Cecostomy Tubes R Us

Here we are - two posts in 30 days! It's scandalous, I tell you!  Seriously though, I bet you all thought that I abandoned ship and would revert back to neglecting this blog.  Well. . . ahem, *whispers* I thought about it, but then I figured I'd present you with more random medical stuff, some pics, and the usual Ellie Bear antics.  Yes, antics on steroids because you know, hormones and puberty.

If you've been following The Chronicles of Ellie Bellie Bear for a while, you know that I love to get into all things medical.  I am a former pediatric nurse practitioner turned mama bear to a sassy 13 year-old with Down syndrome and other random, semi-complex, but not serious medical issues that pretty much have nothing to do with her having Ds. Please the disclaimers listed on the side of my blog.  

Now on to the actual point of this post.  There is a point?  In case you cannot tell by the title, Twinkletoes still has her cecostomy and for the most part, it has been a Godsend as her constipation is finally well controlled and she is no longer fighting us with the ante-grade enema administration. She seems to be happier now that she doesn't have weeks'-worth of stool packed into her gut and she is eating better and finally no longer failure to thrive.  It took us some time to find a good regime and we have to tweak it here and there but she is now getting 175cc of SMOG through her tube 3 x week.  SMOG is saline, mineral oil, and glycerin. Sometimes we toss some Milk of Magnesia directly through the tube if she seems to be getting backed up.  The type of enema through a cecostomy is different for everyone -some people only need water, others need saline, etc.  We were hoping that she would only have it for 2 years to allow her colon to snap back to its original size and elasticity but here we are 6 years later and she just cannot seem to go on her own.  The kiddo may be a cecostomy lifer and I am actually okay with that. 

That being said as with any ostomy and with any medical device (Chait Trapdoor), there can be things that go wrong.  The list includes infection, appliance failure, skin breakdown, infection, parastomal hernia, and tube dislodgment.   

**Even with all of the possible complications, I firmly believe that for Ellie, this cecostomy has vastly improved her quality of life and my husband and I do not regret her undergoing this procedure.

Photo Credits:

Left: Cook Medical

Right: Arya, Shruti & Gupta, Nancy & Gupta, Rahul & Aggarwal, Arun. (2016). Constipation and Outcomes of Cecostomy. American Journal of Therapeutics.

Ellie has what is called a Chait Trapdoor.  It is a rectangular-shaped button that sits flush with her abdomen.  The tube itself has a straight piece turning into a "pig tail" or corkscrew.  This tube is typically replaced under fluoroscopy with sedation every 9-12 months.  Ellie spends more time in recovery than the length of the actual procedure.   The usual risks include bleeding, infection, device failure, and gut perforation.  I think she has had it replaced 6 times.  One for each year and then a little extra one from way back in the day when the Princess StinkyPants pulled the tube out. 

Taken a few years ago, this is how the Chait Trapdoor should look.

Chait Trapdoors come in Small, Medium, and Large.  The size is not determined by its diameter, but rather the length.  As a person grows, the colon grows and a longer tube will eventually be needed.  Having the wrong size tube can lead to all sorts of issues.  Unfortunately, I sort of feel like it is Goldilocks and Three Bears in trying to get the size right. 

Photo Credit: Science Direct

*Some of the images in this post may be considered graphic by some.*

WHEN THE TUBE IS TOO LARGE 

For years, the tube was the perfect size sitting flush to her abdomen, but then last year the tube placed was too loose.  I was told it was the Small but the thing was sticking way out from her abdomen.  When she would go to defecate, it would come out to the first coil.  I would say "Ellie fix your tube!" and she'd push it back in.  It wasn't a horribly huge deal because she wears an abdominal binder (see this post) so it was keeping it in place.  Nonetheless, it was a defective tube because it was labeled as a Small in the sterile package but it wasn't actually a small.  With a tube that is too loose, there was stool leakage around the site which lead to skin breakdown.  She has alway had leaking but this was a bit excessive.  Think about a baby in diapers, they are at risk for skin breakdown and yeast infections.  Preventatively, I would use Critic-Aid-AF around the site and keep covered with 4x4 gauze folded into quarters. 

This was Ellie's tube from last year - notice how far it sticks out.  

Even with all the preventative care, such a moist environment would lead to skin breakdown, like what you would see with a diaper rash and eventually infection.  Typically, I would slap on some diaper cream. Well maybe not slap, but very carefully and gentle apply.  I prefer Boudreaux butt paste, but really any diaper cream with zinc works.  If that didn't resolve it, then adding some Lotrimin cream - yes the athlete's foot medicine- twice a day for 2 weeks would also help.  Unfortunately, my skin sensitive little Bearity-Bear would still occasionally need us to whip out the big guns in the form of an oral antibiotic such as Keflex and once she needed oral Diflucan because it was yeast and bacterial.  (Note: Ellie is notorious for getting infections in general, not just at her cecostomy site)

Left: Just after Ellie had her ostomy made, she had a bad reaction to the dressing adhesive

Right: excessive drainage saturated the gauze dressing resulting in skin irritation.  

