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. 

Building your medical vocabulary

If there is one thing Angela has done for me throughout her 19 1/2 years on this earth, its to build my understanding of medical terminology and anatomy.

Angela had an ultrasound yesterday, and another more detailed study today. We're looking for something very specific, and we're hoping we find it. We need to find it.

Our vocabulary lesson for today includes the following term:

Congenital Intrahepatic Portosystemic Venous Shunt

Congenital = present at birth

Intra - inside

hepatic = liver

Porto = point of entry

Venous = vessel that carries blood

shunt = bypass or diversion

On ultrasound, it appears that Angela has an obstruction of blood flow within her liver. Liver blood flow should be smooth, and pulse with the heartbeat. Angela's liver blood flow is turbulent, meaning it is being blocked or hindered by something. The body's blood supply needs to go through the liver to be filtered of waste products, including ammonia. When the blood flow is "shunted" (diverted) in any way, that blood is not getting filtered. That would cause her ammonia levels to spike and fall continuously.

Orders have been written for her to have an MRI under anesthesia (because it will take a long time and she can't lay still that long). We're hoping it can be done tomorrow but if she isn't stable enough we'll have to wait until Monday.

Please pray this is it. There are a few different ways to address a liver shunt, some more invasive than others, and they all depend upon where, how big or small. If we can solve the problem it is curative. That is best case scenario and its what we NEED to happen.

Angela needs this. 

Afraid to get our hopes up

The team of Angela's metabolic geneticists have a theory about why she keeps having bouts of hyperammonemia, and why even after treatment she quickly spikes and needs another treatment. Get this...

If their theory is correct, it could be

curative.

CURATIVE.

We are afraid to get our hopes up. But prayers. Oh please pray their theory is correct! It would involve a major and invasive procedure, but it is far better than the way Angela is surviving now! 

Gtube Care Kids

PLEASE SHARE THIS POST!

When Angela was little and had her first g-tube, I made special gtube pads for her to help keep her site clean and infection free. I don't have time for that anymore, but thanks to Amazon and Etsy, I have found a seller who makes GREAT pads! Cutie Button Pads

These pads come in a variety of fabric patterns appropriate for little kids as well as teens and adults. Angela LOVES to pick out her clean pads, and I love I can just toss the used ones in the washer!

 

Angela in the PICU is feeling well enough to pick out her clean Cutie Button Pad

She found her choice for the day. The soiled one will be tossed in the
 washer to be used again another day.

Now Angela has a new gtube, and spends a lot of time in the hospital. Her nurses are always surprised to find a cute pad on her stoma site, and they love the patterns! They are always impressed at the condition of Angela's stoma, with none of the irritation that comes from the medical gauze pads.

So I am starting a new project. I would love to donate a set of new pads kids who are getting a new gtube, and their parents who are learning to care for the stoma for the first time. I have created an Amazon Wish List with pads from Cutie Button Pads, the seller who makes the ones I buy for Angela. I have NO connection to this seller other than I like her button pads and they're the best I have found! If you would like to purchase a set of pads from the list, they will be shipped directly to me. I think you can include a card that says who contributed the pads. Thanks for helping care for these kids!

Random Hospital Things

Last week when Angela was here at the hospital, some darling person was going around popping out the elevator call buttons. The first time I found one I went to grab someone to see how to get the elevator to come up. He was all "oh, go ahead and put your finger in there and push it. It works." And I was all "Umm I am not putting my finger in there with wires and stuff." and so he did it. The next morning when I came back he had taped up this sign. Go ahead, put your finger in there!

There are two cafeterias here. One is part of the Children's hospital but the hours are strange and they never seem to be open when I am here. And yet, I'm here a lot! Most of the time I walk down to the joining adult hospital because they have a cafeteria that seems to be open 90% of the time. Anyway, the woman who seems to be in charge at the Children's cafeteria needs to find some humor in her life. Seriously, she is the most crabby person I've ever met here at the hospital. I'm one of those annoying people who have to crack a joke about the dumbest things and I have never been able to get a smile out of her. One day a few weeks ago I even said, "Smile! It is a beautiful day outside (and it was!) and we're alive and breathing!" and she just started at me. I have even tried signing to her in case she has hearing loss and can't hear me. Nothing. No response. (I have heard her talk to people, by the way) I have now made it my mission to make her smile. I think today I will bring her some flowers.

