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!!

Fifteen Minutes

My baby stopped breathing on her own for 15 minutes today.

Did your stomach drop when you read that? It's been 13 hours since I learned that this is how Verity reacted when she was given fentanyl during a simple, outpatient tenotomy surgery. And my stomach is still churning.

Let's back up a bit and start with the easy part: the tenotomy portion went well. Verity is sporting purple casts attached to a wooden bar. The casts are to keep her Achilles tendons flexed, and the bar is to keep the hip abductors positioned just so. Both sets of tendons underwent this lengthening procedure. Verity will wear the casts/bar for 4 weeks. (Note to self: Call to schedule the cast-removal appointment. My brain could not handle making that phone call today.)

I knew Verity would be completely under anesthesia for this surgery. She has been under twice before (once for her g-tube placement and once for a sedated hearing test), and both times she had zero issues.

What I did NOT know was that fentanyl was part of today's protocol. I had asked upon arrival what the pain management plan was, and a couple different nurses assured me the doctor would go over that with me. It was about 45 minutes between when I asked and when it was surgery time. They allowed me to carry Verity to the operating room and be with her while she went under, and then someone whisked me away. Honestly, I totally forgot to pursue the medication question...(trust me, I've carried the weight of guilt because of my memory lapse)...I had realized that Verity's feeding pump and supplies had fallen out of her diaper bag, and I was anxious to grab them and return before they called me back.

It probably took me about 15 minutes to retrieve those supplies from my vehicle...15 minutes during which I had NO IDEA an anesthesiologist was doing the work of breathing for my daughter.

I had hardly sat down in the surgical waiting area when it was all over: an attendant walked me to the post-op room, where I found a very sound asleep Verity and got the run-down from the post-op nurse.

I did not hear about the fentanyl.

We waited and watched Verity. Her sats and heart rate were good, respirations were low (10-11), but that was to be expected, they said.

Verity slept. And slept. And slept.

The anesthesiologist came by to check on her. He said nothing about the incident. Nothing at all about the fact that my baby had stopped breathing while under his care.

Verity continued to sleep.

The surgeon came by to check on her. He said nothing about the incident. He did say he only recommended ibuprofen for the pain.

Verity continued to sleep. And as the two-hour mark approached and she was still sleeping, we tried some creative measures to wake her up. We got close...and then she would go right back to sleep. I wondered if I should be getting worried, but her color was good and by this time she no longer even needed the blow-by oxygen.

The post-op nurse has 40 years of nursing experience, and she was pretty calm. But I think by this point she was getting a little concerned herself. The nurse talked out loud as if reassuring us both that it wasn't too surprising that it was taking longer for Verity to wake up...after all, she's definitely a "lightweight." After all, it was only a little bit of fentanyl that made her stop breathing for 15 minutes.

WHAT?!

I have learned I am not very quick on my feet when things like this happen. It really took me all afternoon to process all of this: I wasn't told my daughter--my almost-21-month-old, 17-pound daughter with special needs--would be given fentanyl, a drug that has killed children in the Trisomy community. Furthermore, the doctors who had my daughter in their care...literally held her life in their hands...did not tell me that she stopped breathing for 15 minutes. The only reason I learned this happened was because the post-op nurse made a passing comment. I had spent nearly 2 hours with her and even SHE did not mention this staggeringly important detail!

Am I crazy to think this is a rather vital detail to be left out of the post-op narrative?!

I have contacted our PCM and received a reply from his nurse right away--she wants me to call the patient advocate at the hospital to get it documented and request a call back from the provider. I do wish I had been able to say something while I was there in person...I think I was so stunned, and then I also needed reassurance that it was in fact all right for me to take Verity home after all this had happened. She did wake up more or less "for good" about 3 hours after surgery, and we have monitored her all day and she has done better than expected, actually. I anticipated a rough few days and nights...she napped well this afternoon, and though the evening was a bit difficult, she is resting well now with her pulse oximeter on and nasal cannula in place.

