Ellie's Surgery for Sleep Apnea and Recovery

The month of November sped by while at the same time it, seemed to crawl along at a snail's pace.  There were too many things to worry about and I do not mean of the turkey being over-cooked  and a family feud variety.   No, I am talking about Ellie's surgery and my cervical steroid injection procedure.

As someone with a diagnosed anxiety disorder, it wasn't very difficult for the usual worrying and feelings of anxiousness to transcend into full blown panic mode complete with heart palpitations, nausea, sweating, and feeling of impending doom.  Then how the irrational suddenly becomes the rational.

*I apologize in advance for some missing photos - I used Photobucket for free as a means to store my photos and they are now holding a few hundred pics of mine for hostage unless I pay for an expensive plan and I also managed to lose half my photos from my computer*

In the past year, I have had several steroid injections into my back.  Nearly all of them have been in the lumbar (lower) region of my spine as well as my sacroiliac joint.  However, it was the first time I was having a shot into the epidural disc space a my neck in hopes of alleviating the intense neck/should pain with radiating numbness, tingling, and weakness down my entire left arm.  Epidural shots into the cervical region, as opposed to lumbar or thoracic, are a bit more risky given the close proximity of the brain as well as narrowness of the spinal column.  This of course triggered an intense sense of doom.  "It is going to cause a flare up that lasts through Thanksgiving and then I will have to cancel Ellie's surgery.  I am going to have temporary paralysis or a spinal headache!"

Then even more worrisome for me because it involve my baby, Ellie's surgery - the lingual tonsillectomy and glossectomy- which was going to commence exactly one week after my injection.  By the Friday before Monday's procedure, I had talked myself into believing that because I worried, fretted, and had horrid dreams about all the "what ifs"related to all that could go wrong with Bear's surgery and recovery, that I worried all possible complications away.  Of course, then I had myself thinking "well now I just jinxed it and something is going to go horribly wrong!"

As with most things, I worried for nothing.  My shot was awesome with a minimal flare up followed by 50% pain relief and 75% decreased numbness/tingling.  Ellie's procedure went well and she had a relatively smooth recovery.

Pre-operative waiting room Selfies

Ellie's surgery lasted approximately 1.5 hours and she was a little stinker pants coming out of anesthesia.  Then for the next two hours she was agitated and screaming bloody murder.  Kicking, hitting and using her teeth to pull off the sock that was taped around her ankle.  The sock that was covering her pulse oximetry monitor around her toe - the little thing that measures the oxygen saturations in the blood (normal is 98-100%).  Given the thrashing that was already making it difficult of the pulse ox monitor to function proper, I respectfully asked if we could do spot checks every x number of hours rather than continuous monitoring and the doc looked weary.  I totally understand seeing as the child just had her upper airway carved on!  Yet, after spending just 5 minutes in the room with the Tasmanian Devil, the doc said "take it off and check her every 4 hours".

Once Ellie was calmed down using coloring books and watching Sofia the First, she ate some ice cream and drank a bit of juice.  She also started peeing!  As in she peed THREE times that day and 3 times the following day. This is something that we hadn't seen since July (yes, she is still doing that pee every 22-30 hour thing).  When we arrived in pre-op, Ellie hadn't urinated in well over 24 hours.  I knew from past experience that the anesthesia would cause a more acute urinary retention so I convinced the surgical team to perform a urinary cauterization in the OR if she didn't pee while under.  That cath seemed to be a cure . . . for about 3 days.

Busting out!  Poor thing was feeling pretty cruddy, but she was happy
to be headed home after a night in the hospital.

I was extremely concerned about how things were going to go overnight- would she require oxygen since she is so swollen?  Would she be in a lot of pain?  Would she sleep at all?  Again, it was needless worry for our Bearity Bear had oxygen saturations from 91-94% for the entire night!!!  To me, that is proof that her surgery was a success because pre-surgery, she would sit at 81% for most of the night.

Play-doh pajama time with Daddy!  Her cheeks were pretty swollen.

A traditional tonsil and adenoidectomy recovery usually takes 7-10 days with an increase of pain around day 5-10 depending on when the scabs slough off (it was day 6 for Ellie a few years ago).  With the lingual tonsils, there is less raw space and as such, less pain.  The surgeon stated that normal recover for a lingual tonsillectomy is 5-7 days.  Ellie's recovery took about 6 days with her returning to school the following Monday.  I would say that post-op day 3 (Thanksgiving day, of course) was by far the worst where we had a hard time controlling her pain and that left her miserable, crying and rather agitated.  As for the glossectomy, the tongue heals quickly - in a manner of 4-5 days.  It did give her some seriously rank breath that lasted nearly 2 weeks - as in enough to pass out from if she were to breath on you!  She is still snoring a bit, but I believe that is because she has some drainage and congestion due to her throat and tongue healing.  We will have a follow-up sleep study in about 3 months to re-evaluate her sleep apnea.

