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.

I have no idea what I am doing: fostering independence

I really wish that parenthood came with an instruction manual.  Only modify that manual to be about special needs parenting and have it contain the knowledge and wisdom of a parents, teachers, psychiatrists, occupational therapists, speech therapists, patient advocates, and well, everyone!

All children are unique individuals with their own strengths and struggles so really you cannot lump them all into one group, but I do wish that I had some guidance.  I honestly have no idea what I am doing here when it comes to teaching Ellie life skills.  Am I doing too much for her and holding her back?  Or are my expectations too high?

My goal, like most parents, is to help my child to be come self sufficient.  She already is and will be capable of a great many things and while I am not under the delusion that Ellie will grow up, move across the country and live 100% independently without any supervision, I do want to foster her independence as much as possible.  What that will look like in the future -whether she lives in a nearby apartment nearby with someone checking in on her vs. a group home vs. living with us, I do not know. The thing is, I have absolutely not idea how to identify and teach her the necessary life skills to set her up for success.

Right now, I have the following short-term goals in mind:

A. Be able to make her own "meals" (a Nutella sandwich is a meal - don't judge!)

1. Able to remove bread from package
2. Open/close the Nutella jar (she's getting there!)
3. Properly hold knife to remove Nutella from jar and spread onto bread
4. Be able to open a package of food (i.e. remove the wrapper from a nutrition bar)
5. Be able to unscrew the top to her supplemental nutritional shake

B.  Get this kiddo poop trained!

• First and foremost,  this is out of my control and Ellie's control given her complex GI history and colon situation, but it is a goal nonetheless.

C. Able to change her bedding and put her clean laundry in the correct drawers

• Bonus points for folding.

D. Drink from an open cup with all of the contents ending up on the floor or herself.

E. Understand the concept of time.

• Ellie can read both an analog and digital clock to the 1/2 hour mark, but she doesn't really under the concept of time.  For instance, if I were to say that Kyla comes at 4:00, she does not understand that.  If I say you've got 5 more minutes, she only seems to grasp it if I set a timer.

Sounds easy, right?  It isn't.  I have a 10 year-old daughter who thinks like a 4 year-old with the fine motor skills and receptive language understanding of a 3 year-old.  Are the goals that I have set for her appropriate for her developmental level?  Am I pushing her too much or not enough? I feel like a horrible parent when I think it would be so much easier and faster to just do it for her (obviously, I am trying to avoid the "just doing it for her").

Oh and by the way, do not google "how to use a knife" or you will get some weird and disturbing results.

This brings me to changing my mindset.  Rather than focusing and lamenting about what she can't do (yet), instead let's celebrate what she can do all while trying to figure out how to give her the tools the she needs to succeed. . . . once I figure out what those tools are, that is.

After all, she has already attained several life skills in the past few years and I know that she will continue to gain more!

What she has already accomplished:

• Laundry -  This kiddo has been using the washing machine with minimal assistance for years now.  I need to work on the whole folding clothes and sorting thing.  Also, I need to keep reminding her that if she is going to wash a lovey, she should wash her other dirty clothes at the same time.  As in, do not do an entire wash cycle for 1 blankie!
• Cleans house - dust, wipes counters, sweeps/mops floor
• Orders food at a restaurant - this is where her speech device comes in handy
• Swallow pills
• Administer her inhaler with assistance
• Makes her enema for her cecostomy tube
• Differentiates between trash and recycling (can someone educate my husband that cardboard can be recycled and styrofoam cannot?)
• Able to get dressed, including socks and shoes
• Able to put on jacket and pull up zipper
• Uses a key to unlock doors - while this is good, it also means she is now able to get into my office!
• Packs her lunch - granted her idea of a lunch includes  an entire bag of grapes, a bag of baby carrots and an unwrapped zone bar, but at least she didn't forget the ice pack!

So. . . if anyone has that special needs parenting instruction manual please send it on over to me!

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!

   

The Busted Knee, Pediatric Mood Disorder, and a Boyfriend

Goodness!  Last week's blog post was getting w a y  too long as that is what happens when I fail to update the blog regularly. . . that and my kiddo seems to have a ton of random stuff that keep popping up.  Anyway, I left off where I typed:

"BUSTED KNEE WHILE AT THE DOCTOR "

On the first day of winter break, Bear ended up with a busted knee.  She was having a follow up neuropsychiatrist appointment when I mentioned that she was peeing copious amounts of urine and frequently.  To the extent we put her back into pull-ups and it was soaking through them and her clothes about every hour.

The question was: UTI vs. Increased Urination secondary to medication?

