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!"

Thursday, April 28, 2016

Orange and Pink

Angela is doing great! She had a few rough days last week, but seems to have turned the corner now. She isn't able to tolerate bolus feeds yet, (bolus feeds are relatively fast tube feedings) instead needing them timed for a full hour. I'm hoping to work her up to bolus feeds before she goes back to school next week. In the meantime, she is putting in appearances at various social events. Today her music group performed a couple of songs for the annual Highland Friendship Club's "Be A Friend" luncheon. Angela is in her element when she's mingling in a crowd or in front of an audience. This is Axel's first year in HFC and Angela was more than happy to introduce him to everyone. And I do mean, "everyone"!

In just 21 days we're moving to our new house!! This move was my idea. It took me eighteen months of seed planting to convince Dean this is a good idea. Mostly he didn't want the work that comes with a move. He had just warmed up to the idea when Audrey started having trouble sleeping. She and  Angela have shared a room for the last two years, but that didnt work so well once the not sleeping boycott started! We ended up moving Angela's bed to the basement playroom where she has been ever since. Suddenly we were out of space! We put the house on the market in June but had already missed the Minnesota spring real estate rush so the house didn't sell. We took it off the market in September, put on a new roof, new carpet in all the bedrooms and a few other little projects in preparation to try again this year. The first week of March we found a house we fell in love with! We listed our house March 11th and it sold the same day. We close both houses on May 20th!

The little kids are fascinated with the POD and spent half an hour exploring every inch of its emptiness.

Did I mention this move was my idea in the beginning? I remind myself with each box I pack. Moving with 6 kids, five of them being kids who can have problems with change is interesting to say the least! I think our trip back in February ended up being good preparation for the move. The kids understand we all go together, that they are safe, and that even in a different place all their needs are met, AND we're still a family! So far they seem excited about the move.

Because Angela has been a year without a space of her own I promised hers would be the first bedroom decorated. She informed me her new room colors should be orange and pink. Ideas quickly flooded my head, and Angela and I have been looking at bedding to go in her new room. Here are her favorites so far. Oh, and of course occasionally something else slips in there, like dolphins. LOL

As for Dean and I, we are both ready to move on. This house has served us well, and we have added 6 kids to it since we met in 2003. Now its time for someone else to enjoy this space. Let the packing begin!!! 

Sunday, April 17, 2016

She's home!

We had originally planned for Angela to be in the hospital anywhere from 2-4 weeks, allowing her liver time to adjust to it's new blood flow, and her gut restart all its processes. We're thrilled that she's home on day 10!!!! 

Our patient is currently resting on the couch, and has had nothing but Tylenol all day, which is pretty amazing. She has handled this whole event so very well, and accepting some very difficult circumstances. She was even thanking staff after having to do something unpleasant like start a new IV line. We are so proud of her! 

We go back to the surgeon in two weeks for another ultrasound and blood work to make sure all is functioning as it should. Then she'll have several months for her liver to continue to grow and adjust. It will likely be September before we look at the next, and what should be last, step in her liver repair! We are so very thankful for the amazing doctors who are treating her!

Another teenager in the house!

Thirteen years ago today, in the city of Belgrade, a baby boy was born. He was very much wanted, planned for and loved while in his mother's womb.

And then he was born.

Things changed from that point on. At two days old Abel was sent to live in the institution in Belgrade. Left alone for the first three days to "cry it out", a practice meant to teach babies that crying is ineffective, Abel didn't stop crying like he was supposed to. He spent the next 10 years surviving.

Then three years ago this week, Dean and I made our first appearance in his small, sheltered world. He was so tiny, yet SO STRONG!!!

That day we made a decision to love this little boy no matter what. It was his birthday, and the staff could no longer manage him. Had we not agreed, he was being moved to the adult facility in Subotica. Abel was not a happy little boy. He cried constantly and was a bundle of high anxiety. We knew that life with Abel would not be easy, but we chose him anyway.

It took some time, but Abel learned that our showing up every morning and afternoon was a good thing!

Let met tell you how amazing this young man is!!! He is the most observant young man I have ever met. His communication has bypassed most of the other kids. He is strong as an ox, and if you need something heavy moved, Abel is your man! He will happily lug that large object wherever you want it.

Abel is a happy young man now, with broad shoulders and the shadow of a mustache. He has found humor and joy in life. He knows there are people who love him, and he returns that love in his own ways. Although he appears to spend a lot of time in his own world, he is very much connected to the here and now. Although he is not attached to the people here, he IS attached to our home and routine.

This post can't be written without acknowledging the woman who sits weeping on the other side of the world. She has not seen Abel since the day he was born, but still, I know she weeps.