So that was our November and now I can hardly believe that December is already here and soon 2016!


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Obstructive Sleep Apnea - surgical management in children; lingual tonsillectomy, glossectomy

When I first started working as a nurse practitioner, I took a position at a busy, urban pediatric ENT clinic.  I used to spend my days in pre-/post-op appointments and teaching parents about various ear, nose, and throat surgeries.  Now, I am the on the other side, the parent side. It is a weird feeling.  On one hand, I feel more prepared than ever given my background.  On the other hand,  it is a great source of stress to know all the details of the various ENT procedures / anatomy related to my daughter's ears and airway issues.  Sometimes, it just means more to needlessly worry about and get fixated on.

Today, we finally had our appointment with an ENT specialist who works specifically with children who have Down syndrome and sleep apnea.  She is amazing and very knowledgeable.  I know that Ellie is in safe hands.

Rather excited about the horse picture in the waiting room
Note: I took a quick pic first and then told her no feet on the chair - priorities

Background:
Ellie was diagnosed with Obstructive Sleep Apnea via a sleep study aka polysomnography back in January.  Essentially, Ellie would have pauses [apnea] in her breathing while asleep.  Even more worrisome is that she has very slow, very shallow breathing. This means she isn't taking in adequate oxygen to feed her brain and that she isn't exhaling enough carbon dioxide. [hypopnea] Her oxygen saturation would sit in the low 80s for most of the night.  Normal oxygen saturation are 97-100%.  This is a toxic situation that leads to behavior issues, frequent night wakings, and possibly even be part of her failure to thrive.

Photo from Ellie's sleep study.  

Health Risks related to sleep apnea
Photo: www.sleepapneadisorder.info

In most cases of sleep apnea, the child's tonsils and adenoid are removed.  These fatty tissues can be the source of airway obstruction.  By removing the tonsils and adenoid, you are keeping the upper airway open.  Ellie had both of hers removed 2.5 years ago.  It actually resolved her sinus infections and eliminated the need for further sets of ear tubes because the adenoid was so large, it was blocking drainage from the sinuses and Eustachian tubes (a part of the ear).

Beauty Sleep: Tonsils, Adenoid, and Ear Tubes - obstructive sleep apnea and chronic sinusitis


CPAP:
Because we already performed an tonsillectomy and adenoidectomy, we opted to treat Ellie's sleep apnea and hypopnea with sometime called a continuous positive airway pressure device aka CPAP.  It was quite the trial.  Getting Ellie to put on the mask let alone hook it up to the machine and turn it on, was a nightmare.  Social stories.  Pictures.  Bribery with M&Ms. Enlisting the help of therapists and a sleep mask technician.  None resulted in Ellie donning on the mask for more than a few minutes.

As you can see, Ellie was not a happy camper.

CPAP for obstructive sleep apnea


CPAP Failure:
Now, this is where things get interesting:
In most cases, the removal of the tonsils and adenoid "cure" obstructive sleep apnea in up to 75% of people.  It is closer to only 25-45% in kids who are obese, have asthma or have Down syndrome.  Kids with Down syndrome are more prone to having persistent sleep apnea even after a T and A because of their small jaws, smaller mid faces, larger tongue or a narrow, high-arched palate.   So when something like CPAP fails, other options must be explored.

Obviously, you don't want to go hacking away at random structures of the child's airway.  How do you know where to begin?  Back at the beginning of September, Ellie had a Cine MRI under sedation.  Basically she was put into a sleep-like state and had a special moving MRI that is used specifically to look at how the head & neck anatomy acts during sleep.  This is a way to pinpoint exactly what structures (tongue, palate, tonsils, etc). are collapsing while sleeping and cutting off the airflow.

Ellie was pretty loopy coming out of sedation post MRI.  She wanted to rip our her IV until she discovered her pulse ox on her thumb.  Oooh light. . . pretty. . . .  Sometimes I think that sedation is rougher than anesthesia for Ellie.

Cine MRI:

Ellie's Cine MRI revealed that she has a large tongue aka macroglossia - at the base or back of the tongue and at the top of the tongue is large enough that when laying down it abuts the palate.  It also showed Epiglottis Insufficiency - basically where the epiglottis closes off the airway.  The epiglottis is located just below the tongue and it covers the trachea/airway during swallowing.  If the epiglottis is fully closing during sleep, it is blocking the trachea and decreasing airflow.

