On the beach

This trip wasn't about fancy. It wasn't about 5 star restaurants. I didn't pack any make up. We didn't pack and "nice" clothes, just our every-day stuff. The owner of the house in GA asked if we'd like to have some family photos taken. Wow! We have never done that. Ever. We opted for pictures on the beach.

I panicked. Oh my word! What are we going to wear? I looked at the kids, at the boys who *all* were in desperate need of haircuts, as were Dean and I.  I had nothing but t-shirts along for myself. Oh man, these were going to be the real US! The real deal! No hiding under brand new clothes or make up. Just shaggy heads, frumpy mom, sandy faces.

And I love them.

I love every single one of them. I have very few pictures of myself with any of my kids, and now I have pictures of me with every one of them. And pictures of Dean with every one of them. And pictures of all the boys together, and my girls together. And Dean and I....oh how these pictures of Dean and I together mean the world to me. Here are some of our favorites. You'll see everyone's personalities, from those who don't really care for hugs to those who are a bit more mischievous, the serious, the "Teen who can't smile for the camera", and the one who loves the camera most.

I want to say a special thank you to Tamara Gibson Photography, who so generously gave time to our family. These pictures mean the world to us.

A little frustration, but all is still good

Audrey's surgery went very well. Her left knee took a lot longer than excpected, so her total time in surgery was 5 hours. Her ABR results show her hearing to be pefect, as we suspected.

There have been a couple of frustrations with this prococess. First, I was told Audrey would come out in casts that would be on 6 weeks, then move to immobilizers that would be on another 4-6 weeks. Instead she came out in immobilizers, and she will be fitted with a fixed brace with a hinge so her knees can be put in a range of motion machine, adding range of motion in small increments over the next 6-8 weeks. Ok, no biggie I guess, except that I knew it would add time to getting her discharged today (Thursday) and Dean needs to get to work.

The other frustration is she came out with an epidural in place. The anesthesiologist had told me he may do this, but did not tell me it would add 2-3 days to our stay! Audrey's comfort is the most important thing, but we need to be able to plan a bit for childcare for the other kids and all the other things involved. Add to it Audrey is having a problem with the epidural which may add another day or two to the stay.

Oh well. There is nothing we can do about any of this so we just move forward. Dean needs a break and the hoslital is quiet so he and I will switch places today. Then I can be at home getting vacation laundry done and the van cleaned out. Dean can take naps when Audrey
naps. ;-)

Angela came to help send Audrey off to surgery.

Soooo out of it after surgery. Thankfully with the epidural gt
She is very comfortable

Mom

Asher has just started drawing, and he is also learning to write. Last night at parent teacher conferences his teacher gave me this drawing he made, unprompted. It brought tears to my eyes!

We're Home!

I don't have time to post pictures now, but rest assured we had a great trip. The temperatures in the south were perfect for Angela who has anhidrosis and cannot handle the heat of summer at all. We were at a couple different beaches in GA and FL, then headed west to TX and came north from there. We did get trapped in IA overnight due to a winter storm that shut everything down, including the interstate. We found ourselves in a seedy motel to wait it out.

We arrived home at 1:30 yesterday (Tuesday) afternoon. Dean and the kids got out of the van at home, and I started running errands. Picking up new meds for Angela, groceries for home, then parent teacher conferences for Audrey and Amos, and an IEP meeting for Asher.

Today is Audrey's surgery. She'll be having bilateral knee reconstruction along with an ABR (type of hearing test) This knee surgery is the second step of the process started last spring. There has already been a huge difference made in her ankles and knees. Now its time to tighten everything up, place some bony parts she is lacking, and get her back to running. And let me tell you, this girl loves to run! This process has gone much slower for Audrey than expected because she isn't growing very well. She has only grown 1 1/2 inches since she came home almost two years ago. When she wakes up she'll have casts on both legs, from ankle to hip. She'll have those for six weeks, then knee immobilizers for 4-6 weeks after that. This should all prove to be very interesting! Thankfully we never moved last summer, so we're still in a house with only a couple of steps to get Audrey's wheelchair up and down.

