Blogging about life in Minnesota, raising our six kids with Down syndrome while battling Breast Cancer.

Be the kind of woman that when your feet hit the floor in the morning the devil says, "Oh shit! She's up!"

Thursday, December 31, 2015

2016...BRING IT!

For the last 29 years I have done what mothers around the world do; I have taken care of everyone else. The last five years have been even more intense, working on bringing kids home and parenting through some really rough stuff. Then 21 months ago came cancer, and I found myself in for the fight of my life. "Taking care of me" meant keeping me alive.

Now it is time for me to turn the tables. Oh, I have made half-hearted attempts in the past, but this is
it. This is the time for me to take care of me, from the inside out. Getting rid of all the left-over crap that hangs on my body, and replacing it with a new, healthier version of me. One who has the energy and ambition to keep up with the life we have created here. The commitment has been made. My trainer(s) are waiting, the weights are waiting, the cycles are waiting, the sweat is waiting, the pain is waiting.

So here is my "before", for all the world to see. 
Bring it on 2016.
Bring. It. ON!!!!!  

And high praise for my plastic surgeon who gave me new boobs!! If you're in the twin cities area and need a referral for a surgeon specializing in breast reconstruction, hit me up! 

Sunday, December 20, 2015

Guest Post from THE DAD

I would like to start this off by wishing everyone a "Merry Christmas"

I like to cook and on occasion I will make things from scratch, like chili, (okay, I haven't made it for a long time because Leah doesn't like it. No I don't need a new recipe because she just doesn't like chili), spaghetti, lasagna, stews, cookies, brownies, etc, etc. Unlike Leah who buys everything already made or "just add water" type of foods, or orders delivery or take out. Anyway, I learned a few things tonight while making those green wreath things with the little round cinnamon candy on them.

First, don't wait until the last minute to start making stuff. The stores wouldn't have it. I checked 4 stores for those little candy things! I could not find one package of them. Not the Dollar store, not Cub Foods, not Big Lots and my least favorite store Walmart. The guy at Walmart didn't even know what I was talking about.

Second, make sure you have everything you need, before hand, Here again I needed some ingredients that I forgot, like wax paper and a can of Spam, oops I mean PAM. Where did I go? You got it,Walmart. Wouldn't you think that wax paper would be in the aisle with the baking goods? Let me tell you, it's in the aisle with paper plates which is the aisle next to the dog food, away from the grocery part of the store.

Third, never ever measure liquids over what you are making. I didn't think that hole in that little jar of almond extract was that big.(Make sure you close the top good because that stuff pours out really fast when it tips over), I think I put three times the amount it called for.

Forth, marshmallows. Never ever put four cups of marshmallows in a four cup measuring container and put in the microwave. That stuff expands big time!

Fifth, I figured I screwed up the first batch so why not try another batch? After measuring the green dye the almond extract and vanilla extract, make sure you put it all in. I didn't. I think I was gun shy because of putting to much almond extract in the first batch and the smell of the almond extract I spilled on the counter made me not want to put it in like the recipe called for. Lets just say they aren't very green.

Sixth, ALWAYS measure your corn flakes. I figured I had a good eye for measurements and I found out I DON'T. I didn't have enough stuff to mix with the corn flakes and now not all of them are green colored.

I'm thinking of starting a cooking school. Does anyone want to enroll??

P.S. I also found out that milk chocolate chips melt better than semi sweet chocolate chips!!  

Audrey: Looking back and forward

Sooooo many people have asked me for updates on each of the kids. I thought I'd start with Audrey since I found something today.

On April 15, 2014 I posted this picture on Facebook. 
Doesn't she look like an angel?

I don't remember taking this picture.

I don't remember this dress, though Dean says it was sent to us by a friend of mine.

I really don't remember much of Audrey's first 6+ months home. Lost in a chemo-induced fog, going through the mechanical motions of each day. Taking care of the kids between naps. Feeding the kids between naps. Sitting outside, trying to soak up some sunshine until I could excuse myself from life for another nap.