Abel, we are so happy God brought you to our family! You have added the element of surprise, and a special sense of humor. We wouldn't have it any other way!

Happy 13th Birthday Abel!!!!

Wednesday, April 13, 2016


I can't believe it's been five days since Angela's surgery. It has both dragged on and sped past, all at the same time. Hospital time is like island time; everything happens at a different speed than the rest of the world. Sometimes it's too fast for me, and other times not fast enough.

I posted about the change in procedure for fixing Angela's liver. It has been nothing short of amazing! Without the need for any scavenger medications, Angela' ammonia levels have been in the 20s. Can you even believe it? The surgeon had warned me the incision would be large. Still, it was a shock to actually see it in real life. Then there is also a 6 inch incision on her thigh where the graft was harvested from. (pic posted with Angela's permission)

Now, I need to be clear. We have been allowing Angela to eat a few things by mouth for "social eating" as her doctors referred to it. Telling a teenager who loves food that suddenly she cannot eat a single thing does some pretty serious things to that kids' mental health, so we allowed a few things, all zero or very low protein items, and only very small amounts. Plus, when she is well, she is able to keep her lungs clear a bit better, particularly when her protein level is down. We even let her drink a little bit. BUT...that is on a good day! Not when she is high on narcotics that affect muscle tone and her protective reflexes, so in  her current state ZERO eating is allowed. Everyone here knows it. There is a band on her wrist, a sign on her door, and its written on the white board next to her room. Immediately after her surgery she was allowed to suck on toothettes to keep her mouth moist, and an occasional ice chip. As in, a single chip of ice which is nothing.

On Sunday we were moved out of PICU and into a regular room. A couple hours later I left the room to get some dinner. While I was gone one of the nurses gave her tylenol and her reflux meds by mouth. Angela was quit excited to show me her red tongue! I quickly brought to the nurses attention the fact Angela aspirates everything and there are NPO signs all over the place.

Later that evening I left the room to make a phone call. When I  returned Angela had an entire 16oz cup of ice chips in front of her that she was just finishing. (So yeah, great. Let her mouth and throat get nice and numb and expect her to swallow safely, while on heavy drugs!) I said something to the nurse who pretty much blew me off like it wasn't a big deal. I was NOT happy and said something to the charge nurse about it.

We had been waiting for Angela's GI system to wake up and start making noises so she could start getting food through her gtube. Finally it was making quiet noises so we were anxious for the surgical team to do rounds! Monday morning she was doing really well and there was talk of her being discharged by Weds! I had to leave for two hours to switch vehicles with Dean. Two hours. When I came back Angela was just finishing a yogurt cup. She was very exited to tell me, "I had yogurt, and pudding, and jello, and grape juice!" and stuck out her tongue to show me it was indeed stained purple. I immediately called the nurse it. "Why is she eating, and who gave her the food?" The nurse responded, "Oh, the surgical team lifted the NPO orders and said she could go to full liquids (pudding and yogurt count as full liquids) The nurses did not realize Angela's NPO orders had nothing to do with surgery. A few hours later her very wet cough started, so we were all pretty sure she had aspirated. I was very quick to get the patient rep, surgeon, charge nurse and nurse manager involved. Everyone is now on the same page, but I have not let this drop. This was a major negligent act on their part.

That was just the beginning of a very difficult night. Swallowing problems aside, when the doctors lifted the orders for Angela to get food, they did not mean an entire meal worth. "Take it slow" is the rule after major work like Angela had done. Angela's GI system rebelled, shutting down once again. Her abdominal cavity started filling with fluid, and by 2:00 a.m. she was writhing in pain and had bruising showing up all over her belly. The surgical resident was called who was very concerned with what was happening and suspected the graft was no longer working. He ordered an ultrasound for first thing in the morning. In the meantime we couldn't get Angela's pain under control and she was miserable the rest of the night.

Finally and ultrasound was done around 9:30 in the morning. The graft was still working but there was a LOT of free fluid in her abdomen. Also her liver didn't look right but they think its because the liver is adjusting to new blood flow so there are physiological changes happening to it. We came up with a plan to help with the excess fluid. Her poor belly looks like she's 10 months pregnant. Since surgery 5 days ago she has gained TWELVE POUNDS, which of course is all fluid!!! Thankfully the incision is holding tight, but because she is on blood thinners she now has bruising all over the place, with a couple softball size bruises on her belly.

Today (Weds) her belly is still huge, but her pain seems slightly better. Her hemoglobin is very low so they're watching close in case she needs a blood transfusion. But, when her pain meds are doing their job she is game to sit with volunteers in the playroom and do some crafts, or take a walk around the unit. She tires very quickly though. She's not yet ready to try food in her belly yet so we're still discharge is still several days away. When she does come home she'll be on daily injections of Lovenox to prevent clotting since she is at really high risk.