Abel also had hardware placed last spring but he grew very rapidly so most of his hardware was already removed. He still has one screw in one ankle that will probably be ready to come out in April. Luckily he will not need any further work on his joints!

On Friday we meet with Angela's doctors at the University of MN to discuss if they came up with any ways to fix the portal shunt inside her liver. We are hoping they came up with something!!!! When they thought she only had a urea cycle disorder she was admitted to the hospital if her ammonia levels were 50 or over. When she was discharged almost two weeks ago she was over 125. A lot of people with liver disease can run much higher, but it is very unpleasant. The migraine from high glutamine levels is constant, and, for Angela, it makes her very aggressive and irritable. But her doctors made it clear, there is no reason to admit her now unless she has a seizure or falls into coma again. In the meantime we do a constant dance with her protein intake, calorie intake and fluid volume, and we seem to have found a good routine for now. 

Island life

Island life is quiet and serene, which is exactly what we needed. No hustle, no bustle. We drove HARD to get down here to enjoy some sunshine before bad weather rolls in, and enjoy we did! We went to the pier, played on a playground overlooking the beach, went to two different beaches and dipped our toes in at both. Saw dolphins, swam in the pool and loved feeling the sun on our winter skin. Today is rainy and is expected to be for the next couple of days. Alternative plans in the works.

The Car Wash

Angela doesn't have a diseased liver, she has a malformed liver vascular system. Her actual liver cells are (mostly) working fine. I've been trying to come up with an analogy to make it easier to understand. Think of a car wash.... People drive in and their cars get washed. The car wash works great and the cars come out shiny clean. But THOUSANDS of cars drive right past, full of dust and dirt. They could get clean, but they need to drive into the car wash! If only they would go into the car wash!!! But they don't, so they continue to get more and more dirty until they start to rust and erode. That is how Angela's liver functions, like the car wash. The blood cells that are supposed to be going to her liver for cleaning are bypassing her liver, and being shunted back to her heart, causing ammonia to accumulate in her blood. Thankfully Angela is really physically small and she stopped growing at an early age. If she was a larger adult there is no way her liver could keep up.

*People have asked about Angela traveling. She is as stable as she is ever going to be and her doctors gave the green light. No more ammonia checks. She will be forever high (she was at 125 at discharge) until they find a fix or she gets a new liver.  They don't want us to bring her in unless she has an acute change, such as seizure or another coma. She's on two new meds and still on restricted protein to prevent her spiking, which is hard on the body. We have a meeting with her team when we return. In the meantime we hope the other specialists they talk with come up some options. We know she needs a transplant in the future, the medical team needs to decide the "when" part.

** One person has repeatedly asked about Make A Wish and how it is Angela is still alive. Please refer to the Wish.org website for clarification. A child does not have to be terminally ill to qualify for a Wish. They need to have a life-threatening medical condition. "life threatening" and "terminally ill" are not the same. 

Just about ready

Details are done. House sitter arranged, dogs accounted for, laundry done and house cleaned. We're ready to go.

A quiet house on the beach in Georgia. No rushing. No doctors. No crowds. No people. Just us, a road trip, some sunshine, fresh air, skipping rocks, feet in the sand, family time. Reconnect. Regroup. Catch our breath before the next round.

Angela's Liver

You liver people will understand this. I will try my best to explain for those who are trying to understand.

First, here is what the liver vascular anatomy is supposed to look like.

The IVC (thich blue vessel) runs kind of behind the liver, bringing blood back to the heart to be oxygenated. The portal vein (purple) runs directly in front of it, through the liver, bringing blood to the liver to be filtered of toxins.

Here's the picture the doctor drew on the board in Angela's room. This is a side view. Angela's portal vein, instead of running parallel with the IVC, it stuck to it and is open. The dotted lines show where the portal vein is supposed to go. Keep in mind, this is all inside the liver. You look at the picture and think, "So just cut it and sew the opening closed." Only it doesn't work that way. That junction is huge, and more than 1/2 of Angela's blood flow dumps into her IVC instead of going to her liver for cleaning.