I do remember one day in particular when I was trying to potty train Audrey. WHY in the WORLD did I choose THEN to potty train her? Did you all think I was crazy? Someone should have slapped me. Anyway, I had underwear on her and a potty chair in the middle of the living room. I was doing my usual "no pants" day, which is how I potty train my kids. Suddenly I thought, "Why don't I have a pull up on her instead of underwear? Yes, she might take longer, but I can barely get off the couch much less constantly be cleaning up puddles on the floor." Such a simple thought, but one that was so difficult for my brain at the time. Audrey was fully potty trained just a few days later.

My friend Ann came to help me on a couple of my really bad days. I remember sitting outside in the fresh air so thankful my friend was there when I had to make several mad dashes to the bathroom. And she brought me desitin which I desperately needed.

I have to go back and look at pictures to have any idea of Audrey's progress during those first months home. I know from videos that her communication took off, and she was so excited to have a way to tell us what she wanted instead of just pointing. Her love for music is something to be reckoned with, and she can usually be found with an iPhone in her head, and occasionally a small blue-tooth speaker that she can carry around the house. Although, it doesn't really matter if there is no music playing at a give moment, because Audrey has a song in her head at all times. She never stops dancing her own little jig, singing some crazy tune that often sounds like a Native American spiritual dance. She has been so very happy and excited to see the world.

But a couple of months ago things started to change. I'm not sure exactly when, as we were a bit pre-occupied with Angela and probably not noticing some of the more subtle changes. Finally, there came a day when I said to Dean, "I think that Audrey is regressing."

It is very common for kids with Down syndrome, as well as post institutional kids, to make big gains, then regress a bit in one area while they're working hard on another. Kind of the "two steps forward, one step back" concept. Two weeks ago we had Audrey's IEP meeting. We discussed some changes to her programming, and I expressed my concerns about just teaching her to function in the world, and lets worry about the academics later. Audrey is developmentally around 16-18 months old, and kids that age aren't sitting at a table learning letters and numbers. Her classroom staff is excellent and in complete agreement. They know what they're doing, and I'm so thankful to have staff working with my kids who truly do approach teaching as a team effort! Anyway, I also brought up my concerns about regression. Her teacher made it clear that Audrey has regressed in every single area.

Every. Single. Area.

So, here is where I tell you the hard parts about parenting Audrey, and to remind those adopting for the first time that the child you bring home may not be the child you have a year or two down the road. When Audrey came home she was SILENT. I knew she liked to sing because she did it with her foster family in Serbia. I figured the whole language barrier and new sounds she was hearing was too much for her to process. But now, a little over a year and a half home, Audrey is SO VERY LOUD!!!!!! Oh my word!!!! She chants. She sings. She screams. (not like a girl screaming when they play. This is a self-stimming noise that goes right through my spine!!) And when she screams a certain scream she is sent to "sit in the circle", while she laughs hysterically at our attempts to reign her in. She has also gotten very aggressive and will kick, hit, bite, pinch, scratch, spit, tackle, and put other kids into headlocks before you can process that is what's happening right before your eyes. While she smiles at you and bats her eyelashes. Thankfully she saves most of her aggressive behavior for school, laughing at staff's attempts to make her stop. I say "thankfully" not because I'm glad she's naughty at school, but's 6 1/2 hours less per day that I don't have to keep an eye on her. We love this child fiercely, but we also need a break for the eyes-on supervision she needs at all times.


I am pretty sure Audrey was a stripper in another life because she is so very good at being dressed one second, and when I blink my eyes she is naked. Or wearing someone else's pants. Maybe even the pants that person was JUST wearing and she has managed to get them away and onto her own body. While I blinked. This child can vaporize and re-appear in totally different clothes as we all sit here wondering what the hell just happened.