This is a combination of bacterial and yeast infection.  She required both an oral antibiotic (Keflex) and an antifungal (Diflucan) to clear this up.

WHEN THE TUBE IS TOO SMALL 

Guess what happens when you have a child going through a massive growth spurt?  Any guesses?  Just two months ago, Ellie had her annual tube replacement.  She also packed on a bit of weight since then and all the sudden she developed this soft tissue swelling adjacent to the tube seemingly overnight.  I panicked and was worried about a parastomal hernia (a hernia near an ostomy), which is actually not an uncommon thing.  After a quick trip to the Bowel Clinic where she got to visit with her beloved Dr. R, the hernia was thankfully ruled out.  Yes, the little Turkey still loves her doctors. Ellie's tube is officially too small/short.  It is creating an indentation into her abdomen and causing soft tissue swelling and irritation.  She is starting to have the beginnings of a pressure ulcer where the tube is digging into that swelling.  I am now applying Meriplex, a silicone foam bandage under her tube to protect the skin until Interventional Radiology can fit her in for a new, larger tube.  I am so frustrated because she *just* got this current Chait Trapdoor and now we need to sedate her again!  Of course, my medical frequent flyer kiddo just takes all of this in stride.  As long as she still can get her salad with broccoli (she's an odd duck) once she is home, she is a happy camper.

Soft tissue swelling due to the tube being too small.  You can see how the tube is pushing into her abdomen. She has a Stage 1 pressure ulcer forming right where the tube is rubbing against the swelling.

As usual, it's fun times over in our household!  Fingers crossed that I hear from IR soon.  Our children's health system in Austin has only ONE interventional radiologist now and so the wait may be a while.  

More on Cecostomies:

The pre-surgery debacle and ridiculousness

Surgery - Cecostomy

Cecostomy Post-op Complications

The missing cecostomy tube 

Tips on how to prevent your child from pulling out their g-tube or cecostomy tube.

