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!!!

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...

Best. Update. Yet.

Day 14
Today turned out to be a much bigger day than any of us could have anticipated. As I waited for Ted to arrive, I got word from our nurse that the cardiologists had ordered another echocardiogram. We weren't sure whether this was a good thing or a bad thing, so, being able to do absolutely nothing other than wait, I continued with my plans to make the trek to the Other Side of the Hospital Universe to shower. (Interestingly, the Infectious Disease Hallway smelled strongly of freshly baked chocolate chip cookies today...)

After Ted arrived and we shared a lunch from Schlotzky's, we got a phone call from Dr. M, the cardiologist who led the discussion with us last Thursday about Verity's heart. She explained why the team was requesting a new echo; the reasons were twofold:

1. There was some unexplained activity around the pulmonary vein--this is completely different from the issues we discussed last week, and they wanted to check it out more closely.

2. The large VSD, the one that Dr. A had initially told us would require surgery before Verity's first birthday...the large hole that in all likelihood would not close on its own, the way the smaller two probably would (and already have started)...yes, THAT hole...seemed to be starting to close "on its own." They wanted to see another echocardiogram to be sure.

Well! It's always good to get news that is much BETTER than one expects! Dr. M said she would call after the results came back, so Ted and I made plans for a milkshake date for the two of us and a much-needed massage for me.

While we were getting ready to go, Verity had a little episode: she pooped so hard that she had some reflux and aspirated stuff out of her nose. Her oxygen sat went down into the 70s, which meant the alarms started going off, but then the numbers climbed higher slowly but surely. Meanwhile, we were suctioning out her nose and repositioning her to try to help clear her airway. A nurse stepped in to help and was so calm about everything, I didn't think there was much of anything to worry about...but then I watched as the nurse quietly repositioned Verity, listened to her chest, and continued to hover over her and work with her, and then I noticed her little chest caving in with each breath. It was alarming: the numbers on the monitor were perfectly normal by this time, but she was clearly having difficulty breathing. The nurse suctioned out more junk, continued to work with Verity, calmly listened, wash, rinse, repeat. After a tense period, she was breathing more easily and the nurse seemed satisfied with what she was hearing (or not hearing), and the scary part was over. But it was definitely a wake-up call...I was reminded of the warnings from our Trisomy families that our littles can "silently" aspirate. Despite how well Verity has been doing thus far, we can't take anything for granted and must remain alert, especially if/when she gets sick.

I had a hard time leaving Verity after this, but we stayed around long enough to verify that she was doing much, much better and had two nurses saying they would specifically be watching her so that I could indeed go get my massage. I'm sure it won't be the last time I experience Mom guilt for leaving my baby. :-( (As an aside, I scheduled the massage because I haven't been able to turn my head to the right for the last few days; my muscles are that tight and knotted. I'm still sore and having difficulty turning that direction, but my shoulders and back are much less tense.)

Ted headed home after walking me to the massage clinic (about as far away as the showers, but in the opposite direction). As I was headed back to the NICU after my appointment, I got a call from Dr. M with the results of the day's echo. Sure enough, that large VSD is starting to close on its own! Whatever is going on with the pulmonary vein is not anything worrisome. The team wants to continue to monitor things, but as far as they are concerned, we can be released from the NICU whenever the doctor here is comfortable sending us home, and best of all...

THEY DON'T THINK SHE WILL NEED SURGERY!!!!

Verity does a victory dance of joy!

I texted the news to my sweet friend and prayer partner, who was here on Friday and prayed specifically that the holes in Verity's heart would close up and be healed. She wrote back right away to tell me this:

"Yesterday in children's church Zaden asked for prayer for Verity to be here on earth for a long time. We prayed that her heart would heal. Prayers of children I think avail much."

Indeed! Why do I marvel at all...I had wondered why God didn't show us via ultrasound the issues with Verity's heart so that we would know about that before she was born. Now I think what a blessing it is that we didn't know; surely it would have only added to the mental and emotional burdens we already carried throughout my pregnancy.

I praise and thank God for these answered prayers; at the same time, I am determined to stay yielded to His sovereign plans and purposes. I maintain that God is good no matter what our circumstances; I have wept with parents whose little ones were NOT healed, or who were NOT born alive. I don't understand why God has allowed our little Verity to live and (so far) thrive with such a positive potential outlook when so many other sweet children have had parents fighting for and with them and yet had to say goodbye all too soon.

I rejoice...yet I continue to ask God to let us not take anything for granted but rather help us to cherish what we are given.

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.