Years ago, before HIPPA became the law of the land, it was fun when visiting a hospital to go up to the maternity floor and look at all the new babies in the window. Do you remember doing that? I wish that was still allowed. This hospital has a new Mother and Baby center that just opened. Its fun to see the moms coming in who are in obvious labor and know in a few hours there will be a new baby in their arms. Its energizing to me. I also get to see lots of people leaving for home with their new babies, and the first-time parents with a TON of stuff to bring home with them. I've also seen people who have obviously been here a very long time, probably in the NICU, with their babies and have months worth of things they've collected in their hospital rooms. I'm so excited for them going home, even though I know its probably both exciting and scary for them, all at the same time. The entrance to the Mother and Baby center is right next to the elevators up to to Angela's room. I wish I could find a way to sneak in there and see the babies!

Sitting in the PICU with Angela makes me so aware of how lucky we are. We have amazing healthcare available to us, something that is not the case world-wide, or even in our own back yard, sometimes. There are people here who's kids are so much sicker than Angela. I mean, we made her sick by giving her drugs to clean out her blood. It is a mostly-predictable process. (other than the fact she didn't respond!) But there are kids here who are several hours away from home, their parent or caregivers living at the hospital with them. We live just a few minutes away. They are recovering from major trauma, sudden acute illness, or sometimes and end-of-life process. Last night there was a very large family sitting out in the family waiting area which is next to the elevators. Many of them in tears as they try to process what is happening to their loved one. I said a prayer for them all as

If you're not on Facebook, you missed the fact I messed up my back sleeping on the pull-out bed thingy in Angela's room. After a trip to the doctor I'm feeling a bit better. Still, the hospital needs a full-time massage staff. Parents go through a lot with their kids here, and the stress takes a toll on their bodies. A massage every couple of days would be a good thing!

And someone to cut hair! There are so many parents here who can't leave. I have met a few in the last few months who have been living here with their kids, who are feeling desperate for a haircut, but don't feel comfortable leaving the hospital.

All you g-tube parents reading: When Angela got her gtube I started buying gtube pads made by various sellers on Amazon and Etsy. I found one I LOVE!!!

Angela's nurses are always commenting on the condition of Angela's stoma, and how there is never any granular tissue or skin breakdown. The pads I order come in a variety of fabric colors and patterns, for both boys and girls, babies and older kids. I would love to be able to donate a set to new gtube parents who are just learning to care for their child's stoma site. I created a wish list on Amazon if you would like to help me out with this project. You can find the wish list here.

A HUGE shout out to the groups from the Ronald McDonald house who love to feed the families!!! Ok, most of the stuff is not on my approved food list, but its still a great way to take care of people who are really stressed out and tend to eat junk food while caring for their sick kids. Personally, I have not eaten fast food in a month, which is probably a record for me.

Why has the second floor parking pay kiosk thinging ALWAYS broken? I have never yet seen it working. It makes it confusing for people who don't know their way around here to pay for parking and then find their cars.

Shhhhh I have learned how to park down here for free.

Angela is waking up. Time for me to go back to mom duties!!! 

PICU Update

A quick update for those friends and family who aren't on Facebook.

Yesterday morning Angela's ammonia level was coming down, but only very slowly. Then around noon they started spiking again, and by 1:00 she was sleepy and struggling to stay awake. (she does this by talking NON stop! But if she stops her eyes are immediately closed and she's out.) Finally around 3:00 we found her levels to be in the 140's (goal is under 50) Unfortunately that means she has to have another 24 hours of the Ammunol, with the addition of Arginine. Overnight her blood sugar is all over the place so she is off and on Insuline, plus is on lipids and other stuff. I'm looking at her IV stand now and there are 7 bags hanging. Thankfully at the moment she is sound asleep. I wish her whole night had been like that.

She'll be on these two drugs until around 7:00 pm tonight (Weds Jan. 20) and we'll see where she's at. I can't even imagine if they had to do a 3rd round of the Ammunol. It would truly be terrible.

A bit if a funny. Sometime in the night Angela's bed discovered it has a mind of its own. Suddenly the head would raise, or the foot, or the whole bed would lift to the highest position, then back down to the lowest position. Its not Angela doing it. She likes her bed in a certain position and does NOT like it to be messed with. Girlfriend will be getting a new bed today.

The Learning Curve

When Angela was diagnosed with a Urea Cycle Disorder, we were told exactly how dangerous it is, what the warning signs are, and to get her in for treatment immediately, etc. Scared us to death, of course, but we got over it and moved on. We know that Angela is at risk of having another episode if she gets so much as a hint of a cold or ear infection, much less a serious infection such as another round of aspiration pneumonia. Or, you know...sneaks a cheese stick from the fridge.

On Monday the mail brought Angela's emergency protocol. A 12 page document put together by her metabolic geneticist. This protocol stays with us, and with Angela at all times. It is our ticket to immediate treatment should we find her in crisis again. UCD's are very difficult to diagnose, and clearly for 19 years nobody ever thought to test Angela for such a thing. Walking into an ER with a chatty, charismatic young lady doesn't help when the mom is saying "No really. She is very sick."