Colon Manometry 

~~~~~~~~~ 

While this is a Down syndrome related blog, many of the health issues and psychiatric illnesses discussed here are unique to Ellie and not necessarily related to her having Ds.* Please see more information under the Disclaimers tab.

Medical Disclaimer

The information in this blog is not intended or implied to be a substitute for professional medical advice, diagnosis or treatment. All content, including text, graphics, images and information, contained on or available through this web blog is about my parental experiences with Ellie for general information purposes only. All health questions and concerns should be directed at your medical care provider.

The Pre-Surgery Debacle and Ridiculousness

The Pre-Surgery Debacle

Friday 12/2:

This is the big day!  The day for bowel prep.  A day where we administer a gallon of GoLytely through a NG tube in hopes of clearing out Ellie’s colon in preparation for Tuesday’s cecostomy.  Or so I thought.  Friday morning we show up to patient administration for hospital admission.  We had seen Dr. R in GI the afternoon before and we should have been good to go. Only to discover that Dr. R never put in the orders for admission.  Dr. R is, of course, now out of town.

After much calling around, one of the GI nurses was going to put in the orders.  So we waited.  And waited.  Said nurse went into a meeting first and then got around to putting in the orders.  We waited some more. They neglected to call the unit to get a bed.  More phone calls.   They get a hold of Dr. S who is actually the doctor who performed Ellie’s colon manometry and recommended the cecostomy tube placement.  Apparently, she doesn’t need to be admitted on Friday.  Saturday is good enough.  So after pulling Ellie out of school and wasting an entire morning, Ellie and I head back home.

Saturday 12/3:

Ellie gets admitted!  For real this time.  I was apprehensive when we showed up, but it all went smoothly. We get the NG tube into her.  She doesn’t fight us much.  We watch Mickey Mouse’s Choo Choo Express 12 times while coloring in a Color Wonder book for hours.

Plus, she starts to poop in the late evening!  Yes, of all the things to get excited over.  Pooping.  A bowel prep is loads of nastiness fun.  The goal is to be literally pooping water.  As in clear fluid.  That is why we need Monday to be a day of “drying out” so to speak.  As I am helping the nurse change her sheets for the 3rd time, I discover a few interesting orders in Ellie’s patient chart.

Jello. Popsicles.  Broth.  Water.  Juice.  All of those are considered a part of a clear liquid diet.  Ellie was to start a clear diet as soon as the bowel prep started and continue until Tuesday.  However, some genius resident decided to change that order to nothing by mouth, NPO, as in she can’t even drink until after her procedure.  I had the nurse pass along to that resident that  I will be not following that order.  I am not a big fan of detrimental health effects related to dehydration.  Plus, what is the point?  She is getting GoLytely pumped into her stomach.  The whole idea behind NPO is to keep the stomach empty.  Score 1 for mom because she changed the order back to Clears.

The whole plan, to my understanding, is that we would go in for a bowel prep Saturday - Sunday and then get discharged Sunday afternoon.  Tuesday morning we would report to radiology.  Apparently, according to the orders in Ellie’s chart, we would not be going home until after her surgery.  We would get to hang out in the hospital just for fun for Sunday and Monday night even though Ellie had no medical need to be there.   There was a theory that this decisions was related to insurance. That if they discharged between the bowel prep and the colon surgery, insurance wouldn’t cover the bowel prep.  

The resident called the case worker.  She couldn’t help.  She called financial services.  They never called back or weren’t even in the office with it being a weekend.  I call Cigna.  I explain the situation and Cigna informed me that there is no reason why we couldn’t be discharged to home and return Tuesday.  I had her check with her supervisor who confirmed.  I also have a nice reference number and a note in Ellie’s Cigna account too to cover all my bases. 

The on-call GI doctor finally stops by the hospital room as we are watching Mickey Mouse’s Choo Choo Express for the 21st time (I am not exaggerating) and playing with sight word flashcards.  Apparently, she thought we were staying through the procedure because of 1. Insurance and 2. Because Dr. S said we might as well just stay.  Um, right.  The on-call GI doc calls Dr. R who is out of town who says “oh yeah, she can go home”.   

Finally!  Discharged to home on a clear liquid diet until Tuesday.

Tuesday: 

Surgery day. . . or not.  We presented to radiology at 7 am today for an abdominal x-ray.  This is to look at the state of her colon.  Is there too much fluid in there? Too liquidy?  That answer to that question is, yes, she is too liquidy.  So surgery has been postponed to tomorrow provider her colon is in the right state tomorrow morning. 

I nearly cried when I heard.  We have been waiting 6 months for this surgery and it is delayed again.  I am anxious that we will show up tomorrow and hear the same old story.  That her colon isn’t in the right state to proceed.  This entire process has been ridiculous!  At this point, it would have been much easier to have done it back in September laparoscopically.

Please pray, thinking positive thoughts, or send positive vibes that all goes well tomorrow.  That her colon is okay and that we can proceed with the procedure.

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A quick update on Urology/GI procedure plan

When I last left off, I mentioned that both urologists confirmed that they could perform Ellie's cecostomy and tube placement for Monday Sept. 12, but they wanted to discuss the surgical approach.  Well. . . I was confused because at our long awaited urology pre-op appointment, we decided on a laparoscopic approach with the possibility of needing to open her abdomen up completely.

