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.

Everybody poops. . . or not

Welcome to another episode of "Why Won't My Child Poop?" 

Today's cast of characters feature Ellie Bear, a distraught Mama Bear, clueless ER staff, members of the Comprehensive Care Clinic, and a jolly x-ray technician.

Want to know why this little Turkey is smiling?  This is outside Dell Children's Medical Center.
 She was there for her appt with Dr. Simon.  Elli is in luv with him. 

First, a little background:

I am unfortunately known among many parenting groups as "the poop guru" (seriously who wants that title as their claim to fame?) as I can pretty much get any child to poop. . . except my own.

Remember way back when Ellie went 22 days without defecating?  Well, by end of August we were back to that.  Okay, that is an over exaggeration as this go around it was more like 6 days, but given her surgical intervention from 2 years and 9 months ago, constipation shouldn't even be an issue.

Sadly, all of my experience as a former pediatric nurse practitioner and working closely with her gastroenterologist pretty much amounted to big amount of nothing in the Bear's land of excrement.  For those of you who have been following this blog for a while, then you are aware of such lengths we went to in the past to regulate Ellie's GI tract and we now know that she has what is called moderate-to-severe delayed colonic transit.  That's a fancy way of saying Ellie's colon doesn't move stool through it, but instead sits there getting harder and harder thus distending the colon.  If you really want to know the entire story as to what interventions, diagnostic tests, and whatnot that we have tried,  you can search this blog for "constipation" or "cecostomy" or click on the label on the right side bar or if you are feeling extra feisty, google "cecostomy" and this blog pops up.

Cecostomy

For those of you who are new to the Bear's adventures, Her Royal Stinker Butt had a cecostomy performed surgically back in December of 2016.  The purpose of this tube is that it allows for high volume enemas to be given directly into the top of the colon, thus flushing her out.  There are many types of enemas, but we were pretty much sticking with saline.  In theory, I know isn't everything great in theory?  Anyway, in theory, these enemas would keep her colon mostly empty and thus preventing her from getting constipated.  For the most part, it worked . . . until July when we were finding ourselves have to near daily enemas via the tube and even some rectal ones with minimal results.

Medications that cause constipation:

Do you ever read the side effects of medications?  Nearly every single of one of them go like this:

• Headache
• Diarrhea / Constipation
• Nausea
• Fatigue /Insomnia

So . . . Ellie Bear lost weight about 18 months ago.  Some of it was expected because we took her off of the Risperdal which caused excessive eating and excessive weight gain.  What we didn't expect was the amount of weight that she lost - over 7 lb.  Then that was it.  She just stopped growing for the most part.  She was still eating close to 1800 calories/day which is a lot of a child her age with Down syndrome (people with Ds have about 10-20% slower metabolism compared to the general population), but other than growing in height a little less than an inch, she hovers around 46 lb.  She is now 10 years-old.  She is not on the growth chart for weight while she is 18% for height on a Ds chart.

First vs. Last day of school last year

Where am I going with this failure-to-thrive nonsense?  Appetite stimulants!  That's right, GI has determined that she is 5-10 lb(!!!) underweight and she is probably a kiddo that just has a high metabolism and thus needs more calories. (E has a history of failure to thrive and again, there are several posts on this blog that address the FTT and determine what was the cause). Therefore, we started her on cyproheptadine (Periactin), which is a prescription antihistamine that works as an appetite stimulant (and along a migraine preventative FYI).  Again, where am I going with this?  One of the top side effects is. . . ding ding ding freaking constipation!!!

Mismatched shoes are in vogue right now - as per Fashionista Ellie

The point of this blog post:

Okay y'all.  Now that we've establish that my daughter was no longer excreting fecal matter on a regular basis thanks to a medication, she ended up in the ER.  After 2 months of never getting fully cleared out and then 6 days of nothing at all.  Absolutely nothing, I didn't know what else to do from home.  Saline, mineral oil, glycerin enemas.  Enema through the tube.  Rectal enemas.  We maxed out on what we could give her without causing phosphorus poisoning.  She looked pregnant and her stomach was hard.  The gas though. . . this child was tooting like her life depended on it.  The house and classroom smelled like cabbage and dead decaying squirrels.  Believe it or not, this was a good thing because it means there was no obstruction.

