(What you’re about to read is chapter two of Surgeon’s Story, where you meet Rylynn, an adorable two-year old with a deadly heart problem.)
“I just want to be Mommy!”
Andrea Riojas went to her OB/GYN on December 23, 2008 for her four-month sonogram. She was pregnant for the first time, and she and her husband Gilly hoped this visit would be the one where they’d find out if they were going to have a boy or a girl. A successful young couple living in central Texas, Gilly is a broad-shouldered, quiet rancher with alert, piercing eyes while Andrea—blonde, cheerful, and ready to be a mom—is an architect specializing in designing healthcare facilities.
“I went in for my twenty-week ultrasound,” Andrea recalled, “and we got the feeling right away that something wasn’t right. My OB really didn’t tell us anything because this was outside of his specialty. He said he’d need to send us to a specialist.”
Christmas, with its promise of miracle birth, couldn’t have been a more ironic time to get this kind of news and the Riojas family holiday became something less than cheerful. Andrea and Gilly waited two weeks for the specialist appointments the obstetrician recommended. At an appointment with a fetal-maternal specialist, they finally heard the diagnosis. Their baby, a girl, had hypoplastic left heart syndrome. Her left ventricle, which normally sends oxygen-rich blood through the aorta to the rest of the body, was severely underdeveloped. The specialists laid the cards on the table.
“They told us we had three options,” Andrea said. “We could terminate the pregnancy; we could proceed with the pregnancy and then either get hospice care after birth or take our baby home where we’d only have a few days with her; or we could proceed with a course of three surgeries which were usually quite successful, but were full of the risks of open-heart surgery–– the complications and the risk of fatality. Obviously, we had tremendous emotional ups and downs, and everything in between.”
In the end, their choice was really no choice at all. Andrea and Gilly wanted a child and they decided to go ahead with the pregnancy and birth, and the subsequent surgical treatment. The parents-to-be chose the name Rylynn for their unborn daughter, and after numerous meetings with doctors at major hospitals around Texas, they decided Rylynn would be treated at Children’s Medical Center.
More bad news came midway through March, during a meeting with their cardiologist at Children’s. An echocardiogram showed that Rylynn’s patent foramen ovale, the hole between her left and right atria, was closing. This is a normal development once the child has been born, but for Rylynn, still in utero, it was not good. The hole is there to re-route oxygenated blood from the near-useless left ventricle to the right ventricle for pumping around the body. One ventricle has to do the work of two. Moments after she was born, Rylynn would have to undergo a procedure to keep the atrial septum open.
Rylynn Riojas entered the world at 8:37 AM on May 1, 2009. She was delivered by Caesarean section at Parkland Hospital, the county hospital situated next door to Children’s. Due to her delicate medical condition, Rylynn did not get a chance to bond immediately with her mother.
“I didn’t get to hold her, or touch her, or anything. I remember hearing her scream and it sounded like a duck. And I remember Gilly telling me that she had lots of hair. But then, they whisked her off to Children’s, to the cardiac catheterization lab, to treat her atrial septum.
“Not only did I not get to bond with her, but Gilly took off with the doctors and nurses who were wheeling her through the corridors to the cath lab. I finally got to see a picture of her three hours after she was born. It was kind of tough.”
But Gilly returned from the cath lab sooner than Andrea expected, and the news was good. The hole between the atria now allowed for a perfect balance between flow and resistance to get Rylynn off to a good start. In the strange surroundings of a hospital, with a child wired to so many machines that they couldn’t even hold her, the new parents tried their best to enjoy their first moments of family life.
The treatment of HLHS requires three surgical procedures, each of which is a palliative, not a cure. The first of those procedures, called a Norwood, can carry significant risk. One-fifth of the babies who have the surgery die during their first hospitalization, and one-third die within their first year. Rylynn’s procedure would include placement of a Sano shunt to connect the right ventricle to the pulmonary artery, the blood vessel feeding the lungs. Dr. Joseph Forbess, Chief of Pediatric Cardiothoracic Surgery at Children’s, performed this first procedure on May fifth.
“It’s really tough to say good bye to your four-day-old baby like that,” Andrea said. “It was a very long day. We got hourly updates on the surgery from the OR, and finally we got to see her when it was done. After the surgery, day by day, they’d take out another of her chest wires or drains, and cut out another medicine. Finally, she was extubated and we actually got to hear her cry for the first time!”
A normal CVICU stay after a Norwood is 10 to 14 days. Rylynn was moved to the eighth-floor cardiology ward after only six days. To make it even sweeter, Rylynn’s feeding tube was removed. These were the first indications of the toughness this tiny child showed during a battle that would inspire the host of professionals at Children’s Medical Center involved in her care. She became what the hospital staff likes to call a “rock star.”
On Mother’s Day, Andrea held Rylynn in her arms for the first time.
Andrea and Gilly had to remain within quick driving distance of Children’s after Rylynn’s discharge, keeping her in position to be transported for her second surgery when the time came. The wait wasn’t long. By the time she was two months old her oxygen level was fluctuating and doses of Lasix were administered to counteract fluid buildup, all in an effort to buy time. Rylynn and her parents headed back to Children’s in September for the second procedure, called a bi-directional Glenn shunt. On September 24, 2009, Dr. Forbess connected Rylynn’s superior vena cava to the right branch of her pulmonary artery, so that deoxygenated blood from the upper body, head, chest, and arms traveled directly to the lungs, increasing the amount of blood receiving oxygen. Like the Norwood, the Glenn is a palliative. There would be more surgery down the line.