Function of Epiglottis
Photo: www.rise.duke.edu

Tongue causing obstruction of the airway while asleep

It was theorized that the epiglottis insufficiency is the result of enlarged lingual tonsils.  These are tonsils that you cannot see when you open your mouth.  Most people don't even realize we have these tonsils, as the lingual ones sit just below the tongue base.  If they are large, they cause an obstruction and they can also push the epiglottis closed while asleep.  It is thought that more than 30% of kids with Down syndrome who have persistent sleep apnea have large lingual tonsils.  Ellie has large lingual tonsils.

Photo: Anatomy of the Respiratory System Institut Pendidikan Guru Malaysia; slideshare.net

Goody.

Plan:  SMILE
Apparently, everything Ellie is having done can be summed up as the SMILE procedure.  It sort of cracks me up - SMILE!  We are going to do a SMILE on Ellie! Funny, I don't think we will be smiling when the day arrives.  SMILE = submucosal minimally invasive lingual excision

The ENT doc is going to remove the lingual tonsils.  This is going to go a lot like her previous tonsil/adenoid surgery, but with a slightly shorter and hopefully less painful recovery time (5-7 days average recovery).  I am pretty much going to follow what I did way back in April 2013 when she had her T and A with regards of what to bring to the hospital and how to make her comfortable post-op.

Tonsillectomy and Adenoidectomy recovery strategies for children with Down syndrome  - prepare for day of surgery and for post-op.

The other procedure that will be performed at the same time is a posterior-midline glossectomy, which is a fancy way of saying "we are going to make the back of her tongue smaller /less bulky".  This is actually a surgery that I am not fully familiar with, but it has been gaining in popularity among pediatric patients these past 15 years when CPAP and Tonsillectomy fail to alleviate sleep apnea. I will spare you the details, but believe it or not, it is probably the less painful of the 2 surgeries and the recovery time is only 4-5 days.

Photo: www.singhealth.com.sg

I am not exactly thrilled about any of this, but I know it needs to be done.  Sleep apnea is no joke.  It affects all health systems negatively.  Ellie's doctor is an excellent doctor (when doing research - I discovered several articles and studies conducted by her) who will take good care of the Bear.
So now I have approximately 28 days to have mini panic attacks on a daily basis.  Oh Ellie. . . why must things be so difficult?!

References:

Propst, Evan (2015). Lingual tonsillectomy and midline posterior glossectomy in children with obstructive sleep apnea.  Operative Techniques in Otolaryngology http://www.optecoto.com/article/S1043-1810(15)00009-3/fulltext

Ishman, S. (2012). Abstract: Pediatric Sleep Apnea and Surgery; Beyond tonsillectomy. Audio-Digest Ototlaryngology. http://www.audio-digest.org/adfwebcasts/pdfs/ot4518.pdf



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The Pumpkin Patch and Updates - GI, Urinary, Sleep Apnea, Cervical issues, Etc

You all should be proud of me as it has only been 3 weeks since my last blog post.  Longtime Ellie Bellie Bear readers, do you all remember back in the day when I wrote at least 3 posts a week?  Progress!  Seriously, everyone, I have 4 or 5 open blog posts sitting in the queue waiting to be completed.  Such as the one that I wrote way back in June on lice.  I really need to finish that one up because Ellie has lice again back in September!

As usual, things have been chaotic, but I made sure that this past Columbus Day we spent part of the day doing something enjoyable.  We had such beautiful weather that Ellie and I spend the day outside and then too a trip to the local nursery to pick out a pumpkin. . . er pumpkins.  Bear was the only child there and there were two lovely ladies picking out flowers and 3 landscaping guys as the Red Barn Nursery.  They all held little secret smiles as she would go running by (and when she ran up to the landscaping dude and gave him high-5s).  My daughter, the entertainment at the local nursery.  The frolicking fairy princess.

Ellie Bear thought it was quite the game to gather up pumpkins of various sizes to add them to our cart.  The Chunky Chicken was under the mistaken impression that was going bring those 5 or 6 of those personal-sized pumpkins and 12 minute pumpkins.  She would dance (yes, dance, frolic, sashay) her way back to the cart with a mini pumpkin in each hand.  Perhaps the most entertaining part of the trip was Ellie's vast fixation on gourds.  Especially the funky looking green one.  She was adamant that we take that one home with us.  As well as the one that I think looked kind of like a light tan-colored, bowing goose.

Now, what is the Bear going to be for Halloween?  That is a great question because I don't know the answer to that either!  Ellie hasn't been into dress up lately.  I was thinking Flash dance given her propensity to don leg warmers during dance class.  However, I imagine she will saunter about the neighborhood in her glow-in-the-dark Mickey Mouse pajamas like the little diva that she is.

