Sheriff Sarah

Sheriff Sarah

Monday, March 18, 2013

This Fragile Life

I was reminded this past week about the fragility of life. We see this daily in the liver community - babies, children and even teenagers, battling for their lives when their liver fails or as they await a transplant. But this reminder hit even closer to home than liver disease.

A coach from our kids’ school passed away. He was my age or younger (early 40s). One week there was nothing wrong with him. Three weeks later after an infection, kidney dialysis and surgery, he was gone….leaving behind his wife of 17+ years…and his two high school-aged kids.
And the question you know is coming began popping up all over the place: why?

Why is someone so loved, so admired and so important to those around him taken so young and so suddenly. It’s impossible to provide a satisfactory answer to that question, especially for those most impacted by his loss.

Our lives will go on because it wasn’t our husband, father or brother that died. But for his family there will be an emptiness that will be difficult to even explain to others. No graduation celebration with his children. No more anniversaries with his wife. No more laughs with his friends.

This is an inconceivable pain to me. I have four children and an incredible wife. I can't begin to imagine leaving them - to never see my children grow up or get married or have their own children; to not be able to share countless priceless moments with my wife as we grow old together. 

For those of us who believe in Jesus, there is great comfort in the fact this man is now home with the Lord, pain free…forever. We thank God for that and we praise Him for a life well lived. It's not goodbye, just farewell for now. 

But the emptiness remains. And the bitter reminder that no matter how much we try to convince ourselves otherwise, this life is temporal. Truly, no man knows his time.

Live your life well. Every day counts. Every moment matters.

Monday, March 11, 2013

Pediatric Liver Disease Profile #1: Biliary Atresia

Throughout the coming months I'll be writing profiles of different pediatric liver diseases. This world used to be invisible to me before Sarah's diagnosis. I'm grateful to be part of a wonderful community of people who care for each other, advocate for their children and try to bring awareness to their own little parts of the world.

Up first, the disease that affected my family: biliary atresia.

Biliary atresia is a rare pediatric liver disease that affects infants in about every 10,000-20,000 births. Race can factor into this number, and females are generally affected more than males. 

The essence of biliary atresia is the liver's inability to excrete bile (the "garbage"). This is due to bile ducts being too small to be efficient or completely non-existent. Without the ability to rid itself of bile, the liver is poisoned, leading to a hardening of the organ. If not diagnosed within 8-12 weeks of age, serious complications can occur. The disease is already life-threatening to begin with but if diagnosis lags, the danger increases. 

Hardening of the liver (as in cirrhosis) can lead to impeded blood flow which causes varices, as the blood must find "alternate" routes through the body to the heart. These varices (essentially, internal varicose veins) first cause the spleen to increase to an abnormal size before before making a new path through the spleen to the esophagus and beyond. In the absence of immediate care, these varices can burst with serious ramifications. 

The first step in restoring the liver's ideal function is the Kasai surgery. The Kasai takes a piece of the small intestine and attaches it directly to the liver in order to allow bile drainage. Sometimes this is enough to restore the liver's health and no transplant is necessary. 

If the liver has been too badly damaged to continue for the long term, transplant is the only viable option. 

Standard symptoms include jaundice (body and eyes), distended belly, lack of appetite, white stool and lethargy. These may or may not ALL be present but if you have a question, you should request your pediatrician request blood tests. 

Links for Reference and Education:

National Digestive Diseases Information Clearinghouse

Cincinnati Children's Hospital

American Liver Foundation

American Pediatric Surgical Association

The Children's Hospital of Philadelphia