Verity's Next Big Thing

Verity's tenotomy surgery and ensuing cast (and cast removal) adventures seemed to dominate our November and December posts in her group. So not too many people know about our visits to her ENT and the sleep doctor in mid to late December, a long-awaited follow-up to the sleep study she had in September.

Immediately after her study was over--as in, that very same afternoon--we received a phone call from the nurses on duty who had read over the report by the respiratory therapist who observed Verity overnight. The study still indicated severe obstructive sleep apnea (OSA), and we were instructed to continue administering oxygen via nasal cannula at night or during naps as needed.

This was somewhat surprising news: Verity's sleep patterns seemed much improved, and she didn't exhibit any of the typical signs of OSA such as snoring. We had thought reflux was the culprit of the obstruction from months past, and with the start of the continuous feed and significant reduction of vomiting during January through the summer, we thought we were on the right track.

Interestingly, though, as we started monitoring Verity with the pulse oximeter and having oxygen at the ready, we noticed that she either had a number of nights in a row during which her saturations would be in the high 90s without oxygen, OR she would have noticeable snoring and need even more oxygen than originally recommended (only 1/32 - 1/16L). We resigned ourselves to having to wait for more information, since the "soonest" they could get us in to see her doctors was December, nearly 3 months after the sleep study! Meanwhile, we were grateful for a fairly consistent night nurse schedule as well as a friend who filled in (and still does fill in) to give Verity care and monitoring during the night. (As an aside, nowadays Verity does need oxygen almost all the time during her naps and at night. It can fluctuate between 1/32 - 1/8L, only needing more if she is fighting a respiratory illness, which, thankfully, has only happened a couple of times this season, and without a hospital stay! Verity does snore pretty substantially, fairly often, though positioning does affect this of course.)

So our first respiratory-related visit in December was with the ENT, who did a quick upper airway scope through her nose right there in the office, revealing nothing other than inflammation from reflux. (That answered my question of whether reflux was still an ongoing issue--clearly it is, even though we don't always see it coming OUT.) He did decide to put her on some nasal drops (Flonase) to help with some swelling to see if that would help (it hasn't noticeably). And then he said we would do a bronchoscopy (evaluation of the airway) to take a closer look at what is causing the obstruction.

A week later we saw the sleep clinic doctor, and she agreed with the ENT that a pulmonologist should be on board with the bronch, so together they have coordinated their schedules for the procedure, which will be while Verity is fully sedated. If the ENT sees something that can be corrected surgically (enlarged tonsils or adenoids, for example), he will do the surgery while Verity is already sedated. Meanwhile, the pulmonologist will check Verity's lungs, taking fluid samples for examination/culturing. (This is called a bronchial lavage, or lung washing. Sounds fun, eh?!)

The plan is to repeat a sleep study at least 6 weeks following the bronchoscopy to see if her OSA has improved. If it has not, then we will move toward fitting her for CPAP. Since Verity is still so tiny, the sleep doctor is reluctant to make that our first step...or rather, our next step, I guess, since we have been addressing this issue for over a year now.

These procedures and surgeries are pretty common in the Trisomy community, as our kiddos are smaller than usual but their tonsils and adenoids may continue to grow at a more "normal" rate. Still, it's always concerning when our little ones must undergo anesthesia--thankfully we have now noted CLEARLY in Verity's records that she is not to be given fentanyl!

Please pray with us for the February 5 procedure and recovery afterward. Thank you in advance!!

Recent Appointments

Life with Verity is never dull, that's for sure! We have wrapped up another month of appointments, and here are the highlights.

From the GI follow-up:

• Verity is 15 pounds, 1 ounce! I was hoping for 15 pounds by her 15-month birthday, and she reached that! (May 28 was her 15-month bday!)
• We are back to giving 15ml prune juice twice a day to ease constipation. (We had stopped when vomiting was prevalent after giving it.)
• We are continuing to give her Prevacid twice a day for reflux, not wanting to change that AND her feeding regimen just yet, but hopefully later in the summer we will drop to once a day and see how she does.
• We have begun a S-L-O-W transition to one bolus feeding per day as follows:
• At 11:30am we stop the feeding pump.
• At 12:30pm we begin the bolus feeding.
• The first few days we gave 35ml over a 30-minute period.
• When we tried to bump up to 40ml, she vomited significantly, so we backed down to the 35ml for a few more days.
• Today we gave 40ml but did not compress it to a 30-minute period, instead letting it run at the same rate (70ml/hr), and she did great!
• Every 2-5 days we will adjust the volume OR the rate, working them up slowly to the goal of tolerating 60ml over a 30-40-minute period.
• At 2:30pm we resume the continuous feed (30ml/hour for the next 21 hours).

Overall it is clear Verity is growing and thriving, though we still deal with vomit almost daily and constipation (relieved instantly when we give her a liquid suppository--she just needs help getting it started). She is much sturdier, less floppy, with greater head and even trunk control, but still not able to sit on her own yet.

From the Sleep Clinic Meeting:

• A repeat sleep study has been ordered since so much has changed since our last one in November. (Earliest opening = end of September, but we are on a waiting list.)
• The doctor recommended establishing care with a neurologist, which is something I agree with--she has never been seen by one, but Trisomy 18 kiddos often deal with seizures, and I confess this is an area in which I have much fear over what the future may hold for Verity.
• The doctor also ordered a check of Verity's thyroid levels, which turned out to be normal.
• We discussed the fact that studies show propping kids up (laying them on an incline) during sleep may not actually help with reflux and can worsen the airflow obstruction. We have laid Verity flat ever since, and lo and behold, she has slept better overall!
• She noted Verity's high arched palate and said we may want to address this in the future. She gave me info for a dentist who specializes in this.

