Congenital Hepatic Fibrosis: Barbara’s Story

December 31, 2014

by Barbara Ainsley

I can only rember little things when I first got my diagnosis of Congenital Hepatic Fibrosis with ARPKD. I was very young, maybe three or four.

I remember staying in the Floating Hospital in Boston MA and the doctors drawing on my stomach. I was always told that I could not play any contact sports and that I needed to be careful. My grandmother would take me to the hospital twice a year for a visit while I was growing up. I didn’t really understand anything that was going on. I thought all the kids went to the doctors like I did.

When I got older, some time in my early twenties, I got really sick and the next thing I knew I was moved to Beth Israel Hospital in Boston, MA. where they took out my gallblader. The nurse came in and gave me a huge book about transplants. I was overwhelmed. I got better and went home and never thought about it again. Maybe it was my way of trying to live a normal life. I stayed healthy for a long while.

During all of this I was also told that I would probally have a hard time getting pregnant. They said it was due to my health. That they did not recommend it because I would be a high risk pregnancy and the baby, or me, or both of us could die. This was a hard for me to accept. I didn’t really have a choice in the matter, because it never happend and I began to accept it.

I was shocked when, at 36-years-old I found out that I was 12 weeks pregnant.

I cried when I heard. I was more scared than I’d ever been about anything.

I knew deep down what the doctors would say. My health had become more serious. I had developed portal hypertension and had varices in my esphogaues and my PKD was more severe. I took my best friend and went to the hospital. Again they did not recommend me having the baby.

But I began to wonder–Is this to be my chance? Was this God’s plan for me, to have a child after all these years? I struggled to make sense of it all. After so much consideration, I found myself making an appointment to terminate the pregnancy. It was the hardest thing I ever had to do in my life. It still haunts me today.

Six years later I found myself getting even sicker, this time with severe sepsis and septic shock. I was brought to Beth Isreal where I was kept in ICU. My aunt and uncle who I am very close to were called up from PA. I had a heart attack and my liver and kidneys were shutting down. With the help of prayers from friends and the doctor’s skill and guidance, I started to slowly get better. I had to learn how to walk and build my strenght back up from being in the ICU and the hospital for so long.

I was out of work for three months. When I went back to work I still had to use the cane. I was just glad to be back. My kidneys were affected and took a big hit and did not recover so well by this set back. I am now at stage 4 with a GFR number of 20. I was told that I will need a transplant sometime in my future. This scares me. Not only will I need a kidney transplant but the sugery is going to be more complicated due to the varcies and problems with the liver. I am 43 now and I’m just aiming to live life for now. This is all we can do. When I was in my twenties after I got sick the first time, I realized that I might not make it to retirement age or live a long life. This is why I livie my life the way I want and I try to enjoy it as it goes by.

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