Family hoping for gifts for girl with rare disease
December 7, 2013 11:33 PM
The Acosta family, Sandra and Roger with their children, back from left, Brayan and John, front, from left, Kendra and Kianny.
By Ed Blazina / Pittsburgh Post-Gazette
Kendra Acosta's happy disposition belies the life-threatening physical struggles she has faced since the day she was born in Puerto Rico more than three years ago.
Since then, she's had 25 blood transfusions, 21 surgeries and countless hospitalizations and doctors' visits as she deals with an intestinal disorder known as megacystis microcolon intestinal hypoperistalsis syndrome. That condition is so rare there have been fewer than 300 cases worldwide since it was identified in 1976.
In March 2011, parents Sandra and Roger Acosta moved to Pittsburgh with their three other children so Kendra could be treated at Children's Hospital of Pittsburgh of UPMC. She's been at the top of the waiting list for 2 1/2 years for a rare colon and small bowel transplant, but doctors haven't found the perfect match yet.
Put in simplest terms, Kendra's digestive system doesn't work and her bladder doesn't empty, requiring her to be on a catheter four times a day. She's also on a delicately balanced nutritional program that involves intravenous feeding more than 20 hours a day with the supplement Total Parental Nutrition to maintain her health while bypassing her digestive system.
Kendra's medical needs -- combined with adjusting to life in a new environment thousands of miles from home -- have put a major strain on the family's resources, leaving little extra for gifts during the holiday season.
That's where the Pittsburgh Post-Gazette's Goodfellows Fund and the Marine Corps Toys for Tots program can help. Through generous donations by readers of the Post-Gazette, the program provides toys for children who otherwise might not have any.
Life changed for the Acostas in September 2010.
"Kendra was born and the next day she was in surgery," said Mr. Acosta. "The doctors told us she would be dead before she was 7 months old."
The reason: Kendra wasn't able to process any food for nutrition. Kendra's diagnosis set Mr. Acosta on a frantic Internet search to learn about the condition and find the best place to go for treatment. That led to Children's, which has experience in her specific syndrome and has performed more than 200 of the tricky intestinal transplants.
But getting to Pittsburgh wasn't easy.
As Mr. Acosta was saying goodbye to co-workers on the day before they were ready to board a medical ambulance, he got a phone call telling him insurance would cover only 80 percent of the cost. That meant he needed $6,000 immediately or the flight would be canceled.
Co-workers who overheard the conversation passed the hat. By the time their family, church and neighbors pitched in, they had $7,000.
When they arrived here, Kendra looked like a mummy, wrapped in bandages to control an accompanying skin problem that made it look as if she had been in a fire. As part of her initial evaluation at Children's, doctors flushed her system with a special solution. In an unexpected benefit, her skin cleared within four days.
"That was the sign -- this was the place we were supposed to be," Mr. Acosta said. "God is giving us the opportunity to get help for Kendra."
Since then, the whirlwind has continued almost nonstop with a series of hospitalizations, surgeries and doctors' visits over the next two years as doctors try to stabilize Kendra and search for a transplant match.
The couple initially came here only with Kendra, but sons Brayan, now 17, and John, 16, soon joined them. A new baby Kianny, who will be 2 in January, also joined the family.
Along the way, the family spent three months at Ronald McDonald House and lived for a time in Crafton. That proved to be too far from the hospital, so they moved to Highland Park in February.
Keeping Kendra stable has been particularly difficult because she is at extreme risk for infection at sites for her feeding tube and a stoma, as well as regular use of a catheter to empty her bladder. That means keeping nearly sterile conditions in her bedroom.
Traveling has been particularly difficult. The Acostas have family in New York City, but Kendra and her parents spent most of their five days there in a hospital after Kendra developed a fever.
Through Children's, they became involved with Sandra Lojek of Colombia en Pittsburgh, who Mr. Acosta described as "an angel" for helping the family with housing, diapers and other needs. Mr. Acosta lived in Colombia for a time and the organization of about 300 has regular get-togethers, but the family rarely can attend.
"It's not easy to congregate. We are almost always here alone," Mr. Acosta said.
Things have gone well for the past three months with no hospitalizations. Kendra goes to medical day care most weekdays, but there is so much to do at home that Mr. Acosta, 36, works from there as a customer care representative for PNC. Mrs. Acosta, 34, is learning English through the Allegheny Intermediate Unit.
Dr. Jeffrey A. Rudolph, medical director of intestinal care and rehabilitation at Children's, said the ability of children in Kendra's situation to survive has improved substantially over the years. Before the nutritional supplement was available, they almost always died of dehydration in a few months.
That improved to about 25 percent survival in the past 10 to 15 years, and a better balance of nutrition has now extended survival indefinitely, he said. The right nutritional balance is needed to keep the liver healthy.
"Our hope is we can manage Kendra indefinitely," he said. "The fact her liver is stabilized is a very good thing. It's really amazing how resilient these children with chronic problems are. She's really fairly well developed given her condition.