Because I am a medical nerd, I researched Adderall XR and urine production.  I discovered that up to 5% of people on stimulants get urinary tract infections [UTI], but no mention of increased urination.  Consequentially, we tested Ellie for a UTI.  This kiddo usually needs to be catheterized for a urine sample. Sadly, she is relatively used to cathing due to her history of urinary retention.  Turkey Bear is strong.  Very strong.  To do a urinary catheterization, I have to hold her upper body while a nurse takes each leg.  The third nurse does the actual catheterization.  Being in a frog-legged position with pressure being pressed down on her leg while Ellie pushes up is a recipe for disaster.

Photo: limoncello.ie

After the cath, I told her to pull up her pants.  She kept falling down and crying.  Being the horrible mom that I am, I thought that she was being defiant.  I figured the crying was because she was still pissed off.  Nope, her left leg was buckling and she couldn't support her weight.  I refused to leave the clinic until the PCP assessed her.  It didn't seemed fractured, but a knee and bilateral hip X-rays were ordered.  She was finally able to stand without too much pain, but still falling.  I had to carry her around until the X-ray.  Of course, I threw out my back. . . again.  After her X-ray, she was going much better in that she was able to walk without pain or falling, but you could see her knee continue to buckled.  It was determined that she strained either the supporting ligaments or tendons.  She wore an ace bandage for a few days after which she decided it was a stylish fashioned statement and wanted one on each leg.  After a week of this, her ace bandage magically disappeared one night.

Ellie head banging to Like A Storm's "LTWYHM" aka
Love The Way You Hate Me
Like A Storm on Spotify: https://spoti.fi/2ATh0Bl

PSYCHIATRIC CARE

In addition to making her pee, the Adderall XR didn't do anything for her ADHD.  She was super hyper, impulsive and not listening to directions.  It was so bad that her teacher asked if we stopped her medication.  Given the increase urination, the doctor hypothesized that she just didn't absorb the extended release Adderall.  We decided to go back to morning Adderall and add in a booster dose.

This seemed to help.  After nearly a year of having poor concentration, Ellie was finally able to sit down and color, paint, and play with play-doh.  However, it triggered her mood disorder.  She was taking 3 hours to fall asleep most nights and that was with an increase in her sleep meds and she would wake up much earlier than usual.  Note: this is not the Adderall.  Her midday does is at 10:30am and is out of her system at 1:30pm.  Some nights, she would only take an hour (this was her norm).

Soon, she was extremely energetic and very impulsive, but she could still attend to tasks.  She has always been a big repetitive with her speech - "mama!  first school. then dance", but it was nonstop.  As in as soon as she would finish the sentence, it would start all over.  It is sort of like the typical pressured speech you would see in an adult mania.  She was also emotional and defiant.  We'd tell her no on something and she would just melt down.  The neuropsychiatrist witnessed all this and just knew the E couldn't get control of herself.

For people with mood disorders, it is very common for other psychiatric medications such as stimulants and antidepressants to trigger a mixed state (both mania and depression at once) or a mania.  This is why people are placed on mood stabilizers.  So earlier this week, we increased her Trileptal dose.  I am really hoping that this works for her.  It is so trying for us and especially for her.

Hanging out with Brandon, the co-owner for Noble Sandwich Co
Formerly Noble Pig featured on Food Network.
 We go there every Saturday morning and she will not leave until she
gets to talk to him!

Best Friend/Boyfriend

Before Winter Break, The Bear's class had a winter party which parents were invited to.  It was during this party that I discovered Ellie had a best friend. Not to mention that she and S were all lovey dovey.  They are the same age and apparently were besties last year.  His mom said that S talks about her all the time.  This warms my heart to know that Ellie is making friends!  Over break, they were both at the same drop-in daycare and apparently they went off to a corner to play. . . um, should I be worried?!   What is this canoodling that is going on?!   Nonetheless, I always worry about Ellie connecting with other kids because she is predominately nonverbal and also has her impulsive behaviors such as shirt pulling and stealing toys (and putting them away).

Pajama Thief!

Next Friday will be a fun filled day - cecostomy tube replacement, an ABR (fancy hearing screen), and an angiogram of her abdomen (to look at kidneys).  Currently, we are attempting to add on eye surgery, but it is complicated as the ophthalmologist doesn't usually have OR time on Fridays.  Fingers crossed we can get it all worked out!  I believe that this is her 17th or 18th time under anesthesia and I really want to avoid more procedures.

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!

When can we bust out of jail? . . . er hospital

Hello y'all!  Can you believe it?  TWO blog posts in 1 week, but I wish that the posts were about fun stuff.  A few days ago, I mentioned that Ellie was in the hospital for adenovirus, RSV, and an asthma exacerbation.  She was admitted Friday night and we thought that we might get to go home on Saturday.