On the left of the drawing of Portal Vein and IVC is a crude drawing of the liver. You see that bulbous looking thing. That is where the Portal Vein is blocked. The blood flow stops. There have been a lot of little tiny vessels the body created to get blood to the liver. If they just closed off that big Portal/IVC connection, those tiny vessels would explode because they can't handle the blood flow.

But that picture, and the drawing, make it all look so easy, when in reality the NORMAL liver vessels look like this:

Angela's liver has tons more tiny little vessels due to revascularization. The most major risk is blood clots. The IVC goes directly to the heart and *anything* they do around this creates an unbelievable risk for a blood clot, that would go straight to Angela's heart.  Lucky for Angela, because the blood isn't getting to the liver, her liver isn't able to produce enough clotting factor. It is that deficiency that has kept her alive for 19 years before we discovered this problem.

The doctors have proposed one option that has never been tried before. They want to bring this to the other leading liver specialists in the country for their opinion.

Our only other option is a liver transplant.

Because a donated liver has a maximum life of somewhere around 10 years, they want to buy as much time as possible with her current liver. The two drugs she's on to keep her ammonia levels down are very hard on the gut and Angela is already having trouble with them.  They are a band aid that will quickly lose their effectiveness.

The haters have already started commenting on the blog. I'm sorry if you hate us. I really don't care.

Buying Time

I'm going to be very direct in this post. I don't know how else to be. I have no energy to be anything but direct.

In 2008, when Angela was 11, she was granted a wish from Make A Wish. We did Disney World and the other activities that are included with a MAW to Disney.

It was too soon.

Sitting in the meeting with doctors this afternoon was more than I could handle but I did it. I took in all of their information, I asked semi intelligent questions. Then when they walked out of the room I walked into the bathroom and vomited. And I cried the hardest cry of my life. Worse than the day I was told I have cancer.

Angela is being discharged either tomorrow (Friday) or Saturday. She will be relatively stable on band aid drugs, but only for a short time. The doctors are needing 2-3 weeks while they connect with other specialists around the country to get additional opinions and try to come up with something. Anything.

I can't go into details yet. I'm not ready to put them in print. But we want, we need, to get our family out of here for a few days. The last few months have been horrible and we're damaged. What is ahead will be worse.

We need sunshine, warmth and we need time together as a family without any outside intrusions. We need a family "Wish", if you will.

I don't know how we will do this, except a miracle. But on Monday I want to have my family in the van and be driving somewhere private, somewhere with warm sunshine and a pool, somewhere we can leave all of this behind us, if only for a few days. (Audrey has surgery on Feb 10th, so it has to be short.)

Who can help us do this?

Abernethy Type II Malformation - more

Yesterday I explained that Angela has Abernethy Type II Malformation. Today with another ultrasound we have a slightly more clear picture.

Let me just say, I wish we were all stick people on the inside, because medicine would be so much easier.

Angela has a large shunt (like a bypass) from her portal vein directly into the IVC. The reason her ammonia level spikes is because this shunt is HUGE!!! Like, as big around as my thumb. It appears to be the same size as the IVC. That means roughly half of her blood doesn't get toxins filtered.
Unfortunately the problem is made more difficult by the fact she has two main vessels that run parallel to one another, appearing to be fused together.

This is not something they will be able to fix in the catheter lab. She will absolutely need surgery to fix it, and they said, "It is a surgery that is difficult on the body, but she will also feel much better." It will take several months for her body to adjust to the change in blood flow, increase in protein, in addition to just physically recovering from the surgery.

Then there is the large blockage (and the new vessels created because of it) on another part of the portal vein that must be addressed. They cannot see it well enough on the MRA that was done last week at the other hospital so she will need that repeated. It is very likely she is going to need two separate surgeries. One to fix the large portal shunt, let her body recover a bit, then go back in to address the blockage. At their meeting later this morning they will discuss how they want to proceed.

Lord God, only you know how this child survived 19 years with this. There is some reason, probably one we will never know, that you had us wait this long to find out. But doctors learn by experience, either theirs personally or the experience of other doctors. Angela's vascular malformation is exceedingly rare, with only a few documented cases. Doctors all over the world will be learning from our queen.

