zebras in parks

Nobody seems to know what makes a teenager sleep for days on end, getting deeper and deeper until she is not responsive. Sleep through an ambulance ride. Sleep through getting an IV placed. Sleep through hetting a catheter placed. Sleep through the eight different doctors in the room, poking and prodding.  The first thing asked is always the most obvious- could she have gotten into anything? Were meds dosed correctly? Blood test after blood test rules out all those things. Everything is normal. Carbon monoxide poisoning at home? New carbon monoxide detectors rule out poisoning from invisible gas. The sleep lab gets involved to look for obscure things like sleep disorders. More bloodwork to investigate further. Search for the zebras in central park,right? It has to be a reaction to something based on the timing of when she starts to wake up. Tonight we stopped everything. No food. No meds. IV fluids only. Tomorrow we will start over. One change at a time. Find the reaction. Sounds so simple. 

If you could

Angela is in the hospital again. Praying we get this figured out. 

Random stuff

Playing catch up here a bit. You know, since I was awol from blogger! So here are some random pics and videos from the last few...ah...months.

We're working hard with Audrey and Amos on table manners. We have a long way to go, but we're getting there. My main goal with Audrey right now is to keep her hands out of her food. She's coming along! (for those who know where Amos was when he came to us a few months ago, he has bypassed Audrey in this area. His hands are out of his food AND his face is significantly cleaner. YAY Amos!) 

Abel has a new favorite job! 

This morning at 8:30 am. he turned 

on the vacuum ....again.

Axel loves to organize various areas of the house.

Everyone needs a teenager like Axel! He also loves

to clean the kitchen, fold towels, feed the dogs 

and take out the garbage. For real! 

Axel comforting Angela shortly after being discharged

after she had her feeding tube put in. He felt so bad for her. 

About 1/2 our house is still in storage and I refuse to 

move it all back in since we're going to put

our house back on the market in the spring. 

However, I finally bit the bullet and pulled 

out all the winter gear. Asher was excited to find his favorite hat. 

We did actually make it to a pumpkin patch! 

It was an unseasonably hot day, which isn't so great

for Angela. We were only there about 30 minutes 

before she faded and we had to leave. We did get pumpkins though!

This picture is from the top of the observation tower, 

which is about 25 feet high. Can you guess

which of the kids weren't happy to be up there? 

Hint: They're hanging on tight! 

Evidence that Audrey got into the kitchen. 

This is why we're working hard on feeding skills. (and not stealing food, as this

was contraband chocolate!) She's still cute as can be!

Back in August, walking the trails near our house. 

We bought this cheap pool late in the season and it never did warm up. 

The three older boys didn't care. They still lived in the ice cold water!

Axel and Angela get to stay up later than everyone else

on the weekends. One night Axel went to bed and hollered up 

the stairs. "Mom. Dad. Abel no!" In other words, Abel 

did something. We walked in to find Abel's pillow had

"somehow" exploded all over the room. Hmmm

Could it be because of the bite marks in the corner seam?

What you can't see in the picture is that Axel's bed is

covered too. It took a long time to clean up those feathers!

August 6th Angela started her first day of 

her Transition Training program. She was SO excited! 

Asher has started taking selfies!

The last couple of years winter has been tough for me. I normally

spend all my time outside in the summer months, soaking up

the sun's warmth and re-energizing myself. The year I needed the sun the most I 

found out I had cancer. How I craved the sun even more! Only when summer 

finally rolled around chemo happened, which means staying out if the sun and heat. 

When winter rolled aroundagain it dragged on forever and ever and ever. 

Surgery after surgery, infections, hospitalizations and just B.L.A.H!!! 

With the growth of our family and a few other needs, 

Dean and I decided it was time for a fresh start 

in a new house. Since showings require we vacate the house, we 

spent the summer in the van with 6 kids, 2 dogs 

and sometimes the cat! We did almost nothing fun because we always 

had pets with us. And when we didn't have showings we didn't feel like

leaving the house. I feel like it was the summer that never was. 

This last picture, taken in July, is a reminder

for myself that summer will come again. The summer of 2016? 

WE WILL ENJOY IT JUST BECAUSE IT IS! 

Fall Clean Up

Its a beautiful weekend here in Minnesota. Well, in our part anyway. I hear my parents are getting snow way up north. I personally dread winter so that white nastiness can keep its distance as far as I'm concerned!

