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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CPAP for Obstructive Sleep Apnea

*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*

Has it really been over a month since I last blogged about Ellie's sleep issues???  Apparently, it has.  This is where we left off: Treatment plan and health risks associated with obstructive sleep apnea [OSA].  To learn more about Ellie's sleep issues, read here.  But first, a few little photos from Easter:

We finally got in to see Ellie's ENT doc at the beginning of March.  Her sleep specialist fought to get her an appointment back in mid-February, but the ENT was out of town.  Anyway, the purpose of the ENT appointment was to evaluate whether or not Ellie's adenoid had grown back.  If so, we would then proceed with a repeat adenoidectomy (because surgery is so much fun!) in hopes that would "cure" her OSA and "oxygenation instability".  I had 2 choices: lateral neck x-ray or a flexible nasal laryngoscopy.  I went with the laryngoscopy.  Yes, it sounds mean, but it is quick.  It provides more detail information.  Plus no radiation is involved. Plus, I have this perverse obsession with seeing people's insides (it is the nurse practitioner in me - especially being a former ENT NP).

Ellie's procedure was the pic on the right.

This is how it went: the nurse kindly sat with Ellie.  Her legs over Ellie's and her arms around Ellie's. I then stood behind the nurse with my hands holding Ellie's head straight. Now, a laryngoscope is essentially a spaghetti-like noodle with a camera on the end.  It passes through the nose and looks at the structures of the upper airway.  It can be used to diagnosis enlarged adenoid and tonsils, enlarged base of tongue, laryngomalcia (floppy airway), abnormalities of the vocal cords, and even reflux.  How did Ellie Bear do, you ask?  Well, she fought it all while screaming "NO THANK YOU!  NO THANK YOU!  NO THANK YOU!"  Girlfriend is nothing, but polite.

The findings were not what I was hoping for.  It may sound strange, but I wanted that adenoid to be there.  She is obstructing.  She has had nearly nonstop sinus infections again since August.  If it had regrown, we could have "easily fixed" it.  Nope.  The adenoid did not grow back.  While she does not have an abnormally large tongue, she does have a large tongue base.  She also has not only a high arched palate, but a long palate that dips into the upper airway.  At rest, between the tongue base and the palate, she is already in a partial obstruction - this is while awake and upright.  With sleep, the muscles relax and while laying down, it allows everything to collapse back into her airway.  We also learned that she had reddening of the epiglottis suggesting reflux.  Goody.

Depicts large tongue base causing obstruction of air flow
Photo courtesy: Dental Magazine - unable to find vol #

Take the above pictures and add it to the pic below and that is Ellie's anatomy

Depicts soft palate obstructing air flow

So guess what we have been doing these past few weeks?  CPAP mask desensitization!  The fun never stops.  By the time all was said and done - the doc writing the script, insurance approval, equipment ordered, and equipment training, we finally started on March 26th to desensitize Ellie towards wearing her CPAP mask.  Let us pause for a moment to think about how that.  Desensitize.  Ellie.  Is it going to take years like bath desensitizing did? Save me now!

It's Fighter Pilot Mickey

Continuous Positive Airway Pressure [CPAP] is considered to be a type of ventilation therapy in which positive pressure is blown into the airway as a treatment for obstructive sleep apnea.  We are trying what is called a "Pixi" mask.  This is a mask that fits over her nose only, as opposed to a mask that fits over both the nose and mouth.  You need to create a tight seal or otherwise the pressurized air will leak around the mask.  If she starts to mouth breath, the air will escape so there is a strong possibility she will need the full face mask. 

During Speech and OT.
We have adjusted it since then so that it isn't digging into her face.

You can just imagine how this is going.  My daughter has some significant sensory avoidance issues when it comes to her face, hair, and head.  Tooth brushing requires some interesting gyrations, which involve my legs over hers and my arm holding down her arms.  Combing or washing her hair is considered a form of torture.  Now she has to wear a mask over her face?  One that blows pressurized air into her face?  Right. . . 