Of course, the other surgeon was out of town so they couldn't not discuss immediately.  I gave 2 weeks and left a message this past Monday with the nurse.  Tuesday, I get a phone call from Ellie's secondary GI specialist, the one who performed the colon manometry back in June. He tells me that the procedure will be done in interventional radiology [IR] where they just "pop" the button right in.  It is less invasive and the hospital stay is closer to 48 hour as opposed to 4 days.  Obviously, this is all good, except, at our urology appointment in July, GI relayed to urology to not do it through IR because her colon may be too floppy from being stretched out.  What?!  So do they bring her into IR and if it doesn't work we need to relocated to the OR?

Friday approached and as usual, I never heard back from the urology nurse.  Dr. McQ, the actual urologist calls me personally.  Her surgery on 9/12 was CANCELLED back on Tuesday since her procedure will be performed in interventional radiology and not the OR. She assumed that I knew this already.  Urology is no longer in charge.  GI put in the orders.  Radiology is taking over. If GI was going to put in the orders, why didn't he do so in June?!  I immediately called GI and then googled the number for radiology.  Radiology never calls back, but GI does. . . only it is the other GI's nurse.  According to her, IR is booking into late November, early December.  I may have lost it.  I spent the afternoon angry crying.

first day of 1st grade

Timeline:

June 16th - referred to pediatric surgery by GI, then Dr. C in urology
Dr. C out of town, then takes another week to look at her case
Referred to Dr. McQ in urology and she is out of town
July 24th - urology appointment, tentative surgery date because need to coordinate with Dr. S
Dr. S it out of town so more waiting to coordinate
Both urologist can do the 12th, but need to discuss approach, Dr. S out of town
August 30th - GI calls to change plan
Sept. 2nd - surgery cancelled
No new date yet.

She lost her front tooth!

This is ridiculous!  If she gets booked in December that is SIX months since the ordinal order was put in.  SIX MONTHS.  My daughter is in pain.  PAIN.  She is uncomfortable and only goes with a special enema that I have to constitute myself.  We can only give those enemas twice a week which is not enough to help her.  Her medications do nothing.  She spikes fevers because of this.  She had to have a urinary catheterization due to being unable to pee and screaming secondary to constipation.  This is unacceptable.  I will be rattling radiology's cage.  I will cry and beg for my daughter, but it isn't like I can personally create an opening in IR.  I will contact a patient advocate in hopes of filing a complaint.  Other than that, all I can do is scream and watch Ellie suffer for another 2.5 to 3 months while they all had their thumbs stuck up their *sses.  It seems they all forgot that a real human child is involved.






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July Update: The Fun Stuff and The Medical Stuff

Believe it or not, there is not much to report.  July has been relatively tame with the usual therapies, fun summer activities, and only 2 doctor's appointments.

The Fun Stuff:

At the very end of June and into July, my mom (Baba to Ellie) visited from St. Louis.  We had a full schedule trying to fit in everything we wanted to do.  With each visit, we make sure to get breakfast with awesome coffee and head to the mall to ride the train.  It is tradition, you know!  We went to a 4th of July BBQ at my friend's house - the friend I have known since I was 8 years-old, the one who introduced me to my husband.  We had a great time and Ellie was very well behaved.  You all know what a feat that is.

Ellie and Baba

Ellie is still trying to hard to talk.  She is saying new words such as arms, kick, and fast (sounds like *ss).  Girlfriend certainly has her curse words down. Her speech therapist had me write down 10 of her most favorite foods so that she can work on these words specifically.  At the very top of the list is "cake". Right now, she signs it incessantly while making the "k" sound only.

4th of July BBQ

At the beginning of July,  Ellie participated in a dance camp for the children who are a part of the 2dance2dream program.  The Bear had a great time and I had 2 hours for 3 days to myself.  It was wonderful.  I did grocery shopping and ran a few errands.

Enjoying dance camp

Last year, we started swim lessons with this woman who works solely with kids who have special needs and their siblings.  Ellie made great progress last year and I worried that she would have lost all her skills during the school year.  Not so, she remembered!  As a child who is petrified of water (aside from baths and a splash pad), she is doing great.  With some assistance, she can swim across the pool.  It isn't super wide, but it is amazing progress.  She knows to hold her breathe and come up for air and moves her arms to propel herself through the water.  She still has trouble coordinating kicking and arm movements together, but that will come with practice.  She is also able to pick up diving sticks from the bottom of the pool.

The Medical Stuff:

Weight Gain-- that's right, our girl finally gained weight!  Nearly 2 lbs.  The Bear is going to be a chunky chicken once again.  Well maybe not but her ribs don't hang out as much.  She is still eating the same amount of food, but we started Carafate for her ulcers.  I am thinking she is finally healing and is now able to absorb more nutrients from her food.  Fingers crossed she keeps the weight on.

