Muddling

We're muddling through these last weeks until the cath. It's been harder than I expected, both emotionally and practically, but there's only eight days left.

Job is so very excited to that he's walking. If he wasn't so proud of himself, I probably couldn't let him walk so much - but how do I stop him? Walking IS a good thing... It's just hard to watch him turn so blue.

Likewise, he wants to be outside all the time. His big brothers are outside, so that must be what big boys do! He asks so nicely, so often, but when we do go outside he gets so cold so quickly... And turns blue.



How do you find the balance appreciating and even encouraging such a huge developmental skill (that we have worked SO hard to achieve) that is also hurting him? How do you mostly deny requests to be outside in the fresh air, playing with balls and looking at new spring plants?

I wish he'd be interested in vegging out and binge watching some TV instead. 🙄

It's also been a rough month because we've had a good 4+ appointments a week. I'd forgotten how consuming such a schedule is - however did we make it through the first year of Job's life?!

So that's a weird place to be too: so grateful for so many people working so hard to help Job... But also really wanting to get off the merry-go-round and just stay home and let my toddler walk around outside.

I'd really appreciate prayers for patience and trust and contentment because all of those things are really hard to come by this week.

Interventions Ahead

Job is now walking!

But his heart can't currently sustain this increased activity so he needs intervention this month.



Job was fitted for orthodics in mid February and, amazingly, began taking independent steps the next day.

He's gained confidence really quickly and, over the last two weeks, has completely quit bottom scooting. He just walks everywhere!

But over these last two weeks we've watched him turn bluer and bluer with each subsequent step.

So at our regular cardiology appointment last week we did a stress test, hooking Job up to a pulse oximeter while he walked the hallways (with his brothers' encouragement): video

Job doesn't get enough oxygen-saturated blood and never will. Whereas you and I "sat" at ~98% (that is, 98% of our blood is saturated with oxygen) and with saturations of 90% would be admitted into hospital care, Job will at best sat between 75-85%. (Which has and will continue to cause organ injury and function impairment - we just want to limit this as much as possible.)

When he got off of supplemental oxyegn in August he was satting in the low 80s but we've watched his numbers slowly drop over the last 6 months to the mid 70s. This alone told us he would need some intervention sooner than later but we weren't really expecting to need to do something so soon (I guess I had next fall in mind).

Walking (a sharp increase in energy expenditure) has now forced this issue. At rest in the office, Job was satting at 78. He walked about twenty feet down the hallway and dropped to 50. We had him stop and rest and he climbed back up to the mid 70s. Then we had him walk and he dropped to 48.

It is so exciting that Job is walking! And it's scary. He very visibly turns bluish purple, first in his finger tips and lips, then his cheeks. Then he starts panting for breath. But he's so excited that he can move around like a big boy that he just keeps going and going. He does eventually get really tired and finally sits down but right now I'd rather he just never walk.

Cardiology didn't give any restrictions - we don't want to halt such huge developmental progress! It would be great for him to be on supplemental oxygen but it's virtually impossible to put a toddler on oxygen without using hand restraints and we're not going to do that.

But we need to intervene.

So we have a cardiac catheterization scheduled for April 27th. Caths are the best diagnostic tool to assess the state of the heart. How is the function? How is his tricuspid valve regurgitation? How are the pressures in his Glenn? And why is his blood this oxygen depleted?

We expect them to balloon some vessels or place a stent or two and therefore we plan to stay at SCH for at least one night. But we're now also tentatively planning on a surgery this spring. This will absolutely depend on the results of the cath, but Job's little half heart needs assistance and likely more than a cath can provide right now.

We had a long year off from intervention and I think we were lulled into a bit of a false sense of normalacy. No hospitalizations and only one ED visit!? So this news, though not unexpected, was and still is jarring.

It's also been a hard month as many of our heart friends have been in and out of their respective hospitals for various complications and surgeries and we've also had two deaths at our church. It's been impossible to blissfully ignore cardiac complications and death, like I at least pretended to do for the year previous. The timing of Good Friday/Easter and Job's birthday in all of this is, of course, not coincidental and has also been a large part of our emotional reflections.

We know that God is moving in Job's life for our family's good and for His own glory and we are resting in that, even in our sadness. It's important to grieve and it's important to preach truth to ourselves.

Practically speaking, we need to keep Job cold free over the next few weeks or his procedure gets bumped and we need to find that balance of letting him walk around and gain these necessary skills... but also swoop in and pick him up when he's blue in the face but not willing to take a break yet. We're on high alert right now, hoping his desats hold steady until the cath and monitoring his O2 closely.

