Hello friends and family,
It has been a long time, and I've been meaning to write this update for awhile. Lots of big things happening right now. Life is busy with two big kids, but this goes beyond that, so I'm going to jump right into it. It is time for Reya to have her 4th Open Heart Surgery and this one is going to be a big one.
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As many of you know, Reya has had quite the journey. Three open heart surgeries as a baby / toddler and over the course of her 10 years, 24 (?) cardiac catheterizations. The cath has become routine and familiar. It is a twice yearly tune-up that she has become pretty dependent on to live the way she wants to live. It is the only way she is able to dance, ski, figure skate and keep up with her friends in general. A few years ago it became pretty clear that we were not really making forward progress. We were able to improve her quality of life in the moment, but not really make any progress. Every time Dr Esch (who does her caths) went in, he found the pressures he had worked so hard to bring down were right back to where they were at the beginning of previous caths. We tried a few different medications - Sildenafil (a vasodilator aka Viagra!!), Bosentan (a pulmonary hypertension drug), Eloquis (to help avoid clots). At one point she was taking all three, plus aspirin, which she's taken since birth. It was a lot. Sildenafil was 3 times a day! Recently we switched to Tadalafil, which is only once a day, and she is down to just that and Aspirin. That may seem like progress, but it is only because the other drugs were not beneficial enough to continue taking them that we discontinued them.
Back in December of last year, after another disappointing and frustrating cath that showed no forward progress, I asked a hard question of our team. There have been a lot of hard conversations in the past couple years, such as what the lack of forward progress means for her future. Long story short, no one really knows, but it was not likely to be good. Anyway, back to the hard question, one that had been weighing on me for a few caths - Was it time to seek a second opinion? I hated to ask this question. Our team is so talented and has tried so hard. We are so lucky to have had such great doctors on our team, not just talented medical professionals, but genuinely nice guys as well. After her December cath last year, I could see Dr Esch seemed frustrated and it opened the door for the hard question.....he agreed with me it might be time and that first we'd need to talk to her cardiologist, Dr Friedman, and said he would talk to him about it. We didn't get in to see Dr Friedman until March, at our follow-up, but he also thought it was worth a try. So the ball was rolling.
So many people have expressed surprise that Boston Children's, one of the country's top hospitals, can't do anything else for her. MAPCAs (Major Aortopulmonary Collateral Arteries) are tricky and Reya has an especially tricky case. It is not at the most severe level, but it more than just a moderate case.
BCH treats MAPCAs by stenting and ballooning in the cath lab, known as pulmonary artery rehab. The hope is, as a MAPCAs patient gets older, bigger and stronger, that they are able to stretch out the caths, so that patients are only going in every 18-24 months. This works great for a lot of cases, but there is something about Reya's disease that causes her pulmonary arteries (PAs) to shrink back up every single time. This causes high pressures and has resulted in Pulmonary Hypertension.
Upon doing some research, and a lot of listening in the MAPCAs groups I'm in, we learned that there is a surgery, called the PAR (Pulmonary Artery Reconstruction) at Lucille Packard Children's Hosptial at Stanford. It seemed like a perfect fit for her, so when I asked this question of our team, it was specifically Stanford that we had in mind. They agreed that Stanford is doing amazing things for kids with MAPCAs, it is kind of their niche specialty, for a pretty rare condition. They thought we should go for it and to quote Dr Esch, "If Stanford thinks she is a candidate for this surgery, we should absolutely go." Both doctors said they are doing things far beyond what Boston Children's can do surgically for kids with MAPCAs. However, they said if Stanford came back with new ideas for the cath lab, we would do that here, because our team here is far more advanced in the cath lab.
In May, I had a conversation with another BCH heart mom who had taken her son out there. They had dramatic results, that have greatly improved his life and his future prognosis. She enthusiastically encouraged me to take Reya out there if they accepted us. And she made me very excited about the possibility. Shortly after that, I asked Dr Friedman to get the records sent. We were looking at our June cath a few weeks later, and I wanted to be sure that if there was any imaging they needed they would be able to get it.
The records were sent, and I received confirmation from someone on their team shortly thereafter. I explained that we would be in the cath lab shortly and asked if they could expedite it. A few days later, on a beautiful May night, my phone rang and caller ID said it was Dr Friedman, so of course I answered it. He was calling to tell me he had just gotten off the phone with Dr McIlhenney at Stanford, and wanted to be the one to tell me first that they thought the PAR was a good fit. He did warn me that it was a big surgery. As we were talking to him, a call came in from Palo Alto - It was Dr McIlhenney himself. We called him back and he told us all about the surgery and why it was a good fit. He asked us if we wanted to go through with it and we said yes. A few days later, the scheduler called to get us on the books. November 13th. At the time, that seemed so far away, but here we are two weeks out, and making our final preparations to travel west.
We are excited and nervous. This surgery, if successful will greatly improve her quality of life and address many of the topics of those hard conversations we have had with our team. She looks super healthy and lives her life to the fullest, but breathing and exercise are hard. Her O2 saturations drop dramatically when she exercises because it is so hard to get blood to her lungs through her tiny PAs. In addition, we have heard various things about her lifespan, most not super optimistic. This surgery could add 20-30 years to her life, and help allow her to live long into adulthood. Finally, without this surgery, it is unlikely her body could handle pregnancy, so this is also an attempt to protect her ability to have children of her own.
She left school last week and is currently being tutored at home. The school provided her with a tutor through zoom, two hours a day, and it is working well. Chris works from home, and Hope and I are doing our best not to bring germs home to her. We are still allowing her to see friends outside and she did still go Trick or Treating last night.
Next Sunday she and I will get on a plane and fly out to San Francisco. One week from today She has appointments on the 9th, a cath for imaging on the 10th (they prefer to do their own cath in their own way), we will be overnight in the hospital on the 10th, then a day and a half free before surgery on the 13th. We hope to do the highlights of the Bay Area - Golden Gate Bridge, Cable Car, Ghiardelli Sundae, and maybe some Redwoods! We might even ride up to Sonoma - Reya would love to see the vineyards. Chris will fly out on the 12th, while Hope stays at home with her grandparents so she can go to school and stay in her routine. My parents first and then Chris' parents.
I have a lot more to say, and will continue to update here as the date approaches. I will also update here and on Facebook / Instagram the day of surgery and during her recovery.
Many people have asked how to support us. I don't really know the answer to this, and am still thinking about it. Reya will need things to keep her busy and crazy socks might be fun for her! I have been compiling an amazon wishlist for her and I am trying to figure out the best place to have things sent out there. She would probably also really enjoy cards and letters. Hope will need to be made to feel special, she's feeling pretty left out right now, so I am also compiling a wishlist for her. Both of those list will come soon in a future post.
Thank you to everyone who has supported us in this interim as we wait. It has meant a lot. We are so luck to have an amazing village both near and far.
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