So there we were hanging out in the ER for fun and the resident walks in.  I start to relay what all I have shoved into this kid's colon (which would have been the equivalent of a colonoscopy bowel prep for at least 4 adults) and I am getting the whole skeptical "so this is all rectal?" and "are you sure she hasn't gone to the bathroom with all of that?"  Gah!  What part of tube in her colon or cecostomy tube were they not getting?!  After explaining it all yet again, I finally hear:

"So what is a cecostomy tube?"

Now granted, this was a resident and she was still learning and cecostomies are not common so I went with the:  "So a cecostomy is when  the appendix is brought to the surface of the abdomen and it is used for high volume enemas to essentially flush out the colon ..... and here are some pictures. . . "  For tips on preventing your child from pulling out tubes (i.e. g-tube and/or cecostomy tube), click here.

An x-ray was ordered to rule out bowel obstruction. Because the x-ray tech was a male, Ellie wanted to show off her awesomeness and be-bops over to the exam table for her x-ray and poses like she is a fashion model.

Then nothing.  I hear nothing!  The attending never comes in.  The resident doesn't come back in.  However, the nurse comes in with another enema which we could have done at home and I ask her for the x-ray results which to summarize involved a large volume of solid stool (no kidding, Sherlock). Let us not forget the 1cm x 1.5 cm foreign body that left me sighing and saying "again?"!  Twinkletoes has a history of this - see here.  Of course, they are all, "she'll poop it out." except my kid wasn't emptying her bowel at all!  After painfully pushing out the equivalent of 2 small meatballs, we were discharged without ever seeing the attending or resident.

