Volume 35 No. 2 "Spring 2012"

Feature

Baby Brenna
SIU providers come together to help a very special baby

Written by Rebecca Budde • Photography by James Hawker

On December 19, 2011, Courtney Westlake of Springfield called her husband, Evan, and told him, “It’s time!” Hurrying back to Springfield from a business trip in Indiana, he made it to the hospital with just over an hour to spare. Brenna Helen Marie Westlake’s easy, quick delivery at 36-and-a-half weeks seemed like an early Christmas gift for the young Westlake family. But something was wrong.

After delivery, Courtney saw baby Brenna laying on her chest for only fleeting moment before nurses took her away. “She looked shocking,” says Courtney. “Her eyes looked bright red because her eyelids were flipped out and her skin looked completely white. If we hadn’t heard her cry, we would’ve thought she was still-born.”

Brenna’s first photo, taken on an iPhone® by Ginger Darling, M.D., assistant professor, was sent to Daniel Batton, M.D., professor and chief of the neonatology division, for consultation. “Our immediate reaction was that this baby might not make it,” says Dharmendra Nimavat, M.D., assistant professor and neonatologist. “Fortunately, Brenna was breathing on her own, though she needed oxygen.” A team of SIU providers cared for Brenna at St. John’s Children’s Hospital (SJCH) for the next 38 days.

It was unlike anything the physicians had seen before. Brenna’s skin was so scaly, thick and tight that they enlisted the expertise of Joseph Conlon, M.D., assistant clinical professor of pediatric dermatology and SIU School of Medicine Class of 1999. As the only board certified pediatric dermatologist practicing in Springfield, Dr. Conlon often collaborates with SIU physicians.

A RARE DISEASE

Dr. Conlon diagnosed Brenna with Harlequin Ichthyosis (HI), a very rare and extremely serious skin disease characterized by thick scales with crevices between. The scales of skin on babies born with HI often present themselves in a diamond-like pattern, reminiscent of the Harlequin clown costumes from the Renaissance. HI is considered the most severe and rarest skin condition in a spectrum of related Ichthyosis skin conditions. According to Dr. Conlon, even the national expert on Ichthyosis skin conditions has treated only one patient with HI from the neonatal stage.

Dr. Nimavat examines Brenna during her "graduation" from the NICU.Until recently, babies with HI usually died within a couple of weeks of birth. According to Dr. Nimavat, the two major causes of death in infants with HI are sepsis and dehydration. Patients with HI lack a protein that transports fats to the surface layer of the skin to make a strong barrier. Without this protein, the skin loses water quickly, develops thick, dry scales and hinders the body from maintaining a consistent temperature. The tight scales cause the baby’s eyelids to be pulled inside-out. Ears are pulled close to the head, noses are turned upward and their lips are pulled taut. Cracks arise where normal skin would fold, leaving the area susceptible to bacterial infection.

Faced with an extraordinary condition, physicians worked quickly to keep her stable. “We have the best resources, and because we are a smaller institution, we have a personal, caring touch,” says Dr. Nimavat.

To help Brenna maintain consistent body temperature and remain hydrated, Dr. Conlon developed a detailed skin care regimen: maintaining temperature, humidity and oxygen level in her Isolette™. Humidity in Brenna’s incubator was so high that it was raining inside the chamber. Antibiotics prevented infection. Every few hours, nurses applied emollients to hydrate her skin and eye drops to protect her eyes. “Without these nurses’ commitment to Brenna’s care, I don’t think she would have made it through the early days,” says Dr. Nimavat.

On Christmas Eve, despite the tremendous care that Brenna was receiving, test results showed that she had developed an infection. Doctors had to make more critical decisions.

From the time of her birth, Brenna’s medications and fluids were fed to her through a central umbilical line. Subhash C. Chaudhary, M.D., professor of pediatric infectious diseases, knew that the E. coli was present in her central line and recommended that the line be removed. “Antibiotics wouldn’t help in this situation because they can’t penetrate plastic or foreign material if it is contaminated,” says Dr. Chaudhary. Though removing the central line might save Brenna from the infection, it would literally remove her lifeline of medication and hydration. Replacing the central line was an option but also very risky.

“If there is any tricky situation involved in the patient’s care, we don’t make an individual decision,” says Dr. Nimavat. “We sit down as a group, make the best judgment as a group and abide by it.” Despite being the only venous access, the team decided to remove the central line. Four days later, test results showed that Brenna had beaten the infection, and the team had made a tough choice that saved her life.

With confidence in the medical care Brenna was receiving, the couple was able to spend Christmas with their two-year- old son, Connor. “We were able to give Connor the carefree Christmas that every child deserves,” says Courtney.

