The Medical City performs 3rd liver transplant in girl with rare condition

MANILA, Philippines - The Medical City recently performed its third liver transplant in a pediatric patient, five-year-old Marilda Agcaoile Guzman, who suffered from end-stage liver disease and a rare congenital condition called Situs Inversus Totalis (SIT).

Persons with SIT are said to have all their internal organs “flipped” to the opposite side of the body, like the heart is found in the right side of the chest instead of the left, or the stomach, normally in the upper left side of the abdomen, is found in the right side while the liver is in the left of the abdomen instead of the normal position in the right.

Because of the unusual position of the organs, organ transplantation in patients with SIT becomes a more complicated procedure which makes Marilda only one of a few SIT patients worldwide to have undergone a successful liver transplant.

Two operations took place simultaneously. Marilda’s diseased liver was removed, after which she received a graft taken from the liver of her father, Mario, 42, who was her donor. She was operated on in February 2013 and was discharged a month later.

Credit goes to The Medical City’s liver transplant team, led by Dr. Vanessa de Villa, head of the Center for Liver Disease Management and Transplantation (CLDMT), which undertook the delicate 21-hour surgery and gave Marilda a new lease on life.

De Villa said Marilda’s liver transplant was a highly complex procedure because of her SIT and her donor father’s complex vascular anatomy. In the past, this would have been considered as a contraindication, but through careful pre-transplant preparation and surgical planning with her liver transplant team, De Villa was confident in performing Marilda’s life-saving procedure despite the complexity of her anatomy.

“Once again, the TMC team of liver transplant experts, surgeons, nurses, and critical care specialists saved another Filipino life. They have shown what Filipinos can do for fellow Filipinos — in the Philippines — when given the opportunity to do the right thing… in an exemplary manner,” said Dr. Eugenio Jose Ramos, senior vice president of TMC Medical Services Group.

The Medical City’s liver transplant team, led by Dr. Vanessa de Villa, head of the Center for Liver Disease Management and Transplantation, undertook the delicate 21-hour surgery and gave Marilda a new lease on life.

Marilda was born in a hospital in Riyadh, Saudi Arabia on Feb. 24, 2008. Her parents, Imelda and Mario, both from Dagupan, Pangasinan, are overseas Filipino workers. The child was noted to have jaundice soon after birth and was subjected to phototherapy. At two months, Marilda’s jaundice persisted so an abdominal ultrasound was done which indicated her SIT condition.

A liver biopsy done when she was eight months old revealed cirrhosis or end-stage liver disease secondary to “sclerosing cholangitis,” which refers to inflammation, destruction and scarring of the bile ducts inside and outside of the liver. This leads to cirrhosis of the liver which is fatal unless liver transplant is performed.

According to Dr. Karen Calixto-Mercado, a pediatric gastroenterologist, a liver transplant became imminent for Marilda in August 2012 when she started to have variceal bleeding from dilated blood vessels around the esophagus and stomach due to cirrhosis.

However, the high cost of hospitalization and treatment in Riyadh forced Marilda’s parents to bring her to the Philippines for her liver transplant. The Guzman family went first to a government hospital whose doctors, in turn, referred them to TMC. “They told us if our child needed a liver transplant, we had to go to The Medical City,” said Imelda Guzman.

At TMC CLDMT, Marilda and her father was seen by De Villa and Calixto-Mercado on the first week of September 2012, and then subjected both to various tests to prepare them for the liver transplant.

But she again vomited blood in October 2012 which led to her admission at the TMC Pediatric Intensive Care Unit (PICU). “Her bleeding was controlled but her liver function was deteriorating. She underwent catch-up vaccinations, medical clearance and supportive treatment of decompensated liver disease to prepare for her liver transplant,” said Calixto-Mercado.

The operation was originally scheduled in December last year but was rescheduled when Marilda developed bacterial sepsis. The operation finally pushed through last Feb. 15, 10 days before Marilda’s fifth birthday. Marilda’s parents said they just had a small cake for their daughter’s birthday but still, it was the happiest.

 â€œMarilda’s successful liver transplant was the best birthday gift for her,” said Imelda, who was also thankful for her husband’s quick recovery. De Villa said that although Marilda’s condition is stable and her “new” liver is functioning well, she still needs to be observed and monitored regularly by her doctors.

But after the procedure, Marilda developed two life-threatening conditions — obscure intestinal bleeding and hepatic artery thrombosis. Fortunately, both complications were managed without the need for a second operation.

It was in January 2011 when TMC performed the first successful pediatric liver transplant in the country in three-year-old Catherine Erica Buenaventura. In July 2012, TMC performed its second liver transplant on Amytis Batao, a doctor and former mayor from Cebu that gave her a “second” life.

Erica learned to walk at four years old after her liver transplant and was able to realize her dream of going to school. However, though her liver transplant was successful, Erica tragically succumbed to the dengue virus in September 2011.

On the other hand, TMC’s second liver transplant patient, Batao, is now back in Cebu, enjoying a normal and healthy life.

Aside from De Villa and Calixto-Mercado, Marilda’s and Mario’s core team of doctors included transplant hepatologist Dr. Janus Ong; pediatric intensivist Dr. Neva Batayola; pediatric cardiologist Dr. Dexter Cheng; infectious disease specialist Dr. Cynthia Aguirre; hepatobiliary surgeons Drs. Dante Ang, Anthony Yap, and Ramon de Vera; and microvascular surgeons Drs. Eric Arcilla, Glenn Genuino and Catherine Yap-Asedillo.

Drs. Grace Herbosa, Celine Ancheta, Alexandra Odi, Elena Malong, Elke Sauz, and Ace Prodigalidad completed the team as special anesthesiologists for both the donor and the recipient. Other specialists who helped manage the patient were interventional gastroenterologist Dr. Mark Anthony de Lusong, radiologist Dr. Rommel Galsim, interventional radiologist Dr. Ramon Santos-Ocampo, and rehabilitation specialist Dr. Celso Bate. This multi-disciplinary team of experts was ably supported by equally competent and compassionate nurses and counselors.

The Guzman family is grateful to their doctors and nurses for their dedication, to their family and friends who helped them raise funds for the transplant, to TMC for giving them generous discounts and to the Philippine Charity Sweepstakes Office (PCSO), through its board of directors, which donated a significant amount to defray the cost of the operation.

The CLDMT is a specialty unit that offers a comprehensive array of diagnostic and therapeutic options for patients with liver diseases and liver tumors through an integrated and highly skilled multidisciplinary, patient-partnership approach. It is otherwise known as The Medical City Liver Center and may be reached through 988-7000 local 6506 or 0932-88LIVER.