Lies!  All lies!

We are still here.  Ellie loves it so much that we decided to stay.  On Saturday, we attempted to wean her off the oxygen during the day, but that was a fail after 2.5 hours. Even with oxygen, her O2 sats stay between 88-92%.  (normal oxygenation is above 95% and oxygen therapy is typically started if under 88%).  Sunday, her lungs sounded much better and she was coughing a lot more (she coughed so hard that she projectile vomited all over me.)  True to Ellie fashion, she decided throw in a few more, non-major medical things. Because, why?

1. Peeing.  Why does my kid not pee!?  She seems to think that it is appropriate to urinate every 12 hours.  The doctors are not a big fan of this, but I told them I am not concerned.  The did a bladder scanner on Sunday night and showed she had tons of urine.  Just as the nurse gets the order to do a urinary catheterization, Bear pees.  Woohoo!

2. Ellie developed significant abdominal distention and harness.  She was not a fan of us even looking at her belly let alone touching it, but she did not cry out.  The x-ray revealed extensive gas. Most likely from her rapid breathing and inhaling so much air as well as stool at the lower colon.  We were still having constipation at home issues even with the enemas through her cecostomy tube as well as rectal enemas so she hadn't gone in a week.  Thankfully, yesterday she had two small BMs after giving a large volume SMOG (saline, mineral oil, and glycerin) enema rectally.  Her abdomen is still a little distended and hard, but it is still much better.

Fever, poor fluid intake, and more oxygen. Early Monday morning, the Turkey Bear thought it would be appropriate to drop her oxygen saturations and require an increase in oxygen twice.  We couldn't figure out why because her lungs were even clearer than before and she was coughing (this is good news).  We did get her out of bed for a while yesterday, she started to eat more again, and she was in a great mood.  She had trouble drinking and we were considering an IV, but we got her to consume small amounts of fluid throughout the day.

Ellie Bear also developed a fever of 102.5 so the doctor decided that if she continues to spike that high, we will do a chest x-ray to rule out pneumonia.  Fortunately, after the Tylenol wore off, she never went above 101.4.

Now for the good news! Last night (Monday night), we were able to decrease her oxygen a fair amount while sleeping.  This is a BIG step in the right direction.  She really needs to be able to go all night without oxygen while maintaining her oxygen saturations before 88% before we go home. I a really hopeful that she will be able to go a few hours today, while awake, without her oxygen mask.

Thank you, The Wish Connection for the visit and balloons!

The nurses, respiratory therapists, and doctors at Dell Children's Medical Center are great!  They value what I have to say.  They accept that I know Ellie better than anyone and they understand that I have a fair amount of medical knowledge given my professional history of being a PICU nurse (okay, that was 14 years ago and back when our charts were on paper) and practicing as a pediatric nurse practitioner.

Andrew had to post-pone his business trip on Sunday due to Ellie's hospitalization, but he has to fly out today and will be back on Friday.  Hopefully we will be home by then!  He has been visiting and relieving me for coffee breaks and meals as well also bringing snacks, toys, and changes of clothes and toiletries for me.  I don't to go home other than after the first day because, but I rather stay near the hospital as it is a 30-40 minute drive for us.  I would like to thank everyone who has been praying for us, sending positive thoughts and virtual hugs, brining me coffee, and offering to help.  I appreciate it more than you will ever know!

Hypertension In a Child

In previous blog posts I have written about Ellie's high blood pressure.  I have an update er, well updates.

According to the new pediatric guidelines, for Ellie's age, height %tile, and gender, her systolic blood pressure [top # of BP] should fall around 111 mmHg.  Ellie's average without blood pressure medication is 124 in the LEFT arm only and normal in the right arm.

High blood pressure in a child is usually due to cardiac issues such as narrowing of the aorta (a large artery that branches off the heart) or renal [kidney] issues.  However, with the increasing rates of childhood obesity, doctors are seeing more and more hypertension related to weight, similar to adults.

