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

Job Stood Up!

Wow wow wow! Job stood up completely independently!

He's 22 months old, about to turn 23 months, and I really didn't think we'd hit this milestone before two years old. Ahhhhhhh!!!!

December Recap

December was a rough month for Job. He had several little colds and while they didn't send him to the hospital like they would have last year, he was still uncomfortable and unhappy.

Job also got incredibly frustrated by his communication difficulties and our inability to understand him accurately (and quickly!).

Put together, these two issues manifested (through the middle of December throught the middle of January) in the grumpiest attitude I have ever seen Job exhibit. It was like living with a stranger! He was almost constantly angry at our feeble attempts to follow his requests and if he wasn't angry, he just didn't feel well.

This last week has shown such a stark contrast in his demeanor: he is back to his cheerful, contented, interactive little self.



The first week of January I started making some waves to get Job into a more intensive track for Speech Therapy. That's a whole other post that I will write soon, but he is now in weekly SLT and I'm really excited about our plan.

In addition to the facts that he now feels better and we're now communicating a bit better, Job also hit a new "ability spurt".

I'm making that phrase up, I suppose, to try to describe a very pronounced phenomenon that we see regularly in Job... at least after the fact.

This is the new area of research for CHD kids that I am so excited about and desperate for: how does Job's constantly inadequate oxygenation affect his physical and cognitive development?

The theory (that I absolutely believe to be true for Job) is that this inadequate oxygenation impacts cognitive function, especially in the area of executive function. This has been studied in patients on ECMO (a heart lung machine that temporarily keeps these organs working for critically ill patients as they recover from a trauma), but hasn't really been studied for kids who live on low oxygen usually (like Job who is at best getting 75% of the oxygen we with typical circulations get into our blood flow).

Job can really only work on one skill at a time. We've seen this so many times, but I always forget again as he's working on the next skill. If he's learning to take steps, he can't communicate. Well, he learned how to take steps in October and lost a huge chunk of his verbal/non communication. Once he was somewhat steady on his feet, he did gain back a few words and so I mentally checked off that "ability spurt" because he was again making sounds and he was still taking steps. Great! Done with that single focus on steps and back to talking.

But it has become so clear in the last two weeks that he was still working so hard on gaining confidence in his ability to take steps that he still just couldn't communicate well. He couldn't coordinate his body and brain and mouth to cruise around on furniture with confidence AND be receptive to our efforts to help him communicate. He couldn't try any new signs or words or sounds or gain back what he had known before. It was too much for his brain to process all at once.

I was so frustrated and worried that he refused to mimic us or work with us on anything to aid his communication even though he was so annoyed (and therefore should be motivated)! I think we have picked up more tools for how we communicate with him, as we've strategized with his various therapists or a few friends who have experience in this area. I think he's just gotten older and learned more. But there is an ability to participate now that there just wasn't a month ago, two months ago.

It's exciting to see Job be so much more receptive to working with us. But really, he's most interested in mimicking his big brothers again. 'Oh, Isaac is doing something? I want to do it!' They are my best helpers to help Job. Here's an example I recorded yesterday, of Job learning to sign "please":https://youtu.be/zHsXldeCXBg

November Update

Job is practically all grown up! This is one of my new favorite photos. He was so proud of himself for climbing up onto the arm of the couch all by himself, multiple times, and is studiously avoiding looking at me so he won't see or hear me tell him to come down.

Short update: Job's fine. No big changes!

Long update: Job climbs up and down the couch and stairs with ease. He cruises (takes steps, while holding onto something for stability) along couches or tables or stools or our legs but refuses to make the next jump to reaching out between a stool and couch, if there's a bit of a gap. He will take some steps holding onto someone's hands or his push cart, but he has to be interested; he won't just perform for us on cue.

Job has always been so sweet and cheerful, but we're starting to see him insist on his Definite Opinions. It's developmentally totally appropriate, but it is jarring to see him be so demanding suddenly.

He gets especially frustrated because communication is still difficult for him. We've made some gains but are definitely working hard to help him help us to understand what it is he needs.

He can say "Gan-pa," "uh oh," "hi," "peek boo" and "'at" ("cat" but used for all animals); he signs for "more" and nods his head "yes" and shakes his head "no"; and he makes a quacking noise and pants like a dog. He said "Gan-pa" for the first time back in May, so it's not as if we're rapidly progressing in our language acquisition attempts. Still, I remind myself that we are progressing. We see our speech therapist as I request but since there is progress, we're holding off on weekly therapy/other interventions.

It is very hard for Job to work on his gross motor skills AND increasing his language (whether by sign or speech). He can only focus on one or the other, so we'll see some language gain (a new word/sign) and then a few weeks later he'll switch to some new gross motor gain (more stable foot placement), then a few weeks after that, some new language gain.

This is almost certainly because of the way his body and brain are (inadequately) oxygenated. I am very interested in the current studies on low oxygenation/oxygen deprivation and how this affects motor planning and mental processing. This is one of the big new areas of research for HLHS kids (as part of the general trend of researching how quality of life can be increased rather than "just" keeping these kids alive), and I can't wait for conferences/papers to be put together/published on the topic. I want this to be a more frequent discussion than it has been.

After his cold and then GI issues stemming from his cold (mid November) we went back on two of his digestive meds. So we're back up to 8 meds and 5 med times. It feels like backwards progress, but I am grateful he's keeping food down again. We're going to make a push to get off of these two meds in early January.

Job had his big cardiology check up last week and is doing ok. We need to adjust some of his medications and perhaps go on a few additional supplements/meds because his heart function isn't quite where we want it. Though his tricuspid valve regurgitation is unchanged, it's likely we won't be able to wait until OHS #3 (the Fontan) to deal with that problem and will be getting an extra OHS next year or so. Currently, his blood pressures and flow are still too high for him to be eligible for the Fontan (part of why we'll likely do a valve repair/replacement sooner rather than later), which is part of the med adjustments we're doing.

None of that is new news, and he's stable, but I still struggle to not be disappointed that there wasn't some miraculous improvement.

We'll continue to dialogue often with cardiology, but don't have another cardiology appointment on the schedule until April. And we'll likely do a cath in the spring/early summer, to get some hard numbers on his pressure problems so we can perhaps then start to attack that issue more intensely in preparation for Fontan eligibility. I am NOT used to only seeing them every four months. It's exciting, yes, but it's also a bit intimidating. And it's very odd! I still expect at least monthly appointments, if not more often, because that was our life for so long.

I've been trying to figure out how to articulate what it's like to be in this new, awkward stage, of Job's medical journey, but just can't figure out what to say (thus no posts). There isn't some clear cut goal to strive for anymore (like getting off oxygen), and he looks so much healthier. Everything is better! But it's still hard. It's not "normal". He's not actually "healthy". So how do we proceed? No answers here, though it's probably just "day by day".