Down Syndrome Awareness - Our Tonsillectomy/Adenoidectomy Outcome

I know I've posted some disjointed information regarding the boys' recent experience with their back-to-back intracapsular tonsillectomy/adenoidectomy surgeries. I've noticed some interest in my blogs traffic report and I've received questions from other parents -- whose children also happen to have Down syndrome and may suffer from obstructive sleep apnea -- about the process we went through of identifying the need, undergoing the surgery, surviving the recovery and, finally, the positive outcome we've experienced. As such, I thought I'd put it all down in writing here to serve as a point of reference for others.

Note: this documents our experience including the information I ferreted out on the internet; gathered from other mothers; received from medical personnel. This story specifically pertains to my boys' tonsils and adenoids, how my boys reacted to the surgery, their particular recoveries, setbacks and, finally, our impression of the outcome. You may or may not agree with the information I was given, get the same recommendation for your child, or have the same outcome. I am simply sharing the information as another point of reference for other parents going through it. It would have been less worry and research for me if someone had shared all this with me 2 years ago. Hopefully it helps.

After recognizing that the boys had unusually large tonsils, at age 2, I asked my pediatrician to refer us to a local Pediatric ENT. We visited the new doctor eyeballed their tonsils and told us that although their tonsils were a 3.5 and 4 on a scale of 1-4 (4 being the largest), he didn't do surgery on children before they were 3 because they were still growing and their tonsils may not grow with them. "Better to wait a year and see." At 3, I returned because we were experiencing sleeping difficulties and snoring AND their tonsils were still HUGE to my untrained eyes. Now, the Pediatric ENT said they rated a 3 and 3.5 (reversing the twins' charts... but that's a different problem). Once again he suggested we wait a year because, he said, they didn't like doing surgery on such young children. Additionally, the doctor quoted adult-level statistics on sleep apnea and stop-breathing indicating that if my children were not experiencing this level of sleep interruption, then surgery was not warranted. I said I didn't think my children were experiencing that adult-level of stop-breathing and apnea episodes. So, we left with a recommendation to, once again, wait another year and see if their tonsils shrunk or the apnea miraculously went away. Meanwhile, I knew that my children were losing sleep and suffering extended illnesses -- though not strep or tonsillitis -- due to their lack of sleep. I was worried about how this might affect them in the short- and long-term.

Dissatisfied with the doctor's answer for the 2nd year in a row, I decided to do a little research on my own. I discovered that there is an increased propensity for children with Down syndrome to suffer from obstructive sleep apnea (OSA) because their typically smaller-than-average oral cavities -- a common developmental side effect of DS -- crowded by over sized tonsils and adenoids often results in blockage of the already narrowed airway especially when the child is at rest in the prone position (laying on back) where the tongue would relax into the back of his/her small mouth and further prevent the passage of air into the lungs.

In asking other parents of children with Down syndrome about their specific experiences, I found that the majority had undergone the tonsillectomy/adenoidectomy surgery by the time they were 5-7 years of age (typically earlier) to prevent illness and/or to unblock the airway and relieve OSA symptoms. Some had endured the painstaking process of proving the need based on scientific sleep studies that did or did not support the OSA diagnoses because they're not really designed for children. Others had forgone the intrusive and time-consuming sleep studies either because their ENTs were familiar with the common issues faced by children with DS and accepted their description of the OSA-patterns or because they provided their ENTs with video tape evidence of the OSA-patterns. These patterns, which my children experienced, included frequent stop-breathing episodes that lasted 10-15 seconds and occurred sporadically and/or frequently throughout the night resulting in a deep sucking in of the abdomen and sudden gasping for air as well as frequently sleeping in the upright/sitting-up position, sleeping folded in half (sitting then falling forward), balled up on hands and knees, restless sleep, crawling all over the bed, propping themselves up against the headboard, footboard, me, my husband, pillows, bed rails etc. all in an effort to open the airway, breathe and stay asleep! To no avail!