But let the records show...fentanyl will NOT be given to Verity AGAIN!!!

Thank you, Heavenly Father, for protecting our precious girl today. May she (and we) ever glorify you no matter what...

Current Status (Quo)

I can't believe it's been 2 months since I wrote a blog post! Do you know what that means?! We've been experiencing some kind of NORMAL! That is to say...we've been living the busy life with our kids, and Verity has been growing and developing and thriving in the midst of it all.

So with a bit of time and a quiet house, I decided an update was way past due, especially since we do have some things to report that we haven't detailed on Verity's Voice (our Facebook page).

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First of all, I am pleased to report that Verity's feeding regimen continues to improve as we work toward a more "normal" feeding routine. We are up to 3 bolus feedings now, with only 14 hours of slow continuous feeding, running 4pm to 7am. This means Verity has quite a bit more TUBE-FREE time during which she can practice her moving and grooving! It is SO nice to be able to hold her, carry her, and help her with her physical skills when she doesn't have her "leash" on! While she still isn't eating by mouth, we are doing more and more tasting and doing so while she is having her bolus feeds so that she can begin associating food with a full (or filling) tummy.

So her feeding schedule looks like this:

• 0700: Turn off the feeding pump (ending the continuous feeding)
• 0800: Begin bolus #1: 75ml over a 30-minute period (rate of 150ml/hr)
• 1100: Begin bolus #2: 75ml over a 30-minute period (rate of 150ml/hr)
• 1400: Begin bolus #3: 75ml currently at a rate of 85ml/hr, with rate increases of 5ml/hr every 3 days.
• 1600: Begin continuous feeding: 34ml/hr for the next 14 hours.

Typically during the first 2 bolus feeds, Verity will sit upright at the table in her chair. She has had tastes of applesauce and sweet potatoes by spoon (or on her pacifier), and we also frequently give her a food "stick" to hold and explore with her mouth. She has tried carrot and celery sticks, cucumbers, and apple slices so far. One of her day nurses wants to try a big pretzel rod! Verity's interest level varies from day to day, but I'm in no hurry. She's GROWING!

And speaking of which, I don't have exact measurements--we should get some this week since we have a couple of appointments--but last time we weighed her she was about 16lbs, 12oz, although that was with clothes on. STILL...she had dropped under 16 pounds awhile back when she was fighting off a sickness, so this is encouraging!!

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Last week we had a 6-month check-up with the orthopedic doctor. The good news: Verity's hip X-ray shows all is continuing to look good. The doctor says she should have yearly X-rays as long as she is growing.

The bad news: Verity's Achilles tendons have tightened considerably, and the tendons in her groin are likewise still very, very tight despite the stretching exercises we do faithfully and frequently. So, a November 19 surgery date is scheduled for a tenotomy to release those tendons. After the brief procedure (which will require sedation), Verity will get full-leg casts with a brace between her legs. She will need to wear those for 3-4 weeks, and then we will resume the boots and bar (which we have discontinued at this time).

So...if you think of us during Thanksgiving week, please pray that Verity's surgery goes uneventfully and that she has minimal pain and recovers quickly. We are already dreading the lack of sleep that will undoubtedly accompany this process!

Below you can see her left foot, which is "rocker bottomed," a common feature in Trisomy 18 kiddos. Until our appointment last week, I had not heard a doctor describe her foot in this way. I assumed it looked different from the other foot simply because they were both severely clubbed and had to go through the casting process (a year and a half ago!). You can also see the back of the heel, which will not flex any more than this even if we are trying to help her do so. The right heel is much more affected than the left...we have not been able to get that foot flat onto her boots for some time now, so I was not terribly surprised when I heard the doctor's recommendations.