I think that brings us about the current. I'm hoping the next couple of days things smooth out a bit. I have Monday in mind for a discharge goal. I guess we'll see!

Friday, April 08, 2016

A functioning liver

For the first 19 years and 6 months, we didn't know that Angela's liver wasn't able to function properly. We spent 11 years treating crazy wild mood swings, *severe aggression*, seizures, grogginess, a stroke and eventually two comas before figuring out that she had an Abernethy type II malformation.

A normal ammonia level is 15-45 mcg/dl. When we first discovered the hyperammonemia, and they thought she had a Urea Cycle Disorder, our goal was to keep her ammonia levels under 50. With heavy scavenger drugs it was a struggle, but we could do it. Then suddenly in January she started spiking again, and not responding to the rescue drug Ammunol. A very intense and dangerous drug, with one dose being a 24hr IV drip in the intensive care unit, only she needed two back to back doses then spiked again. That's when they finally moved to additional testing and found she had the portal liver shunt.  The old goal was tossed out the window. Now it didn't matter how high her ammonia was, they told us not to bring her back unless she was having seizures or fell into a coma again. Really, not very comforting, but they needed time to discuss with other doctors around the country an come up with a fix for this. She was discharged from the hospital with her ammonia at 175. 

When we set a date for surgery, her medical team had come up with a plan, something that had never been attempted before. Over the course of 3-4 surgeries the would use a graft vein from her groin and gradually close the shunt, a little more with each surgery, allowing her liver time to adjust to this new blood flow.

This morning her doctor came in and explained they had change of plans, and yesterday met to discuss this new idea. It is what they did today. Without going into the long explanation, they used the graft vein to make a smaller shunt, small enough that with her next surgery it can be occluded in the cath lab without having to make another huge incision in her belly. They also found a bunch of tiny shunts that went "every which way" into and out of her liver, and were able to just clamp them without problems. 

What does all this mean? It means that Angela's liver now has adequate blood flow and should be able to cleans her blood without ANY of the nasty drugs she's been taking for the last few months. It means she gets her independence back. It **MAY** mean she will no longer need her feeding tube because her swallow is so greatly affected by the ammonia and that will no longer be an issue. So this afternoon, three hours out of surgery, her ammonia levels were checked. Her liver ROCKS!!!! Her ammonia is at 28!!!!!! That is without ANY SCAVENGER DRUGS!!!

Just the thought of all this, of the potential for her life, of this new young lady we have seen emerge from the ammonia fog....all of it...has me sitting here sobbing tears as I type. This is a true miracle for Angela. For us. 

Pain management has been an issue this afternoon. Now that I've updated everyone here, I'm going to take a nap while she has a few minutes resting quietly in the Intensive Care Unit.  I cannot thank you all enough for your love, support and prayers!!!!! 

Update from the OR

This morning we were given fantastic news. Angela's surgeons met again yesterday to discuss a new option for closing the Portal shunt. (Later I can make a video and draw it out) He felt it was very possible the could close the shunt in one surgery, but if not they could place a graft, making a smaller shunt, bringing the number of surgeries down to one or two, instead of 3 or 4. It would also mean an end of having to taking the horrible medication.

Just now they called from the OR. We're 4 1/2 hrs in. Unfortunately they weren't able to do it all at once, so have just started phase two, which is to place the graft. Still, before this it was estimated less than 1/2 the necessary blood was going through her liver. This step means her hepatic blood flow has increased significantly.

Friday, April 01, 2016

One week

I've been a bit quiet, I know. I need to post updates on Audrey. It has been a *very* long two months, with another couple of months go, getting her through this knee reconstruction. I don't think we were at all prepared for how physically demanding this process was going to be. It was not explained well to us at all. Getting Audrey in and out of the big van isn't easy, and that combined with Angela's liver related hygiene issues have left us largely home-bound. Like I said, its been a long two months.

One week from today, on Friday April 8th, Angela is scheduled for the first of  3-4 surgeries. This will be an attempt to gradually close the vascular malformation in her liver. This procedure has never been attempted before for this type of malformation. 

I'm pretty much scared.

The only thing that scared me this much was being told I have cancer. Actually, I think this scares me more.

I cannot convey to you the seriousness of this surgery. I cannot convey to you how high risk it is. But I can show you some more images.

Do you see the large blue vein that runs behind the organs? That is the Inferior Vena Cava, or IVC. It brings the blood from the body back to the heart to be re-oxygenated. It is huge, to handle the high volume of blood. In front of it lies the Portal Vein, which brings blood into the liver to be cleansed of impurities, like ammonia. Do you see, right at the base of the liver where the portal vein starts to branch out into the liver? In Angela that first branch is inside her liver. However, instead of just moving into the liver, it re-connects to the IVC right there. It is a very large connection, which causes the majority of Angela's blood to go straight back to her heart without being cleansed of ammonia and other toxic blood waste.