Co-therapy between speech therapist and occupational therapist
in the sensory gym.  Also trying out a compression vest since Bear has
been behaving like the Tasmanian Devil on steroids.

So desensitizing is a  s l o w  process.  A step by step approach.  Ellie is to build up to wearing her mask for 15-20 minutes, 3 x day.  Then we add hooking it to the machine and turning it on - again 15-20 minutes, 3 x day.  After that, falling asleep with the mask hooked up to the machine and turned on.  We are making progresses. . . sort of.

"yeah, I'll totally wear it if Papa is next to me."

At therapy (ST/OT), she will wear the mask without too much difficulty.  She has worn it for 2 therapy sessions for ~30 minutes!  At home, she will touch it, but shout "NOOOOO!" when she sees me hold it.  She will allow Mickey Mouse to wear it. She will try to put it on Minnie Mouse.  Once, she tried to put it on herself - in the waiting room at therapy.  Apparently, she associates therapy with the CPAP mask now.  Once, she allowed me to put the mask over her face just before we went for a ride in the car.  I am also able to place the mask on her once she is already asleep, but am not able to turn on the actual CPAP.  Progress is progress.  No matter how slow it is.  

Traitor!  You said this would be fun. You said I was going to be a fighter pilot.

Our sleep doctor told us to follow up in 2 months time.  It is said that if she will not wear the mask with CPAP within 2 months, she will most likely never wear the mask (well at least in the near future wear the mask.  Maybe in 10 years she'll wear it, but that doesn't really help the now).  That means Ellie would have something called a Cine MRI, which is basically an MRI in motion to look at the airway while the patient is under deep sedation.  The Cine MRI helps determine if surgery is necessary and if so, what part of the upper airway needs to addressed.  So yeah, I want the CPAP to be tolerated and to work.

My little dancer who recently discovered her shadow.

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Part 2: Treatment Plan and the Health Risks Associated with Obstructive Sleep Apnea

This is a follow up to Part 1: Polysomnography, Ellie's sleep study results, and OSA

The sleep study results landed Ellie with the official diagnosis of Obstructive Sleep Apnea with hypoventilation and oxygenation instability.  Isn't that a mouthful? 

Obstructive sleep apnea [OSA] and sleep disordered breathing [SDB] can have a huge impact on health.  Actually, it can be very detrimental to one’s well-being.  I still shutter when I think about what Ellie is at risk for if we do not treat her OSA.

The symptoms above are what we in the health care field refer to as "red flags".  Some of the symptoms may mean nothing at all or may signal a sleep disorder.   Yes, snoring is a sign of OSA, but not everyone who snores has OSA and not everyone who has OSA snores.  That being said, chocking and gasping while sleeping is a neon sign indicative of OSA.

In addition to the signs listed above, kids can present with a few other symptoms (Ellie's are in purple): 

• anger control issues
• difficulty swallowing 
• irritability
• daytime sleepiness
• frequent runny nose
• mental brain fog
• hyperactivity
• impulsivity
• inattentiveness
• poor school performance
• memory loss
• headaches
• learning difficulties

This pic is from 2 years ago before her T & A.  Notice that she is propping herself up or sitting up to sleep?
These are also signs of sleep apnea in children.

If you have been following this blog for any amount of time, you know that my daughter has severe ADHD  and had shown signs prior to turning 3 years-old.  You also know that when she has poor sleep, it is like ADHD on steroids—as in it is like she never took her Adderall.  Her ability to function at school is pretty much nonexistent.  She has trouble participating in class.  Trouble sitting in circle time, lunch, art, music, therapy.   Trouble concentrating and following directions.  Impaired social interaction as she is “grabby, impulsive, irritable”.  