We spent a lot of time in the van this past summer, killing time while strangers walked through our house. I cleaned out the van whenever I could, but it has really needed a thorough cleaning. I got new tires on my van today and was a bit embarrassed that someone was doing to actually see inside! Ugh! Abel and Axel love a job so I put them to work.

Asher gave it a try too. 

But he mostly just wanted to climb trees. This boy, he is 

all about climbing and being up high as he can possibly get! 

Can you find some of the other kids in the background?
Audrey and Amos are on the trampoline and Abel is on the swing. 

What do we do with our rotting jack o'lanterns? The deer LOVE them so

we put them at the tree line for a snack as they pass through the yard. 

Axel decided to rake the back patio. As you can see, the little

kids were a huge help said.nobody.ever. 

Amos was the most help of all. LOL

A funny story: 

I wear orthotics on my feet and it can be difficult to find 

shoes that fit right with them. About a month ago I 

wrecked one of my tennis shoes. I was in a hurry to

get out the door and saw this pair of shoes. I thought

they were Deans. They're about 1/2 size too

big for me, which is what I need for a good fit 

with the orthotics. They are the most comfortable 

shoes I've owned since getting them. Only I was mistaken!

They are Axel's old shoes that he has outgrown. 

Yes, "little" Axel's feet are now about 2 sizes bigger than mine. 

Even more shocking? Abel's feet are even bigger! 

Axel is now wearing a size 8 mens, and Abel is wearing 8 1/2. 

(Yes, Angela is missing from the pictures. She came out to help with the van cleaning up, climbed into the back seat and promptly fell asleep. She went back in the house after a few minutes. I wish I could get her to sit outside with us. She just avoids outdoors lately. We're working on it. ;-) 

She's HOME!

As I type this, Angela is sleeping peacefully on the couch next to me. She wasn't super thrilled about coming home. You know, at the hospital she has complete reign of the TV, and the nurses love coming in to visit with because she's so adorable. Yeah, I am immune to the cute. Totally over it. ;-)

 Admittedly, they don't often have patients on the PICU who are quite so active and chatty, so I guess Angela was a welcome change. Errr maybe not! She was awake all last night, finally falling asleep around 6:00 a.m. She slept for two hours and was wide awake until 8:30 tonight when she finally crashed. When I arrived at 11:00 am the PICU doctor said it was a "rough night" because Angela was quite wound up. Hmm maybe because she had essentially slept for the last month, and then there was that deep coma-sleep for her first 24 hours in the hospital. She was feeling the best she had in ages! Why would she want to sleep? LOL

The ultimate conclusion is Angela's gtube has changed the way she's absorbing meds, probably because we're dissolving them before putting them through the tube. Slow release meds are being absorbed immediately. Since they got to see first-hand what Angela is like when she's NOT sleeping, the doctors didn't like the idea of her being completely off one of her meds so wanted to start it back up, but at a half dose. I reminded them she's been off all but her seizure meds since Saturday, but they still wanted her back on the one medication. 

I'm just thrilled that our chipper Angela is back. Of course, she is anxious to be back at her transition program but they're on a break through next week. It might be a looooong week keeping her busy! 

ICU Update 3

Well, this will be my last update from the Intensive Care Unit! Angela was actually moved to the medical/surgical floor around dinner time, but they don't have a bed available so she is still in ICU, but her level of care has been significantly reduced!

Angela is fully awake! She has been awake and talking, talking, talking  all day long. She has been more awake today than she has been since October 6th.

This morning we were told her EEG showed slightly decreased activity on the right side so they ordered an MRI+MRA. Those tests showed no change from her last scans done in 2007. The doctors had a pow-wow in her room, and the conclusion is she just cannot be on all her meds anymore. Her medication blood levels were within normal ranges but for whatever reason that seems to high for her. This is most likely due to her "sustained release" medications no longer being slow to release when they are crushed and put directly into her stomach via gtube. As I mentioned in a previous post, the neurologist said he has seen this a couple of times over the years, but the ICU staff had never seen it. Nobody has any ideas other than the medications. When asked about the left-sided weakness this morning I was reminded that she has had hemiparesis since she was an infant. Normally the only time its noticeable is when she's swimming. She isn't able to kick her left leg very well, and her left arm doesn't have full range of motion. But this event left her very weak, making that left-sided weakness more apparent.