BUSY BABY

Shortly after Christmas, Brenna’s skin and health began to show noticeable improvement. Her thick scales began to shed and reveal a smoother, flaky pink skin underneath. But as she continued to lose skin, her list of doctors grew. Brenna’s extensive care team grew to include not only Dr. Conlon and Dr. Nimavat, but many other SIU neonatologists, genetic counselors, occupational therapists, infectious disease physicians, otolaryngologists (ENT), plastic surgeons and feeding team specialists who all collaborated on the baby’s treatments and therapies.

Brenna’s tight skin limits her range of movement. Reuben Bueno, Jr., M.D., associate professor of plastic surgery, informed the family that Brenna did not need surgery at that point. He will monitor Brenna as she learns to move her rigid limbs to be sure that her unyielding skin isn’t causing wounds that affect her joints and muscles. SIU occupational therapists worked to increase the range of motion in Brenna’s hamstrings, ankles, feet and hands. As she grows and her skin continues to shed, Courtney and Evan will continue to help her stretch and strengthen the tight muscles and tendons. Left, Diedra Stewart,RN, weighs Brenna during her NICU "graduation". Right, The daily routine at home includes a long bath, as Evan and Courtney gently slough Brenna's skin and apply lotion every few hours.

Brenna will also have regular appointments with Sandra Ettema, M.D., assistant professor in otolaryngology-head and neck surgery. Though Brenna’s condition doesn’t affect the insides of her mouth and nose, it does affect the insides of her ears and the skin around her nose. According to Dr. Ettema, the constant build-up of skin in Brenna’s ear and pressure on her tympanic membrane can cause hearing damage and infection. Thick skin around Brenna’s nose could create breathing difficulties, irritating her throat and adenoids, which can lead to possible infections. Dr. Ettema estimates that Brenna will need appointments for ear cleanings approximately every six to eight weeks.

And the Westlake family’s relationship with Dr. Conlon has just begun. For the moment, Brenna is a good-natured baby, easy to slather with lotion as she lies on the changing table. As she grows, she will enter and exit rebellious stages as children do. “She’s going to be pretty slippery,” Dr. Conlon says with a bit of a laugh.
“I can see her running away from them, looking back and laughing.” As she grows, she will likely use different products and learn how and when to care for her skin on her own, a life-long commitment.

HEADING HOME

Brenna’s time in the NICU was crucial both for her and her parents. Courtney and Evan learned the best ways to care for their baby and keep her infection-free. “We refuse to keep her in a bubble,” says Courtney.

Although Brenna’s condition is rare, multi-disciplinary teamwork is the hallmark of care at SIU. With great medical care, this very special baby has proven to be a fighter.

“Everyone told us that the best care was right here with the SIU doctors,” says Evan. “We are so fortunate to have all these doctors to help us.”

As she grew stronger, the doctors and nurses began to make changes in Brenna’s environment to prepare her for the transition to home. The humidity in the Isolette™ was gradually lowered, and Brenna was moved to an open-air crib in a room with 35% humidity. Courtney and Evan participated in her feedings, therapy sessions and skin care regimen as often as they could. “Our time at the children’s hospital was imperative in learning how to care for Brenna,” says Courtney. “The medical staff was very helpful and encouraged us to take part in her care at the hospital, so that we felt comfortable with the extensive routine at home.”

On January 24, after 38 long days, doctors said the word Courtney and Evan had been yearning to hear: home. “The care we received from the SIU doctors was second to none,” says Evan. “The entire crew made the experience better than expected under less than ideal circumstances. Not only was the medical knowledge good, but the bedside manner of all the doctors put us at ease on a consistent basis.”

Since Brenna’s discharge from the NICU, she has been re-admitted to SJCH twice for medical treatment. At mid-February, the Westlakes met with neonatologist Magali Jean-Louis, M.D. While conversing about Brenna’s recent loss of appetite and inability to keep food down, Dr. Jean-Louis became concerned and readmitted Brenna to the NICU. “Evan and I are so incredibly thankful that we happened to meet Dr. Jean-Louis, so that Brenna could get the proper amount of fluids and nutrients and be under the care of the SIU medical staff,” says Courtney. Brenna returned home a few days later, just in time to celebrate her two-month birthday.

She was re-admitted in March for a staph infection, but under the care of Lynn Kink, M.D., assistant professor and hospitalist, Brenna went home with an antibiotic treatment.

“We want Brenna to live as normal and happy of a life as possible,” says Courtney. And with the arrival of an early spring, Brenna is doing well, growing strong and gaining weight. The Westlakes, including Connor, are enjoying time together as a family.

Brenna’s doctors are also optimistic about Brenna’s future. “Brenna’s going to be great,” says Dr. Conlon. “Maybe she’ll be a pediatric dermatologist someday; there’s no reason she can’t.”