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!"

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. 

Tuesday, October 06, 2015

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.

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.


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.