Nighttime Oxygen

A typical oxygen saturation is about 98-99%. I believe most people are hospitalized if they drop to 90%.

But thanks to Job's surgeries, his body can function, is supposed to function, in the 70s-80s. These low levels of oxygen do take their toll on his body, both in the short term (his digestive issues, for example) and the long term (predicted organ failure).

However, when he started walking back in February we quickly realized his oxygen saturations were dropping really low as he exerted himself. It's been hard to get good reads while he's now running around, but the guess was that he hit 38% on Friday at our appointment.

Job bounces back pretty quickly once he rests, but the downward trend isn't sustainable (thus heart transplant list).

Because Job gets so "blue" when he's active, he should be on oxygen all the time. But he's an opinionated toddler who doesn't want a nasal cannula on his face, thank you very much. Most toddlers don't want oxygen so for this whole summer his teams didn't even want to bother to try.

But in his trials on Friday, we did get him to wear the mask (thanks to Isaac modeling it and Ezra talking about how cool it looked) and his sats bounced up 15 points!

Oxygen wasn't supposed to help him so drastically, but Dr. Files insisted on trying it and sure enough, it worked and well. Well enough to try to force it at night.

The best guess (because Job won't cooperate with expectations) is that his pulmonary AVMs ("bad," narrowed veins) aren't allowing enough oxygenated blood through. This was the reason Job was on oxygen from September 2016-August 2017, but the combo of his axillary fistula (surgery) and sildenafil (medication) worked to get him off.

Since PAVMs are so rare for kids like Job, the fistula is pretty rare too. He was only the second kid at SCH to have it done, and the smallest, so they made their best estimate for the size of the fistula and they ultimately made it too small because the PAVMs are back. They've been back for a few months actually.

So we are now going to start putting him on oxygen at night (or trying to, at least). The thought is that he'll then wake up properly oxygenated and have more stamina throughout the day. I'm skeptical that he'll keep it on but excited to see if it will help!

We kept some of the oxygen supplies from our last oxygen experience but are waiting for a new delivery of masks and larger canulas to officially start this up.

Tricuspid Valve Regurgitation

Short version: Job's heart is steadily failing and he now needs another procedure this month. He's still on the transplant list.

Long version: A friend asked me if yesterday's cardiology appointment was "just routine?" and I responded "yes, routine bad news every month".

That has definitely been the case these past eight months, and really, it's to be expected since Job's in heart failure and listed for heart transplant. I guess I just didn't expect the decline to be so very noticeable, week after week. I thought we'd have a longer trajectory.

I keep reminding myself of how sick he can get still and how many interventions are available still, but I'm just so sad and so weary after this last appointment.

The heart has four chambers and therefore four valves. The tricuspid valve is supposed to allow blood to flow from the right atrium to the right ventricle but not let that blood "leak" back into the atrium.

Job's tricuspid valve does indeed leak blood back to the atrium which causes two significant problems: his atrium is filling up with blood and essentially getting stretched out and he's not sending as much blood along to his body to use as he should.

This first became a problem after Job's first surgery, when his circulation was first reconfigured, because the right ventricle is so stressed from over work. If you remember, Job's left ventricle was the size of a sesame seed and not at all functional, which is why his right ventricle had to take over the job of pumping blood to the body.

The right ventricle is smaller and less powerful than the left ventricle, as it is only supposed to pump blood to the lungs as opposed to the whole body like the left ventricle. Think lawn motor engine vs cadilac engine.

We always expected Job would need a tricuspid valve replacement, until we realized his other issues necessitated a transplant instead of the other HLHS interventions.

We have been carefully monitoring Job's tricuspid valve "leakage" (regurgitation). It was increasing (to "moderate") but then stabilized about 6 months ago and actually seemed to be improving (to "mild").

But since his last echo three months ago, his tricuspid valve regurge is suddenly very severe.

This could be because his heart is failing and failing quickly and this is just one expression of this failure.



But it could be because of narrowing in his aorta which would need surgical intervention. His aorta has been patched and enlarged twice in open heart surgery and has had two cath interventions but the flow is fine at these intervention sites (where we would expect scar tissue to create narrowing).

The team debated about sending Job to the cath lab where they could assess his heart, figure out why the tricuspid valve is suddenly so leaky, figure out if there is narrowing somewhere else, and then potentially intervene. But they decided the risks of an exploratory surgical procedure and anesthesia are too high for him right now. Job is too fragile around with unless it's an emergency.

So we'll be doing a CT angiogram which I know nothing about yet but will be learning about soon.

Job is still on the transplant list and this new finding hasn't "hurt" or "helped" his status. But we need to optimize his health so that he can wait however long for a transplant match to come up.

His huffing and puffing and increased cyanosis (blueness) are likely because of this regurge and they will probably continue to get worse.

