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
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.|
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; slideshare.net|
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.
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 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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