Pediatric cardiology patient's family showers care team members with love

When Ryan Henson shows up at the MUSC Children’s Health R. Keith Summey Medical Pavilion with his son, James, for one of James’ many medical appointments, he calls ahead to get the staffers’ Starbucks orders.

When the pair arrived on a recent Tuesday in December, they also brought Christmas gifts for James’ cardiology providers and cards addressed to individual members of the care team from the Henson family. It’s a small way of showing appreciation for the large team that cares for the busy toddler, but it’s part and parcel of the Henson family’s way to remember and single out individual care team members for praise and thanks.

Certainly Ryan and his wife, Jessica, have had plenty of opportunities over the past two years to get to know the people who work at MUSC Children’s Health, whether at the MUSC Shawn Jenkins Children’s Hospital on the peninsula or at the medical pavilion in North Charleston.

From the parking lot attendants who greet them with banter about what’s happening in the outside world, like the Super Bowl – while also asking after James – to the people inside the building who keep everything running, like Renee Wallington, who in her former role as concierge offered support to families in surgical waiting areas and connected out-of-town families to local hotels, the Hensons say that the MUSC team helped to make a traumatic experience bearable.

They are particularly appreciative of how team members kept their focus on patients during the earliest, most uncertain days of the pandemic.

“It’s a testament to everybody there because they had all of the same craziness. They had to deal with COVID and uncertainties. But, you know, they had to check it at the door, so to speak, and take care of us. And I'm forever grateful for that,” Ryan said.

 

When James was born at East Cooper Medical Center in May 2019, the Hensons had no reason to believe their third child would be anything other than healthy. Prenatal exams had raised no issues. But it was clear to doctors there that something was amiss, and James was sent to MUSC Children’s Hospital for a full workup.

An echocardiogram revealed he had a thickened heart muscle. Pediatric cardiologist Heather Henderson, M.D., said the team was concerned he had hypertrophic cardiomyopathy, although his case didn’t quite fit all the usual signs.

 

He had another abnormality, too – what's called an accessory papillary muscle. The papillary muscles are inside the heart’s ventricles and prevent the valves from collapsing when the heart contracts, but James had an accessory, or extra, papillary muscle that looked like it might be obstructing blood flow. 

But it wasn’t initially clear how serious the issue might prove to be, at least to the Hensons, who had never had cause to spend time in the MUSC Children’s Health system before. For months, James returned for appointments as doctors monitored his status, adjusting medications, until they said it was time to go the surgical route.

It was March 2020. The new children’s hospital had opened the month before. James was scheduled for surgery to remove the accessory papillary muscle, when suddenly, it seemed that the entire country shut down as COVID began to overwhelm New York City.

James’s surgery was technically considered elective, but the doctors felt it was important to move ahead. Ryan remembers the hospital as a ghost town when they showed up the day before to sign paperwork.

In retrospect, it was lucky for James that the doctors pushed to go forward with the surgery, Ryan said, even though “It all went haywire.”

 

He said that pediatric cardiothoracic surgeon Minoo Kavarana, M.D., later told him that the obstruction in James’s heart was so severe that James would have lived for only weeks without intervention. Yet the surgery proved far more complicated than expected.

Kavarana had planned to cut off that extra muscle to relieve obstruction of the aortic valve, which is nestled next to the mitral valve. But the accessory muscle was connected to the mitral valve in an unusual way, and relieving the aortic valve obstruction worsened the mitral valve. A surgery that was meant to last a few hours wore on into the night as Kavarana maneuvered in the 10-month-old's heart.

The initial outcome wasn’t good. James had to be placed on extracorporeal membrane oxygenation, or ECMO, to pump blood through his body mechanically as the team in the intensive care unit worked to stabilize him.

Kavarana had to consider options to address the mitral valve problem. Repairs often don’t last long, he said. Replacement was another option but not an easy one for a small child. Metal valves require that the patient take high levels of a blood thinner for life – not the best idea for an active child prone to typical childhood bumps and bruises – and they have to be regularly replaced as the child grows. Bioprosthetic valves don’t require blood thinners, but they don’t last as long, and would need to be regularly replaced.

Babies in James’s situation can be offered heart transplantation as an option, but Kavarana tries to avoid that whenever possible. First, the wait for a pediatric heart is long. And most importantly, heart transplants are, at best, a medium-term solution, Kavarana said. By 15 years after surgery, half of patients are either dying or getting sick from their body rejecting the heart, he said. At that point, James would still be only a teenager.

"I consider it a success only when they leave the hospital, not just alive, but alive and well.”

Minoo Kavarana, M.D.

Kavarana didn’t like any of the options.

Instead, he decided to adapt a procedure that a few other surgeons were doing. Some surgeons had started using a Melody valve – which is a valve constructed from a bovine jugular vein and intended to replace the pulmonary valve in the heart – trimming it to size and implanting it upside down in the mitral position.

But Kavarana wanted the ability to use minimally invasive procedures to expand the valve as James grows, thereby stretching out the amount of time between major surgeries to replace the valve. So he proposed implanting the valve within a Gore-Tex tube graft, which would allow him to expand the valve by 2 to 4 millimeters at a later time.

Ryan remembers talking with Kavarana about the potential surgery and Kavarana saying, essentially, “We can try this thing that’s never been done before.”

“He's like, ‘What do you think?’ And I'm like, ‘What do you mean what do I think? I think I'll do whatever you say,’” Ryan recalled. “The only alternative was to take that Hail Mary shot or to sit in the ICU waiting on a transplant.”

The surgery went well, and within weeks, James was discharged home where he finally got to see his sisters and grandparents again, who hadn’t been allowed into the hospital because of COVID visitor restrictions.

 

That was not the end of their medical journey, however.

Ryan ruefully refers to James’s surgeries as 1A, 1B, 2A and 2B. The first two – 1A and 1B – occurred in March 2020. The hope was that the valve would last for a couple of years. But echocardiograms showed that it was failing, so James was scheduled for yet another surgery the following January.

It’s not clear why the valve failed so much earlier than expected, Kavarana said, except that it developed a lot of scar tissue.

Kavarana replaced the valve, repeating his innovative technique from the year before, and all was well – until it wasn’t.

Within a few days, it was clear that the valve wasn’t working properly. Kavarana needed to do another surgery, but Ryan was hesitant at first to put his little boy through a fourth surgery.

“I'm like, ‘Well, wait, what are the options?’ He says, ‘Well, I already cleared my whole day tomorrow to make this happen,’” Ryan recalled.

After that surgery – 2B – the valve began operating properly. Doctors and the Hensons hope it will last a couple of years, but, as Henderson, his cardiologist, points out, the procedure is so unusual that there is no body of established literature to which they can point to say the average patient can expect this valve to last “X” number of years.

“I would love if he gets a few more years out of it,” Henderson said.

The second set of surgeries – the emotional whiplash going from seeming success to failure overnight – is indicative of the ups and downs of James’s path.

In addition to the major surgeries, James has dealt with an array of other health concerns, from an electrical problem in his heart that causes fast heartbeats to a leukemia scare, which, thankfully, was a false alarm.

“James has just kept us on our toes since birth, but he’s done remarkably well since he got over the hurdles of the last surgery,” Henderson said.

Ryan and Jessica say the team members at MUSC Children’s Health made the hospital feel like home, and they can name many team members off the top of their heads.

“There's a long list of people – I wish I could just give you a block paragraph, like an Oscar speech,” Ryan said.

 

Angie McKeta, PA, a member of the inpatient cardiology team: “We nicknamed her ‘the glue’ because she holds everything together.”

Lauren Haney, Pharm.D.; Andrew Savage, M.D.; and nurse practitioners Ali Burnette and Vanessa Adams are all part of the cardiac team: “That’s like the Navy SEALS right there.”

And Carol-Ann Ward, R.N., especially helped to make moments in the ICU a little better.

“We had great interactions with almost every nurse, but she just stood out because she was so happy and fun,” Jessica said. “And when it is a hard, scary dark time, to show up and have a nurse that has that sort of personality – you know, she was happy, and she made us look forward to spending the day there.”

“She made us laugh a lot in there, which is important and hard. You don't always feel like laughing,” Jessica added.

They are, of course, grateful for Henderson and Kavarana. Henderson has been a blessing to the family, Jessica said.

“She goes above and beyond what could ever be expected as far as a doctor, in my opinion,” she said.

Kavarana, for his part, said it’s gratifying to be able to help patients like James. Seeing kids going about their lives makes the long grueling hours in the operating room worthwhile, he said.

“It does feel good when you are done with the technical part of the operation but not as good as when you see them leave the hospital,” he said. “I consider it a success only when they leave the hospital, not just alive, but alive and well.”

As the family continues their journey, coming in for regular echocardiograms and waiting for the day when tests show it’s time to replace the valve again, they have a hard-won perspective that helps them to maintain a positive attitude.

“It's all perspective. Yeah, a lot of crappy things have happened,” Ryan said. “But we've gotten so lucky in so many answered prayers and so many different things that have made it infinitely better than what it could have been.”