Ellie spends time in both special education classroom [FAC] and general kindergarten.  Of course I signed up to be room mom in the kinder class.  Several people have asked "are you kidding me?!  Don't you know that room mom is a lot of work?".  Yes, it is yet it is always a great way to meet people and be involved at the school. (Maybe now isn't the time to also mention that I am the inclusion parent liaison as well).  Ellie's school no longer offers Halloween parties.  Pumpkin Math does not count, folks.  So this past Saturday, all of us room mom's in Ellie's kinder class though it was be fun to host a mini Fall -Festival at a local park with snacks, crafts, and goodie bags and with costumes being optional.  All of the children had great fun and it was exciting to hear several of the boys say "Ellie's here!  Hi Ellie".  Ellie liked her alone time, but she still did well socializing with the other kiddos.  Several parents all came up to me to share that their child talks about Ellie at home saying that "she is in the class some of the time, but she is in two classes. I like it better when she is in my class". Andrew and I had the chance to meet many of the parents. To say that her school and her kinder class is welcoming is an understatement.  We are thrilled to be a part of this community!

Pumpkin Dancer

Now for some very quick updates.  I promise to write more in-depth posts later, but since I have been asked by a few people for news, here you go:


GI / Urology
For a while the constipation was worse.  We were doing a lot of enemas just to get her to go.  When I called GI, they recommended lactulose.  I imagine you all heard my maniacal laughter at you own homes as this child has been on lactulose in the past (you know that neon green liquid that she has to swallow 1 TABLESPOON twice a day) and it did not work.  The telephone call seemed to be the best treatment because she has been uncorked now.

Urology - she was seen 2 days after my post! No real answers.  Kidneys are good.  Yay!  Possibly related to constipation (um, not completely because she has been backed up way worse and was still urinating several times a day) and possibly a disconnect between bladder contraction and urinary sphincter.  We watch and wait.  If she gets kidney damage, we will look into doing routine, daily catheterizing - please say a prayer that we do not get to that!

Weight - so she has either gained a 1/2 pound or 1.5 lbs. depending on the scale since we started the appetite stimulant in July.  Wooohooooo!!!!!!  AND she has grown in height as all her pants are too short.  First time in over 2 years that I have needed to purchase new clothing for Ellie

Sleep Apnea/ENT:
Last week we met for the Sleep Apnea Clinic which is a double appointment with an airway ENT and the sleep pulmonologist.   It didn't go as planned as the ENT got called away to an emergency.  These things happen, but I was practically in tears after we left the clinic.  We waited months for the appointment.  Her apnea/snoring is worse.  And I pulled her out of school for nothing.

Later that night I got a call from the nurse.  The ENT reviewed the Cine MRI of her head and neck.  She has complete obstruction at the level of the epiglottis.  It could be epiglottis insufficiency from laryngomalacia (aka floppy airway) or due to large lingual tonsils (tonsils below the base of the tongue - not the ones you see when you open your mouth) pushing on the glottis.  We will do a laryngoscope at the rescheduled visit which is in 1.5 weeks.

Surprise MRI Finding: upon looking at the Cine MRI, it was determined that she has widening at the C1-C2 vertebrae suggesting instability.  Aka Atlanta-Axial Instability.  About 15% of people with Down syndrome have this, but for only 1-2% of the people, this is an issue. Right now, Ellie is scheduled to get repeat lateral neck X-rays to determine if she does indeed have AAI.  It does make me wonder, can the instability cause Ellie's bowel and bladder issues?

Me: I think this also needs to be its own blog post, but it will explain why I have neglected the blog lately.  I am still having massive issues with my lower back and sacroiliac.  The sciatica is mostly resolved.  I did receive a nerve block back in May, but it wore off after only 2 months.  The last 4-5 months my neck has been a mess. With lots of pain and signs of arthritis from C3-T1.  Certain movements cause pain and/or numbness down my left arm.  I have had several shots, chiropractic appointments, therapeutic massages, and visits with my pain and physical rehab doctor.  I have also been diagnosed with fibromyalgia.  I was in a car accident last month which exacerbated everything.  Last week I got a neck X-ray which showed that my clavicle is really messed up where it connects to the breastbone and I am waiting for MRI results of my neck and lower back.  Some days are difficult to get out of bed, but I am trying my best to eat well and continue with my PiYo exercises.



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Urology, Oliguria and Happy 6th Birthday, Ellie!

About 3 weeks ago I left off with the results of Ellies MRIs, Renal ultrasound, and blood work results - all to look at why she isn't regaining weight even with the appetite stimulant, why she isn't peeing, why she isn't pooping, and why she doesn't breath at night  -  ah, basic life functions going haywire.

