P.F.I.C. - Bethany's Liver

Progressive Familial Intrahepatic Cholestasis - a rare genetic liver condition affecting our daughter, Bethany

Above: Bethany at 11 months old and very ill, then two hours after her liver transplant in January 2009. The other pictures show Bethany's glowing health and energy later in 2009 - miracles do happen!

Our daughter, Bethany, was born on 29th March 2003. It was soon apparent that all was not well. Extensive tests at St James's Hospital ("Jimmy's") in Leeds concluded that she had PFIC2.

This is a recessive genetic condition. Lack of liver function from birth leads to a failure to thrive. Symptoms in the first weeks of life include swollen abdomen, jaundice and lack of appetite. The condition is fatal. A liver transplant is the only solution. At present there are less than 30 cases in the United Kingdom, although it is more prevalent in Canada and North America. Death normally occurs in mid-teens.

Bethany spent most of the first year of her life in hospital, during which the situation was severely complicated twice - when she caught a "Rota Virus" on the ward, and later when a Hickman line into the main artery leading to her heart became infected, when "sterile field" protocol was not followed. She also developed rickets due to a severe calcium deficiency.

Her condition was eventually stabilised. Against the expectations of the medical staff, Bethany no longer needed artificial feeding through a naso-gastric tube, and took her medicines orally. This enabled us to dispense with the tube, and the painful monthly ritual of changing it. Although taking 15 doses of medication per day, and two injections every three weeks, Bethany managed to attend school full-time until November 2008 and went to dancing classes, where she has won several prizes. In October 2008 the decision was taken to place her on the waiting list for a donor liver.

After two "false starts" in December 2008, a donor liver became available and Bethany received her transplant on 16th January 2009. Within ten days, she was being reprimanded by nurses for riding a tricycle too fast on the ward, and only 20 days after the transplant she was home, building a snowman in the garden! By May she was back at school full-time and crammed so much adventure into the long, hot summer.

A minor setback occurred in October when a tummy bug laid her low for a few weeks and she was hospitalised for a few days, but this sort of thing is to be expected. She was back in business in time to do the round of Christmas parties.

The skill and dedication of the surgical and nursing team, and all who work in the Paediatric Liver Unit at St James's Hospital in Leeds never cease to astound us. Messages of encouragement and many gifts have come in from around the world, and for this we are eternally grateful. But nothing can match the debt we owe to the young man whose liver saved Bethany and, of course, to his family.

Fortunately our youngest daughter, Eloise, shows no sign of the condition. Bethany's mum, Samantha, keeps the world up to date with her progress on her "Caringbridge" website.

Now, if you haven't already done so, please visit www.uktransplant.org.uk and sign up to save more lives like Bethany's. If you have any doubts, read the TRANSPLANTS page of this website.

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