Stripping can happen anywhere, such as while we wait in line to see Santa. And I am holding her hand. And without ever letting go. I listen to her sing and feel her dancing around as I talk to the people next to us, when I suddenly realize they are looking down with their mouths hanging open And so I, too, look down to see Audrey smiling the biggest smile at me, and her jacket and shirt are now hanging from our joined hands. And her pants are at her ankles. And where the hell are her shoes and socks???????  This is something that happens multiple times per day. And don't even try to say, "You must not be watching her close enough" because it happens at school too, where she has a 1:1 staff person with her at all times. On the bus she is strapped into a harness for the 3 minute and 42 second ride home. When she arrives she is usually missing her jacket, shirt, shoes, socks and is working on her pants when they arrive at our driveway. How is she even able to do this strapped into the harness?

One of her best moments was when I had a county person here for a visit for one of the kids. Audrey was outside jumping on the trampoline when I ran to answer the door. When I ran back to bring the kids up to the house Audrey naked, laughing and jumping on the trampoline with wild abandon. I looked up to see the county rep. watching out the window, trying not to laugh.


In addition to stripping, Audrey also changes clothes multiple times per day. We still haven't figured out if this is a sensory thing or a developmental stage. Probably a combination of the two. She will find anything to wear. I'm considering putting together a dress-up box for her, but really the "dress up as someone/something else" part is beyond her yet. She just wants to change clothes. I have taken all of the clothes out of her bedroom to save me having to pick up the mess of strewn clothes at the end of every day.

Have I mentioned that Audrey is extremely hyperactive? Now, Angela was very hyperactive when she was little, and there were many professionals in her life who, along the way, commented, "I have never seen a child with Down syndrome quite so hyperactive."

Let me tell you, Angela was a sloth compared to Audrey. Remember the cartoon of the Tasmanian devil? This is Audrey, on one of her calmer days. Complete with the noises.

And so, where Audrey is concerned, our focus over the next few months will be figuring out exactly what is happening in that pretty little head of hers. Maybe we won't find out at all (look, it took us 19 years to get Angela diagnosed!) but we have to figure out something to help her. She is not even functioning well in the world right now. In the meantime we're looking at moving her to a different program. Like I said above, Audrey's staff at school are excellent. But these kids with severe institutional delays, behaviors and attachment problems? They are no walk in the park and not everyone can be expected to know how to manage it all. We don't know how, and we sure can't expect her school staff to just know how either. Thankfully our district does have another option for Audrey, and I think it will be a very good fit for her. A place where she can learn to function in the world, and if she learns some academics along the way? That would be just great!

So that is my update on Audrey. She really is a beautiful little girl, but man, she is a HANDFUL!! I'm so very grateful we have to parental tools needed to get her to adulthood. To help her become the most independent she is capable of being. To allow her to see the world in all it's glory while the rest of us run to keep up with her. 

Tuesday, December 01, 2015

The end of a season

Do you know what today is? Well let me tell you! Its the end of the season of my life called "cancer".  Yes, I finished treatment ages ago (16 months ago, in fact), but I've still been dealing with this stupid reconstruction process. 14 months ago I had a bilateral mastectomy, and today I'm in surgery, having my horribly uncomfortable tissue expanders removed and replaced with implants. Also, because of the way breast reconstruction works, it also involves liposuction at the sides of the ribcage to even everything out.

Looking back at the last 20 or so months since I was diagnosed, there are some things I wish I had known. Here is a letter to that self.

Dear Me,

Today you were told the worst news you've ever heard in your life. You have breast cancer. You felt your knees give out beneath you as you best friend and life partner held you up. You sobbed the longest, loudest sobs. As you cried, noises escaped from your mouth you didn't know were even possible. Primal fear. What you are feeling now is primal fear.

I'm not going to tell you, "Don't worry, you'll be fine." because its impossible to not worry. You're going to spend the next few days (or even weeks or months!) numb. You will think you're losing your mind because you keep forgetting odd little things and can't concentrate on anything. Its ok. You're normal. Your brain is on overload and it can only process so much right now. Remember that primal fear? This is part of it. You are in survival mode. Don't worry about the laundry right now. If it doesn't get done the world won't end, but if it helps you by all means do it.

Start a list of questions for your doctor. Even if they seem silly, write them down.

