When Mr Lau Chin Kwee was diagnosed with Familial Amyloid Polyneuropathy (FAP) in 2005, it might as well have been a death sentence. The rare genetic condition, which disrupts the functions of the nervous system, heart, gut and kidney through a mutant protein produced in the liver, has no known cure - and the only solution was to replace the liver.

In September 2006, the 58-year-old retired pastor was referred to SGH’s Liver Transplant Service for a possible liver transplant. His heart, found to have been affected by the disease, would also need to be replaced. In November 2008, Mr Lau was put on the waiting list for a combined heart and liver transplant.

The surgery had posed a dilemma for doctors: Singapore doctors had never performed the complicated procedure. As of 30 June 2008, only 17 heart and liver transplants had been successfully performed worldwide on patients with Mr Lau’s condition - and the mortality rate for such procedures was estimated at 30%.

“While it is usually not advisable to perform transplantation surgeries where the mortality rate is above 5%, we kept in mind that without the operation, it would have been certain death for him,” said Associate Professor Tan Chee Kiat, Director of SGH’s Liver Transplant Service and Senior Consultant of SGH’s Department of Gastroenerology & Hapatology.

Doctors also worried if Mr Lau, extremely malnourished and weakened by prolonged diarrhoea after the abnormal protein began to affect his gut, would be able to cope with the stress of a double organ transplant.

Sequence of procedures crucial

“Surgically, there were not too many technical challenges because both the heart and liver teams performed the same procedures as they would in a usual heart or liver transplant,” said Dr Tan Yu Meng, Surgical Director of the Liver Transplant Programme and Deputy Head & Senior Consultant Of National Cancer Centre Singapore (NCCS)’s Department of Surgical Oncology.

“The main challenge was in getting the sequence right while doing a technically perfect surgical operation.” The complex surgery – involving the donor, heart and liver transplant teams – would have to work like clockwork with the handover between each team seamless to make sure the recovery of the transplanted organs wasn't compromised.

The teams wouldn’t know when the surgery would be scheduled – it would depend on when a donor became available. Thus, well before that happened, the teams had to ready a plan with detailed steps for the transplant procedures, and the sequence of these steps. The transplant teams brainstormed about the problems that could arise, and formulated backup plans in the event they do surface.

It was also crucial for the support teams to know their role and responsibilities. The coordinators, anaesthetists, operating room staff, ICU (intensive care unit) staff, pharmacists, physiotherapists, medical social workers – all needed to know how and when they should work with others if the surgery was to be performed with as few hitches as possible.

“It was like conducting an orchestra,” said Dr C Sivathasan, Co-Director of NHC's Heart & Lung Transplant Programme, who oversaw the preparation and coordination between the two teams.

“As it was a long surgery, there was no room for error. Every person had to know exactly which key to press and what tune to play.”

Race against time

It would also be a race against time for the surgeons involved in the combined transplant.

Both the organs would have to be recovered and transplanted into the recipient as quickly as possible to avoid complications setting in. The heart would have to be transplanted first as the organ had to be pumping blood in the recipient’s body within four hours after it was recovered to keep the risks of complications low. In comparison, liver transplant surgeons have a longer time to work with – 12 hours. But to save time, the liver team would first mobilise the patient’s liver before the donor heart arrived.

“Within a matter of hours, we had to get the newly transplanted heart to work very hard for the second stage of the surgery. In fact, we had a standby recipient for the liver in case the donor heart failed,” said Associate Professor Tan.

In other words, the heart had to hit the ground running. Fortunately, the donated heart was in good condition, and to check that the organ was functioning well and able to sustain a proper heart rate, “we allowed the heart to gradually receive the circulatory volume of blood,” said Associate Professor Hwang Nian Chih, Head of NHC’s Department of Cardiothoracic Anaesthesia.

Once it was established that the heart was functioning and in a stable condition, liver transplantation would proceed as planned. To partially relieve the newly transplanted heart’s work of circulating blood and oxygen throughout the body during the liver transplantation, the patient would be kept on a cardiopulmonary bypass machine (CPB).

“We were pleasantly surprised that everything went smoothly,” said Dr Lai Fook Onn, Senior Consultant at SGH’s Department of Anaesthesiology.

“The greatest risk came from the unexpected. Many things could have gone wrong, as heart and liver transplants in themselves are already major operations where many uncertainties abound - what more a joint transplant. In a worst case scenario, the heart or the liver could have malfunctioned in the recipient’s body, or uncontrollable bleeding could have occurred during the surgery.” Dr Lai said

Two months after (around the time this paper went to print), Mr Lau’s recovery continued to progress well, with both “his heart and liver functioning well,” said Associate Professor Tan.

Still, Mr Lau will remain on lifelong medication to prevent complications arising from organ rejection, among other kinds of heart medication. Additionally, he will have to be continuously treated for other symptoms because while the transplantation was able to halt the progression of the disease, it will not be able to remove the mutant protein already deposited in other parts of his body.

What is undisputed, however, is that through a combination of strategic planning, cohesive teamwork, generosity of strangers and a stroke of luck, Mr Lau is now able to enjoy a new lease of life.

“While every transplant we undertake is special to us, this one is especially memorable given the scope and the odds. After all, it is challenges like these that keep us in the field,” said Dr Tan.