Urology: Six weeks people.  Six weeks to get a pre-op appointment!!!  Apparently, GI does not do the cecostomy ostomy and button procedure [Malone procedure].  Urology does.  So on June 16th, we got referred to urology.  She already has a urologist for her chronic urinary retention (which has resolved!  Woooohooooo!), yet she was referred to a different doctor, Dr. C.  Dr. C was out of town.  Once he returned, he took an entire week to get to her chart before referring her to her primary Urologist, Dr. McQ.  At this point, Dr. McQ was out of town.  She was pretty quick to review Ellie's chart and set up phone conversations and emails with Ellie's gastroenterologist.  However, the GI doc wanted to meet with the urologist in person.  They were going to meet up in 2 weeks.  Both docs conversed this past Tuesday and Dr. McQ worked us into the schedule yesterday.  It is a good thing we had a pre-op appointment as GI told the urologist that we just wanted to catheterize and not have a button.  Um, no.  We want the button.  This is why it is good to confer with all people involved in your child's care.

Now, there will be two urologists involved in this 2-4 hours procedure.  The goal is to do this laparoscopically, only Ellie may have a super floppy colon due to the musculature issues and being stretched out.  If they are not able to do it laparoscopically, they will open her up.  I am quite apprehensive about this.  I do not want to do this if they have to make a big incision, but we also need to treat her issues.  We won't really know what will happen until they get into the OR. So coordination for surgery is tricky as both surgeons need to be available for 4 hours on the same day.  Fortunately, we have a tentative date for September 12th.  Ellie will be admitted the day before for a bowel prep and then surgery in the afternoon on the 12th.  After that, she has a 2-4 day post-op hospital stay.  How will I keep this child occupied for that long?! Fingers crossed and prayers that she can have surgery during this time or we are looking at a much later surgical date that may be affected by insurance changes. Fingers crossed that our primary insurance covers this procedure, the bowel prep, and hospital stay (recall they refused to cover Ellie's hospital stay and manometry last month).

Hanging out at the Dentist

Finally, Bear had a blast at the dentist.  I don't know why other than the toys and getting her own tube of toothpaste.  She was fabulous until it was time to floss.  It took two of use to hold her down, but she was great for the exam, brushing, and fluoride treatment.  If only she was that good at home!

As for me, my uterine ablation from last mont failed.  I am looking at a hysterectomy come November.  I am not looking forward to this.  It is a big decision and I flip flop back and forth between going through with it.  It is a 6 week recovery which should be interesting while caring for Ellie.  I will definitely need help.




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Where to begin - manometry results and surgery

Oh where to begin.  I feel as though this blog has basically turned into medical updates for Ellie.  I apologize for that.  Please do not think there are no positives in our lives. There are!  How about I start with some of the fun, exciting things?

Ellie is trying to talk.  We don't always understand what she wants and if Drew doesn't get it he says "go ask mama".  If Ellie doesn't know the name of something, she says "ish" [this].  Usually she is pointing in the general direction and we have no idea just what "this" is.  The other day she said as clear as day "help please"!  She also has this flashcard with a clock on it.  Guess what she says?  Come on, guess?  Cock!  I shouldn't laugh, but well, it is funny.

Ellie is a big fan of bubble baths.  She constantly says, a clear as day,
"MORE BUBBLES"

Now that summer is underway, Ellie will be starting swim lessons.  We go to this amazing instructor. She teaches children who have special needs as well as their siblings in her backyard pool on a 1:1indiviaual basis.  Ellie made more progress in her first lesson last summer than in 6 weeks at group Y lessons.  She always wants to swim, but gets scared when the water goes above her waist.  I may have flashed a few people at a kids pool party when Bear clung to me like a spider monkey and pulled my suit down.  Whoops!  Not everyone wants to get a full show of my saggy boobs.

Horse therapy has been moved to earlier in the day to avoid the hottest time of the day.  Her beloved horse, Charlie, has been retired as he is considered "elderly" in equine terms.  She has been riding this sweet horse, Jake, and it has been going well. Jack has been taking a break this summer from therapy sessions which means Ellie is rather reluctant at first to get on the horse.

Here she is trying to make the horse go "fast fast fast"!

Playdates!  The nice thing about summer is the flexibility and free time.  We have been aiming for weekly playdates with Jack and his sister, Maggie.  You all know how much the Bear loves her "Hack".  This also means I get a much needed coffee gab session with their mama and my dear friend, Sheryl.

Onward to the medical stuff.  Bear with me her as I try to explain this in layman's terms.  Recall that 2 weeks ago was hospitalized for a series of tests.  One of which was a colon manometry, which looks at the motility (movement, peristalsis) of the colon.  Another was an upper GI which looks at the esophagus, stomach, and duodenum (upper portion of the small intestine).

Upper GI:
She continues to have esophageal gastritis, which is inflammation of the esophagus and stomach.  The biopsies revealed she is more inflamed that what was seen by the naked eye during the endoscopy.  She has been started on carafate (for ulcers) and will continue her protonix.  Here's the not so exciting part, the carafate's #1 side effect is. . . you guessed it! Constipation!  Yes, I am not kidding.  Consti-freaking-pation.  Because, you know, she needs more of that.

The biopsies again ruled out: celiac disease, lactose intolerance, and H. pylori.

Colon Manometry:
