Brooke Harrison comes across as a surprisingly easy-going mom, but she does worry a bit about germs.
It started when she noticed extensive bruising on her daughter Trinity’s body when she was about one month old. Test results showed a number of measures of her liver function were out of whack, but doctors couldn’t put a name to a condition to explain her symptoms.
A liver biopsy would lead to a diagnosis when Trin was five months old. She suffered from a rare condition known as progressive familial intrahepatic cholestasis (PFIC). It was only then that she developed some of the telltale signs of the condition—jaundice and intense itching known as pruritus.
PFIC is a life-threatening liver disease with several subtypes. In the case of Trin, she has type 2, which involves a problem with her ability to move bile acids out of her body. Children with PFIC have a grim prognosis. It leads to cirrhosis and liver failure within the first 10 years of life. For patients with type 2, there is an increased risk of developing hepatocellular carcinoma, a form of liver cancer.
There are no medicines currently approved for PFIC, only surgical options. Those include a procedure known as a partial external biliary diversion or liver transplantation. Before Trin turned 2, she would undergo three surgical procedure because of her PFIC.
The buildup of bile in the body causes itching all over, but scratching doesn’t provide any relief because the itch is coming from within the body. Trin would scratch day and night, so much so that she would cause herself to bleed. She twice had surgery to direct the bile from her liver to empty into her bowel. After the procedures failed to give her adequate relief, she underwent a nine-hour surgery to remove her liver and have a piece of her aunt’s liver transplanted in its place.
Trin’s PFIC symptoms are gone, but her mom doesn’t consider Trin cured because she must live the rest of her life on immunosuppressants to prevent her body from rejecting the donated liver inside her.
“’Cured’ is a big word for us. I wouldn’t say cured. You’re switching one problem for another. Gratefully, we haven’t had a lot of issues after the transplant,” said Harrison. “Infections are bad. Regular everyday stuff is bad, but you are not getting a cure. You are trading the itching for not having an immune system.”
Trin is 7 today and in many ways lives the normal life of a 7-year-old girl, but that can be a perilous existence for someone who is immunosuppressed. Harrison has taught Trin to wash her hands frequently, keeps Trin’s teacher well stocked with antibacterial hand sanitizer, and is quick to cancel playdates for Trin if whoever she is supposed to get together with has so much as a sniffle.
Harrison understands the problem people sometimes have keeping a sick child home from school. She knows people sometimes can’t afford to miss a day’s pay or risk angering their employer by missing a day’s work. She said before she had children, she wouldn’t have considered it a serious problem to send a kid to school if they were sick the night before.
But exposing Trin to a child with a cold or flu can be disastrous and lead to a health crisis for her. Now that Trin goes to school and interacts with more people, it’s a big concern. While getting a cold for most kids may not be that bad, it could mean an extended stay in a hospital on a nebulizer for Trin.
“Keeping your kid home one day could save me a trip to the hospital for a week or more, or maybe even save my child’s life,” said Harrison. “It’s a lot bigger deal then people think.”
Wash your hands, cover your mouth when you cough, keep your vaccines up to date. And if you or your child is sick, stay home. Following such practices can have big implications for others like Trin.
Photo: Brooke Harrison and daughter Trinity