Hi, I'm Fiona but you can call me Fi. I'm 21 yrs old and I have biliary atresia with no transplant. I currently live at home. I'm not currently in school at the moment, but I was attending FIDM (Fashion Institute of Design and Merchandising). I was majored in fashion design so that I could become a costume designer for movies. I have two older sisters Mary-Jane and Lauren. I also have a very loving boyfriend, Don. We have been dating for two years now. I'm very lucky to have someone, besides my family, that is there with me through my ups and downs. Above all I'm thankful to have my mom. She literally gives me strength to fight and carry on through my illness. Without her I don't know where I'd be today. I must say that I really do have the best support system that anyone could ask for.
Biliary atresia is a condition in which the ducts that carry bile from the liver to the gallbladder and small intestine are not normal. These ducts can be injured, blocked, or missing. When the ducts that carry bile out of the liver are closed, bile backs up and damages liver tissue. In time the liver can develop scarring or cirrhosis. The gallbladder may also be abnormal or missing. The cause of Biliary Atresia is unknown. It occurs in one infant in every 15,000 live births. It is a serious disease which requires immediate surgical and medical treatment.
Arteriovenous Malformations AVMS
I have been diagnosed with AVMS in my lungs do to my liver disease. These have caused me to have 2 lung bleeds which were very scary. I still have more AVMS and they are causing me breathing problems. Below is an explanation of what the AVMs are and a picture of what is happening in my lungs.
Arteries and veins are part of the human cardiovascular system. Normally, the arteries in the vascular system carry oxygen-rich blood at a relatively high pressure. Structurally, arteries divide and sub-divide repeatedly, eventually forming a sponge-like capillary bed. Blood moves through the capillaries, giving up oxygen and taking up waste products from the surrounding cells. Capillaries successively join together, one upon the other, to form the veins that carry blood away at a relatively low pressure. The heart acts to pump blood from the low pressure veins to the high pressure arteries. If the capillary bed is thought of as a sponge, then an AVM is the rough equivalent of jamming a tangle of flexible soda straws from artery to vein through that sponge. On arteriogram films AVM formation often resemble a tangle of spaghetti noodles. This tangle of blood vessels forms a relatively direct connection between high pressure arteries and low pressure veins. The result is a collection of blood vessels with abnormal connections and no capillaries. This collection, often called a nidus, can be extremely fragile and prone to bleeding.
AVM picture
Liver Terms
I realize that we have become so accustom to speaking in medical lingo that we confuse people along the way. A list of commonly used terms in the liver world are displayed at: Children's Liver Association for Support Serviceshttp://classkids.org/Resources/glossary.htm )
This is the non-profit organization we work with that serves families with children with liver disease.
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