UCLA doctors operate, cure rare illness in Filipino youth

Monday, August 17, 1998

UCLA doctors operate, cure rare illness in Filipino youth

MIRACLE: Overwhelming odds overcome in quest to save child from
muscle-afflicting disease

By Laura Luu

Daily Bruin Contributor

When doctors in the Philippines told Menchi Estares that nothing
more could be done for her son’s life-threatening condition, she
didn’t listen.

With the help of a nurse at the hospital where her son spent
nearly half of his life, Menchi conducted her own search for a cure
on the World Wide Web.

Her son, Paul Anthony Estares, was born with Prune Belly
Syndrome, a rare condition where those afflicted lack stomach
muscles and suffer from severe urinary tract dysfunction.

Paul, who recently turned one, suffered from serious
constipation, breathing difficulty and damaging psychological
ramifications due to the abnormal appearance of his stomach
area.

Despite the doctors’ lack of faith, Menchi was determined to
find help for her son.

She soon found the home page of the National Organization for
Rare Disorders (NORD) and learned about Dr. Richard Ehrlich and Dr.
Malcolm A. Lesavoy, who pioneered a revolutionary method for
dealing with Prune Belly Syndrome in 1994.

"When I saw Dr. Ehrlich’s name, I felt something – hope," Menchi
said.

She soon wrote a letter to Dr. Richard Ehrlich at the UCLA
Children’s Hospital, asking for his help.

"I asked him if he could save my son’s life," she said.

Not only did Dr. Ehrlich agree to help Paul, but he also
enlisted the support of a generous donor.

This mysterious donor, a close friend of Dr. Ehrlich’s and a
past patient, has chosen to remain anonymous.

Having donated $50,000 to cover hospital fees and travel
expenses for both Menchi and her child, this donor’s contribution
cannot be ignored.

"I want to meet him," said Menchi of the donor.

"There is no question in my mind he would have died," without
the surgery, Ehrlich said.

Ehrlic, along with a team of urologists and plastic surgeons,
performed a three-part, 10-hour-long operation reconstructing
Estares’ abdominal wall, his urinary tract and bringing down the
testes.

"It was an honor to work with (the child)," said Lesavoy, the
plastic surgeon on the team.

After the operation, "(Paul) cried when he saw me," Menchi
said.

Since this method was formulated in 1994, 35 to 40 patients have
undergone the surgery and all have survived.

"If his problem was not fixed, it would have led to his demise,"
Lesavoy said.

In most cases of the syndrome, the patient is born with a
congenital problem, marked by distinct urological problems, such as
a dilated kidney and bladder. In addition, the testicles are not
fully descended and the abdominal wall lacks the proper
muscles.

Since there are no muscles to hold the shape of the stomach, the
belly protrudes and wrinkles abnormally, causing a prune-like
appearance.

The most damaging ramification of this abnormal physical
appearance is the psychological damage afflicted on the patient,
according to Ehrlich and Lesavoy.

Most patients who suffer from this condition refuse to leave
their homes or associate with others due to their appearance.

"After the procedure, the patient can function normally,"
Lesavoy said.

"We are very fortunate that the mother was able to find us on
the Internet."

Paul is expected to fully recover and is scheduled to leave UCLA
Children’s Hospital in the next week, after which he will return to
the Philippines in less than two weeks.

"We never gave up hope," Menchi said.