We also did an oxygen trial but I'll write about that later because this post is already so long.

Transplant FAQ

I wanted to try to write up a list of facts about what we understand about the transplant process. I'm very much learning as we go, so I'm presenting this information with plenty of caveats! But almost everyone I've talked to has asked the same questions, so I thought another post was due.

Sitting in a fire truck at the Shoreline Festival this weekend.

Short version: 

Job is listed for heart transplant. The organ matching process is complex: we could get a call tonight or in a few months or in a year. Post transplant, we'll likely be inpatient for about three weeks and then home but relatively quarantined for some time as his anti-rejection meds are gradually adjusted.

Long version:

• Job is (as of 8/17/18) listed for transplant at Seattle Children's Hospital.
• Job is listed as status 2. There are four status levels, increasing in urgency: 7 = inactive; 2 = at home; 1b = usually in the hospital; 1a = in the hospital on heavy medical support.
• Now that we are listed, we could get a call at any moment (unless we temporarily deactivate, which would happen if we do any traveling away from the hospital - even if just a few hours or if Job gets sick).
• We have no way of knowing when a call might come. A (very dynamic) list is populated for every available donor heart, and Job is assigned a number on said list for each heart. He may gradually work his way up the list or he may leapfrog over other kids who have been on the list for a longer period of time or who are more critical because he is the best candidate for that particular heart being offered.
• Donor matching is based on the size of the heart (measured by weight), blood type, antibodies and geographical location.
• Job, miraculously, has no antibodies.  Because he's received so many transfusions and because donor tissue has been used in his surgeries to reconstruct his aorta and make other reconfigurations, we were expecting him to have developed some antibodies to certain human tissues, which would limit him to only match with a select number of donor hearts.
• This means that Job can match with a much greater range of donor hearts, which means he will likely receive a match in less than a year - perhaps even just a few weeks or months.
• The United States is divided into eleven geographical regions for organ donation. We're in region 5, which includes Alaska, Montana, Idaho, Oregon and Washington. SCH is the only pediatric heart transplant center in region 5. It's possible that we might get an offer from a neighboring region, but we probably wouldn't accept such an offer unless Job declines and his need is more urgent.
• Geographic location is key because it is so important that the donor heart spends as little time "on ice" as possible (or in cold ischemia time). Different organs have differing acceptable ischemic times, but for the heart they try to keep it under 4 hours. Obtaining a heart from a different region increases the ischemic time (I've heard 8 hours is the absolute max possible for a heart).
• There is an extensive"behind the scenes" process to coordinate the matching process when a donor organ becomes available. It's so complicated and so intricate I have yet to wrap my head around it. It all has to happen so quickly, too!
• When SCH receives a donor offer they send a recovery team (surgeon and fellow and nurses) via chartered jet to the donor's hospital to assess the organ's viability and then make the recovery.
• We will get a call when there's a match and (almost always) immediately make our way to the hospital. We'll keep in contact with the team to let them know where we are so they can plan (to the minute!) the rest of the process.
• Job will be prepped for surgery - he'll get his chlorhexidine bath and his IV and so on. When Job goes under anesthesia depends on when the recovery team makes it to the donor's hospital and when they have begun the recovery process, because they won't make an incision on Job until they have confirmed that the donor heart is viable.
• Of course a great deal of imaging of the donor heart will be sent to SCH to enable them to even accept the offer, but the final determination that the donor heart is a viable transplant option won't be made until the recovery team has actually visualized the donor heart beating with their own eyes.
• It takes an hour or so to properly anesthetize Job and then two or so hours to open his chest because of the scar tissue he has from his previous surgeries. So that process is perfectly timed with the recovery process so that the recovery team walks into the OR with the donor heart when Job is ready to receive it (hopefully under four hours from when it was recovered, as already mentioned).
• Comparative to the two previous open heart surgeries Job has already had (the Norwood at five days old and the Glenn at five months old), the actual surgical procedure of a transplant is rather simple. Our surgeon said "it's just a few cuts and then some sewing,"which is obviously a gross understatement but one that underscores how intricate his other surgeries were. The mortality rate for the surgery itself is, of course, also much lower.
• The total time Job will likely be in the OR is about 10-12 hours.
• Post surgical care will begin in the CICU but transition to the step down ward when Job is stabilized. Typical inpatient care is about 3-4 weeks, post transplant, and is primarily focused on the course of antirejection meds Job will require.
• We were told that after about a week of recovery Job will feel better than he's ever felt before, as he will, for the first time in his life, have proper oxygenation. He'll of course be sore from surgery, but our team said he ought to be quite energetic and want to run and play.
• The biggest issue in Job's life will no longer be desaturation but immunosuppression. This probably deserves a post of its own, but his transplant team will be carefully looking for any sign of rejection, which is his body attacking the new foreign object (the donor heart) in his body. Even a cold can sometimes trigger an episode of rejection so we'll be quite careful about germ exposure.
• Post transplant, Job will have a regimen of doctor appointments and blood draws and medications that sounds rather similar to what we experienced the months after his Norwood. Of course many of the specifics will be different, but because we've something somewhat similar before, this doesn't sound as intimidating as it otherwise might.
• We are overwhelmed at the reality that a donor match means another child has died and are already grieving for the donor's family. The big boys ask so many questions about this aspect in particular and we are very sobered by this awful, awful fact, even though we are also already grateful that families do chose to donate life.
• This is especially grim because most transplant recipients do, in fact, need another transplant after 10 or so years, because of chronic rejection. Medical science is always advancing but a heart transplant is no "fix". Job will never be "cured" with a new heart.

