Update: Spring Appointments

Recent posts have detailed the couple of respiratory illnesses that Verity had in March and April, so now it's time for a general update on the slew of other appointments we've had over the past few months! October was our busy month in the fall as the referrals finally went through and we started seeing her specialty providers, and April of course was the 6-month mark for follow-up. So here is the list of what all we've been up to!

March 22: Pulmonology
The pulmonology follow-up was scheduled even before Verity's first illness struck, but it was timed well as she had been out of the hospital about 10 days or so. At that time her lungs sounded amazingly good, and since we had never had any lung issues before, the doctor said she would leave it up to the Sleep Clinic doctor as to whether or not she would continue to follow Verity. Of course, little did we know that Verity would have pneumonia about a month later! Sleep clinic is scheduled for May 25...

April 2: Ophthalmology
We drove 45 minutes to get to Ft. Carson to spend a whopping 5 minutes with Dr. B, who assured us Verity's eyes are looking great and he doesn't need to dilate them again until our fall appointment. Okie dokie then.

April 17: Orthopedics
At this follow-up appointment we got another hip X-ray, which indicated her left hip is still stiff but not any worse than in the fall. Her feet are looking good and we are to continue using the bar (supposedly naps and nighttime but in reality more like 2-3 hour chunks a couple of times a day).

April 19: Pediatric Visit
We needed a doctor to look at Verity's umbilical hernia, which was repaired with her G-tube surgery May 2017 but has been flaring up again recently. She ordered an abdominal ultrasound. (This is the day we realized Verity was getting sick, and at 3am the next morning we took her to the hospital, where she was diagnosed with aspiration pneumonia.)

April 24: Renal ultrasound and barium enema
The ultrasound looked normal (per the follow-up the next day), and the barium enema did not reveal any particular reason for Verity's difficulty pooping, although it did show a "tortuous" pathway. (Once she gets started going she does pretty well, but we frequently have to give her a liquid suppository to start the process.)

April 25: BAHA fitting
Verity got her Bone-Assisted Hearing Aid, which fits on a headband! We learned lots of details about how to use this device and have a whole bag of STUFF to go along with it. She wears it in stints, as it is very overwhelming for her to have it on all the time at this point. Slowly we are working up to it! She isn't terribly responsive, although we know for sure when she is DONE!

April 30: Adaptive Stroller Shopping
We went to an equipment provider that we know of through The Resource Exchange (our home therapy program) to look at and take measurements for an adaptive stroller for Verity.

May 3: Echocardiogram
Our cardiologist is very optimistic about the way Verity's heart looks right now. Her large VSD has completely patched up! She still has a teeny tiny one that causes her heart murmur, but everything is balanced, and he does not think PH will be a problem. Whew! He feels comfortable waiting another year before we do another echo.

May 4: Post-hospital follow-up with PCM
Verity had been off oxygen for some time before we had this appointment, and she looked quite well. Her PCM was pleased.

May 7: Abdominal ultrasound
This was a pretty quick appointment, but we didn't hear anything about the results until I asked a week later. We have a referral to see a pediatric surgeon since clearly the hernia is there.

May 15: Dietitian assessment
Our Verity weighed in at 14 pounds, 14 ounces!! And she is 26 inches long now!! In less than 2 weeks, she will be 15 months old...15 pounds by 15 months!!! Clearly she is growing and thriving on the Nourish formula. Now that she is 100% on this whole-foods mixture (plus plenty of water), our next step is to meet with the GI doc and formulate a plan for compressing her feeds so that she can get on more of a bolus feeding schedule. Her vomiting can still be a problem, particularly when she is bearing down to poo or pass gas, but it is much less frequently to be sure. I'm not going to lie...I would LOVE for her to not be hooked to the feeding pump 24/7. But...counting my blessings. Our baby is growing and thriving and becoming SO much stronger and sturdier!

In between all the above listed appointments were nearly weekly visits to the chiropractor as well as nearly weekly occupational and physical therapy sessions in our home.

COMING UP:
May 25: GI follow-up and Sleep Clinic

May 31: Surgery consult

June 6: Speech evaluation

June 7: BAHA check

Whew. That's all for now, folks!!!

Cardio Report

I posted a video report on Verity's Facebook page the day we visited the cardiologist, so I will just give a very quick rundown here.

The last time we got an echocardiogram was in Omaha in late April. At that time, the docs said that Verity's VSDs were not really a concern any longer and recommended that we get a baseline echo done once we got settled into our new location in Colorado. So, 6 months later, we were able to do just that, getting not only an echocardiogram but also an EKG (pictured below).