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In other very quick news:

• Verity's sleep patterns have improved DRASTICALLY! She usually will take a 2 or even 3-hour nap (although sometimes only 45 minutes or an hour) in the afternoon. If she gets a good nap, she typically does have a pretty decent night of sleep. She is even sleeping all the way through the night on occasion!
• I have successfully completed CNA training and passed the Colorado state exam! This involved 4 weeks of night classes and clinical experience, followed by a full day of written and skills testing. I am officially a certified nurse aid! What does this mean? Well...
• In the state of Colorado, family members are allowed to receive payment to care for their disabled loved one. There is an assessment tool that tells how many hours per day one can qualify for, and when a caregiver is certified (or licensed or registered, depending on what level of care), he or she can be hired through a home health agency. I work through the same agency that provides our skilled nursing care, and I'm quite pleased with the training and preparation I received through this whole process. I chart for 4 hours a day, and it amazes me that we are blessed to receive financial help for the tasks I was already doing with Verity. What a GIFT! The Lord knew we needed to be in this state for multiple reasons, and this is just one way He has tenderly shown His provision for us. Jehovah-Jireh, indeed. 
• I will detail later the results of the sleep study we had at the end of September, as I still need to meet with the ENT and later on the sleep clinic doctor.
• Tomorrow we have a liver scan and kidney ultrasound--we do this every 3 months, although this will be Verity's first liver scan. Our T18 kiddos have a higher chance of cancer/tumors, so it's proactive vigilance. 

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).

G-Tube Surgery = Success

Monday was a big day for Miss Verity! If she were able to retain and speak about the memories of her experiences during our hospital stay, she would probably first mention how incredibly hungry she was for most of that time, lol. We had to end her feedings by 8am, with surgery prep beginning at noon and the surgery scheduled for 2pm. Unfortunately, the previous surgery ran late, so Verity's didn't begin until 3:00.

The nurses adored and fussed over Verity!

Waiting comfortably in Daddy's arms.

Ted and I waited about an hour while Verity was in the operating room. I confess I was a bit anxious; my stomach was in knots all throughout the morning. It was hard having our baby go into surgery, knowing she would be under anesthesia. Thank the Lord, all went well, and after it was all over, a nurse took me back to the recovery area, where I got to hold Verity and comfort her. I was so thrilled to see how she clearly responded to having Mommy hold her--there was a noticeable difference compared to when the nurse was trying to calm her! I loved that she relaxed in my arms and became more peaceful.

We were only in this "holding area" about 15 minutes, and then Ted joined us and we moved to our overnight room. It was nearly dinnertime by this point, so we got Verity settled and then parted ways: Ted to go home to take care of the rest of the family (who had been holding their own all day, bless them!) and I to the hospital cafeteria to grab a soup and salad to take back up to our room.

Verity was not very happy when I returned and took over from the nurse! But rocking and singing to her helped. I ate my dinner very slowly, one-handed, over the course of an hour and a half before she calmed down a bit. She had to wait 6 hours before feeding, and then when it did start, it was to be the slow, continuous feeding that we typically do at nighttime. That seemed to make sense and lined up with our usual schedule; we began the feed at 9pm, and I pumped and hoped to get a bit of sleep.

HA! By midnight it was apparent that Verity was not interested in sleep whatsoever. She seemed to be in considerable pain, at least according to her screams. (There is nothing wrong with her lungs, that's for sure...) We ended up giving her some more pain medication, but she continued to be unhappy. The nurses were tag teaming with me, trying to help soothe her, but nothing was helping. They wondered if her tummy was not doing well and stopped the feeding, restarting the IV instead.

The night was a blur...there would be times when she would seem to settle, just enough for me to crawl onto the sloped couch, spread a blanket over myself, close my eyes, and...jerk back to reality when she screamed again. I think they did restart her feed at one point only to stop it again. They found an infant swing to bring in when I mentioned that she usually sleeps in our swing at night. That helped for a bit as well. But nothing helped longer than a few minutes at a time.

