By Heart

Diagnosed in utero at 16 weeks, Kaiden was born with hypoplastic left heart syndrome. His first of three open heart procedures came at just 4 days old. He had two more before he reached the 6-month mark. But they weren't enough.

By late March 2010, little Kaiden was in heart failure.

Thankfully, St. Joseph's initiated the only pediatric heart transplant program in Phoenix in late 2010, just before Kaiden's own heart failed. Kaiden was evaluated, then listed with the United Network for Organ Sharing (UNOS) April 13. Two days later, he was rushed to surgery to receive a new heart, and his life began again.

By November, he was well enough to "graduate" from the hospital's Complex Congenital Heart Infant Evaluation and Follow-up Program (CHIEF) and return to what his parents are hoping is a normal, active life.

Expert Care

Bedside Visit: Beth Rumack, NNP-BC (left); John Nigro, MD, cardiothoracic surgeon and St. Joseph's heart transplant director; and Ernerio Alboliras, MD, pediatric and fetal cardiologist; tend to Kaiden Ramsey-Hermosillo following his heart transplant at St. Joseph's Hospital and Medical Center. courtesy St. Joseph's Hospital and Medical Center

The heart transplant program brought Susan Park, MSN, PNP, CNS, to Phoenix. Park not only coordinates patient care, but is the point person for donor offers.

"I start being involved with the parents when we find a significant cardiomyopathy or serious congenital heart defect, and immediately when we know a child is in advanced heart failure," Park said. "By this time, they've either gone through several surgeries or have been medically managed with heart failure medications. They have exhausted most options and will now start having to consider a transplant, which is always saved as a last option for parents."

Families are screened extensively before being approved for a transplant. Candidates are not accepted simply based on physiology.

"A genetic component can cause their heart to be weak and the rest of their bodies to weaken. Giving them a new heart is probably not going to save the child," Park said. "When we consider the shortage of organs, we have to make sure their quality of life will get better by giving them this new heart. It's a very difficult process."

Heart to Heart

BAll Smiles: Susan Park, MSN, PNP, CNS, transplant coordinator for the St. Joseph's Hospital and Medical Center pediatric heart transplant program, cuddles with little Sienna Hoagland, the program's third transplant patient, during a clinical visit. courtesy St. Joseph's Hospital and Medical Center

During the initial phase, Park focuses on the introduction of transplant and what that process entails. It's only the beginning of an ongoing relationship.

"You meet parents when their child is extremely sick, and face options of not doing anything at all and losing the child, or considering a transplant," Park said. "With that, you transfer from one chronic illness to another. I make sure to let parents know the transplant will not miraculously make their child 'normal.'

"They are going to have a great quality of life, but these children will be on medications for the rest of their lives," she continued. "It's an important fact for parents to face. Their lives will be different after a transplant, and that's always their biggest question. The half life of a transplant for an infant is 12-15 years. Parents have to consider what changes they will face along with a host of other questions, including financial considerations. We need to discuss a realistic plan for their future."

Continuity of Care

Once the child has a transplant, he or she is followed for a lifetime. Park works in tandem with specialists and pediatricians after the transplant to provide expert care. Many times, there are no physical limitations, so if the child is not around sick children, quality of life can be comparable to a child who hasn't had a transplant. Parents are instructed to watch for signs of rejection at home and Park is available to them at any time they have a concern.

"It can be anything, even as subtle as a headache or fever," she said. "The parents are really on top of any changes on a daily basis. We know [the child's] baseline and can tell how things are progressing."

Gentle Touch: Phoenix Children's Hospital cardiac surgeon Jeffrey Pearl, MD, checks an infant in the pediatric cardiac ICU. courtesy Phoenix Children's Hospital

For Park, the process brings a great deal of satisfaction.

"When you go through the process of seeing a child who's on life support and has minutes or hours to live and you get a phone call from UNOS with an offer to give him or her a new chance at life, it's very rewarding," she said. "And then you see the joy in the parents' eyes afterwards, to see their child pink and with a healthy heart, it's an incredible experience."

