PFIC Progressive Familial Intrahepatic Cholestasis-Resource for Pediatric Liver Disease

The First PBED - By Shawn Roebuck

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My name is Shawn Roebuck and I am 29 yrs old. This is my story living with Progressive Familial Intrahepatic Cholestasis. I was born in February 26 1977 in a small town called Vernon Alabama. I have lived in Aberdeen Mississippi most of my life. Between the ages of  5 months to a year I had jaundice and severe itching. I don’t remember much, but from what my mother told me I used to itch a lot and dig at my skin. I dug so much they had to put cooking mitts on my hands but that did not help because some how I always got them off.

The doctors where I lived, around Tupelo Mississippi, thought it was just skin allergies but my mother did not believe that. She took me to LeBonheur Children's Hospital to see Dr Peter Whitington and his brother Dr Gene Whitington. They diagnosed me with what is now called PFIC. Without treatment they gave me to the age of 6 to live. They decided to try a new procedure that was called a cholecystostmy tube which drained some of the excess bile salt. It helped relieve some of the itching, but had to be drained a few times a day. I remember a few times when the drain came out and I had to be rushed at the local hospital in Amory Mississippi.

 

After having the drain for a bit and seeing that it was helping, Dr Peter Whitington and Dr Gene Whitington decided to do a new procedure called cutaneous biliary diversion. At the time I was only three years old and would be first patient to have this surgery done. The procedure is where they took a small piece of the small intestine and hooked it up where it drains the excess bile salt into a colostomy bag, which would need to be emptied at least 8 times a day.

 

After this procedure my itching stopped and I had no jaundice. It was night and day and we were very thankful.  I had followed up with visits with Dr Peter Whitington until he left for Chicago. Then Dr Gene Whitington took over my care. I remember many tests especially the biopsies, because I use to play bingo with my mother as I lay on my side.

 

I had as much of a normal life after the last surgery as possible, I even played little league baseball. I went to school up to ninth grade. I quit school for un-related reasons and got my GED. The only main problem’s I had growing up with PFIC and the colostomy was that a lot of the times my colostomy did leak. I also got really tired quickly and I do not have the strength that I should. But that did not stop me from trying my hardest to live a full and normal life.

 

At age 15 my mother got me on disability. Everyone in my family told me that I should work or try to work. So, at the age of 18, I got off of disability and decided to work the hardest job I could find, construction jobs. I did everything from shoveling to carrying heavy equipment. My body still tired easily. I would work for 10 minutes and then take a 15 minute break. I did not want to quit the job no matter how tired I felt. I have to thank my mother for giving me the never quit attitude.

 

I did construction for five years.  One day a freak accident happened where I was working on a three story building and the ladder slipped. I fell two stories and crushed my ankle in one hundred pieces. Two surgeries later, I had two plates and twelve screws put in my ankle. I could not run anymore and I could not stay on my ankle much without it hurting. I tried to go back to work but was unable to continue working and made the decision to get back on disability.

 

Not working was a complete let down for me. I always told myself never quit and never give up, but that day my health and then my ankle told me  I could not hold up to it. I am still disability and suffer from depression and anxiety. I live behind my mother in Aberdeen Mississippi. Without my mother, step dad, and family I don’t know what I would do. They are my rock and if you get anything out of this story please get this one thing. If you are a parent of a child with liver disease or PFIC you are the rock in the hard times your child will face.  You are the support when they have to have there biopsy or blood work and with your love they will get threw it.

 

This is what I do today. I try to help families with PFIC and other liver diseases cope with the disease and/or the loss of their child. I also spend time with my little sister Chelsey, who is 6 yrs old. I would not change anything in my life. I feel lucky and very grateful, even though my life has not been perfect I am still alive and have a positive outlook on life. If I had one wish it is that every kid and adult with any liver disease, where it be PFIC or other disease would have a full life and a good healthy life.

This is my story and I would not change anything, because the battle with PFIC disease has made me a stronger person.

Shawn Roebuck

Shawn@pfic.org

 

Reference Research Articles below from Shawn’s PBED Surgery 

1. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology.  1988; 95(1):130-6 (ISSN: 0016-5085)Whitington PF ; Whitington GL

2. Selective Surgical management of Progressive Familial Intrahepatic Cholestasis (Byler’s Disease) by Jean C.     Edmond and Peter F Whitington; Journal of Pediatric Surgery V30, No12,