Funny thing here, when Ellie was born and we learned that she had Down syndrome, I immediately worried about her heart.  After all, it is said that up to 50% of kiddos with Ds can have a heart condition.  Well, Ellie did have a hole in her heart.  A small one.  Not real exciting.  Closed at a year old.  That was that.  Never did it cross my mind that Bear would have a whole slew of health issues completely unrelated to sporting an extra chromosome (well, aside from the sleep apnea which tends to be more common in people with Ds).

These two turkeys crack me up!

So. . . not peeing.  I would LOVE to tell you how Bear's urology appointment went only she hasn't been seen!

Brief history:

Ellie is not potty trained.  She is not purposefully holding onto urine and refusing to void.  She is not secretly peeing in the toilet without us knowing.  She isn't leaking small amounts of urine into her diaper.  She never acts like she has to go, but will occasionally have to "push" to pee, usually after 20+ hours.  She is peeing once every 21 - 24.5 hours.  She is peeing at an average rate of 0.7 cc/kg/hr.  Normal urinary volume for a child her age/size is 1-2 cc/kg/hr.  So, she has decreased voiding frequency and decreased urinary output (oliguria).  Not horrendous, but not normal.  Failure to void on a regular basis can lead to urinary tract infections [UTI] and can also cause urine to back up into the kidneys and cause damage.  So not peeing for 24 hours is sort of a problem.  Not to mention uncomfortable.  I am doing the potty dance right now just thinking about it.

Her renal ultrasound was performed under sedation on 9/4 after 20 hours of not peeing.  Her bladder was moderately full.  Her kidney anatomy was normal.  Her right kidney is 1.1cm larger than the left.  It isn't uncommon for one kidney to be larger than the other - usually the left one, but the right can be normal too.  Now the size differential may be statistically significant since it is >1 cm size difference. Also, the good news is that her kidneys have grown since a previous ultrasound.

"Hi everyone!"

Ellie's BUN  and Creatinine levels were normal - blood work that measures kidney function.  Her spinal MRI, ordered by her GI specialist, was also normal.  This ruled out tethered cord as a cause for her constipation and urinary issues. We were referred to urology on 9/9/15.  Austin is and has been having a huge growth in population.  Lots of young adults and young families are flocking to the Live  Music Capital of the World or Silicon Dessert.  Unfortunately, the number of pediatric urologists have not caught caught up to this population boom.  There is 1 pediatric urology clinic in the Austin area.  This clinic has 4 doctors.  Two of them work with voiding issues.  When I call, just to talk to a scheduler "you are caller # 1" is 17 minutes average on-hold time.  SEVENTEEN!

Anyway, I finally talk to a real live person and I kind of flabbergasted her when she asked what we needed the appointment for.  I know a lot of key words - see early paragraph about "brief history".  Spouting off kidney sizes, history of Ds, failure to thrive, GI issues, urinary output kind of startled her.   She connects me to a nurse.  The nurse then goes "let me see if one of our urologist can access Ellie's ultrasound because we are booking in November and I don't think she should wait that long".  I was told I would get a call back.  I never did so I followed up myself.  They created a chart for Ellie and in her chart it says that the urologist wants to see her in 2-3 weeks.  I am told that they will call with an appointment.  They never did.  Notice a theme?  As it turns out, when they say they want her seen in 2-3 weeks and that she shouldn't wait, it means that they are NOT squeezing her in but rather adding her to a rather long wait list that anyone can be added to.  It also means that nearly 3 weeks have passed and she has still not been seen.  Her current appointment is for November 30th.  Over 2.5 months from the time of my first phone call.

Ellie was so excited about the cake. . . until Andrew took away the remainder of the cake.  Apparently, Chunky Chicken though that she would get to eat the ENTIRE cake and not just one piece.

To say I am peeved is a bit of an understatement.  What kind of practice is this???  I am not ignorant of how things work in the medical field.  I used to work as a pediatric ENT nurse practitioner in Philadelphia where our first available was over 4 months out.  I was the one who would work certain new patients with complex medical issues into the schedule.  Overbook appointments because some of these kiddos couldn't wait the 4 months.  Discuss with the doctor and make sure the patient was seen.  Being a squeaky wheel doesn't see to be working either.

Again, these two crack me up.

So what now?  We wait and wait and wait.  She finally gets seen at the end of November only for the doc to order tests.  Like a VCUG or something along those lines.  Then we wait to get scheduled for that and then wait some more for the follow up.  At this rate, the poor kid will have damaged kidneys.  Right now, I am trying to come up with a plan of action which will involve enlisting the help of Ellie's pediatrician.  I just feel so helpless.