I am home to help Dean tonight. Now that Angela is awake and mostly herself (she's not walking without help yet) she and I were both comfortable with me going home. She will likely be discharged tomorrow.

Thank you so much everyone for your prayers and support. They mean a lot to us as we navigate this very scary event.

Now, I shall go change out of the pajamas I've been wearing since Saturday evening! 

Update 2

Dr just came in and said neuro says her EEG shows slower activity on the right side of brain. This is the side at risk of stroke so she'll have an MRI done to check for a small stroke that couldnt be seen on CT when she was admitted. It could also be she had seizure on that side. We'll see what the tests show. 

ICU morning update

Last night Angela had several moments of being able to answer simple questions. Things like "Do you want a blanket?" Or saying hi back to someone. All with her eyes closed.

About midnight I went to a parent sleeping room. I prayed for a miracle overnight. That I would come back to find my girl awake. The nurse told me that at about 6:00 this morning she very clearly said, (eyes still closed) "Its Monday. I need to brush my teeth. The bus is coming." The nursing staff frequently tells her that she is in the hospital, that mom is here, what day it is, etc to help her reorient every time she wakes up. The nurse went to get her a tooth brush and when she came back Angela was out again.

Meanwhile at home Dean is managing with the other kids. He truly is the most amazing dad. The kids woke him up early this morning (gotta love daylight savings time!) So he had everyone up, dressed and breakfast done by 6:45. All that was left was to brush teeth and comb hair, then wait for those beloved buses to come!

Praying we continue to see her wake up. And that she is able to start opening her eyes. She's in there somewhere, trying hard to get to the surface.

2014 was.the year of cancer and Audrey. What label should we give to 2015?

Intensive care

*note this post was written on my phone over the course of 8 hours. Lots of typose and time lapses I'm sure.
....................

I'm sitting in the intensive care unit with Angela who is unresponsive. I'm a bit stunned we have come to this point.

I recently said to someone that Angela has not been right since the day of her surgery on October 6th. She was her usual bubbly self as she walked herself into the OR. But something happened that day, and it now seems as if its more serious than any of us were aware.

She has been so off. So lethargic. Struggling to stay awake more than a couple of hours. Falling asleep everywhere and generally jusy struggling to stay awake. Ambulances have been called to school when they couldn't get a blood pressure on her, and just yesterday the blod pressure monitor I ordered arrived. I was able to get accurate readings on everyone in the house, but no reading at all on Angela.  Just a week ago I told someone I feel like she's slipping away from us. Like we're somehow losing her. Like she was swimming underwater and couldn't get to the surface.

We went trick or treating. Angela participated but still in her fog with a few moments here or there when we saw flashes of the girl we know so well. We even let her have a few bites of Ashers birthday cake (mostly frosting that would disolve) but she was too sleepy to enjoy even that.

She had her evening food and about 10:00 I moved her to her bed. But she was sooooo out of it. I told Dean if she was still like this in the morning I was taking her into the ER. Remember, she went trick or treating with us just a few hours before.

At 3:00 am I rolled over in bed and heard very clearly in my ear, "go check on her." I sleep very soundly from my own cancer related drugs. I rolled over and thought I will. In just a few.minutes I will. But again, louder, "No, check on her NOW."

I  went downstairs to her room.her mattress has been on the floor because we packed up all the beds thinking our house was sold. She was on the other side of the room, face in the carpet, arms and legs all contorted the wrong way. I couldn't wake her up! I ran to get Dean to call 911. She was completely unresponsive, breathing but with a horrible croupy sounding cough (later I would be told the cough was because her airway was too relaxed.)

The paramedics came and still no response. It took a bit but they finally loaded her into the ambulance. She became VERY combative but could not follow directions. I followed behind in my van. About 1 mile from home they turned on lights and sirens and took off. Another mile and they pulled over. I was trying not to freak out but my sister was on the phone with me. Finally one of the crew came back to tell me they were trying to get an airway on her and to stay in my car. Then another ambulance came, and that crew ran from thier rig to the ine Angela was in. It seemed like there were 20 people in there! Finally they came back again, saying they weren't going to do an airway. That they were going with lights and sirens with both crews and to just meet them at the hospital.