Clinic Time

Like Park, Beth Rumack, NNP-BC, finds great satisfaction in helping her small cardiac patients and their families. As clinical coordinator for the CHIEF program, Rumack coordinates care for high-risk infants like Kaiden who are born with complex congenital heart defects. Begun in mid-2007, 91 infants have been enrolled and 79 graduates have so far successfully completed the program and phased into another form of follow-up care.

Rumack said they routinely care for babies born with one functioning ventricle or two ventricles with a defect. Graduation for single-ventricle babies generally happens after the second of three staged surgeries (usually at around 6 months), because the second surgery stabilizes circulation. Most of these children are still followed in the high-risk cardiac clinic until being transitioned out of the program. Most two-ventricle babies have had a shunt placed, allowing oxygenated blood to get to the body, and are fully repaired between 4-12 months of age, when they graduate to other outpatient care.

"We developed a comprehensive outpatient program to manage these patients, who were primarily managed by pediatricians before," Rumack said. "They may have had wonderful surgeries, but there was no coordinated continuity of care. Because of the nature of the complexity of heart defects, they need comprehensive management and we can provide that, working in concert with their pediatricians."

Quality of Life

The program's goal is to decrease inner stage mortality, to get patients to the right place of treatment quickly so they can be stabilized.

"We have very close follow-up at the office," Rumack said. "It's important for us to see them frequently, so we can follow these subtle changes, then trend them so we know what direction we're heading and start preparing for the next surgery or cardiac catheterization for intervention to be preemptive."

Parents can call 24/7 with questions and concerns.

"We do lots of educating with parents before they go home following open heart surgery, because parents don't know what normal is," Rumack said. "What was normal for other children without a heart defect may not be normal for this child. We're there to help the parents decide whether what's going on with their child is OK or not OK, and take the burden off of them for making medical decisions. It gives them a sense of security and helps facilitate and create a better team environment that includes the family. We do family-centered care. The families are part of the team - this is their child. It's a very integrated team, both inside and outside the hospital."

Having a phone triage and providers who can communicate with parents is key. The process builds a unique bond with families, allowing for a more comprehensive and higher quality level of care.

"It takes a community to manage these children, from the cardiologists to cardiothoracic surgeons to pediatricians, to therapists and nurses - it takes all of us working together," Rumack said. "We really work in a coordinated manner with the pediatricians, so when that patient is in the pediatrician's office, they have direct access to our high-risk team when they have concerns."

Future Plans

For the past several years, St. Joseph's has been in negotiations with Phoenix Children's Hospital, about a mile down the road, to form a strategic alliance when Phoenix Children's opens its new 11-story tower in June. That will mean St. Joseph's pediatric heart programs will move to Phoenix Children's in several months, joining the cardiac program already in place. The pediatric heart transplant program will move when Phoenix Children's is UNOS-certified.

As the St. Joseph's team readies for a transfer of its pediatric heart services to Phoenix Children's Hospital this summer, nurses and administrators at both hospitals are working hard to make sure the program is stronger than ever and continues to grow.

Julie Reisetter, MS, RN, director of critical care at Phoenix Children's, said she is looking forward to the signing of a permanent agreement between the two hospitals, noting the changes in store with a new tower provide a new enthusiasm for nursing staff.

"From a nursing perspective, this is a very exciting time," Reisetter said. "We've had a 40-bed ICU with 12 beds within that are predominantly focused on the care of cardiac patients. Now we'll have an entire floor, with a 24-bed cardiac ICU that's expandable to 48 beds."

Reisetter said Phoenix Children's will be prepared to serve St. Joseph's pediatric cardiology patients. "There's a lot of excitement from the standpoint of taking the Children's heart center that has been onsite for many years to a different level, to having the clinics and services those kids will need all in one area," she said. "The staff has been anxious to have their own area [in the cardiac ICU] and move forward, to have everything the child needs right there. This will take our cardiac care up another notch."

Candy Goulette is regional editor at ADVANCE.