Rylynn again displayed amazing recuperative powers, going from the CVICU to the ward only 24 hours after surgery. However, along with the usual post-operative recovery discomfort, this time she had severe headaches.
“She screamed hysterically every 15 to 20 minutes,” according to Andrea, “but after a while, she’d gotten through the worst of it and was able to quiet down and rest.”
Andrea and Gilly tried resting as well, sleeping on the narrow pullout couch in Rylynn’s room. The uncomfortable bed, and more, did not go unnoticed by Andrea, the designer of healthcare facilities. The time spent with her daughter in all those hospitals would have a major impact on Andrea’s design work later on.
Everything looked great in June of 2010, when 13-month-old Rylynn had a visit with her cardiologist. Her oxygen saturation was normal, her heart function good. The doctor told the pleased parents they wouldn’t need to bring their daughter back for at least six months. But Rylynn developed a viral infection on October first, which led to a severe earache. She spent a couple of days at Dell Children’s Hospital in Austin, where doctors tried to get her oxygen level corrected and help her shed the bug she’d picked up. Even a routine virus is a severe threat to a child with a damaged heart. Rylynn visited her Austin cardiologist in late October, and once again the news was not good. Her single ventricle heart wasn’t working well enough to keep her growing properly.
“I asked the cardiologist how concerned we should be,” Andrea said, “and before I even finished the question, he said, ‘Very.’”
Another holiday came to be associated with bad news. This time, it was Thanksgiving. Dr. Matthew Lemler, Rylynn’s cardiologist at Children’s, recommended that she be brought back to Dallas as soon as possible for transplant evaluation. Rylynn was admitted for a cardiac catheterization and a number of other procedures the first week of December. At the same time, she underwent a rigorous workup for transplant. After the exhaustive evaluation for various criteria—psychological, physical, financial, nutritional, and more—Rylynn was listed for transplant as United Network for Organ Sharing (UNOS) status two, meaning she was on the list, but nowhere near the top. She might wait for years.
Now came the part Andrea and Gilly hoped to avoid. From the moment she was put on the transplant list Rylynn could never be more than two hours away from Dallas. The family home in Lampasas, 180 miles to the southwest, was outside the two-hour area. A duplex was found near Waco, 90 minutes south of Dallas. Gilly would now have to split time between his family and his ranch responsibilities.
“They told us that a transplant was now our only option. They also let us know that a transplant replaces one set of problems with another. But Gilly and I both felt this would give her the best quality of life. We knew she’d be on medication the rest of her life. We knew the new heart would grow with her, but that eventually it would have to be replaced. But we wanted to give her that quality of life.”
Just after her second birthday, in May of 2011, Rylynn took a big turn for the worse. Her oxygen saturation levels were dangerously low. Her pediatrician in Waco put Rylynn on oxygen and told Andrea they needed to go straight to Children’s. The pediatrician said Rylynn would have to stay on oxygen, and that it was too dangerous for Andrea to drive her in that condition. A transport aircraft was dispatched from Children’s to Waco to pick up mother and daughter for yet another chapter in the lengthening hospital saga. Rylynn was admitted directly to the CVICU. Doctors put in a peripherally inserted central catheter (PICC) line to deliver drugs directly to her heart.
“While there were no major changes in her physically,” said Andrea, “it became apparent that her tough little heart just couldn’t keep up anymore. Gilly and I were really scared, because at this point we were no longer just treading water, we were going under a little bit.”
The tough two-year-old weathered this storm, too, and they went back to their temporary home on June fourth. Four days later the truth of Rylynn’s condition, and the 24-hour-a-day vigil needed to keep her safe, began to overwhelm Andrea.
“I hate PICC lines,” she wrote in the blog she kept during Rylynn’s illness. “I have kept it dry, clean, and I check it at least four times per day, but that does no good at all. We have something leaking that we can’t figure out. We have to have it redressed for the third time in five days. With each redressing, we risk infection, and it just plain hurts Rylynn.
“I’m not a nurse. I don’t want to be a nurse. I want to be Mommy. Only Mommy!”
The problems with the PICC line were more than the local home-health nurses could deal with. The line began to leak, and Andrea found herself once again heading to Children’s with Rylynn, this time by ambulance.
“It was a little nerve-wracking. Before we even left Waco, the driver locked himself out of the ambulance. Then, while we were speeding up I-35, he was texting a lot of the time. Finally, I asked him if he drove to Children’s often and he said he had, and that he’d driven to Dallas at least 300 times in the last four years. Then, he asked me if I knew what exit he should take to get to the hospital.”
Rylynn’s PICC line had to be replaced. She was released after a three-day stay, and she and Andrea returned to Waco.
Rylynn was back at Children’s in June, first to have the PICC line replaced with a Broviac, a different type of central catheter that’s a bit more secure in placement, especially in a two-year old. The main purpose of this visit, though, was to confront the reality of her failing liver and kidneys. Her severely compromised heart couldn’t support her vital functions much longer. Her failing heart was beginning to affect her other vital organs. She was on maximal medical therapy, which supported her failing ventricular function. Because of the increased medication, she was now status 1A for transplant, at the top of the list.
Andrea and Gilly noticed the distinct changes in their little girl’s health and attitude. Her breathing was labored and she was showing a lot of swelling. Her heart was failing. It was time to meet with Dr. Guleserian.