Do NOT, I repeat DO NOT set any kind of timeline for when you'll be done with treatments and/or surgeries. There is no way to predict how each woman will respond to various steps in the process and its easy to get disappointed when your treatment or healing doesn't go according plan. Mine was supposed to be a "three month speed bump" in my life, and here I am 20 months later, just finishing up. Don't count on "you won't need chemo" or any other treatment predictors until EVERY test has been completed.

Everyone is going to send you emails and private messages with the most outrageous "cures" for your cancer. Use your "delete" button generously. Everyone means well and only want whats best for you, but they forget your doctors spent years in medical school, and more years treating other women just like you. He or she knows the latest TRUE research. Listen to your doctors, but if you don't feel comfortable get a second or 3rd opinion.

Nipples aren't necessary for life, and if you don't have them there is no "show through" so no need to wear a bra. Oh, and you can buy nipples online. For real. You can order any color you want.

When something doesn't sit right with you, trust your gut and ask questions. Remember that you are your own best advocate. If you don't feel comfortable speaking up, have your partner help you.

Speaking of your partner, he's hurting too, in totally different ways. He's scared to death about losing you. Suddenly the weight of the entire household is on his shoulders. Remember to encourage him to take a break when he needs it. Spending a few hours with his friends or brothers can make a world of difference for him.

Sleep. Sleep and don't feel guilty for sleeping. Your body needs to regenerate healthy cells and the rest of you needs to rest while that happens.

Everything tastes bad when you're on chemo. Moutain Dew will taste like salt water, but banana cream pie blizzards taste heavenly. Oh, and Jimmy Johns delivers to the chemo clinic.

In the coming weeks you're going to be very run down. Try to organize your house in such a way that if someone comes in to help, its easy for them to see what needs to be done. Let go of your need to control all things, even if that means turning on the white noise machine in the bedroom so you can't hear whats happening in the rest of the house. Resist the urge to take over. RESIST!

Don't try to potty train a child during chemo. Really, its a terrible idea.

You may look at your body and hate every scar, or you may embrace them as badges of honor. Whatever floats your boat, but do not EVER be ashamed of those scars.

Let the kids do washable marker art on your head. It feels awesome and they have a blast.

Steroids really do make you crabby. When you notice you're snapping at people, its better to just excuse yourself for a nap.

Chemo brain is a real thing. Unfortunately sometimes it sticks with you long after the chemo is complete.

You have a long road ahead of you. Right now you're trying to see into the future and its just plain impossible. Try your best to live for today. Notice the smiles on the kids' faces. Notice when they seem worried or anxious. Take the time to give them an extra hug. Let them sit on the bed with you and watch movies while you nap. Let them take care of you in their own ways. Its really important to them.

You don't need to make excuses to anyone. When you feel tired, its ok to say, "You know, I know you wanted to have coffee today, but I'm just too tired to even listen to talking, much less get out of bed."

Its ok to complain, and its ok to cry. But, if you're feeling overwhelmed by it all, its also ok to talk to your doctor about a little pharmaceutical help.

Just say "No" to Effexor. Its terrible to get off of!

If you're sitting in the warm summer sunshine shivering, take your temperature. You're probably running a fever. Likewise, if you feel you can't make it up a flight of stairs without sitting to rest, its time for some IV fluids!

Take care of yourself however you feel is right for YOU.

 A lot of you reading are cancer survivors yourself. What would you tell yourself on that day you were diagnosed?

Monday, November 30, 2015

The first real snow

Minnesota moms, did you feel my pain this morning? Did you wake before the kids, look out your window to find the carpet white and PANIC like I did?

Tonight! The snow was supposed to start TONIGHT! I scrambled to find the boots. "Where is the big box of boots? I know I took it out of storage weeks ago!"

 While Dean was looking for that box, I was looking for the snow pants. Oh my word, the snow pants! I found bright pink pair of 4t's to shove Audrey's size 6 legs into. I found a pair of black 4t's for Amos (thankfully they're perfect for him!) Asher, who is a size 7, got shoved into Audrey's 5t's from last year. They were black, so they work. Well, except for the very feminine snowflake design that runs up the leg.

