Readership Institute: Obituary Best Practices Gallery: Sophia Maria Litynski

Web version of Page 1 above-the-fold obituary, Milwaukee Journal-Sentinel, February 14, 2002

To touch her was to love her
9-month-old Sophia taught many about caring and cuddling and courage
By Amy Rabideau Silvers of the Journal Sentinel staff

A baby was born nine months ago with skin so fragile that she could be touched with only the most tender loving care.

The miracle was that she fought to live, soaking up all the gentle cuddling her mother and father and family could share.

She learned to smile, then laugh and coo, delighting in the attention of her big sister. And when her little body began losing the fight, everything possible was done to relieve the pain of her damaged skin and organs.

Sophia Maria Litynski died Saturday at her family's home in Greenfield. She was the daughter of Ernest and Jennifer Litynski, each 29, and the little sister of Amelia, 3 1/2. She died of a rare genetic disease, something called epidermolysis bullosa, specifically an even rarer and lethal form.

"What Sophia had was the worst of the worst," said her mother.

Arthur Dorrington, the family pediatrician, agreed.
"I've been in practice 26 years," he said. "This is something I'll never forget. It's the worst disease I've ever seen."

Sophia was born May 7, 2001, at St. Luke's Medical Center - a seemingly healthy 7 pounds, 13 ounces - but doctors quickly suspected that something was very wrong.

"The only clue was that her fingers and toes looked like they had sores," Dorrington said.

He suspected it was a disease so rare that he had never seen a case.

The family was quickly referred to Children's Hospital of Wisconsin. As soon as infant and mother were discharged, when Sophia was only 36 hours old, they went directly to their first appointment at Children's.

"It's a skin disease," Litynski said. "She was missing certain proteins in the skin. Her skin would break down; ...any trauma, pressure, friction could cause blisters, sores. Eighty-five percent of her body had open sores - similar to a burn victim but worse, because you can't heal."

Each year, only about five to 10 severe cases of the disease occur in the United States, with more cases of its mild or moderate forms, according to Beth Drolet, a pediatric dermatologist at Children's Hospital.

Fatal in infancy
Sophia was believed to have one of the most severe forms, typically fatal in infancy. Her family used extra care in picking her up, dressing and diapering her, bathing and drying her skin.

The care kept most symptoms at bay for the first months. Then the disease began to affect her internal organs, causing nutrition, blood and breathing problems.

By coincidence, and only coincidence, Sophia was the third baby in Wisconsin to die of the disease in the last year. Before that, the last case occurred at least five years before, Drolet said.

Epidermolysis bullosa occurs only when a mother and father are both genetic carriers. Experts estimate a one-in-a-million chance of two carriers marrying. Even then, there is a one-in-four chance that the disease will occur in any given pregnancy. The Litynskis, for example, had their healthy daughter before Sophia was born.
At the time of Sophia's death, genetic testing was already under way to determine where the defect occurs in her DNA. Research is continuing on the disorder.

"She was a fighter," her mother said. "She had touch and love. Mommy knew exactly how to hold her. She was cuddled and loved and hugged and kissed. She loved to be held... She was always in my arms or looking to see where I was."

She had, Litynski said, the most gorgeous eyes.

"You could see the world in Sophia's eyes," she said.

"We just did everything we could. Nine months is pretty long for this. She fought long and hard, and we fought as long and hard as she let us... She was loved so much, why would she give up?"

A team of specialists at Children's Hospital didn't want to give up, either, Dorrington said. They included: dermatologists Nancy Esterly and Drolet; Colin Rudolph for gastrointestinal medicine; Peter Havens for infectious diseases; Joe Kerschner, the ear, nose and throat specialist; J. Paul Scott, a blood specialist. Holly Colby was the special needs nurse and case manager. Christine Weitekamp was the home health care nurse for the family.

It finally became clear Sophia could not survive. Sophia's parents gave up dreams of their daughter somehow entering kindergarten, graduating from high school.

"Our emphasis shifted toward how can we give love to Sophia and dignity in death," Dorrington said. "This little baby touched literally hundreds of lives. She taught us all about love and how to give a baby dignity."

Bruce Himelstein joined the team for pain management, providing palliative care for Sophia. The little girl was eventually placed on morphine and methadone to control her pain.

"They really had a love for Sophia," her mother said. "She wasn't just a patient to them."

What Sophia saw
In her last month, Sophia sometimes watched a spot in her room, a place without toys or pictures or shadows. Litynski saw her daughter smile, something that she hadn't had the energy to do.

Once, she called her daughter's name. For the first time, her daughter didn't turn to her voice.

"I called her name again, and she kind of sighed and then turned to me," she said. "And she smiled at me like it's OK."

The mother believes she knows what Sophia was watching.
"Mrs. Litynski and I have no doubt," Dorrington said. "She was seeing angels."

On the day she died, Sophia's mother left her sleeping, returning less than an hour later.

"She died at home, in her crib, in the room where there was nothing but love," Jennifer said.

Dorrington came to the house, pronouncing his little patient dead.

"Mrs. Litynski was holding Sophia, and I held her for a while and kissed her goodbye," he said. Weitekamp, the home health nurse, came to the house, gently making clay impressions of Sophia's hands and feet for her family. Amelia could hold her little sister again.

Nothing could hurt Sophia anymore.

Visitation will be held at 10 a.m. today at St. Alphonsus Church, 6060 W. Loomis Road. The funeral will follow at 11 a.m. In lieu of flowers, the family will forward any donations for research on epidermolysis bullosa.