Okay, I am done complaining.  Nothing is accomplished by ranting other than me feeling a bit more sane!  The pictures in this post are from from a small birthday celebration that included Bear's best friend and a few family members and close family friends.  It was a great deal of fun and I know that Bear got a kick out of the Mickey Mouse cake.

Happy 6th Birthday, Ellie Bear!

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Results of Ellie's MRIs, Ultrasound, and Blood Work

Last Friday, Ellie had a battery of test run at the Children's Hospital - to learn more, click here.

MRI of the lumbar spine = normal (Yay, no tethered cord!)

Renal / Bladder Ultrasound = normal (Yay, no kidney problems!)

Cine MRI for sleep apnea = awaiting results

Routine blood work of CBC, metabolic panel [this also shows kidney function], Thyroid panel, and vitamin D = normal

Weight Gain since starting predication = possibly 3oz give or take a few

Explanation for why this child is only peeing once every 19-24 hours = unknown

Explanation for Ellie's crazy GI issues = unknown

Do you see that the Princess Bear lost her first tooth?!

Ellie's pediatrician has recommended that she be evaluated by a urologist. I have been in touch with the only pediatric urology clinic in Austin and the first available appointment is in November. I think the scheduler was a bit flabbergasted when I explained what the appointment was for because she then had a nurse talk to me. I am pretty sure it was when I said "Ellie has decreased voiding frequency to one every 19-24 hours at a rate of 0.7 cc/kg/hour" that did it. . . The nurse then said "she really seems to have a lot of things going on so I will talk to the urologist and see if we can fit her in earlier".  I am waiting for a call back. What do you want to bet that some of this is related to her constipation and Periactin? Or maybe she is just being a urine withholding toot?

I also put a call into the sleep clinic and am waiting to hear back as we are trying to move up her follow up appointment (it is in December). The MRI results will be discussed at this appointment.

Debating on taking a vote on what Ellie's GI specialist will do given her lack of weight gain and GI issues. All in all, I am relieved that there is nothing wrong with my precious baby girl, but frustrated because we still don't have answers. Lack of answers means we don't know how to help my Bear.

So now. . . . we wait. . . 

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An Overall Update - failure to thrive, kidneys, GI issues, sleep apnea

I cannot believe that it has been over a month since I last updated the blog.  Things have been chaotic over here so I am going to just pump out a big fat update.  Normally, I like to add in a lot of cute pics, medical photos, and lots of resources, but I will skip that for now.  Forgive me?

Obstructive Sleep Apnea:
Operation CPAP has been a massive fail.  Am I shocked?  Nope!  We had a sleep clinic appointment back in July to discuss options.  I consulted with a sleep tech who works specifically with getting children to wear their  CPAP face masks.  We made sure that the mask was adjusted properly, but I pretty much had to restrain Ellie the entire time.  The doc witnessed the huge fit that Ellie pitched when I attempted to bribe her to wear place the mask over her nose.  I considered it a HUGE success that she actually wore it for an entire minute in clinic.  The thing is, Ellie wore it more when we first started this CPAP desensitization and she has become progressively more frisky and clever at yanking it off.  Did I mention that this is just the mask?  As in the mask isn't hooked up to the machine and the machine turned on?  Oh I didn't mention that?  In speech therapy a month ago, we were able to get her fully hooked up with the machine on for 10 seconds - with us holding her hands down and her crying.

At this point, we are waiting for her to undergo something called a Cine MRI while under sedation.  This MRI will look at the anatomy of her upper airway and how it works while she is sleeping.  We will use these images to identify what structures are causing the most obstruction.  From there, we will determine what type of surgery will best alleviate her sleep apnea.  Sounds like fun!

My "baby" started Kindergarten!

Weight and Failure to Thrive:
When we last left off, we were starting Ellie on an appetite stimulant.  Can I just say that on day 5, things started to happen.  I have never ever seen a child each so much!  Ellie's starts eating dinner at 4:30pm and will continue to eat until 7pm.  She is back to eating breakfast, now lunch, and occasionally a snack. Her current breakfast is what she used to consume in an entire day.  I do not know where she is putting it all.  Like a bottomless pit.  Human garbage disposal.

Because of her hunger, we are now able to get her to try new foods.  She has always been a picky eater and that includes freaking out over different foods intermingling with each other - like having icing on top of her cake.  Or pretty much having something other than Nutella touching bread products.  So I about passed out from shock when she stole my healthy spaghetti bolognese, and just chowed down, I was shocked.  Different foods of different textures all touching!