When Dean found the box of boots I discovered there has been a lot of growth in the house this year. Everyone was put into boots a size or two too small, except for Abel who couldn't even get his foot into them. Hmm Maybe because his feet grew four shoe sizes this year.

After my pre-op exam I made a quick run to the winter clothing section at the local retail place then dropped off appropriately sized snowpants and boots at the kids' school. Guess what! There were a lot of other moms there, doing exactly what I was doing!

When the morning was finally over I was reminded of this scene in "A Christmas Story".

Tuesday, November 24, 2015

Discharge today!

Angela was admitted to the hospital two weeks ago today, and today she's coming home! Axel and Asher in particular are excited to have her back, and just in time for Thanksgiving! We certainly have a lot to be thankful for this year.

I wish I could say coming home is going to be easy. It will be anything but! Angela has had an insult to her brain due to the high ammonia and glutamine levels. In addition, all her meds were stopped about two weeks ago. The nurses have been struggling with managing her. At this point we really don't know what is "Angela", what is "Brain injury" and what is "I've been in the hospital for two weeks and everyone gives me whatever I want all the time to avoid having a confrontation with me." She is also tired and taking a couple naps per day. I wonder how long that will last?

As for formula, she is now on a combination of two formulas. One gives her amino acids, and the other is free of non-essential amino acids, and has additional nutrients to compensate for the medication she'll be on, along with protein. Everything requires very careful measuring and mixing. Before she got sick we were (per doctor recommendation) allowing her to eat one thing per day by mouth. Usually a pudding cup, apple sauce or other foods she is less likely to aspirate. We will still be able to do that, but need to be very careful that whatever she has includes no protein or we have to compensate with the protein included in her formula.

We're anxious for the long weekend and having Angela home. We have to move bedrooms around again so Angela is closer to us. This whole event started for us on October 6th and we're ready to re-establish our routine! 

Sunday, November 22, 2015

Hepathic Encephalopthy; when the liver hurts the brain

The when the urea cycle- which is the process of breaking down protein in the body and removing the waste products into the kidneys - isn't working right, the chemicals glutamate and ammonia build in the brain, which can cause Hepatic Encephalopathy, or HE.

Since Angela's gtube was started on her formula on October 8th, her brain has been swimming in a toxic bath. She displays nearly all of the symptoms of HE. I've marked them in red.

mild confusion
short attention span
mood swings
personality changes
inappropriate behavior
difficultly doing basic math
change in sleep patterns
decrease in fine motor skills
"musty" or "sweet" smelling breath
slurred speech
marked confusion
severe anxiety or fearfulness
disorientation regarding time and place
extreme sleepiness
slowed or sluggish movement
shaking of hands and arms
speech that is difficult to understand

We don't know if we will get our girl back or how long that will take. The doctors say it can be weeks or months. She has a nearly non-existent frustration tolerance, which makes her aggressive and prone to lashing out in the form of throwing things or hitting. She cries because she is confused by what is happening and she doesn't like how she feels. As her body recovers she has very little energy. She can have one "big" activity in the morning (going to the teen room, doing an art project with a volunteer, 20 minutes of music therapy) before she starts to shut down. The dark mood takes over and she really can't function well. It takes a lot of convincing that she needs a "rest time". Finally the nurses or I will shut everything down, turning off lights and removing attention from volunteers and favorite nurses. After pouting for a few minutes she will curl herself up and sleep.

We just want our smiley, bubbly girl back.

Friday, November 20, 2015

Back in a room

Monday was spent doing lots of labs and watching Angela's ammonia levels fluctuate, but she was waking up a little more every hour. Unfortunately waking up didn't mean being her happy self. Unfortunately the glutamine (neuron protector in the brain) was too high, causing her to have a migraine. Like a person with a brain injury, Angela was extremely irritable, irrational, didn't/wouldn't/couldn't talk to anyone. Didn't want to be touched. Didn't want the tv on. Didn't want her glasses on. Didn't want to get out of her bed. Didn't want to touch anything. Didn't want anything.

We have seen this mood before. Years before. It is when she would have sudden, unprovoked aggression. Our neurologist said she was either having simple partial seizures or migraines, and the treatment was the same: Topiramate (a seizure drug that also treats migraines) When she was treated with the drug the aggression improved about 90%, having breakthrough episodes about every 4 weeks. (ahem!) When this mood returned this week, Angela's ammonia level was hovering around 80. This would cause her glutamine level in her brain to rise, causing the migraine and this very dark mood. Now we know when we see this mood, Angela's ammonia levels are on the rise and we need to get her into the hospital to have them checked. For now, it can take several weeks for her glutamine levels to come down and her mood to improve.

Let me go back a bit and explain the Urea cycle, at least in my limited understanding. Maybe this graphic will help? Or confuse you more?

Here is a brochure for patients and families to better understand Urea cycle disorders. Somehow I need a different type of graph because I still don't really get it. Stuff goes into the liver, stuff comes out of the liver. Somewhere in there the body creates the waste product ammonia, and breaks it down with agrinine. Originally it was thought that Angela had OTC Deficiency, which is a deficiency of Orinithine (its in green in the graphic above.) For her to be deficient in that, she would have to be deficient in Arginine. 

Back on Saturday Angela's blood was sent down to Mayo for genetic testing. On Weds her tests came back to show that her arginine levels were actually high. If she had OTC her arginine level would have been low. This means she is more likely to have HHH syndrome (hyperorinthinemia - Hyperammonemia - Hypocitrullinurea)  which is caused by a mutation in the gene SLC25A15, or a condition called LPI (Lisinuric Protein Intolerance) New bloodwork was taken on Weds and has been sent for molecular testing. This will take 2-3 months to come back. In the meantime, we need to get Angela healthy again.

On Weds we started the slow process of finding Angela's protein threshold - the limit of protein she can have before getting into another crisis situation. The fact we found her in a coma - twice - scares me to death. That is the last step in the urea process before a person dies. We don't ever want her getting to that point in the process again.

Angela is now off TPN and on a continuous formula drip. This is a very specialized formula, created just for her, that she will be on the rest of her life, along with daily medications. It is a very fine dance between amino acids and protein to keep her urea cycle functioning properly.

Understanding the whole Urea process has been overwhelming to me. I need to make a model of it in order to understand all the steps. We're talking steps that take place at the molecular level. Mostly I'm just glad we didn't lose Angela last week. I'm thankful for doctors who didn't give up looking for a cause for the comas. Those same doctors have come to visit Angela every day, even when they don't have a medical reason to. This is the first time this hospital  has treated this type of disorder because it is extremely rare. (which is why only 6 doses of the rescue drug were available in the entire 5 state region!) Angela is a bit of an enigma there. This doesn't surprise you, right?

Wednesday, November 18, 2015

Playing catch up

Now that I have borrowed a computer from the hospital's family resource center (thank you Geek Squad!) that I can keep in the room during our stay, I can also update the blog. Typing on the phone is so not fun. Its probably not fun for you to read through the typos either!

So...back to last week...

On Saturday we were finally given the possible diagnosis of OTC Deficiency. This is an x-linked Urea process disorder. Of course it is very rare, but of Urea Processing disorders it is the momst common. Angela's body is missing the enzyme needed to process ammonia in the body. Isn't it crazy that our bodies produce ammonia? I knew that, but..yeah...didn't really need to know that.

It was urgent that Angela receive a rescue drug called Ammunol, which is in class of drugs called "scavenger" drugs. It does just as it sounds, runs around the blood stream eating up the protein based molecules and converting them to products that can be excreted through the urine. The problem is there were only 6 doses of the drug in the 5 state region, and of course not where we are, St. Paul Children's Hospital. The drug was flown up from Mayo.

Because this hospital has never administered this drug before, all the staff was in a tizzy. A bit excited, really. Oh boy! We get a new drug for the first time! I wasn't really feeling it, since this drug has some potentially very harmful side effects, is caustic, and CANNOT mix with anything else!