Abernethy Tyle II Malformation

We're almost there. Almost to the point of a final decision what we're doing. If only she could have just one thing going on!

1) She has Abernethy Type II Malformation - this is a malformation between the portal vein and IVC (the main artery through the body) which prevents blood from going into the liver to get filtered of toxins, like ammonia.

2) Her Portal Vein inside the liver has a large blockage in the form of a clot. They need to look more closely at that to determine if there is blood flow behind it or not.

3) Because of the Portal Vein blockage, she has developed lots of new little vessels, and they need to determine if any of them need to be closed off, left alone, or what.

4) they are not convinced her liver cells themselves are working fine, that all of this business may have caused damage to her liver over the years. They are taking her off ALL THE DRUGS that are keeping her ammonia down so they can see exactly how her liver works. They are also putting her back on full protein. This is probably the scariest thing for me. We will watch her very quickly fall asleep.

5) Her PICC line has failed so they're removing that in just a little while. We need to pray she doesn't need another line right away because it will be done under and emergency situation which is never what we want.

Whats the plan, Stan?

Today they did nothing.

Angela sat around all day while we waited to hear what "The plan" is for further testing. At rounds this morning they said they would be spending the day going over every stitch of her previous records and tests to determine the next step. Tonight when I came back to the hospital I paged the resident. "What's up? What's the plan?" Because I know that any further testing is invasive and requires anesthesia, and there are no orders for Angela to be NPO (without food) meaning there is no anesthesia in her near future.

The resident explains "It was a hectic day. They didn't get to it."

I get it. There a lot of very sick people in this hospital. Many worse than Angela, and others here with just "routine" things. And yet we know the maxed out meds are what's keeping Angela stable enough to bop around the hospital but not stable enough to be home. But, there are how many doctors in this place? Like a million or something? So really, they "couldn't get to it"?

Angela could have sat around at the other hospital and done the same "nothing" that she did here, only in a place where she knows everyone and I feel comfortable leaving her. I had to leave today, because we hae 5 other kids who also need their mom and I didn't feel comfortable leaving her.  Oh she had a good time, don't get me wrong. There was no shortage of volunteers to hang out with her, and she truly enjoys that. But she is wasting. Her body is eating her own protein - muscle - because we can't raise her protein because everyone is worried what will happen to her ammonia levels when they do. So they're keeping her protein at half what she should be getting while they "come up with a plan". And while they "come up with a plan" there are nurses who don't think about the fact the kid  who is hooked up to a continuous drip of fluid may need to pee more than once every 6 hours and perhaps they should get her into the bathroom. And that perhaps the aroma emenating from the playroom while she's hanging out with the volunteers means she cannot manage her stomach with the ton of drugs she's on and maybe they should take care of that. That maybe when the mom is gone they should peek in on her more than once per hour.

And while we're sitting at the hospital doing nothing, Dean is at home with the 5 other kids trying to figure out how to be Mr. Mom while also going to work. And cutting his short hours even shorter so he can be home to get the kids of buses. And when he's tired, and his crappy knees are killing him and he just wants to sit down for a bit after work, he can't because there are 5 pairs of eyes starting at him wondering "Is it time for dinner yet?" And he is learning about keeping the house full of kids running, getting all the laundry done so kids have clothes for school, keeping up on any school forms, permission slips, etc. without Mom's help. Because Mom is sitting at the hospital with Angela who is doing nothing.

I promise, when the resident came to the room tonight,  I tried very hard to contain my snark and sarcasm as I explained that when tomorrow morning rolls around and the staff makes rounds, that I'm expecting to hear about this big plan everyone keeps talking about. 

Its time for a change of scenery

We love Children's Hospital St. Paul . The staff has been nothing less than wonderful, the nurses caring and compassionate. We love this place, and Angela loves the staff. We're here often enough that the nurses all know Angela, and whenever she is admitted they made sure the new nurses are paired with familiar nurses as they get to know the very subtle changes Angela displays when she's not doing well or declining. I feel comfortable leaving her to go home at night and know Angela is safe, and the nurses call me with any questions they might come up. In addition, all the staff here: doctors, nurses...everyone...respects my opinion as Angela's parent and truly treats me as part of the team.