When we received Angela's protocol in the mail, I read it to Dean. My stomach went into knots of anxiety. To see the words in print was very sobering, indeed.

"This acute illness protocol is a guideline for healthcare professionals treating (Angela), a patient known to have hyperammonemia related to suspected Urea Cycle Disorder....
Metabolic crises  in individuals with urea cycle disorders are complex medical emergencies amd must be treated as such to avoid death or serious brain injury....
Angela can rapidly decompensate even if she looks stable or only presents with an apparent minor illness. Therefore, she needs higher acuity triage in the emergency room and needs to be immediately seen by a physician. Please discuss her clinical findings with (insert physician group name) on call ASAP by calling the number listed below."

Gulp

The protocol goes on to list what specific tests to do upon admission, EVEN if Angela appears "fine" to the ER doctor examining her. It lists, step by step, which order meds are to be given, and under what circumstances the various meds should be given. It is all very clear.

So Tuesday, January 5th, I brought a copy of the protocol to Angela's school so it is available for the staff and first responders should they need to be called, or anyone else who may come into contact with her in a medical capacity. When I arrived school staff told me she had complained of being cold, so she added a sweatshirt. She also had a bit of a headache. And at one point in the morning she just decided to leave and walked out the door. Hmmm The "Old Angela", before being diagnosed with OTC, would have done this on a daily basis, but the "new Angela" would never act like that. I decided to bring her into the ER and have her ammonia levels checked. "Just getting checked out" resulted in a one week hospital stay. It seems her meds and feeding schedule needed some tweaking. This is a bit of a learning process for all of us because every patient with OTC responds a bit differently, in addition to having the endochrine system of a person with Down syndrome. And, I know you won't be surprised when I say Angela isn't quite following the rules. She had her blood drawn every 8 hours for seven days (and she kept a running tally of pokes, announcing to the lab tech what number they're on. She is getting tough to get blood on.)

Angela's goal ammonia level is below 50 and we struggled to get her there. She was finally discharged at 63. She was a bit irritable but otherwise ok. She went back to school on January 12 but it was a rough week. On Friday she was hitting staff and just not herself at all. She did go to a dance on Friday night, with both Dean and I as chaperones so we could keep a close eye on her while also making sure she didn't eat anything there. Saturday she was a BEAR, and Sunday morning not much better. But, when I gave her morning food on Sunday (yesterday) I notice she had a bit of a stuffy nose and her eye was kind of gunky. Very minor cold symptoms that under normal circumstances I would barely bat an eyelash at. But this is Angela, and even a simple cold can land her in the ICU.

We have three different formula recipes to follow for Angela's feedings. 1) every day "she's doing great" 2) 5 grams of protien, for days there are special events and she is going to be eating more than normal orally. It allows her to have 5 grams of protein by mouth and 3) sick days recipe. If she starts showing signs of illness - even these minor cold symptoms - we switch her to this one. So on Sunday at her 11:00 am feeding I switched to her sick day recipe, but knowing it was already too late. She was headed downhill and was acting sleepy. At about 1:00 I called her metabolic Dr and we agreed it was time to go in for levels. We're having record cold here in MN (It was -18 here ) so nobody wants to go out unless absolutely necessary!

I wasn't surprised when we got her levels back to find she was at 95. If she hits 100 she has to go back on Ammunol, which is that super expensive ($100K per dose!) drug to clean her blood. She was moved up to a room pretty quickly and we took labs again, which showed her at 52. WAH???? Everyone was confused and scratching their heads. One of those levels was wrong. Because of the way ammonia levels are processed, we know the high one would be incorrect. We drew again this morning (January 18) to find her at 112. Oh, but the doctors felt that was incorrect so they drew it again and she was 123. She is clearly symptomatic (she can barely keep her eyes open to carry a conversation and she looks like she's been hit by a mack truck).

So here we are. She has just been moved to the PICU. We're getting ready to put a PICC line in then the Ammunol will be started. This drug makes her very sick. She will sleep through much of the first 12 hours. She won't be able to walk, or talk, and is just a very very sick person. She'll be on/off insuline and a couple other drugs I can't remember.

Last night the metabolics doctor said he wanted her to get a port because she is getting really hard to get blood from. She is just poked too much and there are too many errors. The port will change all that. Unfortunately she isn't stable enough right now to have a port put in, so PICC line it is.