Basically test this looks at the colon contractions without food in the gut, after eating, and after stimulant laxative administration.  You can tell if it is nerve related or smooth muscle related.  Ellie's  manometry showed that she has low-amplitude decreased contractions.  Essentially, the smooth muscle of the colon wall doesn't not contract enough.  This means that her colon has trouble pushing stool forward and as such allows more water to be reabsorbed into the gut thus causing the stool to become harder.  Eventually, with chronic constipation, the colon stretches out which makes it even more difficult to push out stool.  Essentially, chronic constipation worsens constipation.

We learned that Ellie's colon has absolutely no response to the stimulant laxatives.  This is explains why all the medications she is on do not really help.  Before getting the official results of the manometry, we were to do a trial of high dose SennaLax (she was on a 1/2 dose before) to see if it would work.  It did not.  She was in so much pain on Tuesday and Wednesday due to being unable to go.  Crying, moaning, and clutching her stomach along with having a fever.  Perhaps a bit of colitis secondary to constipation?  It was the worst I had seen her in a long time and I was ready to take her to the ER when I couldn't get the enema fluid in at first.  I finally got the enema in and it helped, but we cannot keep violating her like that.

Anyway, when the doctor called with the results, we were referred directly to pediatric surgery.  Three surgical options were discussed, but both GI docs and Drew and I went with the one that would be most appropriate for our family.  (As in an ileostomy would not work because Drew wouldn't change it and Ellie would remove the bag getting liquid poop everywhere.  I'd be performing 24 hour surveillance.)

We are going with the Malone Procedure or ACE which stands for antegrade colonic enema. Antegrade means "above".  Enemas traditionally go through the rectum and only clear out the lower portion of the colon and do not affect higher up.  This doesn't truly treat the constipation.  By shooting an enema at the beginning of the colon, the colon can remain flushed and clear.  Plus, Ellie could come off all her laxatives and perhaps her urinary retention would be resolved. To do this, the appendix is brought to the surface of the abdomen and a button is placed (called an appendicostomy).  A saline enema is administered through the button It is scary, but we have learned that all the laxatives in the world, dietary measures, natural remedies, and behavior management are not alleviating the constipation.

Photo: www.nationwidechildrens.org

At this point, we have been referred to a different urologist to the one that we already see.  He is the one who will perform the surgery.  His medical assistant has been out all week and they are not sure if they can work her in.  Their first available appointment is in nearly 2 months.  Then who knows how long before surgery is booked.  I was really hoping to have this done before school starts, but it isn't looking that way.

A week ago, I had my surgery and it went well. The recovery period was only 3 days which I am relieved because I was told to allow up to 2 weeks.

So that's the big scoop.  Life is never dull in the Theurer household.



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Hospitalization and Procedure Update

Last week Ellie had her 3 day hospital stay.  It took a while to get to that point.  There was a lot of organization on behalf of 2 GI specialists, Infectious Disease, and Pulmonary.   Cigna denied coverage for the bowel prep and colon manometry stating that it was "experimental" and that even if it was covered, they would not consider an overnight stay.  Right. . . the bowel prep was needed to clear the colon for the colonoscopy and then the manometry gets performed the following day to allow the bowel "wake up" from the anesthesia.  There were appeals on my end and the doctor's end and panic. Fortunately, we have a Medicaid Waiver for children with disabilities program called MDCP.  Not many of our kids with Ds qualify anymore, but Ellie does and surprisingly, Medicaid approved her procedures.  So clearly not experimental.  The thing is, even if it wasn't covered, we would have found a way to make it work.  She needed those tests.

Disclosure: I know that there are a lot of new parents of children with Down syndrome who read this blog.  I do not want you to think that what is going on with my daughter is the future for your child.  Ellie's issues are unique to Ellie.  They are not related to Down syndrome.

I made sure to pack all the important things for a hospital stay!

Ellie did totally awesome during her stay.  As in she was well behaved aside from trying to yank out her NG tube and she stayed in bed for the entire manometry study (6 hours!).  She did great with anesthesia and she SLEPT!  Now I think the first night was because she was up late, had versed, and then was up a lot in the middle of the night.  The next night might have been due to post-anesthesia, and fentanyl.  At home, she is still continuing to wake all of us at 3am.  (It was 2:30am before the hospitalization). She is, of course, up for the entire day.  Yes, awake from 3am - 7pm.  That is another post entirely

Top right: you can see how distended her tummy is from all the GoLytely

So. . . we still don't really have answers.

Bowel Prep:
This was to prepare her for the colonscopy the next day.  An entire gallon of GoLytely was run through and NG tube and was, in theory, supposed to clear all the crap out of her intestines.  I say "in theory", because it took way longer than anticipated.  So long that she wasn't fully cleared out in time for her surgery!  Dr. S called it "good enough" and opted not to post-pone the procedures.

Brain MRI with Contrast: Normal!
This was performed because Ellie had a new finding of central sleep apnea on her repeat sleep study from March.

Blood Work:
10 vials, my friends.  Then it still wasn't enough so another vial was drawn.  We looked at Thyroid levels, Ha1c, CBC, metabolic panel, inflammatory markers, celiac, and IgG/IgM/IgA gammaglobulins, etc.