The following morning, I informed GI that I would no longer be administering the cyproheptadine to Ellie.  The ER visit was August 26th and it wasn't until Sept 9th that things finally became regular.  It should be noted that while it appeared that Ellie gained 2 lb while on the appetite stimulant, she has lost those two pounds.  I am taking a survey on how many ounces you believe was stool.  Also of note, I never did find that foreign body.

~~~~~~~~~ 

*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.

How to prevent your child from pulling out their g-tube / cecostomy tube

For those of you who have been following along on this blog for a while, you know that some of my friends and acquaintances have referred to me as the 'poop guru' - yeah, not exactly what I want to be known for!  Thanks to Ellie's unique gastrointestinal system, I have learned just about everything there is under the sun to relieve constipation and get a child to defecate regularly. . . except my daughter, that is.  Anyway, this blog post comes about because on Friday, I get this call from the Interventional Radiology nurse.

IR: Anna, remember when Ellie pulled out her [cecostomy] tube?

Me: Unfortunately.  I'd like to erase that experience from my memory.

IR: She hasn't pulled it out lately (*me: Thank goodness!) and I need to know how you what you are doing because we have a child who keeps trying to pull his out.


Well okay then.  Here are a few easy strategies that have barricaded the Bear from deftly removing her indwelling colon tube:

Check that out!  I think that this is the first pic that I have shared where the  tube isn't infected and there is no granulation tissue.  Gorgeous!

For all of you The Chronicles newbies - this is a Chait Trapdoor aka cecostomy tube.  It is a button/tube goes into the ascending colon and is used to administer very high volume enemas.  Twinkletoes here thought it would be fun to only have a bowel movement every 12-22 days even with multiple dietary, lifestyle, and pharmacological help.  Puking because you have stool squashing your stomach generally isn't a good thing. That is what happens when your colon has moderate -to -severe delayed colon transit.

Moving on to what this blog post is actually about!  After Ellie pulled the tube out, I tried using those belly bands that you could get off Etsy for kiddos with g-tubes and I tried making them myself.  As adorable as they were with their fun cartoon characters and bright patterns, they didn't work for the following 2 reasons:

1. The belly band would either bunch up with movement or completely ride up on her stomach above the tube.  I think that it would have been fine if it was a g-tube, but since the cecostomy is lower on the abdomen, it just wouldn't stay covered.

2. She could take them off.  Mainly because it was fastened with wimpy velcro.

What I am about to tell you will just blow you away - okay, it most likely won't.  I went over to Amazon, my online addiction, and looked at those surgical abdominal binders that people use after pregnancy and gut surgeries.  These things are full on elastic and have the industrial strength velcro.  The problem was that I bought the generic one-size-fits all and I had to cut it in half width-wise and remove a significant portion of the length.  Then the edges would fray.  The link below is for the brand that works best for Ellie and has various option sizes. It is latex-free and it is available on Amazon Prime. (heads up: while it is Amazon Prime, it usually takes 1-2 weeks to arrive, but hey, you get free shipping!) Ellie wears the small. She is currently the size of a very skinny 6 year-old child at 44 lbs, but she did wear the same size when she weighed 7 lb heavier [someone explained to me why no one is concerned about her weight loss?! Yes, she lost weight a year ago when she stopped taking Risperdal, but still!].

Abdominal Binder by Trademark Supplies

That would be crayon all over her binder.  Once upon a time, I labeled her binders with a sharpie for Camp.  She now thinks all of her binders should say "Ellie" so she grabbed crayons and wrote her name all over it.  

Ellie loves the binder and I think it is because it provides a bit of compression - sensory input.  

But what about when my kid is in the bathtub or shower, you ask?  Turkey Bear wears a one-piece swimming suit for bathing.  Now I know that for all the boys out there, it wouldn't exactly be fashionable, but I will tell you it works!

There is one more thing that helps detour her from yanking out the tube.  Ellie's tube is too big for her and so it is moves around a lot and leaks horribly - gross!  Because of this, we keep a piece 4 x 4 non-woven gauze folded into quarters and secured with paper tape over it.  (if your kiddo has sensitive skin like Ellie Bear - use Critic-Aid AF around it before covering and stick with paper tape).  Notice that I said non-woven gauze.  Woven gauze, which is most commonly used in the hospitals, tends to get stuck on the tube and then you are at risk for accidentally pulling it out when you go to remove the gauze. 

4 x 4 non-woven gauze

Look at that horrible bandage job of mine!  Did y'all know that I used to be a pediatric ICU nurse?  That means wound care was in my job description!  No one said it had to be pretty.

Fingers crossed that some of these tips will help prevent your kiddo from yanking out his/her g-tube or cecostomy tube!

   

Updates: Happy New Year, insurance companies, psych/ADHD

I know. I know.  I said back in October that I would write about Morgan's Wonderland (an all abilities theme park) in San Antonio and Sea World.  It is partially written and then I forgot because life was busy happening. I promise though, I will get the post up hopefully soon.

I hope that you all had a great holiday and New Year!  Earlier in December, my parents came to visit and we had a nice time dining out for family dinners (Jack Allen's Kitchen and the historical Threadgills), visiting Santa, and decorating the tree.  On Christmas Eve, Bear's best friend, Jack, and his siblings came over to bake and decorate cookies.  I think that there were cookies buried under the mound of icing and entire bottle of sprinkles.