She was so very combative at the hospital. Thrashing and rolling but unresponsive to anything but deep pain stimuli. Nobody could figure out what was wrong. All i could think of was omg this is the big stroke we were warned about years ago, when she got her make a wish. She had a CT that thankfully ruled that out.

She was moved to ICU. A million labs were drawn including her medication levels, looking for infections, organ functuon. Really anything they could think of. Finally in the afternoon the neurologist came in he said they were all grasping at straws be he felt this was all neurologocal and not organ related. One thing he has seen, only a very few times, is kids who were switched to gtube, and went from oral meds to gtubed meds. Oral meds you dont get 100% of the dose in your blood stream, plus what goes to the stoach is disolved and subsequently absorbed at different rates. But when you switch to gtube you disolve the meds first so 100% of the dose goes to the blood stream. Angela has always been hypersensitive to meds, so it could be for the last month we have inadvertently been over dosing her on the meds! That would make sense that my saying she has been off since she woke up from surgery because she got her meds right on schedule that night, but disolved in the gtube!

She has been hooked up to the EEG all day to rule out seizure activity. It has shown that she has been in the equivalent of a drug induced coma and is just sleeping. The deepest sleep ever. Unresponsive. At about 6:30 tonight she 1/2 opened her eyes. Over the course of an hour she was able to wiggle her toes on command, but not open her eyes. She could not squeeze my hand but shook her head no when asked if she wanted a blanket. I put the ohone to her ear so she could hear Dean talking to her and she managed to mumble "I got a IV Dad." And was back to sleeping.

The doctors here are stumped. The neurologist said he has seen this once or twice before in his years of practice. It is highly unusual, but if you know Angela, that is her norm, to not follow the rules. The nurses keep saying "this is so odd. This is how you give meds to a gtubed person!"

So that is where we are now. She is in/out of consciousness, with "conscious" being a very altered state. We hope to see her continuing to wake up iver the next 24 hours, then can evaluate what if any damage has been done

Happy Birthday Asher!

It is Halloween, but it is more than that to us.

Eleven years ago, on the other side of the world in Kragujevac, Serbia, a baby boy was born. His name was Lazar.

When Angela was born I was immediately surrounded by a community of parents who already knew how raising their children with Down syndrome was a positive thing in their lives. I lived in a community where every possible support service was already in place. I didn't have to fight for Angela for anything, it was all handed to her.

Baby Lazar's parents were not so lucky. Eleven years ago Serbia had next to nothing in the way of services, and society frowned upon those who chose to bring their "defective" children into their homes and neighborhoods. I can only imagine the anguish little Lazar's parents felt as they made the best decision they could: to place him into institutional care.

Four years ago today we didn't know it was his birthday as we hit "send" on an email to the Serbian adoption authorities. We said yes to a tiny 7 year old boy who's face we had never seen. We said yes based on the very diagnosis that caused him to be rejected by the society of his birth. Just a few weeks later we brought him home. We named him Asher.

Genesis 30:13

Then Leah said, “How happy I am! The women will call me happy.” So she named him Asher.

Today we sang Happy Birthday to our boy. Our Asher.  I hope the mother who carried him in her belly for nine months understands how much we love this boy. I hope the father who was so excited to meet his first son knows how much thankful we are for this gift we kiss each morning. The fact our blessing means sadness to another family is not lost to us, and there is not one day that goes by when I don't think of them.

Happy Birthday Asher Lazar! We're so happy you're part of our family!
(please excuse my terrible singing!)

A whirlwind!

Angela has not been doing so well since her surgery. A variety of problems, most of them related to chronic hypotension (low blood pressure) that we can't seem to find a cause for. We've had an ambulance at school for her twice, and lots of "Hmm, do we bring her in or not?" I did bring her in a couple weeks ago because she was just generally off and very lethargic. Thankfully they were quickly able to find the problem. Though it wasn't fun to remedy, it only took a couple of hours and we returned home by midnight that night. We're hoping to get all this stuff sorted out so we can get our bubbly girl back. She is still really enjoying her current program and really misses it the days she can't go. 

On the 22nd I took a much needed vacation with a group of moms who are also adoptive parents, some to kids who are very difficult to parent like a couple of our kids. We talked about parenting a bit, vented some about our kids as all parents will done on occasion, but mostly just enjoyed the break! There was sleeping in, naps and some time in the sun to replenish the Vitamin D stores! 

Abel had surgery on his knees/ankles in September. He had a recheck right before my trip and is doing well. We'll repeat xrays in a couple of months to see if the hardware is ready to come out, and whether or not he's ready for the soft-tissue repair. We know for sure, without this surgery he was destined for vary serious knee, hip and ankle problems in the very near future. 

Audrey has an upcoming recheck of her hardware. She is growing very slowly so it is taking a bit longer. At her check up we'll determine if the hardware is ready to come out and whether or not she's ready for the soft tissue repair. She is able to completely dislocate her knees, ankles, hips, shoulders, elbows and wrists. We don't think we'll be doing anything to fix her upper extremities but the lower extremities have to be addressed or she will lose mobility. Other than the joint problems, Audrey is extremely healthy, which we are thankful for. Behaviorally she has certainly come out of her shell in the last few months! She is a very busy (and noisy) young lady with a lot to say. We can't wait until we cab understand all that she wants to tell us. 

Asher, he just keeps plugging along. He rarely gets even a sniffle. He is loving school this year and really taking off with his academics. His understanding of English has finally clicked too. His sign vocabulary has grown exponentially which is fun to see! He will turn 11 on 10/31 and he is very excited for his birthday!

Axel is just finishing up his first soccer season on the adaptive high school league. He doesn't really understand the game yet. but he is trying hard to participate. He plays on the JV where all the players are really just learning to understand the game. His reading is taking off, as is his understanding of math. His favorite thing to do is find a magazine, cereal box, or really anything with print and copy the words and sentences into a notebook. We have LOTS of papers around here that he has autographed.

Amos continues to progress in our family. He has learned so much in the 6 months he's been here. He came to us refusing to walk much and unable to really do anything for himself. Now he feeds himself, dresses himself, uses the bathroom, gets his shoes/jacket/backpack on, gets himself into the van and his car seat straps on. All things he should be able to do, but didn't when he came. Every day we increase our expectations to the next step so that he can become more independent. 

As for me, I have just one surgery left. On Nov. 6th I will have my tissue expanders removed and my implants placed. That is the very last step in my reconstruction process and I am eager to have it behind me! 

Our lives are centered around food

Physically Angela is doing as well as can be expected. Her system is adjusting to her new way of eating as well as her new diet. (gluten free, dairy free, casiene free) Never before has it been more apparent to me how much our lives our centered around food. Every major holiday is scheduled around the meal, family reunions we discuss what dish we're bringing to pass, mother/daughter dates a dinner out, or starbucks, or some other food related item always seems to be included. Class parties at school are about pizza or cake.

And then there is Angela.

One week ago tonight was the last time she had anything by mouth. I know the cravings are driving her crazy! Of course she has done better than I thought she would, but we're still putting locks on the kitchen pantry (It started out as a necessity for Audrey but now we have Angela in the mix too) In the beginning when we were discussing the tube we thought maybe she could have a pudding or jello at lunch with her friends or when eating with family. Unfortunately after surgery we were told no because her airway is so inflamed due to severe irritation due to chronic aspiration. Finally, for the first time in months, Angela does not have a horrible cough and we don't want to push her back over that edge.

Today we went to visit her transition program. She has missed a lot of school and really misses her friends. It was great to witness their warm welcome when she entered the room. Then I met with her program case manager and the school nurses to discuss Angela's new plan of care. After a bit Angela joined us and she was already pale and tired looking. Clearly she is not yet ready for full days. She will attend half day Tuesday and Weds, then there is a statewide school break until Monday. I think by then she'll be ready to return to full days.

I cannot tell you how hard it is to tell my child she cannot have french fries or pizza, ice cream or waffles, pop or juice. She has even requested milk and eggs, both things that she dislikes I am praying her cravings go away sooner than later as she forgets what food tastes like.

This past week has been exhausting. Combining all the things affecting my health has wiped me out, and Dean is feeling it too. We started out the school year with Angela in the hospital and have yet to find our morning routine. We hope that next week we can find the calm, peaceful mornings we cherish with our kids. 

This is tough

Angela and I arrived home tonight, just a few minutes after 6:00. Dean and the kids had just sat down to dinner, the aroma of hamburgers and french fries heavy in the air. I was thankful Angela had just had a feeding 30 minutes prior.

Angela went around the table, hugging every one of her little sibs (and Tyler too!), showing each of them her new appendage. I excused myself from the room for a moment, and when I returned she had taken her place at the table. "Mmm French fries. My favorite!"

I gave her a gentle hug. "Remember sweetie? You're not able to eat french fries anymore. Eating with your mouth is making you very sick, so you're eating through your tube instead."

"I can't have a hamburger?"

"No honey. I'm sorry you cannot. Come downstairs with me and we'll find your pajamas."

"No." she said in the saddest voice I have ever heard, then hung her chin on her chest and sobbed great choking sobs as the other kids continued to eat around her.

Oh my sweet child. How do I take away what we all love the most? Something we all find such joy in every single day of our lives?

Tonight she is sleeping in our bed with me. Every time I check in on her, I'm stunned by her color, or lack of. She is white as a sheet with giant dark circles around her eyes. Every time I check in on her, for my own piece of mind, I wait to see her taking breaths.

God, I don't know what you have in store for us. I don't like that my child is being tormented by food she cannot have. But I thank you. I thank you for a formula that should bring her to the healthiest state of her entire life. I thank you for doctors who worked. and are working, to find solutions for our girl. 

Going Home!

Surprises

Surgery was nice and quick. The GI doctor came out and said her stomach and duodenum are severely inflamed and red, which is indicative of celiac disease. This isnt super surprising since she has the genetic markers for celiac, and yet she has no sumptoms. The good thing is the formula she'll be eating via tube is gluten free.

Angela is doing well, refusing to let herself sleep but she is at least resting quietly.

Last supper, plus dessert!

I thought it odd that I didn't get the usual "night before admission" phone call from the children's hospital where Angela would be having her surgery. I was on the phone at 6:00 am to verify what time we were supposed to check in. I'm so glad I did that!

As it turned out, there had been no phone call because Angela wasn't on the surgery schedule. At all Monday or in the near future. To say I was a bit irritated would be a huge understatement.

It was explained to me that after our consultation the surgeon called the adult GI we saw a couple weeks ago to verify which type of surgical approach was written in the orders. The surgeon - who has done all of Angela's surgeries in the past - explained to the adult GI that given Angela's history and the amount of scar tissue she has in her abdomen and stomach, he didn't feel comfortable doing an open procedure. Instead he requested the pediatric GI specialist be in the OR as well and together they do an endoscopic procedure. The scheduling people for the two doctors would need to get that set up, but I was to receive a phone call letting me know that surgery had to be moved to another date. I didn't get that phone call.

I didn't blow a gasket on the phone. I remained calm as Angela sat on the nebulizer in the background treating her current case of aspiration pneumonia. Yes, I remained calm. Ultra calm, ultra cool, and 100% clear that I expected this to be scheduled immediately.

Angela was so excited to be going to the hospital today. It is as exciting to her as a Taylor Swift concert, or a trip to Disney. She wasn't able to eat because of her not-scheduled surgery, and we were hopeful we could get her a last-minute slot. That wasn't to be, so when I finally made her some lunch she was a bit annoyed with me. "I can't eat. I can't eat that. I'm having surgery and I can't eat." Thankfully her hunger won out and eat she did.

This afternoon she wanted me to show her pictures of kids with gtubes and PEGs again (a PEG is a type of tube, usually temporary until the stoma is healed and a button can be put in a few weeks later)

Angela's surgery has been rescheduled for this morning, October 6th. Check in is at 8:00. She went to bed reminding me of the schedule, "You will wake me up, right mom? You will tell me its time to get dressed, but I can't eat. Nope. No breakfast for me. Then we will go to the hospital and I will get my I.V."  I swear, Angela is the only kid I know who likes IVs.

I'll try to post an update in the afternoon after surgery.

The last supper

Its 2:11 a.m. on Sunday morning. Monday morning she'll be admitted to the hospital to have her gtube placed. That means today is her last day to eat.

Can you even imagine????

I have a lot of hopes and prayers right now:

I pray that this is more difficult for us than it is for Angela.

I pray that she has been so uncomfortable eating that she is is *relieved* she doesn't have to anymore.

I pray that she doesn't have the wound problems she did when she was little and had a gtube. (we had one problem after another for four straight years!)