I was sure that Verity just needed a good meal! Of course I don't doubt she was experiencing pain as well, but by 5am, when she was just plain mad, I begged the nurses to PLEASE resume her feed. Imagine if you hadn't eaten for 13 hours, and then when you were finally allowed to eat, you were given one bite of bread every 10 minutes. Would that help you?! I think not! That must be similar to how Verity was feeling! Girlfriend likes her food!!

After going up the chain of command, we got the approval to resume the feeding and stop the IV. (You may hear my eyeballs rolling here...why would a mama not be allowed to feed her baby?! Yes, I understand the surgery and all...but really!) So feeding resumed...and she fell asleep. Hooray! At 6:10am the residents barged in to wake me up and ask me questions. Having just gotten about 20 minutes of sleep (double the amount I had gotten the rest of the night), I was rather disgruntled, and even more so when I realized they had awakened Verity, too. UGH. I held her in the rocking chair and we both dozed off and on until they came an hour later to stop the continuous feed.

But! Proving my point, at 8:00 when we started her full feeding (60ml over a half hour), oh! Was she ever so much more content! She did continue to make little shuddering sighs/moans now and then, similar to the nights after she got new casts on her feet/legs. But it was SO much better. Her little voice was hoarse from all the screaming during the night! By 9am she was resting well enough that I felt comfortable telling the nurse that I was going to go grab breakfast.

In the cafeteria I was able to meet up with another T18 mama! Paige and I had connected via Facebook soon after our diagnosis, and I've followed Addilyn's journey online as well. It was nice to meet in person and chat awhile!

Back in our room, I prepped for Verity's 11:00 feeding, during which I received the G-tube training needed to bring our girl back home and care for her ourselves. I posted this video on Verity's Facebook page with permission from the mama who made it, so if you're curious about what exactly a G-tube is, what it looks like, and how it works, take 6 minutes and check it out! I watched it before the consultation with the surgeon and then showed it to Ted the day of Verity's surgery. It's interesting and was super helpful for me in mentally getting ready for this change.

This is how I found her after I got back from breakfast;
I don't think she missed me a bit!

Ted arrived after his morning work meeting, so together we went over the instructions for using the G-tube for both feedings and medications, cleaning it, venting it (when her tummy needs to release some gas), and general site care. I was just so eager to leave the hospital and get home to NAP!

Now we are at the end of the week. I am happy to say the G-tube has not been yanked out, lol, and Verity's pain seems greatly subsided. I'm still cautious with holding her and don't even like to put her up on my shoulder to burp in case it's still tender. We give her an extra layer of cloth over the belly area when buckling her in her car seat as well. At the 2-month check-up yesterday, our pediatrician said the site looks good. There is some redness but not bad, and some inflammation is to be expected.

We continue to work on oral feeding as time and interest allow, but in the meantime, what a relief to finally be rid of the nasal-gastric tube and see Verity's sweet little face!

GI Scope & G-Tube Surgery

On Monday Verity and I returned to Children's Hospital for a GI evaluation. She had to stop eating 4 hours beforehand, but thankfully she was pretty sleepy in the early morning. The scan itself didn't take very long, but it was strange and a bit sad to see her strapped on her back to a board with large velcro bands covering her tiny body! Her arms were raised so her little clenched fists were above her head. Once the doctor was ready, the tech rotated her on her side and fed her barium from a bottle. I was glad to see that she did suck and swallow from the bottle! After a bit of this, they did switch to a syringe. Similarly to the swallow study, we watched as the barium made its way down her esophagus and into her belly, but then it went further: her stomach seemed to swell up like a balloon as the barium filled it and made it light up on the screen.

Verity was such a little trooper. She had some reflux, and since she had been dealing with congestion for a few days anyway, it was a bit of a mess that I got to clean up and comfort her before we continued the test. After her tummy was all the way full, we waited and watched...and we did see some reflux that didn't come out of her nose/mouth. Granted, she was flat on her back, a position we NEVER have her in when we normally feed her! But still. It makes me wonder how often she deals with reflux like that.