Flashback: October is Down Syndrome Awareness Month

In August, we were able to rule out all cardiac issues so that is not the cause of her hypertension.  We are currently evaluating her kidney function.  Many doctors start off with a basic metabolic panel that looks at things such as glucose, sodium, potassium, etc, but the main values that a physician looks at from that lab study is BUN and Creatinine.  I won't get into what those things are for, but high levels of BUN and Creatine can be a sign of impaired kidney function.  Ellie's were normal.  Other, more in-depth testing include assessing Renin, Aldosterone, and Angiotensin - again, I will spare you the details of their function.  Ellie's Renin levels are high.  Renin is an enzyme that is secreted by the kidney that helps regulate blood pressure. One of the causes of high renin levels is renal artery stenosis, which is is the narrowing of the artery that supplies blood flow to one or both of the kidneys.  This is something that is very rare with systolic BP usually above 170 mmHG and it is highly unlikely that Ellie has this . . . BUT, as the nephrologist said "this is Ellie".  Plus, she has been on clonidine for years and she is also on a calcium channel blocker BP med so we don't really know how high it would be without these medications. At this point, we are working on scheduling a CT with contrast (angiogram) or her kidneys, but it is not considered urgent.

Flashback: October is Down Syndrome Awareness Month

Hypertension in a child is typically diagnosed when the systolic reading is 10 mmHG above the 95% tile for age, height, and gender.  First line treatment for high blood pressure in a child is a calcium channel blocker - i.e. amlodipine. (Calcium Channel blockers tend to end in "ipine.)  A few months ago we started Ellie on Norvasc.  We some mild improvement in her blood pressure.  The nephrologist says that kids typically end up on higher doses than adults due to their higher metabolic rates so he bumped up her dose.

I don't know what happened and I don't understand it.  I measure Ellie's blood pressure with an automated cuff. This is the same cuff that I have been using for months.  After we increased her Norvasc, I noticed that her systolic readings were back into the mid-120s.  A few weekends ago, I took her BP and it was in the 130s.  I waited a few hours and she was 140 mmHg.  I didn't believe it.  I switched out the cuff to an adult one and took mine.  The I compared it to my Qardio cuff that I use with an app.  Both of mine were the same.  So . . . Ellie's reading was accurate.  I waited an hour and she was 147 mm HG.  Note that she had both clonidine and the higher dosage of Norvasc in her system.  A BP that high in a child is dangerous.  I had to call nephrologist on-call who told me to immediately give her clonidine (it is pretty fast acting) and that if it was still in the 140s in a few hours, she'd have to receive IV anti-hypertensives in the hospital.  It was odd.  I don't know why this happened, but the clonidine did work.

Uh huh . . . how long do you think she'll keep that on?  The eye patch is over her good eye.  The thought is the eye that turns in will correct itself.  We've tried glasses and then keeping her good eye dilated with no change (okay, she refused the glasses).  So this is our last ditch effort.

A few days later, the nephrologist upped her dosage of Norvasc again and it seems to be working.  Her BP has been averaging 110/60 so we are pretty happy with that.  Her renal CT scan with contrast still isn't scheduled as we are trying to do it the same day as her ABR and cecostomy tube replacement.  I hate waiting, but I only want to put her under sedation once.

The blood pressure machine that I use for Ellie is by Contec.  It is no longer sold on Amazon, but eBay has it and a few online medical supply sell it.  I have tried several automatic and manual cuffs, but this machine is the ONLY one that has a TRUE pediatric BP cuff.  Most the ones marketed as pedi are actually infant cuffs.  A blood pressure cuff isn't just about length to wrap around the arm.  It is about width.  It is supposed to cover a specific portion on the upper arm.  If the cuff isn't wide enough, the pressure will read higher.  If the cuff is too wide, the pressure may read lower.

Contact 08A Blood Pressure Monitor - pediatric/child cuff sold separately

photo: uptodate.com

Some cuffs are labeled with #s.  i.e. a child cuff may be labeled as size 9 so it is good to read the measurements.

While the Norvasc is working, it comes with an unfortunate side effect.  For Ellie, it is significantly making her constipation worse (it may cause the opposite problem in some people).  She used to get 250 mL of saline enemas through her cecostomy every other day.  She is now getting 250 mL SMOG enemas [saline, mineral oil, and glycerin] through her tube 2-3 x week - the max we can do and then 2 rectal ones a week - also the max.  It is frustrating because she is still only having a bowel movement every 5-7 days.  Seriously?!?!  She has a freaking tube in her colon for this stuff!  Unfortunately, it is something that Ellie will have to suffer through because I really don't want to her to be at risk for a stroke from a really really really high blood pressure.  If it gets to where is going more than a week, then we will have to figure something out because colitis and bowel perforation would suck.

Anyway, that is the big blood pressure update.  My next post on The Chronicles of Ellie Bellie Bear will be fun!  Horse therapy with Jack?  Yes!  Seeing the dolphins at Sea World?  Yes!  Reuniting with friends from The Wish Connection and seeing a Tim St. John, the magician?  Absolutely!  What about Morgan's Wonderland, an amusement park designed for children and adults of ALL abilities?  What fun!

Fun Photo Flashback: