After a prolonged NICU stay, Cecilia (better known as “Cece”) received a diagnosis of pulmonary vein stenosis (PVS), a rare cardiovascular condition. Over the next few months, Cece underwent two heart catheterizations and two hospitalizations at OHSU Doernbecher. Cece is now 6 months old (4 months corrected for prematurity) and we sat down with her parents, Rachel and Chris, to learn more about their family’s story.

Can you share more about Cece’s diagnosis and treatment?

Our pregnancy was dreamy until week 26, when we received a diagnosis of intrauterine growth restriction – which means that Cece was really small. She was monitored for a few weeks and then delivered dramatically by emergency cesarean section on July 26 – at 30 weeks and 5 days. She was a tiny 1.5 pounds and spent the first 11 weeks of her life in the OHSU Doernbecher Neonatal Intensive Care Unit (NICU).

Our cardiac journey didn’t truly start until the fall. In fact, we thought we weren’t going to have a cardiac journey. Cece had numerous echocardiograms done in the NICU and had been cleared by the cardiac team. However, at a November follow-up appointment with Dr. Patrick Evers, we learned about the pulmonary vein stenosis. Most people are born with four pulmonary veins that go from their lungs to their heart. Two of Cece’s are narrower than they should be, which creates blood flow issues. It’s unbelievably crappy.

While this is an unlucky diagnosis, we got really lucky on several other counts! It had been a last-minute decision to send Cece home from the NICU on oxygen, and that was the only reason this echo had even been scheduled. Additionally, it just so happens that Dr. Evers is the PVS guy for several hundred miles, and he has a fantastically qualified (and fun) team at OHSU. We made the mistake of Googling PVS after our appointment. Lesson learned: Do not Google. It is a terrifying diagnosis, but our team has been supportive, positive and has (thankfully) had a plan for each stage thus far.

After her initial diagnosis, the plan was to monitor Cece with monthly echocardiograms – until she told us she needed more intervention. She did well for a few weeks, but one day started needing slightly more oxygen. Luckily, her tells are subtle. Dr. Evers got us in for a stat echo that same day, which showed the stenosis had progressed significantly. She needed a heart cath, which is when a tiny catheter is threaded through one of her femoral veins and up to her heart. Once there, the team assesses need – can the veins be widened with a balloon that is then removed? Or is a more permanent stent needed to keep them open?

To add unnecessary drama, this was during the height of the RSV surge, when pediatric beds were virtually nonexistent in the state. Jenny Mustard (a physician assistant in the cath lab and miracle worker,) along with the rest of the cath team, went to bat for our girl and were able to pull some strings to get her taken care of. There were no available beds in the Pediatric ICU (PICU), so the NICU worked some magic and made space for her for a 48-hour period. NICU readmissions are nearly as rare as PVS so we felt very fortunate despite the scary situation. On December 5, she underwent a successful catheterization with Dr. Laurie Armsby, who used two balloons to widen both of her stenosed veins (venoplasty.) A high energy, smiley girl was discharged the next day!

Being back in the NICU definitely brought back a lot of mixed emotions for us. It was really nice that we knew the staff, but it was also where we experienced a great deal of trauma. Ultimately, we felt primarily grateful to witness what they were willing and able to do for Cece.

Just shy of three weeks later, on Christmas morning, Cece was readmitted to Doernbecher in respiratory failure. We wound up in the PICU this time, and she needed to be on a ventilator. All of the diagnostic testing conveyed that her heart hadn’t changed, but her lungs looked awful. Even though she tested negative on two separate viral panels, it was suspected that she had contracted an unknown virus that we couldn’t test for. Thanks to the PICU team, her health started to improve. She was extubated after five days and moved to 10South (Doernbecher’s inpatient Hematology/Oncology unit) where thankfully there happened to be a bed waiting for her.

We were preparing to go home on Jan. 8, but that morning, Cece rapidly declined again – to the point she was back in the PICU and reintubated that evening. A repeat echo showed her heart looking worse, and the team remarkably pulled off another stat cath two days later. Both of her veins were far more stenosed than the echo had shown, so Dr. Armsby ended up putting in two stents to open them back up. Cece’s recovery post cath was incredible! She was extubated two days later and home on Jan. 18.

It was a roller coaster of 25 days, but those stents have been a miracle for Cece. She is still on a tiny bit of oxygen, which makes sense given all that she went through. But she is an alert and hilarious 6-month-old. Chatty and quick to smile, she is currently very focused on rolling over and loves trying to stand up, playing with Dad’s beard, and being sung to. We have those tiny stents to thank for all of that.

We’re learning, though, that a particularly unfortunate part of PVS is the lack of great diagnostic tests. Echocardiograms are the best we can do for right now, so the plan going forward is for Cece to get one every month or so until it looks like we need to intervene and place bigger stents. She is also on an immunosuppressant drug to try to delay any restenosing. While we love that this drug exists, it comes with its own challenges. She has to take four additional medications, have routine blood draws and will not be able to get certain vaccines.

Rachel, you’re a nurse at OHSU, and Chris, you’re a family medicine physician. How has your own expertise as healthcare providers impacted your experience as parents?

Rachel: Generally speaking, I think our healthcare backgrounds made it easier to know where to look for information and how to talk to people to learn more about something. It made us better advocates for Cece. I work on an adult inpatient medical unit at OHSU so there was some nice practical overlap – for example, I know the night IV therapist, who can get a line in anyone, no matter what, in about three minutes; so I was able to ask for him when we were struggling to get access in Cece (shout-out to Darrel on the Vascular Access Team!). Additionally, one of my best friends from nursing school works in the NICU, and we made a lot of connections at the hospital – not just from a medical standpoint but from a friendship perspective.

Chris: I like to think of my work as a doc as more of a practice than expertise! Sadly, I don’t see as many kids as I’d like in my practice these days, but having a little bit of knowledge was both a blessing and a curse. I really had no idea what was going on with Cece but her team was very willing to help us learn. We really have been lucky to have such knowledgeable and thoughtful providers throughout her life that we can learn from and trust with her care.

Is there anything you’ve learned along the way that you’d like to share with other families?

We’re still very early on in Cece’s story but there are a few things we’ve learned along the way.

First, try to develop relationships with the people who take care of your kid as much as possible (while still respecting their boundaries!). It makes the whole process better in every way. As Dr. Evers continues to remind us, we are all on this journey together with Cece!

Second, ask questions. In medicine, there’s this feeling of urgency all of the time, but we’ve learned that people are happy to teach you. The best way you can learn to advocate for your kid is to learn what you’re talking about to some degree. Your child’s providers want you to be part of the team and want to teach you – we have lots of Dr. Evers’ lovely heart diagrams hung around our house as proof of that! And Jenny walks us through each cath procedure retroactively, in detail and with videos!

Learn more about pediatric heart care at OHSU Doernbecher

In closing, we want to express how grateful and impressed we’ve been with the care that our whole family has received at Doernbecher. Pulmonary vein stenosis is scary and exhausting. Every so often we talk about how utterly incredible it is that Dr. Evers chose PVS to specialize in, that Jenny is using her talents in the cath lab and that Dr. Armsby excels at the tiniest of catheterizations. Bless their team for choosing to do this with their lives. It’s life changing for the three of us. We’ve tried donuts and pizza as tokens of our appreciation, but how can you possibly thank the people who continue to save your child’s life? We’re not sure, but we’ll keep trying.