Of the blood work that is back, all is normal except for a mildly elevated thyroid stimulating hormone [TSH].  After a brief in-hospital endocrine consult, it was recommended that we repeat her TSH and free T4 in 2 weeks at the pediatrician's office.  I just love adding more doctor's appointments to our schedule. Hypothyroidism is very common in Ds and it is often said a "not if, but when" they get hypothyroid so I am not overly concerned.

She wasn't able to eat during the bowel prep until after her surgeries so 32 hours without food.
The donut was awesome!

Upper Endoscopy aka EGD:
Back at the end of January, we did an upper endoscopy to definitively rule out celiac and lactose intolerance.  All came back normal, but her esophagus, stomach, and duodenum (upper part of small intestine) were inflamed.  We stared her on Protonix which is a proton pump inhibitor for ulcers and reflux.  Last week, we did a repeat EGD to see if the Protonix worked.  I am happy to report that the esophagus and duodenum look good and her gastritis is now mild.  The doctor also took 8 EIGHT biopsies of the duodenum as opposed to the usual 2 to double check the celiac screen.

Someone was pretty excited when she found out the bed could move.

Colonoscopy: Normal!  No need for any biopsies either

Colon Manometry:
This was the big test to determine how Ellie's colon functions - think contractions or peristalsis.  Is she missing nerve ganglion in the colon, delayed transit, etc.?  So. . . it was mostly normal.  No absence of nerve cells or other motility stuff.  She could not eat anything for over 8 hours before the test.  She showed normal colon contractions at the beginning.  Two hours in, we gave her food.  Her colon showed an appropriate response.  An hour later, they gave her laxatives.  Her colon did not respond, at all.  Not a real shocker.  There is no explanation for her severe constipation.

Colon Manometry or Colon Motility Study set-up

So now what?
It will be another week or so before we get the biopsy results.  It will be another week before we get the full Manometry results.  In 2 weeks, we repeat the thyroid panel.  We are doing high dose stimulant laxatives (as opposed to her osmotic ones) for 2 weeks on and 2 weeks off.  You cannot do stimulant laxatives every day because the gut gets dependent on them.  We follow up in 1 month.  If not a big improvement, we have to make some big decisions.

Severe constipation is a huge deal.  It has lead to Ellie's weight loss.  There is an increased risk for gut infection, intestinal perforation which can lead to sepsis.  It is causing Ellie's chronic urinary retention, which puts her at high risk for UTIs and even kidney damage.  This is Ellie's life.  It is getting worse, not better.  We have tried everything natural, medical, and everything in between. I was really hoping for more answers so that we would have a "fix" a good treatment plan.







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A bunch of updates: Miss Sassy Pants, Sleep surprise, GI/Urology, etc.

Ha!  I am going to spare you my usual spiel of "I am so sorry that I neglected the blog and I won't do it again".  However, I will promise you that this post isn't entirely about medical stuff. . . sort of.

The Sleep Study Results:
Why does it take so long to get results?  Three weeks to get the results.  Actually, it was 2 weeks, but the doctor neglected to call me because he was flabbergasted and needed to do a more comparative analysis of her previous sleep study.  Yeah, "flabbergasted" is never something you want to hear from a doctor.

To see her previous sleep study results (as in pre-surgery), click here.  Ellie Bear's hypoventilation (very low rate of breathing) has resolved!  As a result, her inhaled CO2 levels are near normal.  Her oxygen saturations for the majority of the night ranged from 92-94% (compared to 80% in the previous sleep study.  In more exciting news, she now has central sleep apnea.  Girlfriend can never doing anything half-*ssed and likes to keep us all guessing.  So. . . where did the central sleep apnea come from?  We do not know, which is why we are going to do an MRI of her brain just for sh*ts & giggles. . . can you all tell that I am just sick of this stuff.  Why the MRI, you ask?  Central sleep apnea tends to originate from the brainstem (or in some cases the heart).  Essentially, your brainstem forgets to tell your to breath.

Speaking of sleep apnea, I have it and so does Andrew.  A few months ago, Drew woke me up because I "wasn't breathing".  My allergies were horrible and he basically shoved Afrin up my nose and it cured me.  Last month, I was in Vegas with some friends.  Vegas, baby!  The cigarette smoke also mucked up my breathing and my friend informed me that I was obstructing.  Because I don't have enough doctors appointments before school lets out in 2 weeks (I have 5 and Bear has 2), I now have an appt. with my PCP.  I am wondering if this is why the past 3 month I have been averaging about 3.5 hours of sleep?  Very restless sleep.

My Fitness Best Friends (and texting buddies):
Michelle (my Ds blogging buddy and first time meeting her!)
Lindsey (former Canadian, former Austinite)
Lisa (fellow Canadian Beachbody coach!)

Motivation Fitness Takes Vegas:
Oh yes, my friends, I went on a girl's trip to Vegas.  That would be a trip without the child.  As in alone.  I am still a beachbody coach running my health and fitness group called Motivation Fitness.  Our Facebook group is full of amazing ladies.  We not only share our victories and support each other during health-related struggles, we have become friends.  Many of us had never met.  Last month, 10 of us took a 4 day trip to the Sin City.  From Vancouver, Canada, Montana, Florida, New York, Texas, Missouri, and Ohio, all of us had a wonderful time.  While we managed to clock in an average of 24,000 steps a day and while we also were a health group, it was our main mission to eat (and to pee -we are all moms with weak bladders) in every hotel room along the strip.

