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!