Rare Good News

Ahhhh! I just got the call about the ultrasounds done today and can hardly believe it!

Both Job's femoral arteries (at his groin - think femur - thigh - bone) were completely occluded (blocked) from previous caths (left side May 2016, right side August 2016) because his arteries were so very small at that time (barely bigger than the catheter).

This has made subsequent caths difficult because that prime cath access was inaccessible and meant a long course of lovenox (anticoagulant) therapy. For certain views and access points the cath team needs femoral artery access or they have to go through the liver. That's no lonher necessary!

I found out today that they did actually get access in one artery in his May 2018 cath but I didn't remember hearing that news because the rest of the news from that cath was so very bad.

Wow!! This is so exciting to me! This means that future cath access is wide open and it means he'll have proper blood flow to his legs! (His body has compensated for the inadequate flow because of the clots by making collateral vessels to get blood to his legs - think side streets instead of freeways).

The ultrasound of his left diaphragm showed (as I expected) no appreciable movement. It's still paralyzed and plicating it (stapling it to the chest wall) back in May 2016 was in fact the right decision (so that it wouldn't hamper breathing). It's been two years, so if it was going to regain function it would have already.

I've been wanting this information for the last 8 months as we've been working so hard on learning to walk and talk. The diaphragm is so integral to the core support, posture and breath support all necessary to, in fact, walk and talk and the left side being paralyzed has no doubt slowed his progress down. I wanted confirmation but to order an ultrasound just for my informational purposes when he still was making (slow) progress in those areas wasn't worth the cost and effort...

But now that we're prepping for a big surgery they ordered this ultrasound to have a more thorough picture of what they're working with for his respiratory recovery post surgery. And, much to my excitement, now I can push speech therapy a bit harder to help Job learn to compensate.

So yay! Femoral artery access and confirmation of continued left hemidiaphragm paralysis!

And another picture of my towhead:

August Cardiology Appointment

We had a regular ole cardiology appointment today and got ultrasounds of Job's arteries ans his diaphragm.

We will be alternating appointments for the foreseeable future: single ventricle clinic (August), transplant clinic (September), SV + echo (October), TX (November), SV (December), TX + echo (January) and so on. Monthly appointments and echos every three months.

It's sad to be back on this schedule after just two every-three-month appointments, but we're used to it (and if we do get a transplant we'll be back to our old once a week schedule).

These appointments will just provide monitoring and probably mean med dose changes as the two teams work to titrate Job's meds to keep him as stable as possible.

SCH staff/doctors/nurses as always make each appointment as pleasant as possible and we are so grateful for this special hospital. It was early enough in the day that we even went out to play at the playground for a bit between appointments and Job had a blast fast walking around the structures (even if he did keep asking for his brothers to play with him - apparently I'm not quite as enjoyable company as they are).

LISTING APPROVED!

We got the call: Job was approved. We list for heart transplant on Friday, August 17th!

June 2018 Recap

Job did two days at Bible Day Camp at our church this week and felt so special and important as a big kid. It was so sweet to secretly watch him go around church with such a big smile on his face. Of course, when I took a picture he gives this scrunched up grimace.

This week I spent much of my mostly kid free time on the phone with doctors and transplant coordinators and have a few more answers now.

Job has passed every test or evaluation that we have done so far. We have two big days of evaluation coming up (July 23 and 25) where we will meet with numerous specialists and do more testing. This next month, until then, ought to be relatively uneventful.

The Transplant Selection Committee meets every Wednesday afternoon, so Job will probably be presented on August 1st and they will likely make a decision that day to approve or deny him for transplant. And then we ought to know within a few days after that.

If he is approved for transplant all that paperwork would happen immediately and he would be listed by mid August. MID AUGUST.

And then, once listed, we wait.

This is all happening so much more quickly than I ever dreamed it could or would. Job's heart is pretty sick and this is our remaining option, so they are trying to push him through quickly.