We are pleased to report that Verity's heart looks great! Tissue is growing over the large VSD (the one initially big enough to require surgery in her first year), and the medium-sized one is not causing problems, allowing blood flow appropriately. The pressures are balanced, and the cardiologist said that the way Verity's heart is, we EXPECT a heart murmur, and it is nothing to be concerned about (as I was following the ENT's report of hearing a murmur, the first time anyone has said anything about it).

For a more detailed report, showing the diagram the cardiologist gave me, check out the video on her Facebook page. For now, I'll leave it at this: it was a positive report, Verity did great during the exams, and we don't have to go back for another 6 months!

All Kinds of Check-ups

Here are the nitty-gritty after-visit summaries of Verity's recent appointments and a quick word about an upcoming visit.

GI, September 29, 2017
Stats:
BMI: 15.33 (14th percentile)
Weight: 11lbs, 13.5oz (< 1st percentile)
Height: 23.31" (< 1st percentile)

1. Swallow study ordered. [Scheduled for November 30--in the meantime, we need to work with Verity to help her swallow more than she currently is!! The study requires at least an ounce, and she only takes about 5ml on a good day.]
2. Zarbees multi-vitamin recommended [now using daily]
3. Trial of a powdered probiotic recommended [now using daily]
4. Follow-up in 2-3 months to check on weight, feeds, etc.
5. Call or message with weights every 2-3 weeks.
6. Work toward 105-110ml per feeding.

Since this appointment, Verity's reflux problems have resurfaced. Every time we try to bump up her feeding amounts, it seems we see an increase in vomiting and discomfort. We had gotten her up to 95ml, but we went back to 90 the last couple of days and she is still acting uncomfortable during her feedings and shortly after. We've slowed the rate down as well. I spoke with a nurse from the GI office and we are planning to do a pH scope to determine the level of reflux and use the information to help us decide the next course of action. Seemingly, there are two options: medication and a Nissen fundoplication. We do not yet have a date for this test.

ENT, October 16, 2017
Stats:
BMI: 15.80 (23rd percentile)
Weight: 12lbs, 3.9oz (< 1st percentile)
Height: 23.35" (< 1st percentile)

[This doctor was very thorough with his notes! I'm going to copy most of them here and simply link to definitions of the more unfamiliar terms and place explanations in brackets.]

"Verity is a 7-month-old female with a hx of Trisomy 18, hypotonia, aspiration with G-tube placement and bilateral hearing loss. Most feeds are happening through the G-tube. Family had a sleep ABR [Auditory Brainstem Response] back in May of this year that showed severe CHL [Conductive Hearing Loss] in the right ear. MOC feels that the hearing has improved over the last couple of months, no wearing hearing aids at this time. Family has not been evaluated by an ENT prior to today. There are concerns about sleep apnea based on her sleep patterns, retrognathia, hypotonia and her diagnosis of Trisomy 18.

Examination today showed poor tone, not able to hold her head up. Bilateral external auditory canal stenosis, unable to view the ear drums [her ear canals are abnormally narrow]. Some soft tissue narrowing of the nose at the vestibule, improved nasal airway on the mucosal side. Flexible laryngoscopy was performed and showed some mild nasal narrowing in the midportion of the nose, no adenoid obstruction, intermittent collapse of the pharynx when she was bearing down. The vocal cords were mobile with a good view of the larynx, no prolapse of the tongue.

I would like to repeat the ABR during sedation and obtain bone conduction for both sides. I would recommend hearing aids of some form sooner than later. We will order a sleep study to rule out sleep apnea. I would like to see the family back after the sleep study and the hearing test."

Currently the sleep study is scheduled for January 8, but we are on the waiting list in case something opens up sooner. If so, it would be a same-day deal, so we'd need to drop everything and make haste to the sleep lab!! I hope we can get some answers sooner rather than later...these awful nights are killing us!

Orthopedic, October 17, 2017

I don't have a lot of written notes on my paperwork, so I'll try to remember the main points...

1. Verity has responded very well to the boots and bar--we are cleared to use it for nights only and not concern ourselves with nap time since her sleeping habits leave much to be desired.

2. They did change the angle her boots were fixed to the bar so that her feet are not turned outward nearly as much. We hope this is making it more comfortable for nighttime sleeping! 

3. New boots will be fitted on Monday, as her toes are nearly hanging out of her old pair!

4. They took an X-ray of her hips, as there was concern about less movement in her left hip. Things look all right for now, but there is danger of her hip coming out of socket. We are to do certain stretches with her diaper changes to help with this issue.