This past Monday we went in for her well child check. Even though she has been eating like a teenage boy going through a growth spurt of the past 6 weeks, she has not gained any weight.  Not even an ounce and her height has stayed the same.  I was shocked.  She is eating so much and I thought that her face looked much fuller.  So she still has not gained back all the weight she lost back 15 months ago, BUT she isn't losing any weight so that is something.  I am still placing the blame at whatever is going on with her GI tract.


GI Issues:
When will it ever end!?  The basic update is: we still can't get a handle on it.  She is still requiring lots of intervention which also include enemas.  I am frustrated.  Andrew is frustrated.  The pediatrician is frustrated.  In fact, her pediatrician is so frustrated that she called Ellie's gastroenterologist herself.  At every visit, for the past 15 months, her GI doc has said "if she doesn't gain weight, then we will do an upper GI to officially rule out celiac".  We have had 4 visits since then and quite a few phone calls - each time Ellie has not gained weight and each time he has delayed the upper GI.  After speaking with the pedi on Monday, he has decided to rule out tethered cord (abnormal attachment of the distal portion of the spinal cord to surrounding tissues ordering) by ordering a MRI of the spine.  Unfortunately, due to scheduling issues at the imaging center, they are not able to do the spinal MRI the same day as her Cine MRI.  I seriously doubt she has a tethered cord as she does not exhibit any other symptoms such as dimples or hair patches on her lower back, weakness of her legs, or obvious changes in her gate, but it is important to rule these things out.  She does have signs of urinary retention (more on that later) and of course the GI issues.

Now this all brings me to my next topic:

Decreased Urination:
Ellie hasn't been what one would call a "frequent" urinator in the past couple years.  She has been able hold her urine for 4- 6 hours for ~ 2 years. Then the time between voids grew farther apart.  Last year, for instance, she would pee 2-3 x during the day and then over night.  When she did urinate, it was huge.  She as having to wear nighttime pull-ups during the day because of her adult-sized bladder.  The past 3 weeks, however, things have changed.  She has been waking up dry.  I thought this was a huge potty training milestone.  Basically, she is now peeing once very 18-24 hours.  She may pee at 4pm and then not pee again until 12 noon the following day.  When she does pee, it is a fair amount.  As in it fills up a diaper.  She will be 6 years-old in a few weeks.  She drinks plenty of fluid for a 30 lb kiddo.  She should be peeing much more.  More in terms of both frequency and in volume.

She is signing "school" and "dance".  Someone was excited for her first day!

I brought this issue up with Ellie's pediatrician.  Ellie isn't potty trained so I do not believe this is an issues of 1. her going and not telling us or 2. holding her urine on purpose.  It is most likely some urinary retention caused by either one of her medications or by constipation rather than a kidney issues.  However, it is important to assess her renal function.  So in addition to Ellie's annual CBC and thyroid panel, we are adding in lab work that will look at her kidney function - think BUN and Creatinine.  A renal and bladder ultrasound has also been ordered.  I know from a previous abdominal ultrasound back when Ellie had her weird clay-colored stools that her kidneys are small for her age (but not necessarily small for someone her size).  The good news is that this ultrasound can be performed the same day as her Cine MRI.

So how's that for an update?  I hope to report back more late next week with regards to the cine MRI and ultrasound.  Until then, fingers crossed that we get her spine MRI on the schedule soon!

More on Ellie's GI Issues:
Sitzmarker Study and Delayed Colonic Transit.
Stomach Issues, Surgery, Anorectal Manometry

More on Ellie's Sleep Issues:
Sleepless in Austin
Polysomnography, Sleep Study Results, Obstructive Sleep Apnea and other things
CPAP for Obstructive Sleep Apnea
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What happened to my Chunky Chicken?

Those of you who were following The Chronicles of Ellie Bellie Bear "back in the day" know that my Ellie was and still is affectionately referred to as "The Chunky Chicken".  She came by this nickname because she was, well chunky.  She had delicious rolls of fat.  My wee little, chubby, sweet cheeks Ellie Bear.  Thighs that you could just munch on.  My Bear was CHUNKY!

Don't you just want much on those cheeks???

Rolls of baby fat!

As with all children, the fat tends to melt away as they grown in height and age.  The baby face disappears.  Usually, the big belly follows, but in the many cases of those with low muscles tone and Down syndrome, the buddha belly is a permanent accessory through adulthood.

Now, my Chunky Chicken has skinny little chicken legs and skinny, little bony chicken wings for shoulders.  Her upper ribs are sticking out of her chest.  She is "chunky" chicken no more.  My almost 6 year-old daughter barely weighs 30 lbs and is in 3T-4T clothing!  She is pretty much wearing the same clothes that she wore last year except for a few items that are now too short.

