The Wrong Kind of One-percenters

There’s a secret part of all of us, and not so secret for some, that wishes they were part of that elite one-percenter club. Those ~34k affluent families enjoying a luxurious lifestyle. We are not that kind of one-percenter (to the shock of no one who knows us). We’re in an even MORE exclusive club and with these kinds of odds, I swear we should play the lottery. The Centers for Disease Control and Prevention (CDC) estimates that each year about 960 babies in the United States are born with hypoplastic left heart syndrome. In other words, about 1 out of every 4,344 babies born in the United States each year is born with hypoplastic left heart syndrome. Of those, very few pull out their right atrial line post-op. Even fewer pull that line out twice. In fact, her surgeon is one of the most world-renowned surgeons for the Norwood and he said it was rare to see one come out and that he’d never seen two! After this Hope continues to play in small chances only. Only a small percentage of Norwood survivors suffer from a stroke.  Only an extremely small percentage are so sick they cannot pursue the single ventricle palliative treatment (the series of three surgeries we thought Hope would have) due to heart failure. Of those that must pursue a transplant, many don’t actually survive to transplant. Of those that receive the gift of life, about 1% exhibit arrhythmias (abnormal heart rhythms that can range in severity from benign to very dangerous). Fewer than 1% of that 1% are multifocal (coming from more than one place in the atrium in the new heart) non-resolving (meaning they didn’t go away within the first few weeks after the transplant) arrhythmias. Yes, people. This. Is. Hope. Last week Hope’s Electrophysiology Cardiologist walked John and I through the latter part of this journey and we felt like the statistics played out to literally equal that she is the one case that has manifested in this way.

Her tachycardia has been so persistent that we felt resigned to say we had to just find a way to go back to “normal” life and accept that we may be inpatient for months and months into the future.  We continued to trudge along with life in the step-down unit continuing a somewhat predictable pattern: one day shave off a bit of methadone off her dose, the next day shave off a bit of Ativan, the next day make a feeding adjustment and then let her rest, followed by rinse and repeat.  Her new weaning plan has taken quite a while to work through – nearly 8 weeks in total.  We are excited to share that our little junkie is finally off the juice!!!  The funniest part of this weaning plan was when I tried to convince the attending that was on during the step-down withdrawal debacle and she outright laughed out loud at the suggested and insisted that she get at least one good day with her so she doesn’t get badly labeled as putting her into withdrawal!  We quickly relented and enjoyed the doctor ordered quiet day!

She has gained weight steadily and is up to 6.25 kilos – putting her close to 14 pounds.  We’re fairly convinced she’s holding most of her weight in her cheeks!  Enjoy some fat-cheeked baby pics below 🙂  It’s pretty amazing to see her growth chart.  She held the same weight for nearly a month – from Halloween to Thanksgiving, as she faced and recovered from back-to-back surgeries.  Check out her growth chart below – you don’t need a medical degree to take a guess at what point her transplant happened.  Her growth has been incredible.

In preparation for her being able to go home in early January, we arranged to do our 24-hour care test on a weekend [yes – we have to pass a test to take home – and quite frankly it might be a good idea for all parents to do the same!].  I had hoped to come home on the 12th – in time for Aunt Priscilla’s visit!  I even tried to suggest it as a rumor to the transplant team, “I heard Hope is going home on the 12th!!!”  Sadly, they saw right through this and we didn’t go home on the 12th.  I am beyond thrilled to say that they have cleared us to go home TOMORROW on the 16th, provided that Hope doesn’t give them a reason to keep her longer.  Call us gunshy, but we can’t help but feel like we need to temper our excitement until we are walking out the door.  If she gives herself a reason to stay, it’s not a reason to be sad, but a reason to be relieved she showed the issue before we got home.

Her 24-hr care was a bit bumpy as there seemed to be a slight miscommunication and the nurses weren’t aware we were doing it.  It led to some pretty intense quizzing of Hope’s med schedule to see if we truly were ready for 24-hour care.  My compliments to the nurse for ensuring that Hope is in safe hands, but I think she missed the Dr. Nash-type Beautiful Mind scribbling that takes up the entire back of Hope’s door. I’ve had to find a way to keep track of her med schedule and our desired changes to work towards a home-friendly schedule and I just figured the door was one big dry erase board.  I know that thing backward and forwards.

After we established that we do know her schedule and how to give meds, our 24-hour care was fairly uneventful and made both John and I feel a bit more ready to bring our little girl home.  However, we broke the rules – or shall we say asked for forgiveness rather than permission – Saturday night.  We were bathing Hope when Dad took the initiative to remove all her telemetry/heart monitor leads, which is normal, except he KNEW that the nurses were right in the middle of the shift change so he had about 30 minutes of lead-free time with Hope.  He was practically giddy at the idea of busting out of the room as he put her in the Baby Bjorn (thanks to Stefan for this gift!) and took off on an unauthorized walk through the Step-Down unit hallways.  I’ve never seen a prouder look on his face than what I saw during this walk.  He was literally beaming as he walked up and down the hall with her.  She loved the motion and new viewpoint offered from her 6’5″ Daddy.  This was her very first time she’d ever left her hospital room and not been headed to a procedure or surgery!  The enormity of not being able to take her anywhere hit us after the walk and we agreed we must do this more often so we’d better ask forgiveness and request to make it officially allowable.  The transplant team jokingly threatened to flunk our 24-hour care, but gave us full approval for daily walks in the unit (we just have to cover Hope up or have her wear a mask).  Hope has made some friends in the step-down unit and she’s gotten to see her hospital buddies – Bennett, Tessa and Samuel – a bit more often with her daily walks.  It’s adorable to watch these kids and see the community that’s created for them to grow up together through things like Summer Heart Camp when they are older.  We are so looking forward to helping be a part of a supportive network of Heart Parents and watching our children raise a little hell together!

The most overwhelming part of 24-hour care was her home med schedule.  She had close to 20 medicines dosed multiple times a day for a total of nearly 40 doses of medicine administered every day via oral syringe and sub-Q injection.  Now between then and now we’ve been able to reduce the number of doses of some meds and eliminate some meds altogether, like Ativan, methadone.  She owes her chubby cheeks in part to the continued steroid treatment they give transplant recipients, but we are happy to say she’s off those, too!  With that, she also lost a mouth swab that makes her puke all the time.   So now we are down to only 2 dozen doses of medicine spread throughout the day.  It’s incredible that we can use the world only in the preceding sentence.  As a heart parent, you constantly feel like you are challenged with the impossible and somehow, someway you find it within yourselves to do what’s needed.

The biggest news is that she finally laughed for the very first time!  Of course, it was for Aunt Priscilla.  She’s been on the verge for the last week or so and Priscilla finally wiggled it out of her with some impressive Patty-caking.  We hope you enjoy the video & pics of her enjoying her aunt’s visit .

We hope to report our next update from the comfort the home we’ve spent so little time in for the past 6 months.  In closing, we want to thank everyone for their friendship and support.  Many people may imagine that we spend our days sad or scared, but because of the love, friendship and support of our friends and family we mostly spend each day feeling grateful for all that’s been done to help us – all the prayers, the hope, the meals, the chats, the coffee – they’ve done more than just give us nutrition – they’ve given us a connection and a real sense of family – beyond bloodlines.

we’ll leave you with the pics from her five month photo shoot – she just happened to turn 5 months on Christmas Day! Fingers crossed until tomorrow!