Thursday, October 29, 2015

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

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

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

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
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;


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.


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?!


Propst, Evan (2015). Lingual tonsillectomy and midline posterior glossectomy in children with obstructive sleep apnea.  Operative Techniques in Otolaryngology

Ishman, S. (2012). Abstract: Pediatric Sleep Apnea and Surgery; Beyond tonsillectomy. Audio-Digest Ototlaryngology.

 photo IMG_2291_zpsudykfi0h.jpg

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Tuesday, October 20, 2015

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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A mask for kids with Down syndrome

Do you hear crickets?  Yes, we are here!  We are alive!  We are safe and healthy!  We haven't been inside a restaurant or store since m...