School: The IEP meeting
Nothing screams "welcome back from vacation" like an IEP meeting the morning after your flight lands.  The IEP meeting went well.  We have good goals in place.  Ellie will spend a majority of her time in a Functional Academic spec ed classroom [FAC], with appropriate push-ins for specials, lunch, and home work and other classes as tolerate.  The "as tolerated" = behavior is good.  On a sad note, Ellie will not be at the same school as next year.  Currently, Ellie goes to a school that is 30 minutes away.  While that is not ideal, her teacher and aides were/are amazing.  Next year, her home school that is just down the street from our house is going to have an FAC room.  Fingers crossed that we have an excellent teacher and aides next year.  Change is difficult.  Being shuffled around from different schools, different classmates, and different teachers can be tough on Ellie.

The Dysfunctional Voiding Clinic: NPs vs MDs and Gastroenterology + Urology
I love joint appointments.  It is so nice to kill 2 specialists with 1 stone.  As a follow up, Ellie is now peeing more.  As in 3-5 times a day now, compared to once every 18-29 hours.  Unfortunately, she likes to do things the opposite of "normal".  When she poops regularly, she doesn't pee.  When she doesn't poop, she pees more.  Go figure.

The Bear has lost weight again.  Not much, but this child should be gaining.  Like a ton.  She is on an appetite stimulant and she eats all the freaking time.  At first I figured "different scale", but after having 4 doctors appointments in 10 days and all 4 scales showing a similar weight, it could no longer be ignored.

Play-Doh Fun!
You can see that she lost a big of weight in her face.  We have also gone down a size in clothes.

This brings me to the awesomeness of Nurse Practitioners.  No, I am totally not biased given that I am also a NP.  I have battled with Ellie GI doc for a few years now.  Always dragging his heels on trying to figure WHY she has severe constipation and WHY she loses weight.

GI NP: She has had a colonoscopy, right?

Me: Nope.  She needs one and will get one next month with her testing

GI NP: She has had stool studies, right?

Me: Nope.

GI NP: How about an upper GI?

Me: Yes!  Just a few months ago after begging the doc for one for 2 years.  We ruled out celiac, h. pylori, and lactose intolerance and discovered esophageal gastritis instead.

So, we did a bunch of stool studies to look for malabsorption.  Good news!  The studies were normal.  Bear's poop is normal.  Yay!

Disney On Ice:  
Two weekends ago, we took Ellie to Disney on Ice.  I wasn't sure how she was going to do during the 2 hours show.  She was so excited!  Before it even started, she was clapping and grinning.  Her enthusiasm was priceless and infectious.  She did great. . . provide Mickey Mouse was on the ice.  When he wasn't, Ellie was telling us "truck, buh bye, Noble Pig".

This all brings me to next month: Surgeries and Hospitalization:

It is always great fun to kick of summer break with a 3 day hospital stay.  Right?!  Okay, this is gross, but we will be admitting Ellie to the hospital for a bowel cleanse.  Think dropping and NG tube from her nose to stomach and pumping her with a full gallon or two of GoLytely.  The following day, Ellie will have a colonscopy and the placement of a colon manometry tube.  Essentially this tube will remain in her colon and be taped to her butt or thigh until the following day when we will hook it up to this manometry machine.  While she is under anesthesia, she will also have her brain MRI with contrast and her yearly routine blood work.  The following day, is the colon manometry which will look at pressures throughout the colon - identifying if there is delayed colon transit and if so, if there is a specific part of the colon where this occurs.  Then, home!

The following week, I get to have surgery.  For the past 2 years, I have been battling woman's health issues.  A variety of treatments have been tried with no relief.  More drastic measures will be taken and I will essentially lose my fertility.  No more kids for us, so if one more person asks if we will have more. . . I feel like I am way too young for this nonsense, but at 35 years-old, I am apparently of
"advanced maternal age" even though I have quite a few older friends who are currently pregnant.

Haircut:
I cut the Bear's hair myself.  We keep it short for a few reasons: 1. She chews her hair; 2. It sticks to her messy Nutella face; 3. Drew loves it short; and 4. It gets scraggly when it is longer.  The reason I am the one to cut her hair is because she hates it.  It is like medieval torture to her.  She does okay with the back, but the sides freak her out.  Perhaps the sound of the scissors? Or maybe having a sharp object near her face?  Sensory issues?  The thing is, if I take her somewhere, she is still a wild banshee child and no amount of fun toys, movies, little riding things help the matter.  I end up paying for the haircut that looks just like my hack job and I feel the need to tip a ridiculous amount because the Bear is a tornado.

Tama Boo:
In sad news, our beloved, black cat, Tama, has been missing for a week now.  She is usually indoors by 9pm and comes when called.  There were storms last Tuesday night and Tama is petrified of thunder.  I have called/ searched for her, all the neighbors in a 1/2 mile radius are looking for her, and I have posted her picture on Next-door.

So even though it sounds like there is a ton of chaos over in the Theurer household, we are staying afloat.  Bear's new favorite word are "oops" and "buh bye".  Any time someone drops something "oose!" [oops].  Any time we leave a room, a toy, or someone leaves a room, it is "buh bye" with great drama and a princess wave.

School is almost over and I am really looking forward to July.  When all this medical stuff is behind us and when I can sleep past 5:45am. . . and instead wake at 6:45am.






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