Enough

To say that the last two months have been rough is something of an understatement.  Every time I think I have reached my limit on the amount of stress I can handle, something else got dropped onto our plates, and we had to make room for more.  Fortunately, we have been well provided for, and each of these episodes* has been resolved positively.  Still, it would be nice if we didn't have to keep figuring out how to solve more problems.

*My brother and sister have decided that we are experiencing a saga.  As such, I have come to refer to the stressor events as episodes, a la Star Wars.

Episode 1:  The Grandmothers

On September 27, one of my grandmothers passed away on her 96th brthday.  Six days later, her funeral was held.  Because of my pregnancy, I could not attend.  That evening, we received word that my other grandmother, who was 97, had passed away.  Again, I could not make the funeral.  Everyone understood, but I was crushed not just by the double loss, but by my inability to join my family in mourning.

Episode 2:  The Bathroom

Our bathroom needed fixing for close to two years now, but it finally reached the point where it was unusuable, between the non-working tub drain and tiles falling off the shower wall.  So, we finaly made arrangments to get it redone.  The original plan was for it to be finished before Jellybean arrived.  However, the day after work started, we found out that the subcontractor who was scheduled to start the next day had just been rescued after a week lost in the wilderness and was in the hospital close to death.  This put things a week behind as we got a new subcontractor.  Several other hiccups came along, as they always do in remodel projects, and it finally got finished this week.  It looks amazing, and we are totally thrilled with it and the people who did it.  However, next time, I will not schedule remodel work for when I am 9 months pregnant or about to have a child undergo heart surgery.

Episode 3:  Jellybean

So, we knew Jellybean was going to have issues and expected the heart surgery, but just because we knew about it didn't make it any less stressful.  He did so well with his recovery the first few days after surgery that we got our hopes up that he was going to beat the curve.  However, progress slowed and then things went backwards for a time as he lost weight, got congested, and refused to be weaned off the oxygen.  Progress has started again, but it is slow-going and the waiting is sometimes tedious.  This is, by far, the biggest stressor, and the unknowns don't help.  Still, we know we're getting amazing care and we are in the very best place for him.  We just hope we get to bring Jellybean home soon.

Episode 4:  The Mentor

On November 11, we learned that Phil's mentor, Rev. Dr. C, was going into hospice.  Just as we were starting to process the news, we found out that he passed away.  This was a huge hit not just for Phil, but also his parents, who are busy watching Lil' Bit for us in absentia, because Rev. Dr. C was their pastor and friend for so many years.  Because, you know, what we really needed was more death and grief stress while our son is fighting for his life.

Episode 5:  The Emergency Appendectomy

Yesterday, we got a call that Phil's mother was in the hospital with what was ultimately determined to be appendecitis.  They called the surgical team in last night so that they could take care of it before it ruptured.  Still, we had to figure out arrangements for Lil' Bit for the afternoon and evening since Phil's folks were out of service.  Fortunately, her godparents stepped up and Lil' Bit got to spend her birthday with four of her favorite people.  Chuch members also stepped up and started planning meals to bring to the house.  So, everything turned out well, but again, lots of extra stress.

Episode 6:  The Furnace

While my mother-in-law was convelescing in the hospital overnight, our furnace quit working and my father-in-law woke up to a cold house.  Phil headed home to oversee repair stuff and also to visit his mom and see Lil' Bit, while I held down the fort at the hospital.  Fortunately, the furnace problem was just a pilot light issue, so it was an easy and relatively cheap fix.  Even so, it was one more d*** thing.

So, here's the deal.  I am done.  I have had enough.  I know better than to ask if anything else could go wrong; there's always something more that could happen.  So, instead, I would like to kindly request the universe to give us a break.  Let us get through the rest of Jellybean's hospitalization without additional episodes.  I'm glad we've been amazing and handled all kinds of stuff so well (so far), but I am tired of having to be amazing.  I'd like a little quiet, please.  But, if that's not in the cards, then I'll just ask that we continue to find positive resolutions for whatever comes; that we have enough emotional and physical energy left to do whatever needs to be done; that we have enough love, prayers, and support to see us through.  My simple prayer is for "enough."

One of a Kind

Jellybean arrived on October 29 around 11:00 p.m.  He was 8 lbs even and 21 inches.  He had a few issues and was transferred to Children's Hospital that night.  After performing a four-hour echo on him and all of the cardiologists conferring with each other, they found that they were still confused by what they saw.  They sent him for a cardiac catheterization, which provided a few more answers, but they still needed more information.  After a cardiac MRI, they had exhausted all methods of looking at his heart structure and finally had enough to make a diagnosis and surgical plan.

What follows is going to be fairly technical and my best description/regurgitation of what we have been told.  The short version boils down to this:  Jellybean's heart structure is one of a kind.  The reason the doctors had such a hard time coming up with a diagnosis--either from the fetal echo or the recent testing--is because he doesn't have just one issue.  Instead, he has roughly three separate issues, none of them standard, which then combined to create a very complex and problematic heart.  His surgery today is not a complete fix and, until medicine progresses to a point that stents can grow, he will need additional open-heart surgeries throughout his entire life.  This information has been overwhelming, to say the least.  What normal approximation of life we have created to cope with Lil' Bit's medical issues will likely disappear as we learn to adjust to Jellybean's uncertain future.  And yet, we feel blessed to have Jellybean, for however long we get to have him.  We will do our best to enjoy every minute we have with him.  Other than that, all we can do is make sure we get Jellybean the best care we can and the rest is out of our hands.  A very hard lesson for this control freak, to be sure, but I'm working on it.

Without further delay, here's as much technical information as we have:

After our last fetal echo, the diagnosis was likely hypoplastic left heart but possible truncus arteriosus.  The official diagnosis is:  truncus arteriosus with discontinuous pulmonary arteries and mild to moderate hypooplasia of the left ventricle.  In other words, some of both, plus more.