From the Surgical Consult:

• Verity's umbilical hernia does not require surgery now.
• If it has not resolved by age 4, surgery may be done to correct it.
• No concerns here!

Whew! Coming up next week is an evaluation with a speech therapist and a check-in with the audiologist to see how things are coming with her BAHA (which she is wearing anywhere from 3-10 hours a day in various stints).

Pulmonology

Today, a visit to the newest member of Verity's Village: Dr. Tina, our pulmonologist. After chatting with other Trisomy moms, I am kind of amazed that we haven't made it into the Breathing Institute before now, especially since we already did a sleep study! I guess typically those are ordered and evaluated by a pulmonologist, but anyway, the important thing is we had a study AND that today we had the opportunity to speak with another specialist. Even though Verity has had oxygen at night, we have not noticed ANY improvement in her sleep patterns, and if anything, it has been even more difficult because she hates the nasal cannula!

Here are the notes from today's visit...but first, the exciting news: Verity is ALMOST to 13 pounds! Her weight today was 12lb, 15.4oz, a nearly 10-ounce gain since December 19 when we discovered she was losing weight. So, the continuous feed is working! (She has had a few vomiting episodes, but not nearly the volume as before--we can tell she still suffers from reflux, but at least she is keeping most of her food down.)

Dr. Tina would like Verity to go back to the sleep clinic, this time with the intention of looking at CPAP/BiPAP. She thinks we can do better with having Verity do some desensitization with a mask prior to going in, and then having the specialists adjust settings to see what works best for Verity. My only fear with all of this is...how will Verity suck on her pacifier with a mask?! LOL But hopefully if she is able to breathe well, she can sleep deeply and not wake every few minutes wanting her pacifier...?

Because Verity has not had any respiratory infections or pneumonia, Dr. Tina doesn't feel she needs a bronchoscopy at this time, something that most Trisomy 18 kiddos end up having (this is a test that requires sedation). I was reluctant to push for one just for the sake of having one--I really like this doctor and trust her judgment.

Finally, she is putting in a request for us to get another pulse-oximeter, which we haven't had since we turned ours in when we left Omaha. Our ENT didn't seem to think we needed one, even though Verity's sleep study showed her sats went as low as 74%!! So, I will be glad to have one so we can monitor her more regularly.

Catching Up...a Bit: Sleep Study & Results

Oh, my goodness...it has been about a month since I updated Verity's blog!! Aside from what I already wrote from last month, November was the Month o' Dental Appointments: myself plus 7 children for regular cleaning and exams, plus 3 children needed sealants fixed/replaced. Only two of those days allowed for back-to-back exams, which meant I was taking someone to the dentist 7 days in November and 2 in December! Oy!

Anyway...as far as Verity's schedule, she didn't have much going on until toward the end of the month aside from her weekly PT and OT sessions. But we did have a couple of significant things happen that last week in November, one of which isn't really worth writing too much about other than to say we had a three-hour, in-home assessment with a navigation coordinator who is helping us write up our application for Medicaid waivers, which can help Verity get more assistance in the future. Since I won't know anything for awhile on that, I will just leave that there and continue with the next and more pertinent item.

Our sleep study, which was originally scheduled for January 8, got bumped up when someone cancelled and we were able to get in on the night of November 26. This was a huge blessing we weren't expecting! It was a L-O-N-G night for both Verity and me...Verity was NOT thrilled with all the wires and gear she had on, but after an hour of screaming (!!), she finally gave up and fell asleep for a couple of minutes, sneezed, woke herself up, and screamed another 10-15 minutes before finally sleeping about an hour. We woke several times in the next couple of hours, and then she had a really rough patch from about 2-3am, finally settling down and sleeping about an hour before waking a few times before the respiratory therapist finally put us out of our misery at 5:45am and let us go home, lol.

The results of the study were sent to our ENT immediately, and we followed up with him a few days later at the earliest appointment they could give us. Not too surprisingly, we learned that Verity has a form of sleep apnea, severe obstructive apnea, although apparently not as severe as some of the kiddos we've met online! (One poor little guy had over 50 episodes an hour! Verity's average was 17.) The good news is that with just a TINY bit of oxygen, those episodes nearly disappeared altogether. So after about 24 hours of various phone calls, we got our oxygen and supplies on Friday, December 1. She only needs 1/32 of a liter per minute, and she only needs it at night, but we haven't QUITE convinced her that it is in her best interest to keep the cannula in!

Now that it's been over a week, we have a bit of a regimen...unfortunately, it still does not include the boots and bar, which seemed to be a bridge too far in getting her to sleep. So we are working on giving her some B&B time in the day until we can finish this transition with the oxygen. It works best when she is pretty sleepy if not already asleep and we can sneak the cannula under her nose and secure it onto her cheek patches.

We are making incremental progress...while we are still waking multiple times at night, it does seem to be less often, and it seems we all get deeper sleep when we are sleeping. Last night was the best yet...though it took a bit of time to settle her down, Verity slept maybe 2 hours, woke briefly, slept an hour and 20 minutes, had an absolute meltdown over the cannula from 1:50 - 2:15am, finally fell asleep again, and didn't wake up until nearly 9:00 this morning!!

I will end this update here, as the GI issues warrant a separate post...but meanwhile, we praise God for revealing the apnea to us sooner rather than later and for allowing us to get started helping Verity on the road (hopefully) to better sleep patterns.