My life seems to be full of nothing but doctor's appointments and phone calls to either insurance companies or the CDS company that manages our respite care provider's paycheck.  Last week, it was hours of taking politely, but forcefully on the phone with both.  In some ways, I am happy to have a degree in health care because I know how to approach things or when something isn't right.  On the other hand, none of this is my responsibility.  

MEDICAL PROCEDURES AND INSURANCE ISSUES

Ellie was scheduled to have her cecostomy tube replacement, CT angiogram of her abdomen, and an ABR (a fancy hearing test) under sedation on January 4th.  I was so proud of myself for getting all 3 coordinated during winter break.  Nope!  Due to Ellie's hospitalization last month for RSV, adenovirus, pneumonia, asthma, and a pleural effusion, her procedures all had to be rescheduled. I also had to reschedule her pre-op surgical exam. I am so thankful that the interventional radiology nurse was able to get them all together on the same day.  That meant I had to contact GI, ENT, and nephrology to get new orders/authorizations.  Again, this should not be my responsibility.  

Of course, things could not go smoothly.  Two Fridays ago, I got the letter from her disability Medicaid that the CT scan of her abdomen was denied .  It is always on a late Friday afternoon after the offices close and on a holiday weekend. Apparently, she needed to have seen the doctor in the last 60 days and have submitted the reason for test and recent lab results (this sort of seems like common sense to me when ordering something).  I wasn't worried about seeing the doctor as we were seeing the nephrologist this past Monday.  As for the labs, it was unclear if those too needed to be within 60 days.  My concern was having to get them re-drawn.  Back in September, the Dell's lab neglected to tell me that the renal bloodwork needed to be sent to an outside lab an oh, they decided to send it to an out-of-network lab!  I didn't know this until Cigna sent me a letter about it.  The 20% out-of-network coinsurance that I would have been required to pay would have been $1,200!!!!

Ellie's particular disability Medicaid contracts with EviCare for radiology.  The number for questions and appeals on the letter was for EviCare.  That person was incompetent and told me that I am supposed to go through the Medicaid.  The Medicaid rep who was really nice, said that because it is through EviCare, they have no record of the order or denial.  She offered to call them for me.  Gah!  Fortunately, one phone call from the nephrologist (who, by the way, called the same number as me) spoke with the medical director who turned over the decision.

Ellie and Jack

WHY ISN'T MY CARE PROVIDER GETTING PAID?

I actually have no answer for that.  Ellie is approved for respite and PCS services through MDCP.  The consumer directed services company that we went with is paid $202.17 through her MDCP budget to manage K's paychecks.  After years of things going relatively well, K started getting paid for only some of her hours - 3 missing paychecks in the last 4 pay periods.  Sometimes the PCS check was missing while other times it was the respite paycheck. The team members assigned to Bear's case would not return phone calls so I had to speak with a supervisor each time. I would be told that they didn't receive the email for x timesheet.  I will tell you that is BS because they were sent in the same email.  Then their excuse of must be an attachment issue or a computer glitch. Well, I started to send them together and separately.  This last one took the cake because I resent the timesheet 4 times, spoke with a supervisor and emailed him the timesheet who then forwarded it to her two team members.  Nothing.  K still wasn't paid.  I call the team members and left another message followed by calling their direct supervisors.  So I left 4 unanswered messages.  It finally got worked out, after  I threatened to contact Health and Human Services for mismanagement of Medicaid funds.  Clearly this was unacceptable so now I am spending time on the phone this week trying to switch over companies.

MOOD DISORDER AND ADHD

Did you know that stimulants can cause UTIs (urinary tract infections) in about 5% of those who take it?  Yeah, me neither.  Ellie had been on morning Adderall for about a month and it was working great aside from the whole wearing off after 2.5 hour part!  I was so worried about the tics given the debacle 10 months ago, but it never happened.  We ended up trying Adderall XR and well, let's just say the school asked me if I stopped her ADHD medications.  At this point, she went from being almost completely potty trained to urinating every 45 minutes with complete saturation through her pull-up and clothes.  

While at neuropsych, we tested for an UTI and determined that the increased urination is the result of Adderall XR (of course, increased urination is not a known side effect of Adderall XR) and that it wasn't working because she was peeing it out.  She is now on 2 doses of short-acting Adderall a day.  It is completely out of her system by 1:00pm so it should not affect her ability to fall asleep . . . yeah, you see where this is going.  

However, she started to have her weird sleep issues of taking either 1 hour (this is her norm) to 3 hours to fall asleep.  She'll go 3 or 4 nights of not sleeping to a few nights of good rest. This is even after an increase in her sleep medications.  She is now super hyperactive and impulsive.  Now we need to determine if it is ADHD for the hyperactivity or has she has she gone into a mixed state over as a result of making stimulant medications.

You can read more about E's history of ADHD management and her Mood Disorder:

Abnormal reactions to common ADHD medications 

DNA testing for psychotropic medications

