Philip's Story

Philip Michael, our second son was born July 23rd, 2013 at 1:40pm weighing 10lbs 5oz. and 22.5 inches long. We were so excited to meet him and add him to our family. After an uneventful pregnancy it was no surprise we were discharged from the hospital after two days with no issues.
His eyes seemed a little yellow to me but was reassured his bilirubin numbers were fine and that all newborns were a little jaundice and it would clear in time.

We started to settle in and get used to being a family of four. Philip had some feeding issues in the beginning and after many changes of clothes, a trip to the ER and many formulas later, he was finally gaining weight and doing well.
Yet his eyes still looked yellow to me. I brought this up to his pediatrician at our 2 week and 4 weeks appointments but was reassured he was fine. I even called the office at 5 weeks because his eyes were still yellow and I was starting to get concerned. I received a call back from the office and was still reassured that babies can be jaundiced for two months and I didn't need to bring him in, I could wait for our 6 weeks appointment.
Friday, September 6th came and we went to the pediatrician. I mentioned that his eyes still seemed yellow to me and the doctor finally conceded and re tested his bilirubin.
Dan called me later that afternoon and said the doctor called him and to head over to the emergency room, that Philip's bilirubin was high and we should get some blood work done. So off I went to the ER. They did some blood work and it turned out that his Direct Bilirubin was high. That day I learned there are two types of bilirubin:

• Indirect (or unconjugated) bilirubin. This form of bilirubin does not dissolve in water (it is insoluble). Indirect bilirubin travels through the bloodstream to the liver, where it is changed into a soluble form (direct or conjugated).
• Direct (or conjugated) bilirubin. Direct bilirubin dissolves in water (it is soluble) and is made by the liver from indirect bilirubin. Source: Web MD

In simplest terms, elevated Indirect causes your usual newborn jaundice where they put the baby under the lights. Direct bilirubin is related to your liver and elevated number may signal a problem with ones liver.

So then they did an ultrasound to see if any thing else stood out. It showed a contracted gallbladder but he had just fed and that can cause the gallbladder to contract. They also noticed his poop was pale and grayish. I thought the change was due to changes in formulas so it never alarmed me. Turns out it is not a good thing.

After a while the doctor came back and told us she spoke with the GI doctor to see what the cause could be and was told it could be a thyroid problem, trouble with enzymes, a cyst in the bile duct OR even worse ..this is when I heard it for the first time...BILIARY ATRESIA. Google, google, google....and I wanted to throw up.

I was told to NOT google but that is hard to do when you have no clue what is wrong with your baby and the name of the worst possibility sticks out in your head.

What is biliary atresia?

Biliary atresia is a life-threatening condition in infants in which the bile ducts inside or outside the liver do not have normal openings.

Bile ducts in the liver, also called hepatic ducts, are tubes that carry bile from the liver to the gallbladder for storage and to the small intestine for use in digestion. Bile is a fluid made by the liver that serves two main functions: carrying toxins and waste products out of the body and helping the body digest fats and absorb the fat-soluble vitamins A, D, E, and K.

Normal liver and biliary system

With biliary atresia, bile becomes trapped, builds up, and damages the liver. The damage leads to scarring, loss of liver tissue, and cirrhosis. Cirrhosis is a chronic, or long lasting, liver condition caused by scar tissue and cell damage that makes it hard for the liver to remove toxins from the blood. These toxins build up in the blood and the liver slowly deteriorates and malfunctions. Without treatment, the liver eventually fails and the infant needs a liver transplant to stay alive.

The two types of biliary atresia are fetal and perinatal. Fetal biliary atresia appears while the baby is in the womb. Perinatal biliary atresia is much more common and does not become evident until 2 to 4 weeks after birth. Some infants, particularly those with the fetal form, also have birth defects in the heart, spleen, or intestines. SOURCE: http://digestive.niddk.nih.gov/ddiseases/pubs/atresia/

So we were sent home with an appointment to see a pediatric GI in a couple of days.

The GI doc called Monday the 9th and based on the blood work form the ER our next step would be to have a liver biopsy. WHAT??!! His vitamin K levels were low so she wanted to start him on vitamin k pills before the biopsy. Vitamin K helps our blood clot and when there may be a blockage in the liver, it hinders our bodies ability to absorb certain vitamins. SO we met the GI Tuesday and they mentioned this BILIARY ATRESIA again.

They seemed to think this is what Philip has. They actually said this is most likely what Philip has. He had some more blood work done and his coagulation numbers did not improve enough so we had to delay the biopsy one day and get him a shot of vitamin K.

September 12th, 2013

was biopsy day and he could not eat for 6 hours before the biopsy. Philip was a champ and slept most of the time and did not fuss much. They put him under light sedation for it and they were finished within 20 minutes.

He then had to wait another 4 hours to eat because they had to re check his blood for sign os bleeding.

He did great again but started to get fussy the last hour or so. He was kept overnight for observation.

September 13th, 2013

We heard from the doctor and the biopsy results came back...consistent with BILIARY ATRESIA.

Next step was to meet with a surgeon:

How is biliary atresia treated?

Biliary atresia is treated with surgery, called the Kasai procedure, or a liver transplant.

Kasai Procedure

The Kasai procedure, named after the surgeon who invented the operation, is usually the first treatment for biliary atresia. During a Kasai procedure, the pediatric surgeon removes the infant’s damaged bile ducts and brings up a loop of intestine to replace them. As a result, bile flows straight to the small intestine.

While this operation doesn’t cure biliary atresia, it can restore bile flow and correct many problems caused by biliary atresia. Without surgery, infants with biliary atresia are unlikely to live past age 2. This procedure is most effective in infants younger than 3 months old, because they usually haven’t yet developed permanent liver damage. Some infants with biliary atresia who undergo a successful Kasai procedure regain good health and no longer have jaundice or major liver problems.

The Kasai procedure

If the Kasai procedure is not successful, infants usually need a liver transplant within 1 to 2 years. Even after a successful surgery, most infants with biliary atresia slowly develop cirrhosis over the years and require a liver transplant by adulthood. Source: http://digestive.niddk.nih.gov/ddiseases/pubs/atresia/

September 16,2013

Today we met with the surgeon and scheduled the surgery for Thursday the 19th. Philip will be 8 weeks 2 days old. He will insert a camera first to confirm the suspicion of Biliary Atresia. There is a tiny chance it is not but most likely they will confirm it is and continue with the Kasai procedure as explained above.

There are so many mixed feelings getting a diagnosis for your child like this. Most are indescribable.

But the surgeon spoke today of a lot of feelings of having to mourn for the life you expected for your child and yourself and that struck a chord with me. It is so true on so many levels.

Our lives will forever be changed with a diagnosis like this and we will face many challenges over the days, months and years to come. We are so blessed though to have the family and friends we do who have shown insurmountable support, love, concern and caring over the last week. We thank you all from the bottom of our hearts.