Sadly, Angela's very complicated liver is beyond the scope of care that Children's Hospital St. Paul is able to offer. They are sending us on to the University of Minnesota Liver Center. Angela will be moved sometime today or tomorrow, as soon as they have a bed ready for her. I don't yet know if she'll be in the pediatric hospital or the adult hospital. The staff here has made it clear to the U of M the Pediatric hospital would be most appropriate.

Once at the UofM there are more tests arranged, that are more invasive that what she's had done so far. Angela is just excited she gets another ambulance ride. 

More but not enough information

Words for today:

Portal Hypertension - Increase in blood pressure in the veins within the liver, usually caused by a blockage.

Portal Thrombosis - Blockage of the portal vein, usually by blood clot.

Revascularization- Where the blood supply inside Angela's liver is not working correctly, she has revascularized into a "multitude" of new vessels. Some of you have known her long enough to remember she also has a revascularized area in her brain that for many years they classified as MoyaMoya Disease. After many more studies it was found the circle of Willis on the right side of her brain was not fully formed, and the left side had crossed over the mid-brain and had taken over the job. When a person has an area of revasularization anywhere in the body its possible they can have other areas. 

Focal Nodular Hyperplasia - benign tumors of the liver. They do not usually grown and it is unknown if they are related to all the other things wrong with her liver, or if they are just an incidental finding. Angela has "multiple" area of FNH, ranging in size from 1 - 1 1/2 cm.

Now take all those words, put them into Angela's liver, add a little "We don't know enough yet" and "You may need to go down to Mayo instead" and you have a picture of Angela's liver.

So Angela has portal thrombosis, with a multitude of revascularized areas where the body is creating new pathways for the blood. Because of all this narrowing and new, tiny vessels, she has developed Portal Hypertension. The interesting, and good part, is that her Vitamin K (which is needed by the liver to produce clotting factors) is low. If it had been at a good level all this time she likely would have thrown a major clot and suffered a catastrophic stroke. The other interesting thing is that Angela's liver should not be functioning well, but her liver function is actually just fine. Its just that she has blood bypassing the cleaning that needs to be done by the liver.

Still, we don't yet know if this is fixable. The radiologist who read the scans today felt they should be read by a specialist with more experience. That will happen tomorrow. The new radiologist will take all three scans (ultrasound, MRI and CT) compare image to image to come up with information we can use to make treatment decisions.

Unfortunately we may have surpassed the skills of our excellent doctors. The liver specialist at Childrens says this is likely beyond their range of expertise. This is a very rare, complicated problem Angela has, and we may need to move to a special liver center: either the University of MN or Mayo. The UofM is 20 minutes away, Mayo is 90 miles away. Tomorrow's report will give us a better idea where we're headed.

There were a few other scary terms tossed around today that I'm not even willing to discuss yet. We're not there. We need more information.

Angela is now on all the drugs she could possibly be on, at the max doses, and her ammonia is staying down to normal levels. However, she's not getting protein yet. Tonight and tomorrow we'll see if all these drugs can keep her system stable even with a protein load. Unfortunately none of the doctors seem very hopeful.

Today Angela was very chatty and perky. She's not walking around much but I'm sure tomorrow she will be. We're stopping the TPN tonight and adding in her formula with protein. That means disconnecting all but her gtube line. Please pray her system can keep up to the protein load. She is HUNGRY and is having a hard time with that. 

Very Special Thankyou!!!

I need to say some very special "Thank you!!!!"s to several people.

There was Carie who had a huge order of Schwanz delivered to our doorstep, and a POLICE hat that Angela loves! THANK YOU!!!!

Bonnie who delivered a basket-full of dinner and other goodies to our doorstep. THANK YOU!!!!

Dean's aunt Linette and her husband Brad brought a delicious meat loaf, sweet potatoes and other fixings.

Dean's cousin Eli and Jennie came over one evening and made a night lasagna dinner.

Randy & Sheila who delivered a lovely crockpot meal that we'll have tomorrow night.

Kathie who delivered some heavenly soup, biscuits and other stuff the kids enjoyed for dinner a couple of nights. THANK YOU!!!!