In my ongoing research, I discovered that the full removal of tonsils in a child with a structurally different oral cavity (as is possible with DS) could result in the potential collapse of the soft pallate as the base and tonsil act as a sort of retaining wall for that structure. Though rare, collapse would mean extensive reconstructive surgery. Additionally, full removal could leave two gaping holes in the back of the mouth where ingested food could gather and spoil increasing the propensity for repetitive infection. Another possibility I wanted to avoid. On the flip side, not fully removing the tonsil -- a partial tonsillectomy -- could, though rarely, result in the tonsil growing back.

Reaching out further, I began asking other tri-state area mothers of children with Down syndrome who had already undergone the surgery for their stories and a referrals if it was a good experience; requesting the names and phone numbers of the medical professionals they used. Without exception, the mothers I spoke with noted that the Pediatric ENTs they visited had experience treating children with Down syndrome. I contacted one of these regional medical professionals asking for a recommendation for someone in their field experienced with children with Down syndrome that practiced closer to my home. I got 2 references, checked on availability and insurance and was dead-ended. But, I had received invaluable confirmation that, for a child with Down syndrome, the surgical procedure could be potentially different depending upon my children's specific and DS-related issues and oral cavity structure as determined during the first examination. I had to find a local Pediatric ENT who had specific experience working with children with Down syndrome.

At the boys' school, I asked a mother I knew whose child with special needs had recently gone through the procedure (though he did not have DS) and I cross referenced that with recommendations I received from a Developmental Pediatrician practicing in the same building complex as the boys school who was kind enough to speak with me briefly about our dilemma. Finally, I went back to my Pediatrician armed with extensive information and asked again for a referral to a Pediatric ENT, this time requesting specific experience working with children with Down syndrome. He gave me 2 names, one of whom was already on my short list. I called the doctor's office, confirmed that he accepted our insurance and made an appointment (or 2). I knew I had my man!

Our first visit was pleasant, quick and very educational, ending with my making two back-to-back appointments for their surgeries. The good doctor examined the boys' tonsils and adenoids using ENT tools of the trade (not eyeballing like the first doctor), asked pointed, quantifying questions about their sleep- and illness-patterns (how often/how long) and then explained that he could definitely help and would like to perform a less intrusive version of the typical removal of the tonsils and adenoids because of the boys' specific issues -- or lack thereof. The tonsils didn't really need to be removed as they were still working as germ filters as evidenced by the lack of strep and tonsillitis infections but the repeated long-lived illnesses were a function of their bodies' inability to heal quickly due to lack of sleep and ongoing fatigue. And, oddly [for a child with DS], their adenoids were of near normal size and would also not have to be removed. However, because of their small oral cavities and narrow airways , their significantly enlarged tonsils (all developmental side effects of Down syndrome) definitely presented an airway obstruction and the sleep patterns I described were consistent with obstructive sleep apnea. An intracapsular tonsillectomy/adenoidectomy is less intrusive and is said to be 90% less painful and an 80% quicker recovery. The procedure involved shaving -- rather than partially cutting or fully removing -- the protruding tonsils and any adenoidal bumps in the way leaving some remaining tonsils and nearly all of the adenoids to function as originally designed. This procedure would clear the airway resulting in significantly improved sleep... reducing fatigue and improving their overall health and vitality.