5. They want a follow-up with the PT in 3 months and with the doctor in 6 months.

Coming up: 

Cardiology (Echocardiogram), October 26, 2017

This appointment is to give us a baseline echo of Verity's heart; when we last examined it in April, everything looked wonderful. The VSDs were all closing, everything was balanced, no heart murmur. However...at the ENT visit on Monday, the doctor heard a murmur, the first time this has happened. He said that if HE could hear it, then it definitely needs checked, since that isn't his specialty. :-/ So...I wasn't concerned about the echo appointment before, but now...I am...a bit...

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!

Meeting with Cardiologists

Ted and I met with several cardiologists this morning. We went over the anatomy of the heart and discussed the various issues seen on the echocardiogram from Verity’s first day of life. 

They explained several conditions involving various connections between the arteries and veins immediately outside the heart (one of which is a PDA, patent ductus artenosus, say that five times fast!), all or most of which should resolve.  If by chance they don’t resolve on their own, these minor defects, while not life-threatening, could certainly be fixed if we choose to intervene with surgery on the more concerning issue (in other words, if they are doing surgery anyway, why not go ahead and fix the minor issues as well?). 

The biggest problem with Verity’s heart are the three VSDs: ventricle septal defects. Two are small and are toward the bottom (“meaty”) part of the heart and are not a major cause for concern; VSDs of this nature often resolve on their own, and if they don’t, the size and location are not prohibitive.  The most concerning VSD is considered “large” and will begin to affect the relative pressures between the right and left sides, which are important to the overall flow of blood to the body and lungs. If the pressure is higher in the body, oxygenated blood spills over and gets sent to the lungs, and less saturated blood is all that’s available to the rest of the body. Right now, the blood vessels in her lungs are constricted (as is normal for a newborn recently out of the womb), which keeps the pressure on that side higher, which allows the damaged heart to more closely mimic a normal/healthy heart.  This is a good thing—it means correcting the problem can wait until she’s bigger and can better withstand surgery. We also talked about the fact that the valves to Verity’s heart are thicker than normal, which is not causing any problems now, but bears monitoring as there is a possibility that the thickened valves could become stiffer over time, which would make her heart work harder to get blood in and out.

Three options were presented to us:

1. We could do nothing, possibly monitoring her but allowing her body to function as best as it can on its own, knowing that some of the problems might heal on their own over time (although they might not). As Verity’s heart grows, the smaller holes are certainly more likely to close up on their own than the larger VSD; however, we could choose to avoid the risks associated with surgery altogether in favor of minimally intervening and creating as high quality life as possible without undergoing major surgery.

2. A less invasive treatment option, meaning an option that would not require them to stop her heart and go inside, would involve putting a band around one of the pulmonary blood vessels immediately outside the heart.  This has the effect of artificially raising the blood pressure in the lungs, which keeps her functionality where it is right now with her lungs still operating at higher pressure.  The constriction band would not grow with her, however, and it would need to be replaced in a matter of months.

3. Finally, full surgical repair, which involves stopping her heart and putting her on a heart/lung machine for the duration of the procedure.  This option would allow the surgeons to go in and fully repair the defect(s) in the inner wall of her heart.  The surgery is more risky and more intrusive but offers the possibility of being “one and done” if the repair is successful. 

Before we go further, I will note that, based on the information we received today, we feel the right thing to do is whatever we would choose for a child without Trisomy 18, and that is the full surgical repair. Why bother with option two, which really is only a temporary fix? Verity shows good health overall—we feel she deserves the opportunity to buy more than just a couple months’ worth of time. And we would rather give her the chance to have an extended life span rather than deny her the opportunity simply because of her T18 diagnosis; the risks of surgery, therefore, are rather to be taken than making a decision (i.e. doing nothing) that will almost certainly shorten her life. God will direct the outcome of the surgery, and we trust His sovereign plan.

With that said, it was of interest that Verity’s case will be discussed Monday at the weekly gathering of the full team of cardiologists from this area. This is a time when all the experts evaluate a given situation, discuss all kinds of angles and viewpoints, and collectively come up with a “recommendation.” We of course are not obligated to follow that recommendation; however, with so many minds mulling over all the facts, there may well be other considerations that we have not heard about with only a few people looking at the data. To give the fullest picture, a follow-up echocardiogram was performed this afternoon (which I got to watch—fascinating technology!). This will clearly show whether those minor flaws seen at birth have begun to resolve yet or not and will give a full and current picture of her heart for the doctors.

Meanwhile, we have a list of things to watch for as Verity grows, and the goal is to get her as big and healthy as possible before doing the surgery. If her growth slows, for example, we will need to get surgery scheduled sooner rather than later. The biggest question for us is whether we should do it before we make our big move to Colorado this summer (thank you, Air Force, for sending us closer to family!) or whether we will wait and do it after we get settled there. The change in elevation may be a consideration, but we will be prayerfully waiting for the Lord’s direction and watching Verity herself for indicators as to what will be best for her.