So to clarify, if you have lost track, of the last 10 week Angela as spent a combined total of 6 weeks in the hospital.  Now that she is starting Ammunol, that will run for 24 hours, then it takes about 12-18 hours for her to stabilize enough to be moved out of PICU. After that is several days to get her back to baseline before she can be discharged.

This is it. This is Angela's new life, and our new life. Our new normal. We need to find a way to live with this new normal, and we need to find a new routine that works with dropping everything so that Dean or I can be at the hospital while the other is with home with the rest of the kids.

And just as aside, if the PICU doctor says "ummm" another time I could lose it.

Like Rabbits

I'm so thankful my kids will eat anything set before them.  Oh, but don't think it has always been this way. Each of the kids we adopted came home with his or her own eating or feeding issues. Axel was the only one who knew how to chew or drink from an open cup. Even Amos, who had been in out of the institution for 2 1/2 years before coming here in May didn't yet know how to chew. Some wouldn't eat meat because it was a new texture to them. Raw vegetables? They're a lot of work to chew.

So how is it I have such good eaters now and have gotten into the horrible habit of giving them junk for snacks after school? Somewhere along the way it happened and it is time to stop. I love this video. Amos and Audrey eat up these veggies like little rabbits.  (and those who are watching from the therapy perspective, you'll notice a MUCH improved mess factor for Audrey. That is because Mom instituted "Dinner time bootcamp" again, only I raised the bar.)

Oh Dear

Today was kickoff day with my new workout team. Yes this is both an individual and team process. The group of 40+ women is broken down into three squads, and a couple times per month we do squad workouts  (think biggest loser team competition). I love this part because it will keep me accountable to them.

Today I mostly had my ass handed to me. Oh Dear Lord I can barely move tonight.

First we had introductions. Most of the women are existing members, then there are 8 or so of us who are new and doing the 5 month challenge. In the entire group I am at the more...ahem...older or "mature" end of the group. Even so, at 48 there are several women who older and the rest have a wide range of ages. Let me tell you though, the women who are older than me are in amazing shape and will take you out!!! Today during kickboxing I kept reminding myself that one day they were new, out of shape, and not bouncing around the gym floor. One day they had cement in their shoes the same as me!

The very first exercise, which was done with partners, reminded me that I hate working out. Here is the coversation I had in my head:

What the hell? It is not truly possible to get from the floor to standing like that at my age.

(Glancing at another memeber.) Hmm, she is older than me and doing it.

Oh dear Lord are we almost done? What? Not even 1/4 of the way.

I cant keep coming here. They will kill me. Oh but crap I already paid for it and Dean would kill me if I turned tail and ran.

She has amazing arms! Remember a few years ago my arms looked like that? They will look like that again.

Dang I just peed a little. I wonder who else is peeing here?

My ass is bigger than this matt. Wonder how long it will take me to fit on it?

GAWD I want a Mountain Dew!!

So when that was done we started the kickboxing class. Pretty sure I am missing some joints in my back since I can't move that way. But I will keep doing it. Because I must.

Then we newbies had a nutrution class. Oh man, so much to change. Never off duty, I did discover some things that will be helpful to Angela.

I came home and was afraid to sit down for fear I wouldn't be able to get up again. Tomorrow is going to be rough, and Monday I'll be at it again. I can't wait to get past this phase that is so painful.

Thinking about it

When I was diagnosed with breast cancer, and contemplating having a single or double mastectomy, I spent hours upon hours scouring the internet for before, during and after pictures. What was I in for? What could I expect for an outcome? What was the rate of failure? What are the complications?

Those pictures were hard to come by unless I wanted to join private forums or groups. I watched videos of women who proudly displayed their scars. Some as a form of awareness, some as a badge of honor, most as a combination of the two.

I need you to know that underneath these clothes I don't look "normal". If you're on my Facebook page I'm sure you remember the fun discussion about ordering nipples online. (I chose not to have reconstructed nipples.) I chose many things along the way, and I don't regret one single choice. Could I still develop breast cancer? Yes, I could. At the moment I'm not taking my aromatase inhibitors because they make me so sick. I hate them. I can't live life on them. But, not taking them puts my risk of recurrence quite high. I just have a really difficult time dealing with the fact I'll be miserable for the next eight years on those drugs. Anyway, a friend of mine saw my pictures privately and was shocked at the process. She had no idea. Even though these surgeries were brutal (some of my wounds made Dean weak in the knees) I walked around as if I was all fine. I had to. I have a family to care for.

So I'm thinking about it. I'm thinking about posting a series of pictures showing the process, starting with my first surgery. I'm still debating. Its  very personal decision to put myself out there like that. Anyway, its just something I'm thinking about doing, just don't be stunned to show up here one day and that's what you find.