March 2018 Cardiology Visit Recap

After Job got off oxygen in August, we had a Septemeber cardiology appointment and switched from monthly appointments to an every three month cardiology schedule!

I missed the December appointment because of a prior commitment, so Seth took Job, so I hadn't been to SCH since SEPTEMEBER 2017. That's right: SIX MONTHS.

I was marveling about this fact as I drove up to the parking garage and the boys and I were talking about how much we had missed SCH during that time. We used to have weekly cardiology appointments and then were shocked on only have them every other week. And then for about nine months we had an appointment every month.

Of course we appreciated fewer appointments, because that meant Job was gaining strength and needed less assessment. But in a weird way, we really do miss SCH. This precious hospital has made really scary medical "stuff" easier to bear because it is such a friendly, welcoming place - sometimes even fun place (at least for the older siblings who aren't being poked and prodded but just get to enjoy the therapy pool and playground and TV).

Well, we're about to be at SCH more often! This news didn't exactly come as a shock, though it is certainly disappointing to make it official.

Job's battered body cannot currently oxygenate his blood sufficiently for his increased physical activity.

He gained weight wonderfully and is happy and healthy for the most part. Except, you know, that heart thing.

It's wonderful that Job is now walking. It's really really wonderful. Buuutttt when he walks around, he desats.

These past two weeks that he's really been moving around, gaining confidence and skill and then speed and endurance, I've been watching him slowly change color and get short of breath. I've checked his sats, but only ever while he's at rest, as wrangling the pulse oximeter and cord and a toddler just proved too difficult to get an accurate assessment here at home.

So when we got to the single ventricle clinic this afternoon I asked that we please do a stress test of sorts and monitor his O2 while he walked around.

Sure enough, his resting oxygen saturations were ~77 but dropped to the low 50s while he was walking down the hallway. Then we'd have him stop and rest and they would climb back to the mid 70s. Then we'd have him walk again (with the encouragement of his big brothers, who love to help!) and they would drop down again.

This, plus the facts that his resting sats have been slowly trending downward and his fistula is no longer audible, lead to the very strong conclusion that we need to do something to help him oxygenate. And need to do it soon.

So cath lab, here we come! We expect a phone call Monday or Tuesday to get it scheduled asap.

Day 5: Cardiologist (Heart Month)

Perhaps I boxed myself into a corner waxing rhapsodic about our hospital and didn't leave myself any room to talk about our cardiologist: Dr. Matthew Files.

Dr. Files was the cardiologist on service the week we were trying to discharge from the hospital June 2016. While we had never met him before and while all of the cardiologists on service at the hospital had been great, he was immediately so accessible and kind to us. At the time, of course, I didn't know how important he would become to our lives, but he cemented himself in my list of favorite people when he let us discharge that week instead of making us stay as he probably should have, since Job wasn't keeping his feeds down.

He saw us a few days later for our first outpatient follow up and was so clearly invested in Job's care that we requested him as our primary cardiologist for the interstage period (between open heart surgeries one and two). After the second surgery HLHS kids are supposed to start thriving, but when Job didn't and it became obvious Job was too critical to transfer his care down to Tacoma's SCH cardiology branch, Dr. File became our permanent cardiologist. Now, of course, I'm expecting that he will live out his professional career exclusively at SCH so that we can continue to see him every few months (! I still thrill at how far apart our appointments are these days!!) for Job's cardiology care.

Dr. Files is incredibly knowledgeable, of course, but he is, most of all, compassionate and personable (perhaps in part because he himself has a CHD?). He has spent so many hours talking to us, writing us emails, calling us and even standing by Job's crib crying with me after some really scary days. His dedicated research to explore all possible options for Job and willingness to order weird scans and try new medications and experimental procedures got Job off of oxygen.

I love his willingness to tell me hard things, without sugar coating, and his willingness to explain even minute details of Job's care that I don't really understand or even need to know because they are so technical but still desperately want to try to understand.

It is also absolutely essential to make note of the RN in charge of the Single Ventricle Program at SCH: Kendra Waldburger (also picured). We first met Kendra when we toured SCH a few weeks before Job was born. She spent so much time giving us such a detailed tour of the hospital and explaining so much about the SV Program and CICU which made our transfer to SCH so much less scary. And she has been such a beacon of sunshine and joy, lighting up whenever she sees Job or responds to my emails. I will never forget the sound of her sweet voice, calling us almost daily while we were interstage, to check in on Job, or how she would pop into our room to chat with me while we were inpatient and help me scheme to advocate for a cath for Job and other such things. If I need anything I still call Kendra because she always has answer for me or can connect me to the people who can. The SV Program is absolutely phenomenal because of Kendra and I'm so grateful Job is a part of it.