The results of the test were then sent to the pediatric surgeon, with whom we consulted the next day. He agreed that the risks of a G-tube surgery would far be outweighed by the benefits to Verity, as she is doing so well overall. I was so pleased that he had obviously read some more current research about Trisomy 18, and we were on the same page as far as proactively improving her quality of life. I really liked this doctor--once again, we are so thankful for God's guiding hand in allowing us to meet with caring, compassionate doctors who are working on Verity's behalf rather than fighting us as so many in the medical community do when it comes to children with "dire" diagnoses.

The surgery could have been done the very next day had it not been for the fact that Charis and I left yesterday to travel to her regional speech tournament! So the surgery date is set for Monday. We would appreciate prayers for the doctor and medical staff as well as for Verity to do well under anesthesia and to recover quickly. The G-tube will allow us to pump Verity's food directly into her tummy, freeing us from the horrors of the NG tube she has had since birth!! I am grateful for the feeding tube for keeping our baby girl alive, but I will NOT miss the paranoia we have lived with being worried that it might come out, even more so now that she is alert more often and flailing her arms and hands so much.

We will continue to offer food orally--from the syringe and/or breast prior to starting the feeding pump. We will use the same feeding pump with her G-tube that we use now with her NG feeding tube. I will try to post a video showing you how we currently do feedings for those who haven't seen anything like this before. It was completely new to me when we began caring for Verity outside my womb...almost two months ago now! TOMORROW IS HER TWO-MONTH BIRTHDAY!!!!!

Heart and Heels

Tuesday was a very long day but overall a success! In the morning Verity had another echocardiogram, which the cardiologists evaluated immediately and then met with us afterward. Some of the problems that were present at birth have completely disappeared (thank you, Jesus!). Two tiny ("pin-prick") holes remain, but they are insignificant and are not causing any problems at this time. Moving to a higher altitude will not present a problem; in fact, it may be even better for Verity! We are all looking forward to relocating to Colorado, even though it is always a chaotic time, getting ready for a PCS (permanent change of station). The doctors recommended that after we get settled, we get another echocardiogram so the cardiologists there will have an up-to-date baseline from which to monitor Verity.

So Tuesday morning was easy! We even finished early and had time to grab lunch in the hospital cafeteria (surprisingly good) before checking in for surgery--a tenotomy, or releasing of the Achilles' tendons, the final step in the Ponseti casting method for clubbed feet. (Well, almost final, I guess, if you count the fact that she will need special shoes and a bar afterward.) Here are some photos of the progression...I didn't think to take photos each week in the same position, but you can get the idea...

Prior to any orthopedic work--this was at a regular check-up.

First casts

This is after the 2nd set of casts came off, so
two weeks in casts total.

After 3 weeks/3 sets of casts, side view.

After 3 weeks/3 sets of casts.

The last two photos were taken the day of the surgery. (Humorous bit: We were in the operating waiting area when we were told that they weren't sure what to do about removing the casts...they didn't think that all the way through, apparently, so we carried Verity to the specialty clinics to get her casts removed, then brought her back. It was fun to hold her without any casts, but she seemed a bit consternated (is that a word?!) at the feel of someone touching her legs and feet!

The surgery was very quick; in fact, it took longer to put her casts on than the actual procedure dealing with the tendons. She got a local anesthetic; actually, they put numbing cream on the area after the casts came off, and then she got the local after they whisked her away for the operation. She was away from us for less than an hour and came back in a sleepy little bundle.

Right before surgery. Isn't she a doll in that gown?!

After surgery...final set of casts. They look huge!

We expected a horrific night after the anesthetic wore off, but it wasn't as bad as it could have been. And from what we can tell, Verity hasn't been in pain or discomfort the last couple of days. In four weeks we go back to get these final casts taken off. Then she will get the "boots and bar," which I have heard is pretty difficult...not much sleep, etc. So let's start praying in advance that she will not be phased by the transition!