic venogram done. At 3 1/2 hrs I was starting to get worried. What could possibly be wrong? As it turned out, her doctor had been called to an emergency case immediately after Angela's, and somehow nobody came to talk to me until Angela was already awake and talking the nurses ears off.

So here's what we found out:

When Angela's last ultrasound was done in July, her liver looked awesome, and the bypass that had been placed was open and functioning well, with good blood flow. Based on that the doctor stopped Angela's blood thinners (yay! No more shots!) in preparation for yesterday's procedure. Well, when the doctor tried to get the catheter into that shunt, the access was too small, plus there was a sharp angle she couldn't get the catheter around. "The access was too small" because it is starting to close, ON ITS OWN!!!! This means the blood flow and pressure in Angela's liver  has normalized so its no longer needing to force blood through the shunt!!! This is pretty amazing, actually!!! Angela's body is trying its best to normalize the liver vascular system. Also, this doctor couldn't get over the difference in the size of Angela's liver now that it has adequate blood flow! 

The plan is that in one month we'll do another CT scan to look closer at that shunt. If it is the same size as now, they will go in from a different angle (remember that sharp angle she couldn't get around?) and close it off. If the CT shows the bypass has gotten smaller, then we will leave it alone and continue to watch. However, all of her doctors suspect that it will be completely closed by then. This would be absolutely amazing!! 

Angela's doctors are to be commended for coming up with this fix! Its now being written about in journals (which, I don't know) as a new option for others who have Abernathy malformations. 

But, it just wouldn't be a surgery day with Angela without a little drama, would it? So in the cath lab an catheter is inserted through a vein in the groin that is run up into the liver (or the heart, or wherever they need to get to) Just one tiny incision in the groin vein is the only evidence anything was done. That hole needs pressure kept on it for awhile so it will close off, then they put a pressure dressing on it. Depending upon what organ was accessed the patient lays flat for 1-2 hours, then slowly work their way to sitting up all the way. 

Angela had been laying flat for nearly two hours. Near the end of that time she was drinking juice and eating some crackers, which, being flat on her back was a bit tough for her and she coughed a couple of times. A couple minutes later her blood pressure took a little dip. Not a lot, but it was odd. She had  been flat long enough so over the course of 15 min or so we let her sit up. She was ready to go home so I signed discharge papers and we had just slipped her shirt on. I pulled back the blanket so we could put her pants on and OH MY GOD!!!!! She had a fountain of blood coming out of her leg!!!! "Uh oh! Problem!" I said. 

Suddenly there was a big flurry of activity. Two nurses putting HEAVY pressure (as in all their body weight) on that hole in her groin, time noted, doctor paged...again and again...IV restarted to push fluid (thankfully we hadn't pulled it yet!) They gave Angela some pain meds because it HURTS to have that pressure on one spot for so long, plus she needed to relax because she was really scared at the sudden activity. Someone pulled the blanket back further to get an idea how much blood she had lost. Holy crap!!!! She had lost a fair amount. Of course we don't know how long she had been bleeding, but the nurses suspected it was the cough that caused it. They held heavy pressure for 20 minutes then gently lifted the bandage to check again and it started right back up. It is really hard on the nurses hands to hold that pressure for so long so they switched nurses, and had to hold pressure for another 20 minutes. All I could think of is what if this had  happened in the van on the way home? I wouldn't have known and she could have bled out!! 

Then one of the nurses went to the cath lab and got a newly designed pressure dressing that allows you to see the wound without removing pressure. It was pretty cool! They put that on the wound. It had to stay in place for two hours, then they could remove half the pressure, then another  half hour the rest of the pressure but leave the bandage in place. 

Angela was finally discharged at 10:00 pm. I was a bit nervous about bringing her home but she did fine. She slept well all night, but I was up frequently to make sure she wasn't bleeding to death in her bed. 

So now we wait to see what Angela's liver looks like in one month. Yay!

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.

Saturday, August 13, 2016


One thing about adopting older kids, is they don't stay little very long. Axel is 16 today. Gone is our little boy, and in his place a man!

I know on the other side of the world is his first mother, father and brother,  who all love him as much as we do. This morning we were able to share pictures, and get birthday greetings from his first family.

Today we will celebrate Axel, and next weekend have a party with his friends and some family. We can't wait to see what 16 brings!

Monday, August 01, 2016

Big sister to the rescue

August is our month for appointments! All the kids will see the eye doctor, dentist, get whatever shots are needed, and get physicals for sports.

Today was Abel's eye appointment. He really has a tough time with doctor appointments. Angela likes to come along to help him and she is great at this! Today we were in the play area waiting for his eyes to dilate. There was a group of siblings who were not very nice, but their mom was busy at the registration desk. One boy about 7 kept taking toys away from Abel. After stepping in a couple of times I was getting annoyed but reminding myself I dont know if this boy has a hidden disability. I was just about to call Abel over to me when Angela jumped in. "Abel, lets read a story!". She sat down on the floor between him and the other kids and started reading a book to him. She is such a good big sister!