"But it's always a hairy situation until we can do the transplant."
A transplant is complicated because the donor must be healthy, almost the same size and the same blood type as Kendra. It is rare for healthy children to die that young, Dr. Rudolph said.
Another complication is that Kendra is slowly learning how to eat even though food passes directly out of her system now without being processed. Eating is a learned experience she hasn't developed yet because of her syndrome.
Even if she receives a transplant, Kendra will need the catheter the rest of her life because the transplant won't address her bladder issue.
Mr. Acosta said the waiting is wearing, but Kendra's recent stability has helped a lot. If she does get a transplant, Kendra faces 18 hours of surgery, followed by a month in a medically induced coma and three months in intensive care while she recovers.
"The risks are enormous," Mr. Acosta said. "It's not going to be easy for us, for Kendra."
In the meantime, Kendra acts like a 3-year-old -- she loves "Dora the Explorer" and its spinoff "Go, Diego, Go," and plays constantly with an iPad. She is bilingual and often begins a sentence in English and finishes in Spanish.
"She's strong, smart. Even when she's in ICU, she's happy, always playing," Mr. Acosta said. "Every day, she says, 'Sorry, Papa; sorry Mama, that I am sick.' "
Donations to the Goodfellows Fund can be made using the coupon that accompanies this story or online at www.post-gazette.com/goodfellows. Donations are tax deductible and will be acknowledged in the newspaper.
How to get toys
The Toys for Tots program will hold open houses this week to distribute toys to parents and guardians of needy children.
They will take place from 9 a.m. to 2 p.m. Friday and Saturday at Guardian Storage Solutions, 2839 Liberty Ave., Strip District.
Parents and guardians are required to bring photo ID for themselves, a birth certificate for each child (children up to age 12 are eligible) and proof of need: an EBT card, Access card or SNAP card.
Today's total: $11,863.57
Grand total: $11,863.57
Robert H. Howland $5,000
Dawn and Richard Moody $1,000
Scott and Leslie Baker $500
William Zumach and Judith Stagg $500
Winston Brodsky Knowles $500
Mary and Keith Reabe $500
John and Britt Vries $250
Bob Jennings and Barbara H. Bott $200
Bradley and Leeann Golish $150
In honor of Marjorie E. Rogers $120
In honor of all of our veterans and their families $100
MaryAnn and Tony Fasciani $100
Ed and Trish $100
Dennis and Jamie Scurletis $100
Dr. and Mrs. Robert Fusco $100
John F. Kraft III $100
K.C. Pfeiffer $100
Bob Breidigan $100
Betty G. Rowland $100
Paul Lane Jr. $100
John and Eileen Olmsted $100
Carol Truschel $75
In memory of Clyde Finney $60
In memory of Margaret Gunter $60
Dennis Hockenberry $50
In memory of Ida Fantino $50
Mary Gwen Phillips $50
Debra Flinner $50
Beatrice L. Zacur $50
Allan and Pat Bobko $50
Miriam Lucarelli, in memory of Edward and Helen Hee $50
Gable-Krieg family $50
Henry W. Jones $50
Fitness Techniques $50
Bill and Marsha Sorba $50
Natalie and Jenna Mangene $40
Robert Gold $30
In honor of Den and Nancy Wagner $27
Jim Leindecker $26
In memory of Grace Bussler $25
In honor of Pudge Cunningham $25
Don Foehringer $25
Leslie L. O'Connor $25
In memory of August C. (Gus) Mann $25
In memory of Salvatore Cerniglia $25
Ronald Mazer $25
In memory of Eileen Arnold Swan and Guzzi. May their memory live on. Tom $25
Tony Costanzo $25
In memory of Jack "Merry Christmas" Walsh $25
Ron and Louise Countryman "Happy Holidays" $25
Louis Stabile $25
In memory of our son Russell, Ray and Arleen Gadomski and family $25
Janet Soltis $25
In honor of Annabelle and Reid Junker $25
In memory of "TC" Thomas Cochran by the Keith Kampert family $25
In memory of Johnathan Cochran by the Keith Kampert family $25
In memory of Richard Cochran by the Keith Kampert family $25
Mr. and Mrs. S.T. Johnson $20
James A. Greenen $20
John Burriss $20
In memory of my late husband Charles Zagan $20
Gerald Janusz $20
In memory of Grandson Michael Wagner, D. Wagner $20
Jon and Joann Strebeck $20
Dan Strebeck $20
Matt, Stefany and Stevalyn $20
Hunter Lynn Gray $20
Anna Marie Mostyn $19.57
Patricia Jacobs $10
Ronald K. Wollett $10
Jennifer Rago $10
Sandra B. Swegman $10
Rose M. Halaja $5
Maryam Bopp $1
Ed Blazina: firstname.lastname@example.org or 412-263-1470.
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