Truncus arteriosus occurs when the truncal vessel fails to split into both the aorta and pulmonary artery during fetal development.  Truncus always appears with a VSD which allows the blood to mix between the two ventricles.  As a result, there is a single great vessel and both the body and the lungs receive mixed (i.e. both oxygenated and unoxygenated) blood.  Because the vessel was supposed to become two separate structures, the valve is usually problematic being stiff (stenosis), leaky (insufficient), or both.  Jellybean's valve is somewhat leaky, but generally good.  It will likely be the least of his issues.

Usually, this condition is repaired by closing the VSD in such a way as to isolate the truncus into the left ventricle and a new pulmonary artery is created and attached to the right ventricle.  The first shunt usually lasts 1-3 years before it needs to be replaced because the body has outgrown it.  Shunts are then replaced through open-heart surgery roughly 3-5 years later, again 5-10 years after that, and by adulthood, roughly every 10-15 years when they wear out.  This is called a "complete" repair.  

This is not an option for Jellybean, however, because his left ventricle is clinically smaller.  Thus, although he does not have a classic hypoplastic left heart, there is some question whether it is capable of pumping sufficiently well to get the oxygenated blood out to the body.  Instead, they will do a partial repair, which involves placing a shunt (a 4mm diameter goretex tube) between the pulmonary arteries and the subclavian or carteroid artery.  By doing only a partial repair, we give Jellybean time to grow to see if the left ventricle continues to increase in size to the point where it looks capable of functioning in a 2-ventricle system.  If so, subsequent surgery would complete the truncus repair.  If not, subsequent surgery would convert his heart into a univentricular system the way they would with a classic hypoplastic left heart condition.

The final complicating factor is the discontinuous pulmonary arteries.  Usually, truncus presents in a variety of types which are classified by the placement of where the pulmonary arteries attach to the truncus vessel.  Jellybean's left pulmonary artery comes off the truncus on one side, but his right pulmonary artery is connected to a ductus arteriosus--a fetal structure that usually disappears after birth.  This type of truncus is incredibly rare and, except for a single case from the 1970s, generally involves the left pulmonary artery coming off a ductus, not the right.  

Jellybean has been on medication, called prostoglandins, to keep this fetal structue intact while they figure out how to fix things.  However, once surgery is over, the medication will stop and the ductus will disappear.  The surgeon will remove the left pulmonary artery from the truncus and then utilize a patch--made of either homograft or pericardial tissue--to connect it to the right pulmonary artery.  This process is called unifocalization.  Unfortunately, because the right pulmonary artery comes off the ductus that will disappear, the distance between the two is fairly significant.  The hope is that the patch will allow the pulmonary arteries to continue to grow and feed the lungs, but the patch, like the stent, will not grow.  So, Jellybean will have a lifetime of heart catheterizations to fix any narrowing of the patch and open-heart surgeries to replace the patch as he grows or it wears out.

Much of the surgeon's work will be done "on the fly" because he has to wait and see what everything looks like when he gets in there.  For eample, he doesn't know how much pulmonary tissue there is on the right side after the end of the ductus and he doesn't know how much pulmonary tissue there is from the left side and how far it will stretch toward the right side.

Recovery from surgery will be roughly 3 weeks on the good side.  Approximately 10 days to recover from surgery and 10 days to learn how to feed (he hasn't ever eaten because, the way his blood flow works, his lower body doesn't get enough blood and feeding him would tax the intestines and kidneys which may not be getting adequate blood supply, thereby causing lots of problems).  He will then be followed closely, likely with weekly doctor visits, to check the status of both the shunt and the patch.  He may need oxygen when he gets home, but we won't know until close to the end of his recovery.  At around 4-6 months, he'll need a heart cath to see how he's doing.  If his growth (weight), echos showing pulmonary development, and blood saturations (sats) are all good, they will likely put off his next surgery until he is closer to one year.  If any or all of them are less than they would like, the second surgery will be closer to six months.  And, as noted above, what that surgery will entail will depend entirely on how the left ventricle has been functioning and whether it is sufficient to pump blood to the body.

If it turns out that the left ventricle cannot function in that capacity, but pulmonary growth has not been sufficient, it is possible that Jellybean will need a heart & lung transplant.  His anatomy is such that you could not place a healthy heart into it and have the system function.  Thus, only a full transplant of both would work.  Although this is certainly on the continuum of things that could happen, at this point, the doctors are feeling good about the chance of fixing things.

Things in Jellybean's favor:  his size.  Although he's lost some weight (just under .5 lbs as of this morning), he's still much larger than most kids who undergo this type of surgery.  We grew him big and it is to his benefit.  He also doesn't have any other health issues other than the heart, so they are only dealing with the heart stuff.  This gives him an excellent prognosis.

As I write this, Jellybean is in surgery.  It's going to be a long day followed by a long recovery, but we are hopeful.  The surgeon gave us a roughly 10% mortality risk with the surgery, but admitted that it was really just a guess because there simply aren't any cases of this condition.  Jellybean is unique, his required repair is unique, and there simply is no case to which he can be compared to guess at survival rates.  All we can do is wait and see.

One final note.  Many people have asked whether Jellybean's condition is similar to what Lil' Bit has.  The answer is sort of.  Jellybean's right pulmonary artery being attached to a ductus arteriosus is likely the same things Lil' Bit had with her left pulmonary artery.  However, because we didn't know she had the condition, she didn't get prostoglandins, and the dutus disappeared and the left pulmonary artery shriveled and was gone.  The surgeon indicated that these similarities are not likely coincidental and, therefore, are likely genetic.  As such, we are certain that we are done having children.  We have our hands more than full with the two we have.