Evelyn requires a lengthy infusion every week because of a rare disease. A nursing unit has embraced Evelyn and her family. Learn more about her bond with the nursing team.
Evelyn sits quietly on the lap of Diane Welch, R.N. A toy stethoscope hangs from Evelyn’s neck as the 1-year-old’s eyes alertly take in the scene at the post-anesthesia care unit (PACU) team meeting.
Evelyn is lucky to be here, and the PACU team is thrilled to have her even though she may seem out of place. Most patients come to the PACU to be monitored as their anesthesia wears off following a surgical procedure. Often, they visit the unit only once, not spending enough time to become a regular at a staff meeting.
Evelyn comes every Thursday.
“We’ve just sort of adopted her,” says Sandra Horan, M.S.N., R.N., NE-BC, perioperative clinical manager of the PACU team at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida.
Who is Evelyn?
Evelyn was born with Wolman disease, an ultra-rare genetic condition. A little over 50 cases in the world have been described so far. Infants with Wolman disease lack enzyme activity that the body needs to process fats — lipids — correctly and those fats and cholesterol build up, usually leading to organ failure. Historically, babies with Wolman die before their first birthday, often as early as 3 months.
But Evelyn needs the six- to eight-hour infusion every week.
“There really are no words to adequately encapsulate how far she has come,” says Kim, who completed pediatrics training at Johns Hopkins All Children’s Hospital and both medical genetics and medical biochemical genetics training at The Johns Hopkins Hospital in Baltimore. “Anyone who learns about Wolman disease in general or reviews her case specifically expects to see a small and frail young girl when they meet her only to find that she is sturdy and sassy, a true fighter who has already overcome so much.”
Managing Her Diet
In addition to the infusions, a key to managing Evelyn’s condition is her diet. Metabolic dietitian Jenna Moore, R.D.N., L.D.N, started a week before Evelyn was first admitted and has been working with her ever since.
“In Evelyn’s case, nutrition therapy for Wolman disease requires severe fat restriction,” says Moore, who meets with the family at each infusion visit and communicates with them by phone or email as needed. “She receives a specialty minimal fat formula to meet her nutritional needs along with controlled amounts of both walnut oil and safflower oil to provide the essential fatty acids Omega-3 and Omega-6 needed for healthy development. In addition, she also receives fat-soluble vitamin supplementation to prevent deficiency. We have gradually been introducing foods including fruits, vegetables, and very low-fat grains and proteins.”
Why the PACU for Infusions?
Johns Hopkins All Children’s has an outpatient infusion center on its main campus where many infusions take place, but Evelyn’s condition requires both intensive medication dose titration and monitoring that the infusion center isn’t equipped to do.
Initially, Evelyn received her infusions in the pediatric intensive care unit (PICU), but having Evelyn in one of the 28 PICU beds wasn’t an ideal situation for use of space and staffing. Horan, a 31-year veteran nurse, offered space in the PACU but wasn’t sure she could provide nursing care every week, worried she was stretching her team too thin. By the third week, the PACU realized how consistent care would benefit this family and decided they would incorporate Evelyn into their weekly staffing and patient census.
“Evelyn's family is absolutely lovely,” Welch says. “They are kind, compassionate and very appreciative of the care received at the hospital. They adore Dr. Kim and what he has done for Evelyn. They regularly praise the staff. They are very grateful people. We essentially spend six to seven hours in the same room with them so we have become close.”
“We love all the nurses in the PACU, and they have become an extension of our family,” says Evelyn’s mother, Megan. “Every one of them has taken such great care of Evelyn, and she loves to see everyone each week. We are forever grateful to everyone on her case to help extend the life of our sweet Evelyn!”
The infusion Evelyn receives is customized for her, so the pharmacy team doesn’t mix the ingredients until Evelyn’s family checks in each week. They arrive at 8 a.m. on Thursdays, and it takes about 30-40 minutes to mix the medication. At first, one nurse would sit with Evelyn but miss the information in the 45-minute weekly staff meeting. At some point, they decided the parents could take a break and the nurse assigned to Evelyn that week would scoop her up and take her to the meeting.
“She is the happiest baby,” Horan says. “We have built up such a rapport with the family, and I love the way the unit puts the family first.”
Enamored with Evelyn
The PACU team loves caring for Evelyn and her family. When Hurricane Idalia forced a change in Evelyn’s infusion schedule, Christy Lawrence, R.N., volunteered to come in on the weekend to ensure Evelyn would stay on track for her infusions.
“It's such a blessing to be one of Evelyn’s nurses,” says Courtney Chitwood, B.S.N, R.N., another nurse who cares for Evelyn. “I love taking care of her and watching her grow. We really try and make her weekly infusions as enjoyable as possible for her and the whole family — we even got to celebrate her first birthday together.”
Evelyn’s baby brother and grandparents also attend her infusions, and the PACU team never miss an opportunity to pop in and say hello.
“When Evelyn's grandfather comes with her, there is a special highlight to the day,” Welch says. “Toward the end of her infusion, her grandfather turns on the song, ‘Celebration’ by Kool and the Gang. The music is pretty loud so everyone can hear. That's when the dance party starts. The educator's office is next to her room. As soon as she hears the music, she (Rosemary Pfeifer, R.N.) joins the party.
“Evelyn smiles and claps, it is a special moment for everyone. One more infusion down.”