Adriene collected from her friends and mine for gift certificates from Lets Dish, along with hunting down codes for specials! THANK YOU!!!!

Roc, Nance and family who took all five kids into their fold of MANY for an entire day so Dean could be with Angela and I at the hospital yesterday.

If I missed you on this list, please let me know. I haven't been home more than an hour at a time this week, and not there to eat, so I don't know what all has been dropped off.

We cannot thank you enough for loving on our family during this really stressful time. We have a couple more weeks (Please God, not more than that!) ahead of us as Angela's liver malformations are treated, and the frozen foods we have set aside will help get us through.

Doing better today

Angela is off the Ammunol now, so she will be moved out of PICU tonight or tomorrow morning. The lactulose is working so her ammonia levels are staying down in the mid 50's, and sometimes even lower. In turn, her belly is feeling much better. The fall-out from lactulose is not fun to deal with, but its doing its job and that is what is most important for now. She is on TPN for the next couple of days, but we've also started her pro-phree (protein free formula) at full volume so she doesn't have to feel hungry anymore. Except for some ice chips here and there, this is the first time she's gotten to eat since Sunday last week.

We do know for sure that she has a liver shunt, which prevents her liver from cleaning all her blood of ammonia, which is why she has these high levels. This is something she has had her entire life, but her liver got really stressed when she got her gtube and her new awesome formula that was high protein pushed her over the edge. If there is some type of urea cycle problem, once you stress the system there is no going back.

The good news is at this point it does look fixable. We're just waiting for some additional testing, and hope to know by Monday or Tuesday what and how to fix it. Angela will likely always need a low protein diet, and the swallowing problem is unrelated so will still need the gtube. We just want this all fixed so we can get our girl home and back to living!

I'm so glad we got her through this crisis. She was one very sick young lady over the past week ammonia levels in the high 300's are a really bad thing. Most people would have been in a coma but over the last 19 years her body had gotten used to living with really high levels.

Multiple problems

We know more than we did this morning, but we don't know enough.

First, Angela's clotting rate is a bit slow. Clotting factors (like puzzle pieces) are made in the liver. Vitamin K increases the liver's ability to produce clotting factors. Angela is being put on 3 days of Vitamin K while they work to figure out the other problems.

The MRI shows *multiple vascular abnormalities*. Some are large. Some are small. Blood flow within the liver is going this way and that. There are also several lesions. There is also regional edema, including in the duodenum, and the head of the pancreas is enlarged due to this edema.

What we do know: It is more and more clear the problem lies within Angela's liver and this is NOT a Urea Cycle Disease. Instead Angela has a liver that is a mess.

The Intensivist is deferring to the Heptologist (Liver Specialist) who will be in to see us tomorrow. In the meantime Angela is miserable and in pain.

Urgent prayers

Overnight it was decided to stop Angela's ammunol and see what ...I don'teven know what. At midnight her ammonia level was at 122. When I come into the PICU her room is furthest away from the door. This morning when I entered the unit I could hear her screaming. She is in a lot of pain and her ammonia is at 375. They're can't give her anything but motrin because they need to be able to monitor her mental status. Right now she is not very coherent but awake. She NEEDS to have this MRI done but her system is not stable enough for anesthesia. We expect her to fall into a coma again soon if we can't get her ammonia level down.

She was started on large doses of Lactulose to help clear the ammonia from her body. This drug causes its own problems as it does its job. 

Back up plans

Angela is still in the PICU and will hopefully be having her tests tomorrow under general anesthesia, as long as she is stable enough. "Stable" is a relative term right now, because we can't get her stable enough to leave the PICU right now.

As badly as I need to be at the hospital tomorrow, all the other kids are out of school tomorrow and Dean cannot miss any more work. (neither can I, but......yeah....there isn't any other option) If any of our back-up people are available to watch the other 5 kids for the day, please contact me or Dean by cell.

Edited to add: Lots of people have volunteered to help out with the kids. We are greatly appreciative of those offers. Sadly it is NOT possible for someone who has never watched our kids, particularly the most difficult of the A's, to walk in here and leave in one piece. And I'm not even exaggerating.