Angela got a PICC line put in, then was moved to the PICU. The drug arrived around 1:00 am. All the nurses in the PICU, along with the doctors, went over the protocol given by Mayo on how to administer the drug, the signs and side effects to watch for, etc. Everyone was hyper-focused and I just stayed out of the way. Finally the Ammunol was started at 2:00 am, and would run for 24 hours. She had 5 other drips running at the same time: lipids, D10, electrolytes, arginine and I forget what else!

Over the course of the next 24 hours her blood glucose levels taken every 30-60 minutes. If she moved over 150 she was started on an insulin drip until she came back down. In addition, her ammonia levels were checked hourly to make sure it was trending down. For the first 12 hours Angela mostly just slept. Then she started complaining of her PICC line and arm hurting her. A couple hours of that and it was full-out crying that her arm hurt. Poor baby. I couldn't do anything to make the hurting stop at all. She was just miserable. It was the longest 24 hours.

Finally at 2:00 a.m. Monday morning the dose was complete. She complained about her arm a bit more. But the worst was that her Ammonia levels jumped right back to where they had started. The doctors thought she was needing to go back on the Ammunol for another 24 hours! Then the metabolic geneticist got back to them and said no. What has happened is she has gone into a catabolic state. Meaning her body was creating its own protien by attacking her own muscle, then converting it to ammonia again. So she was started on TPN (nutrition that goes right into the veins) so her body didn't feel starved and could stop attacking itself.

Finally on Tuesday, when her ammonia levels were consistently sitting at 80, we moved her back up to a regular room.  While she was out of imminent danger at that point, there was still a lot to do.

Tuesday, November 17, 2015

Update from ICU

Posting from my phone so please excuse the million typos.

I cannot begin to tell you how confusing the last 48 hrs have been. Let me recap:

10/06/15 walked into the hospital for surgery her normal chipper self. Had gtube placed. Normal grogginess after surgery.

10/08/15 had first gtube feed with Nestlé Complete.

10/09/15 med supply company delivers our preferred formula (Liquid Hope - amazing stuff!). Nurses prepare discharge papers. Angela is very quiet. We go home.

10/12 she goes back to school but is still not herself. Quiet and tired. She doesn't make it a full day.

10/12-10/31 Angela gets more tired every day. Her blood pressure plummets multiple times. Ambulances are called to school. In general she is not right but nothing we can put our finger on.

10/31 she is very tired and has tremors but really wants to go trick or treating with the little kids. We only do a few houses because I can see she is fading. She is not answering questions and I would call her mental state  a "stupor". We go home, get her last feed for the night as she sleeps on the couch. She is very difficult to wake up to move to her bed.

3 am I wake up and check on her. I find her on the basement floor unconscious and unresponsive. Ambulance is called. On the way she becomes combative, but still unresponsive. She is admitted to ICU in a coma. I am asked about 20 times if she could have gotten into anything or if someone could have put something in her gtube. Over the next 48 hours she gradually wakes up and is more awake than she has been in a month. She is chatty and chipper, although she has these odd tremors.

11/03 she comes home mostly herself again. She starts back to school but cannot make it a full day. She is moving from lethargic to sleepy.

Through all this there were countless conversations with doctors. But she is just sleepy. Not much to do. I decide there is a connection with the formula. Too many calories? Not enough calories? Too much water? Not enough water? She sleeps most of every day.

11/05 we normally give her 4 feeds per day. Our schedule was thrown off and she only gets 3. In the morning she is easier to wake up but very irritable. She is awake all day for the first time in several days. In the evening she goes to a disabilities dance for a couple of hours but does not make it the whole time.

11/06 I email her doctor. As long as we can wake her and her blood pressure is ok we can keep her home. I wake her every hour and give her feeds while she sleeps. Get her to the bathroom every 4 hours.

11/07 repeat of the day before.

11/08 call the doctor first thing in the morning. We agree if she is still sleeping on tues to bring her into clinic. I wake her several times during the night to make sure shes ok.