Uncontrolled movements due to Vyvanse / Adderall XR

Thinks she's awesome because she is wearing my PJs

BUST KNEE WHILE AT THE DOCTOR - sorry, y'all this post is getting long and I am getting finger cramps so I will write about this and some other fun stuff in my next post!

The Guilt

guilt

ɡilt/

noun

noun: guilt

1 1.
the fact of having committed a specified or implied offense or crime."it is the duty of the prosecution to prove the prisoner's guilt"

2 synonyms:

3 culpability, guiltiness, blameworthiness; Morewrongdoing, wrong, criminality, misconduct, sin "the proof of his guilt"

Guilt is a funny thing.  Based off of the definition, I needed to have committed and offense or crime.  The fact of the matter, I feel guilt and yet, I haven’t exactly committed a crime.  Instead, I feel guilt over what I have been putting Ellie through these past 2 months with her ADHD.

I’d love to tell you that we found the right medication and she is now able to sit still, is less impulsive, and is no longer easily distracted, but that would be a big, fat lie.  It has been ADHD baptism by fire medication roulette . . . and I did this to her.  I am the one who wanted to take her off the Risperdal. I had great reasons, but still, I am the one who took away the medication that was working for her.  This goes to the saying “if it ain’t broke, dont’ fix it”.  Well it wasn’t broken and I haven’t fixed it.  Who suffers for it?  My innocent little girl.

Sometimes all you can do is cry in the Starbucks bathroom.

It’s funny how I felt like the sh*t hit the fan on Wednesday with all the following that happened before 10:30am, mind you, and then you lose your calm demeanor a few days later.  Yep, that is what happened. Now of course on Wednesday I was all ready scream out some unpleasant words, but I am trying to be all ladylike and whatnot here in this post.  

Wednesday consisted of a cardiology visit for a high heart rate because endocrine freaked out over her HR of 120 (normal for 8 year-old is 70-110 beats per minute [bpm]).  Ellie’s daytime baseline is 10-137, so yeah, it is rather high.  However, it should be noted her HR has been consistently elevated for the past 3 years and not 1 of her doctors ever cared.  Bear successfully made it through the EKG and echo, but cardio gets the bright idea to do a 24 hour holter monitor to monitor her heart rate and rhythm.  Um. . . right.  Surprisingly, Bear kept the monitor and the 5 electrodes on the entire time. Obviously , I felt it necessary to get that puppy attached to her in such a way she would really have to work to get the thing off. I had tons of tape, 2 abdominal binders, and two shirts to help keep it all on. . . well sort of.  Because I love drama, when I went to remove the monitor, I discovered that the top two electrodes we not completely snapped on!  A few more inappropriate 4 letter words popped out.  Did it happened when I was pulling off the tape?  Did it happen in the middle of the night during her wild acrobatics while sleeping?  Or was it like that the ENTIRE time after the med assistance set it up?  

Holter Monitor

Of course, because she doesn’t have enough going on, I noticed she had thrush (oral yeast infection) while screaming during her exam.

Oh great!  So I call the PCP and beg her to just prescribe the Nystatin over the phone and not have us come into the office.  Okay, I didn’t beg, but I totally would not have been above begging.  Seriously though - Thrush!?  I know it is from her steroid asthma inhaler, Qvar.  It can be a risk which is why you are supposed to wash your mouth out afterwards.  We did that!  

On top of that (yes, I am still on Wednesday), she stank.  Badly like stinky feet mixed with sewer sludge [don't worry, it was better the next day].  I was trying to figure out where the rank odor was coming from.  It was emitting from her cecostomy tube!  It always smells a bit there is some liquid stool back up, which is why we keep it covered with 4 layers of gauze.  Oh no, this smelled way worse.  The ostomy site didn’t look infected and she didn’t have a fever so I didn’t freak out too much.  I decided that rather than figuring out who I am supposed to call - radiology, GI, PCP, wound care nurse, or ostomy nurse, I would do the wait and see approach.  Thankfully, the damn tube which, by the way, does its job, but I bloody loathe with every ounce of my being, will be replaced under anesthesia on the 23rd.

Cecostomy tube: aka Chait Trapdoor.
The surrounding redness is granulation tissue.

Oky, back to cardiology.  The doc refers us back to nephrology for high blood pressure and high heart rate.  For real?  We are really really really trying to go for an even dozen number of specialists here. Some people collect baseball cards, Ellie collects doctors. In her defense, this is something cardiologists typically do and also her urologist referred her back in October (I sort of ignored that recommendation - sigh not sort, I purposefully ignored it).  However, since April, the 3 years of high BP resolved it self.  Her BP has been normal for months!  So here I am trying to figure out why I need to make yet another doctor's appt.  I mean, Bear asks to go to the doctor because she likes the stickers, but I don’t!

Did I mention that all of this happened between 8:00-10:30am?  

The circle stickers on her face are to keep her oxygen tubing in place at night.
They last for 3-4 days so that is why we leave them on.

So back to the whole guilt thing.