No more baby face as Ellie is now a "big girl"

Growing up, I was always a skinny little runt.  I barely registered on the growth chart for both weight and height.  So it would have been no surprise if my own daughter was a bit on the petite side.  For Ellie, she barely registers on the growth chart (typical and Ds chart) for weight and is around 70% for height on the Ds growth chart (okay, she is now 50% on height).  She wasn't always that tiny.  Over the past 12 months, I have watched her weight plummet and then has super tiny growth spurts of gaining a few ounces, dropping a few ounces.  I know that I blogged about this last year when we were dealing with GI issues.

This is a Down syndrome growth chart for girls ages 2-18 years
You can see that she has fallen off and flat lined this past year with her weight.
For her length, the velocity of growth is decreasing and she fell from 75% to 50%.

How her numbers look on a typical growth chart [ Right] as well a calculation of her weight:height ratio [Left].
As you can see, the Ds Growth Chart gives a more accurate portrayal on where she falls within peers her age who also have Down syndrome.

The GI issues haven't changed much other than there have been no new hospitalizations related to constipation (a huge success in my book).  She has a few good weeks of regularity followed by either explosions running down her leg into her boots (we have gone through more pairs of shoes this way) to then 4-9 days of being completely blocked up.  That being said, when she does go a while without a bowel movement, we have seen success with either 2 suppositories or 2 nights of enemas vs. the usual 3 enemas or hospitalization.

The thing is, girlfriend eats.  She is chowing down on a croissant with Nutella in this pic.  Now she may not eat much compared to another child her age, but she is eating more than she was a few months ago.  Actually,y she ate a ton a few weeks ago while visiting my parents and a tried several new foods so I was pretty shocked to learn that she only gained 2 ounces in the past 6 months.

The thing is, 15 months ago when things were really bad, she didn't lose any weight.  July of last year was like a light switch went off.  Ellie had anorectal manometry under anesthesia to rule out Hirschsprung's (which she doesn't have) and then didn't go for 9 days.  In one week, she lost 1.5 lbs and then continued to drop through September, reaching 28.5 lb (original weight 32.5).  With us diligently staying on top of her constipation, implementing various feeding techniques, and trying to add calories to her diet, Ellie's weight has finally crept back up to 30 lbs.  However, we learned at her follow-up GI appointment last week that she only gained 2 ounces since her last office visit . . . 6 months prior.  This is frustrating because Ellie is actually eating more (still not as much as a typical 5-6 year old) - both in quantity and in variety of foods.  Because of this, Ellie gets to keep her Failure to Thrive label and we are starting her on Periactin.

This is how Ellie's BMI falls on the pediatric chart.
I have no idea if there is a BMI chart specific for individuals with Down syndrome.

I knew that Ellie was thin, but she was always wearing t-shirts or dresses and with her buddha belly, she never looked terribly skinny.  So, I never truly realized how bony she was until I snapped this pic of her in a halter top.  Granted, she is running and moving her arms back-and-forth which I believe accentuates her boniness.  

Periactin or Cyproheptadine is a type of antihistamine that also has anticholinergic properties and an affect on serotonin.  So yes, it is used to treat allergies, but it also has great success in the treatment some psych related issues such as serotonin syndrome and also treating in pediatric migraines.  One side effect to Periactin is weight gain.  It is considered to be an appetite stimulant for people who have poor weight gain.  We have discussed this med periodically in the past year, but were reluctant to start her on it as one of its major side effects is constipation.  Right. . . . let's put the kid who doesn't poop for 9 days on a med that causes constipation!  However, we are at a point where even though she is eating more, it still isn't enough and she is not putting on the weight.

She still fits in the dress on the left, but it is a bit shorter.
Same shoes and still too big!

As her doc has been saying repeatedly "if she doesn't start to gain weight in the next x # of months, we will do an upper GI to completely rule out celiacs" -- well, those x # of months keep changing and  I personally feel it is time to officially rule out celiac, especially since her upper GI from 22 months of age showed small white blood cell focalization of the small intestine which could have been an early indicator.  Why is he dragging his feet?  I am not sure, but we will revisit the upper GI in 6 months if she doesn't start putting on the pounds.

My little picky eater ate 5 bits of brisket this past weekend and tried raw onions.
Truth: she spent more time playing with the onions and pickles than eating - telling us "circle".

*Update: We are day 5 into the periactin and haven't really seen anything yet.  In fact, it has made her constipation worse and she is eating less than before this medication.  I can provide her with opportunities to eat, go through all of the techniques we learned speech/feeding therapy and in the end, I cannot force her to eat orally.  Right now she only gets it at night because it can cause fatigue.  We will be increasing it to twice a day by the end of the week.  I am told that can take a few days to increase her appetite so fingers crossed that we start to see some big changes in her appetite and in turn, weight.



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