I pray that Angela understands how much we love her and want her healthy.

I pray we don't have any problems getting her insurance to cover the formula we want her on. The usual stuff is just junk!

I pray she doesn't get sick from all the favorite foods I'm going to let her eat today.

I pray that our spunky girl doesn't lose her spunk in this process.

And I thought telling my child I have cancer was hard

During Angela's last stay at the children's hospital, the pulmonologist said to me, "Whatever you do, don't bring her to one of the adult hospitals. She won't get the right level of care. We will continue to treat her here."

Ok. Well to go to the pediatric hospitals we have to have pediatric doctors. I tried to schedule an appointment with her old ped. GI specialist but was told I couldn't because she is 19. She would have to see one of the adult GI specialists there. I hate seeing new doctors with Angela. I hate having to explain 19+ years of medical history, what surgeries were done when and by whom. I just want simple. It is simple when we can go to someone familiar and I already trust. Its hard for me to not put a wall up before we even walk into the office. Thankfully I really liked this new doctor who agreed he should not be Angela's doctor. He was very nice, and very knowlegable. "You know, this is a very rare disease that she is too young to have." 

Yes. Yes I know this.

We discussed going out to Boston and the options that are available to us to treat this monster called CA. Unfortunately, because Angela's swallow study is horrible, he doesn't think she is a candidate for any of them. They wouldn't solve her problem. Unfortunately, this is the progression of CA. At some point the epiglottis stops functioning properly and instead misfires. It spasms with every swallow (You can't see it on xray video swallow. It is only evident using manometry, and we saw it 6 years ago when she had it done in Boston.) We could dilate her cricoid again, but it wouldn't solve the problem of the spasming epiglottis. We could inject the muscle with botox to loosen it, but we risk damaging the epiglottis further. There is no "win" for Angela. There is only lose and more lose. 

The Dr. confirmed what we already knew. Angela can no longer eat or drink anything safely. Never. Ever. Again. I have dealt with a gtube before. Angela had one until she was 4. She doesn't remember it, but I do. The tube itself is not that big of a deal. Its the psychological part of this process that is tough. 

In March of 2014 I sat in the car with Angela and told her I have cancer. At the time I thought it was the most difficult conversation I could have with my child. I was wrong. I didn't know what was ahead of us. This time I had to tell her she could never eat again. None of her favorite foods. Done. It makes my stomach turn in knots just thinking about it. 

But let me tell you what is scary. Nobody knows what comes next!! Nobody knows enough about this process to know the next step! Medical theory says this disease process moves downward through the GI tract, but does that mean her stomach will shut down? Her small intestine? One of her organs? Nobody knows the answer to this because people who have this disease die before it reaches this point. We don't know what comes next and it scares me.

The xray camera doesn't lie

....so she gets to the hosital via ambulance with a bottomed-out blood pressure. Long story short she had aspiration pneumonia again!! Ok, two bouts in three months is not a good sign. It was decided we would do another upper GI and a video swallow. At least those could get done here and not have to wait until we go to Boston. These are very routine tests. 

Angela is anything but routine. 

We did the upper GI first. Angela's nissen fundoplycation is still intact. She had the open procedure done at 11 months old. Amazing, considering they usually last 7-10 years. She did have distention at the base of her esphagus, and what appeared to be her hiatial hernia having enlarged a bit from previous years, but otherwise not a bad study. 

Next was the video swallow. This did not go so well. Oh, Angela did fine for the test, but the results were very bad. First, for comparison, you need to see what a normal swallow looks like. Sorry these are a bit tedious, but its easier that trying to explain.

This is a normal swallow in an adult.

video courtesy Neinia Ferguson

Now, here is Angela's swallow of thin liquids. It is easy to see where her airway is because the barium goes into it. You can also see the barium sitting in kind of a small bowl-type area just below her airway. This is the cricoid muscle that is so tight, it doesn't let all the liquid through. 

Next they would normally go to nectar consistency liquids, but we skipped that and went straight to honey thick. Not only will you see her aspirate, but you will also see that she has some residual stuck at the back of her throat. You and I would be coughing due to the sensation. Angela gives no reaction. 

Here come the solids. You'll see her aspirate. You'll see her tuck her chin to protect her airway. You'll see the solids stick in her cricoid. You'll see A LOT of residual food sitting in her pharyngeal area. You and I would be gagging. Angela gives no reaction.