Once inside the liver, the portal vein branches out into hundreds of tiny little vessels, which bring the blood further into the liver so the liver cells can do the scrubbing. Remember the car wash? These are all the little stalls in the car wash where the individual cars get washed. Its a crazy mess of vessels.

Unfortunately in Angela's case, because the blood flow through her liver is not adequate, those collateral vessels are not fully formed. Some are there, just smaller than they should be (similar to a balloon that has never before been inflated), and some are completely missing.

When God created the liver, he created a miracle right there. And then God created doctors, giving them the smarts to understand all this stuff and taught them how to fix it when it  doesn't work just right.

Next week three smart doctors will take a small section of veinous material from Angela's leg to use as a patch. In this first surgery they will only close a small section, attempting to reduce the junction by about 1/4. As the doctors slowly close off the huge opening, blood is going to suddenly fill those little vessels. Angela's hepatic pressure (blood pressure within the liver) is already very high at 14+. When the blood flow inside the liver is changed, the hepatic pressure will either increase or decrease, depending upon how the vessels react. If the pressure increases those vessels can begin to rupture. This would cause massive hemorrhaging. If the pressure decreases, the vessels can slowly adjust to the new blood flow. So the doctors will place the patch, then sit back and wait for a bit, constantly measuring the hepatic pressure, with a goal pressure between 10-12 right now. Once they feels she is past the most dangerous time they will finish up the surgery.

Angela will have this surgery repeated 3-4 time over the course of several months, in the hopes the junction can be fully closed and the blood vessels start to function as they should. If successful, it will change her life. It will save her life. After each surgery the biggest risk will be massive blood clots. There is already an old, calcified clot sitting in Angela's portal vein. (The doctors think its possible it was a small piece of this clot that caused Angela's original stroke as an infant.) Angela will be on daily injections of blood thinners for the next several months. As long as it takes to through this process.

If this process does not work, the only option left for Angela is a liver transplant. We really don't want to go there.

I do have some specific prayer requests...

That God comfort Angela as this surgery date approaches. She is so anxious right now. She is starting to pick at her skin and hair, which is how she shows anxiety. I pray for her peace. A calm and quiet body. We also suspect her ammonia levels are climbing. She hasn't been herself.

...for Angela's health, between now and surgery day. She's currently fighting yet another sinus infection and started on antibiotics tonight.

 Angela is going to have a huge incision. They told me the same or similar to the incision if she was having a liver transplant. (whatever you do, DO NOT google "liver transplant scar"!!) I pray that God guides the nurses, doctors and myself to keep Angela's pain to a minimum, and what pain she does have is well managed.

...for wound healing. Some of you have known Angela long enough to remember the horrible problems she has with wound healing. When she was seven she was in the hospital from November to March with a huge open surgical wound that would not close! The doctors think its possible the high ammonia levels were responsible for the poor healing. Each of the surgeries she has for her liver will go through the same incision...again...and again...and again. I pray that God knits these incisions closed with a breath of healing.

...for the nursing staff. We had grown attached to the staff at St. Paul Children's Hospital. When we moved over to the University of Minnesota I didn't feel as comfortable with the care Angela was getting. I don't know if it was just a lack of familiarity with the staff and routine, or if there was truly a difference. Whatever it was, I was  not comfortable leaving Angela alone. My prayer is for Angela to be assigned caring, attentive nurses.

...for the schedule. This is going to be a long hospital stay. We were told anywhere from 3 days to 2 weeks in intensive care, followed by the very slow process of re-introducing food so her liver can slowly adjust to its new workload. This is going to be hard on our family and our finances.

...for Dean. He will be alone with the other kids a lot. We need to get PCA's set up who can help him a couple days per week. I pray for his stamina and patience. I pray for his health that can be affected by the increased stress level.

...for the other kids. Again, this is going to be very hard on our family. When mom is gone a lot it throws off the routine, and at least one of our kids does NOT do well with changes to the routine.

And lastly, for myself. I need to stay physically healthy to keep up with the insane schedule looming before us. The emotional and physical stress really took a toll on me a couple months ago, and I don't want that to happen again. My emotions are already a bit frazzled as I deal with my own anxiety about this surgery.

..for Dean and I as a couple. We have very good communication between us, and it needs to stay that way.

My blog is set up to post to twitter. If you don't already follow us there, you can find us under @GardenofEagan I'll be posting updates throughout this week, and during Angela's surgery day to keep everyone informed.