In fact, studies (several posted in the medical journal Pediatrics between 2009-2013) suggest that there is a blurring of lines between ADHD and OSA. According to the American Sleep Apnea Association, at least 25% of children misdiagnosed with ADHD actually have a sleep disorder, specifically obstructive sleep apnea.  This may be because poor sleep may damage neurons within the prefrontal cortex of the brain.  I have no doubt that Ellie’s sleep apnea and early wakings manifest as ADHD behaviors.  All of that being said, I do believe that she has underlying ADHD just because of our family history.  It makes me wonder though, if we treat her OSA and we treat her ADHD, how well will she do in school?  How will her friendships evolve?  How will our home life change and be improved?

Photo: ADHD Caregiver Day

This goes beyond behavior though.  Obstructive Sleep Apnea MUST be treated.  It CANNOT be ignored.

Untreated OSA and the resulting high levels of inhaled CO2 and poor oxygenation can lead to:

• Increased heart rate and blood pressure
• Cardiovascular disease
• Increased risk for stroke
• Increased blood pressure
• Unstable blood glucose and insulin levels, leading to diabetes
• Memory loss
• Kidney problems
• Hormonal imbalance, especially with testosterone

All health issues that can eventually lead to poor quality of life as well as death.  Death! Oh goody.  Bear is only 5 years-old, so what will her life be like at 20, 40, 50 if this isn't managed?!

In children, the usual culprits are enlarged tonsils and adenoid obstructing the upper airway.  Nearly 2 years ago, this April, Ellie had a tonsillectomy and adenoidectomy [T&A].  She had a never ending sinus infection that was resistant to antibiotics and nasal sprays and it lasted months.  She also had frequent ear infections that have required a set of ear tubes every 6-9 months.  In those last 2 months leading up to her surgery, she was propping herself up to sleep.  The T and A  essentially cured her.  After surgery, she didn’t have a sinus infection for 11 months.  ELEVEN months!  Ellie’s last set of ear tubes was in April 2013. 

Sadly, all of that has changed.  This past August is when she started up again with the recurrent, frequent sinus infections.  August is also when her sleep issues started.  She had fluid in her ears this past fall and an ear infection around Christmas.  In December, I asked her ENT doc if her adenoid might have grown back. . . I was told it is rare and too soon.  We were advised to wait until the summer and if she was still having sinus issues, we would reassess. 

Oh yes, I did say it. THE ADENOID CAN GROW BACK!  Yes, it isn’t common, but I laugh in the face of low percentages and low risk.  After all, my odds ratio of having a child with Down syndrome was 1/1300.  A 2011 study in Laryngoscope, show the rate of adenoid regrowth requiring a secondary adenoidectomy to be 1.3%.  Another study in the International Journal of Otolaryngology revealed that those with adenoid regrowth tended to be younger at the age of their initial adenoidectomy as well as had a much larger adenoid than the general population.  Ellie was 3.5 years-old at the time of her T&A and I was informed that her adenoid was “very massive with extreme crusting” aka large and nasty. 

Ellie’s Treatment Plan :

1. Follow up with ENT to rule out adenoid regrowth and palate abnormalities.
2. If there is no adenoid regrowth, begin desensitization for CPAP (more on that later.)
3. If CPAP is not tolerated, discuss further surgical options dependent upon cause of obstruction a. Palate Surgery for long palate obstruction  or  b. Genioglossus advancement aka base of tongue advancement surgery.  Again, more on this later

Ellie’s Sleep Doc wanted her seen by ENT the week we got her results (2/9) or last week.  Unfortunately, her ENT is out of town for the rest of this month.  Thankfully, ENT and sleep doc coordinated and made sure the Ellie is seen the first day Dr. Z is back in office.  So on March 3rd, Ellie will have her ENT visit.  Meanwhile, we have that many more days until we get answers and even longer before we can treat her OSA.

Bring on the coffee!

Part 1: Polysomnography, sleep study results, and obstructive sleep apnea

Tonsil and Adenoid Removal (T&A) Recovery Tips

From a few years ago--I want to go back to sleeping!

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