Photography by Scott Eklund/Red Box Pictures for this article: https://news.microsoft.com/…/childrens-mercy-app-brings-do…/

Day 4: Hospital (Heart Month)

How do I adequately express my deep appreciation for Seattle Children's Hospital? Every aspect of this place has been a wonderful blessing to our family! I can honestly list our few kerfaffles on one hand. From silly things like clear (but also fun) signage to really important things like personal phone calls from our various specialists, it is so clear that much thought and effort and money has been put into their care for families (not just patients!).

I'm on a number of heart groups/forums on Facebook/online and I can't even tell you how many times different parents have posted questions and concerns that frankly I have never had to ask or worry about because SCH is just so on top of Job's care.



I remember, while pregnant, researching hospitals that would take HLHS kids. I found the parameters I was supposed to be looking for: number of Norwoods performed each year and mortality rate for this surgery and so on. While Boston Children's and Children's Hospital of Philadelphia really pioneered CHD care, a few other hospitals have made pediatric cardiac care their speciality as well. I was so amazed to realize that, of all the places in the world, we lived a mere 43 miles away from one such hospital. How wonderfully providential! So many families have to travel (and then live) out of state for months while receiving care for their heart kiddos. With Seattle traffic it often takes two hours to get there, but I'm so happy to listen to audio books/sermons and make that comparatively short drive!

Job was born at Tacoma General and then spent his first 36 hours in the Mary Bridge NICU before transferring to SCH and also did one inpatient stay in the PICU there last year. I'm grateful for the therapy services we receive through MultiCare as well! But when we had to decide who would direct Job's cardiac care it was easy to pick SCH. I'm so grateful to have the continuity of service and access to so many specialities by staying in their system.

In many places around the country, patients with CHDs who are now adults must be seen at children's hospitals (because there are so few adult CHD programs in existence because adults with CHDs are a relatively new phenomenon!). UW, however, has a great adult program that Job will someday transfer to. I'm grateful that we will have this opportunity! but the day we transfer from SCH will be a very sad day indeed.

I  SCH.

#heartmonth #day4 #hospital

Day 3: Meds (Heart Month)

Job is now down to a mere 8 meds given and 5 med times (though we're about to add another). I hardly think about it as I draw them up and several times a week Isaac gives them... A sharp contrast to when we first came home from the hospital and it took BOTH Seth and I a good hour to draw them all up and give them (and I think we came home on 16 meds and had 7 med times).

He'll be on most of his cardiac meds his whole life to keep his fluid balance level and to help his heart pump and to help prevent clotting. We're hopeful he'll get off his digestive meds some day, but his digestive system currently doesn't get enough oxygen to function properly without these meds.



It takes quite a bit of work to coordinate between his doctors and pharmacies to make sure he gets his refills on time and prior authorizations and dose changes, but it's doable now that I know the system better and have enough of the vocab down and a detailed calendar.

Job has been taking all his meds by mouth (besides his old anti coagulation med that we used to inject 2x/day) since he moved down, out of the CICU, at 10 weeks. Perhaps because he doesn't know any different, he's always taken them so well! I'm incredibly grateful for that blessing and I hope he'll always do so!

#heartmonth #day3 #meds

February 2018 Heart Month!

I've never participated in an "awareness campaign" before, but these past two years of delving into the special needs community on Facebook especially, have brought these kinds of posts to my attention. I don't know if they're at all helpful to anyone, but I thought it might be a way of jump starting me into writing about CHDs and about Job because I've struggled to do so lately. So we'll see how this goes!

Here's the complete list and I'll link back to posts once I've written them!
Day 1: Diagnosis
Day 2: HLHS
Day 3: Meds
Day 4: Hospital
Day 5: Cardiologist
Day 6: Surgeon
Day 7: Staff
Day 8: Nurses
Day 9: Technology
Day 10: Hospital Stays
Day 11: CHD Family
Day 12: Heart Mom/Dad
Day 13: Siblings
Day 14: Heart Warrior
Day 15: Scars
Day 16: Norwood
Day 17: Glenn
Day 18: Fontan
Day 19: Other Surgeries
Day 20: Cardiac Caths
Day 21: Future
Day 22: CHD History
Day 23: CHDs
Day 24: Developmental Delays
Day 25: Therapy
Day 26: Support
Day 27: Financial Impact/Insurance
Day 28: Hope