Last, we send down some honey thickened liquid to help push the solids through the cricoid. The doctor asks her to cough because there is too much residual sitting that Angela isn't reacting to and its not safe. But Angela has lived her whole life like this so it feels normal to her:

Angela was discharged labor day weekend, with an appointment scheduled to see the GI specialist just a few days out. It is clear that the Achalasia has progressed. Her swallow study is significantly worse than it was a few years ago. Not only that, but she cannot swallow *any* consistency safely, including her own saliva. The decision is made that she needs to go back to a gtube.

Two words I hate: Cricopharyngeal Achalasia

I need you to go back on my blog several years. Six years, to be exact. You'll want to read this post, particularly the last two paragraphs. Then come back here.

Key word: Cricopharyngeal Achalasia = CA

I'm not sure what I thought six years ago when we sat in the office of Dr. Nurko at Boston Children's hospital. I remember him telling me, "Well, she's doing really well right now..." and we discussed plan of care.

About a year ago I noticed that Angela was having more episodes of food getting stuck in her esophagus, and just some subtle changes to how she was swallowing. Things like tucking her chin, which I knew she was doing to protect her airway. I started getting my mind prepared for the fact we would need to head back to Boston soon.

Then in April Angela had a very bad case of aspiration pneumonia. Actually her lungs didn't look too horrible on x-ray but she was having a tough time managing her airway. She had to be on oxygen for several days and her blood pressure kept tanking. Getting to Boston was discussed, but the doctors at St. Paul Childrens Hospital had never seen a patient with CA before. Gee, surprised surprise! NOT! Nobody has seen it. There are currently only three kids in the entire USA right now who are living with CA.  Angela is the oldest. Two are treated by Dr. Nurko in Boston. There was another person with DS who had the disease, but at the time we saw Nurko 6 years ago she had already passed away from complications of the disease. If I remember correctly she was 14 or 15 years old.

Let me tell you a tiny bit about CA.

There are three types:
1): acquired CA: Usually happens to elderly stroke victims and is not progressive. Most people with acquired CA die from complications from either the stroke that caused it or aspiration pneumonia. Acquired CA is not progressive.

B: congenital CA: It is not uncommon for newborns to have CA. It is not progressive, but the newborns need to have a myotomy (cut) in the cricoid muscle so they can swallow safely.

C: CA of unknown etiology. There is no known cause, and it is a progressive disease process. It is EXTREMELY RARE. It is NOT related to Esophageal Achalasia which is significantly more common. Again, it is a progressive disease.  This is where the last two paragraphs of that other post become important.

Now back to Angela's pneumonia back in April. She had one really bad bout and got over it. Summer marched on and we put it behind us. In the meantime I started working on getting Angela back out to Boston. One round of aspiration pneumonia in a person with Down syndrome isn't completely shocking. It happens and you move on.

On the August 30th Angela started coughing, which is not unusual for her. By morning her cough had changed a tiny bit. Around noon there was a shift. Her cough was sounding wet. I called the pediatrician's office who said they could get her in at 3:00. At 1:00 I put her in the car and headed for the peds office anyway. Its only a few minutes away. On the way there she said her chest hurt, and thats my indicator that we're headed for trouble. Her chest hurts because of how she's breathing. As always they put us right into a room and took her blood pressure right away. She was low, but not so bad that that she was going to crash on us. We did a chest xray and the tech showed it to me. Oh man...yuck...her lower right lobe was a mess. The doctor got all Angela's paperwork ready for me when I said, "Nope. I want her transported this time. Last time she tanked on me in the parking lot." Looking at how Angela was in that moment the doctor thought transport was a bit overkill but followed my lead anyway. Two young male paramedics walked in and Angela declared, "Oh, you're smok'in hot! I'm riding with you guys!" The paremedics seemed a bit confused that this very with it young lady was going to ambulance. I was following the ambulance when we came to a yellow light about four miles from the hospital. The ambulance made it through, but I did not. As the cross traffic started to move in front of me, the ambulance turned on its lights and sirens. CRAP! CRAP CRAP!!
I got to the hospital just a couple minutes after them. Angela's blood pressure had bottomed out in the ambulance.

Ok...that's about enough for this post, huh? I'll continue with another post so you can rest your weary eyes.