The last time I wrote, Bear was just coming off the Vyvanse due to uncontrolled full body movements and tics.  We decided to try Adderall XR.  Things seemed to be going okay.  School said her behavior has been great, but it was wearing off after 3 - 4 hours.  We saw some oral and arm movements, but she had some back before we ever started stimulants with her 4 years ago.  We increased the dose of Adderall XR [long acting] and later added in a booster dose of Adderall [short acting]. Bear started to get up before the roosters at 2:00am every morning and not falling asleep until 7pm that night.  Hindsight is 20/20.  Years ago, this sleep disturbance was happening.  I never attributed it to the Adderall seeing as Drew and I both have insomnia and she ended up having a sleep study showing apnea.  So the movements continue.  We stopped the Adderall XR and switched to Adderall - AM and afternoon.  Friday afternoon I speak with Ellie’s aide.  Yes, her behavior is great but she is still distracted thus requiring lots of redirection, but her facial movements are mostly gone while she still has some arm movements.  Time to call neuro. . . . again.  Then it happens.  Midway through her after school ABA session, the oral movements return along with the body movements.  Okie dokie.  Time to stop all stimulants immediately and send another frantic message to neuro.  

Ellie was on Adderall for 3 years without anything other ADHD medication.  For 3 years, it was a miracle drug.  So what happened?  Were the movements occurring because the Adderall XR was still possibly in her system?  Or did her brain get rewired and it was the Adderall?  I don’t know and frankly, I don’t care.  There are tons more medications to try, but I am done.  Bear is done. She needs a break.

I don’t know about y’all, but I am going to decompress with a chemed from the Red Horn Coffee House and Brewery Co, a book, and later, an excursion to an office supply place - there is just something about those post-its and colored pens.

Chemex

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Organizing Ellie's Medical Care

It has been an eventful past 2 weeks.  Well, not too much so.  We’ve just had 5 doctor appointments.  Most were anticlimatic, such as ENT where we have graduated to yearly appointments.  Can you say “woooohoooo!?  

First up was ophthalmology.  The Bear still isn’t wearing her glasses.  We’ve been doing atropine drops into her good eye to dilate the pupil thus making seeing out of that eye blurry.  In theory,  by having blurred vision in both eyes, she should more likely to wear her glasses.  Except that didn’t happen.  We discussed patching her good eye as we want to force her bad eye to stop turning in.  I gave the doc my “you are delusional” look because can you see Ellie keeping that on?  So we are now doing atropine twice a month which will act similar as patching.  The thing with nystagmus (turning in of the eye), if left untreated, that eye eventually goes blind.  Obviously we do not want that to happen.

If only she would wear them for more than 2 minutes

Next up was GI. We graduated to 6 month follow - ups!  So let’s do another woohooo! Her constipation seems to be well controlled - I hope I didn’t just jinx it!  She is dealing with some random vomiting issues.  Since June, she throws up about 1/3 of the time with the enemas.  We figured this is because we administer a high volume of saline into her colon and it pushes on her stomach.  Except, this is new.  We’ve had 3 other instances where she throws up once randomly - not associated with enemas, eating or drinking.  We are debating on whether or not they were stomach bugs or reflux.  To be on the safe side, we restarted her reflux meds.

As it turns out, we did have to go back to Infectious Disease.  When they called me while on vacation, saying her labs were mostly normal for her, they only had some of her results.  Her B cell lymphocytes were still very low, but she had a moderate immune response to her vaccine.  ID isn’t worried because she has never had sepsis or required IV antibiotics and she did great with the vaccine.  Yes, her lymphocytes are still low, but her immune system is functioning okay.  

Waiting for audiology at ENT

Friday, we had an appointment with our new pediatrician at the Comprehensive Care Clinic.  We had been waiting months to get in.  Dr. B, coincidentally, was Ellie’s very first GI specialist and she has a son with Ds. The appt was nearly 3 hours long and did great until the very end when the phlebotomist used a band-aid instead of purple coban.  One of the goals of CCC is to minimize specialists visits and try to do most “visits” over the phone.  We have our assigned nurse’s cell # and any time we need to talk to a specialist, Dr. B calls them.  She is going to take over most of her care for pulmonary, ID, ENT and possibly neurology.  We are up in the air about urology since Ellie gets bi-yearly renal ultrasounds and CCC doesn’t have that capability in their office.  Her eye issues and GI issues will continued to be predominantly monitored by her specialists.  

Don't you always take baths with your swimsuit?

We learned yesterday that Ellie has hypothyroidism.  It would explain why she has been sleeping 13 hours and asking for naps all summer.  Hypothyroidism is very common in Down syndrome.  It is why we monitor via yearly blood work.  The signs of hypothyroidism in a child are fatigue, weight loss (in adults it is weight gain), constipation, and cognitive impairment.  Because cognitive delay and mild constipation are common in Ds, it is difficult to differentiate between Ds and symptoms of hypothyroidism.  Thankfully, we do not need to add endocrinology to list of docs we see as the CCC will handle it.

According to the pharmacist, synthroid (synthetic thyroid hormone) is supposed to be taken with water only and not within 4 hours of other medications.  Seeing as Ellie takes meds 4 x day, she would have to get up at 2:30am to take the synthroid.  The pharmacist agreed that it isn’t practical so she will take it with her morning medications and routine lab work will monitor her thyroid levels.

Now the good news is that we have NO doctors’ appts for the months of September and November so I will hopefully get to blog about other exciting things besides medical stuff.

She was so excited to ride the bus and go back to school.

Ellie started 2nd grade yesterday and she had a blast!  Here’s to hoping for a good, successful school year!

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