11/09 wake her up for dr appt. She has been sleeping for 14 straight hours without really moving. I am able to get her out of bed and to the bathroom but with a lot of support. She is not talking or answering questions. I get her to sit down at the table. Her eyes are only 1/2 open when she slumps her head to the table. Dean calls 911. She is able to walk to ambulance with 2 person support but her eyes are closed. After about 2 minutes in the ambulance she is unresponsive.

In the ER they do all the same bloodwork and tests. This is named coma #2. There is nothing they can find. They again ask Dean and I who has access to her gtube besides us. Since she hasnt been in school there is nobody. We make it clear we will not bring her home again until we know whats going on

11/10 observation and tests. She is getting perky.

11/12 she is the life of the party. More questions, along with a list of drugs that would not show up on a toxicology of blood or urine. Do we have any of those in the house? Do Dean or I have prescriptions for any of them? We are asked again and again.

11/13 we start the feeds back up. I insist she is fed the same formula she gets at home so her reaction can be observed. I refuse to do the feeds myself or to do anything with her cares. I won't even go near the beds during feeds. This needs to be all them.

Evening of 11/13 I bring Axel up to the hospital. Angela's hands and arms are trembling. She has feed #2 while we are there.

11/14 she has her morning feed. Dean goes to visit at the hospital. She seems "fine" but I call and insist she get out of bed and walk around. Oh, surprise she can barely stand without support because her legs are too shaky. Dr is seeing the pattern with feeds. 11:00 am she calls me. I told her with the next feed Angela will start to get drowsy. They stop her feeds, contact metabolic specialist who orders an ammonia level. Normal is under 15-45 Angela is at 221. Suddenly there is urgency.

Genetic testing is ordered to test for the x-linked enzyme OTC Deficiency. It is a rare and deadly disorder. She cannot process protein. This amazing formula has given her more protein than she's had in her entire life.

A special rescue drug - Ammunol - is ordered. There are only 6 doses in the entire 5 state region. Coat is $100,000 per dose. One is flown up from Mayo clinic. 11:00pm Angela has a picc line out in. The nurses are all in a tizzy because this hospital has never administered this drug! 12:00 am she is moved to the PICU. This drug requires her blood sugar be tested every 30-60 minutes. Insulin is kept on hand. She needs lipids, dextrose, fluids..everything all at once.

The drug arrives. It should be encased on gold the way everyone is acting
 The nurses and doctors meet outside Angela's room. They go over all the side effects to watch for, when to declare emergency response, blood glucose every 30 minutes, ammonia level every hour, insulin as needed for high sugar levels, watch for burning of tissue because drug is caustic, patient will complain of burning throughout duration because it burns inside. It is a 24 hour continuous drip. 48 finger sticks, 24 ammonia draws, 1.5 liters of fluids per hour. Watch for coma. The drug was started at 2:00am Sunday morning.

Sunday, November 15, 2015

We found the zebra, she wears a pink tutu!

Some of you have known Angela for years in the DS community. Others are more recent additions to our circle, either via the adoption community or connections through friends.  For you lad timers, you remember the days when Angela kept everyone guessing, either due to erratic behavior or unusual medical symptoms. Yesterday we received a diagnosis that now makes sense of all the oddities of previous years.

Partial Ornithine transcarbamylase deficiency

I'm going to post a link to information for you to read at your leisure. Wrap your brain around it a bit. Pick your jaw up off the floor, then come pack and I'll explain how this all came about. I'm sure you'll notice as you read that many features associated with Down syndrome masked the symptoms of POTCD. 

Thursday, November 12, 2015

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. 

Wednesday, November 11, 2015

If you could

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

Monday, November 09, 2015

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? 

Saturday, November 07, 2015

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. ;-) 

Tuesday, November 03, 2015

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! 

Monday, November 02, 2015

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?

Sunday, November 01, 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

Saturday, October 31, 2015

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.

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!)

Thursday, October 29, 2015

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! 

Monday, October 12, 2015

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. 

Friday, October 09, 2015

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. 

Thursday, October 08, 2015

Tuesday, October 06, 2015


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.

Sunday, October 04, 2015

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.