S0- no fibrosis present
S1- mild portal fibrosis
S2- moderate periportal fibrosis
S3- severe fibrosis
S4- cirrhosis (end stage)
In simple terms fibrosis is scarring. The healthy tissue is damaged by scarring which compromises the functionality of the liver. Cirrhosis is developed from fibrosis. When there is significant scarring the liver cannot function properly causing organ failure.
Areli's biopsy at 2 months old showed S0- no fibrosis present, her biopsy at 8 months old, just 6 months later, showed S1- mild portal fibrosis. We knew her liver disease was progressing, but not at this rate! It is hard to estimate how long it takes to progress into the next stage depending on the specific disease, environmental factors etc, each person is a little different. The "average" time it takes to go from S0 to S1 is 15-40 years and Areli did that in just 6 months. We've always known she wasn't average though. I was made aware early on in her medical journey that a transplant was a possibility, but it always seemed so far away, such an incomprehensible thought that my mind did just that, didn't let me comprehend that it just might happen. Before, while in S0 there was always that possibility of reversing the fat in her liver, there was always that option of no liver failure. Now, in S1, there is irreversible damage being done and since there is no cure for her disease the progression will continue and that incomprehensible idea may be reality sooner than originally thought. When she was first diagnosed with liver disease it was hard for my brain to accept and understand, but I overcame that, then she was diagnosed with an incurable progressive disease and I am (still) learning to accept it, now she is in S1 liver failure. I understand the progression of liver failure and her disease, but my mind won't allow me to comprehend that my baby is in stage one liver failure. Although it is only S1 at this point- to be on the spectrum is something inconceivable to me at this point.
Don't get me wrong, I am more than grateful that Areli is as healthy as she is right now, many babies with her disease have died by her age or not met the milestones she has. It is not fair for any child to go through. No child should have diseases, no child should die, no child should suffer, no child should go through organ failure...
I just hope I am a match so I can give my baby my liver.
Love,
Mama Linnea