Our pre-op appointments were uneventful. The staff of the day-op center (non-hospital, outpatient surgical facility) were all friendly, informative and professional. We'd had an unpleasant first experience with anaesthesia during the boys' MRI's as infants. But, the day-op anaesthesiologist explained clearly how he would attend to their slower metabolic out-gassing of the drug (another potential side effect of DS) by giving a lesser dose through a breathing mask which would ensure only the minimum amount needed to keep them asleep during surgery and which would be removed immediately following the surgery, resulting in a much quicker wake-up. The day of our surgery we were prepared thanks to the explicit pre-op instructions we received. I changed my big little man into a miniature hospital gown and suited up in a paper suit to wait in the sterilized pre-op room while my husband waited no longer than a half hour with my littlest man in the waiting room. We were called to surgery quickly -- arriving at our operating room on a motorized Harley Davidson motorcycle. I laid my baby down, sat by his side, kissed him before the mask went on and within a minute and a half he was fast asleep and I was escorted back to the pre-op waiting area where I was met with my 2nd little patient. Within 45 minutes, the doctor/surgeon had finished with our first guy and I made the trip with my 2nd little boy to repeat the process. Within 2 hours from our names being called in the waiting room, we were in recovery with both boys awake and pleasantly-enough (read: not crying) watching "Dora the Explorer" on tv and eventually sipping apple juice. The surgeon came in excited to see the boys so alert as he explained that he was pleasantly surprised with how the procedure went. He was able to shave away more of the tonsil than he expected, further clearing the airway and leaving a much smaller tonsil base which required significantly less area to be cauterized (read: less pain). He provided us post-op instructions that were less restrictive than those for a full tonsillectomy/adenoidectomy and we were cleared to leave -- a mere 5 hours after arriving -- and were home 30 minutes ahead of schedule and ready to facilitate the boys' continued recovery.

The boys were pleasant enough as we fed them a soft diet of ice cream, pudding and yogurt at first then, finally, undercooked hot dogs and pizza because my meat-eating boys don't like pasta and refused absolutely everything else. We medicated the pain with Tylenol (note: research shows that children with DS may not express the pain they feel as accurately as those without DS and so, should be treated for the average pain for any given ailment as opposed to the pain expressed.) and we restricted our use of Tylenol with codeine to night time in order to avoid problems with constipation. Still, as each day passed, their temperament deteriorated, their fevers elevated and their liquid intake waned. By day 3, no longer willing to swallow and 4 days without a bowel movement, we found ourselves contemplating the necessity of an ER visit for rehydration. Finally, at 9PM that night we were advised by the surgeon on call to take the boys to the ER to receive IV fluids. After 3 and 4 brutal IV picks respectively, the boys finally settled down to receive their fluids, sound asleep. Unfortunately, due process in the ER is to perform a chest x-ray if a child comes in with a fever. Despite my best efforts to dissuade them from waking them again and explaining that the fever was an after-effect of the surgery, the tech arrived to take them away for x-rays.... Thankfully. Our first chest x-ray came back positive for pneumonia and the second came back negative. Daddy got to take our littlest guy home to sleep at 4AM while Mom and the big guy checked in for a 3-day stay of rehydration and antibiotics to clear his lungs. When his highest fevers receded and his blood oxygenation leveled off at 96, even during sleep -- proof that the surgery was successful in clearing his airway -- we were released to go home despite NOT meeting the discharge requirements of fever-free and drinking on our own. The next few days were still touch and go as I forced 5ml of Gatorade down his throat every 20 minutes of every waking hour to avoid dehydration -- a hint I wish I'd received on discharge from the day-op center.

Finally, a full 14 days after surgery, both guys were drinking on their own and regularly exceeding 2 wet diapers per 24 hour period... They were sufficiently hydrated and BREATHING freely at night.

Though their sleep is still a bit restless, tossing about, they do not stop-breathe, they rarely sit up or sleep folded in half and they actually do manage to stay asleep through the night without snoring or any significant wake-ups. I can't say the same for me as I wake up just to make sure they're still breathing... I'm not used to the quiet. My husband and I both find them to be happier, falling to sleep and waking up easier and faster, and speaking much more frequently and much more clearly than before the surgery. Their teachers and therapists have reported similar results, noting that they are following routines with less or no redirection needed, often skipping nap time or falling/staying asleep more easily than before, talking and participating more fully in classroom and therapy activities with improved attention and clarity all around. These are the amazing results our wonderful Pediatric ENT (who has experience treating children with Down syndrome) promised us before and after the surgeries.

I just love a happy ending. Don't you?