Sophia (Phia) is our 7 month old daughter that has been diagnosed with a condition called Biliary Atresia, also known as "extrahepatic ductopenia" or "progressive obliterative cholangiopathy”.

 

Her condition is a congenital or acquired disease of the liver. As a birth defect in newborn infants, it has an occurrence of 1/10,000 to 1/15,000 cases in live births in the United States. In the congenital form, the common bile duct between the liver and the small intestine is blocked or absent.

 

On June 19th, 2013, Sophia’s medical journey began. It was on this day that we discovered that Sophia's bilirubin was elevated. Her liver enzymes were evaluated, and something just wasn’t right.

 

On Friday, June 21st, We took Sophia to meet with a gastrointestinal expert in Green Bay. Our hope was that Sophia’s condition could be addressed with a simple medication. We had no idea what we were about to find out.

 

We were instructed to go directly to Childresn's Hospital of Milwaukee. We rushed home, packed a few things and loaded everyone into our van and drove as quickly (and somewhat legally) as we could carrimg our fear and unknowning to Children’s Hospital of Milwaukee. By 4pm, Sophia was admitted to the hospital, blood-work and a liver ultrasound were underway.

 

June 22nd brought a flurry of testing for baby Sophia, including a liver biopsy, CT scan, echocardiogram, cholangiogram, and multiple x-rays. The tests prompted doctors to discharge Sophia with a plan for her to return on Monday June 24th for an open cholangiogram surgical procedure. 
 

Baby Sophia’s June 25th procedure confirmed our fear: Sophia had Biliary Atresia. Her bile ducts were nonexistent. A 10 hour surgery was performed in an effort to correct this. It carried only a 10% chance of success. Sophia spent a week at Children’s Hospital recovering from the surgery, and all seemed well when we returned home. Unfortunately, on the morning of July 2nd, Sophia began vomiting and was taken to the ER. After undergoing testing, she was transported via ambulance back to Children's Hospital of Milwaukee. 
When we arrived at Children’s Hospital behind the racing ambulance, their surgeon met them in ER only to inform them that Sophia’s previous surgery had partially come undone. Sophia would need emergency surgery immediately. After 4 hours in the Operating Room, Sophia was brought to ICU where she spent 3 days.

 

She developed complications that included high blood pressure and asides (fluid build-up in the belly). 700cc's of fluid were drained from her small belly.

Sophia was finally discharged on the 12th of July, 2013. We were informed that she would need a liver transplant. Sophia and our family will continue to travel a difficult road, but with the grace of God and the support of family and friends, we are destined to prevail. Through this entire ordeal, baby Phia has continued to smile, and we are determined to do the same.  Continue reading...