Emily Knakmuhs’s two-and-a-half-year-old Kennedie had just made it past a tough couple of years. When she was born, her family noticed she wasn’t growing, but her pediatrician attributed it to reflux. They put her on reflux medication after reflux medication, but her symptoms didn’t budge.
When she was six months old, Kennedie wound up in the ICU with a spontaneous brain bleed. Here they discovered she had PFIC and unraveled that she had a whole host of vitamin deficiencies. Progressive familial intrahepatic cholestasis (PFIC) is a progressive liver disorder that causes bile to buildup in the liver, usually resulting in liver failure. The symptoms tend to first appear in infancy and include intense itching, jaundice, slow growth, high blood pressure, and an enlarged liver and spleen.
For Kennedie, this meant she was deficient in many necessary vitamins, which caused rickets and meant she needed a feeding tube for nutrition. The worst part was the itch- patients with PFIC have a constant itch due to the liver, causing Kennedie to wake throughout the night. Finally, her family decided she would benefit from a liver transplant. Emily explained it was very scary to put their toddler into the surgery. It wasn’t the option they would have chosen if they had others, but they didn’t. Kennedie was suffering. The transplant did the trick. Emily said,
“It was an immediate turn around. She stopped itching, she started growing, eating on her own, and meeting all of her developmental milestones.”
While the transplant essentially eradicates the disease, it also caused her to become immunocompromised. After organ transplants, there’s always the risk of rejection, and patients have to take precautions to avoid this. Emily explained,
“It’s sort of like we traded one problem for the other.”
After the Transplant
Once she had the transplant, Kennedie continued to take medications to prevent rejection on a strict schedule twice daily. She needed to catch up on the vitamin deficiencies, but they hope to eventually cut down on her medications. She had spent her first couple years overwhelmed by hospital visits, and she needed time to heal from that trauma. Emilie describes the many therapists they needed- to help her physical and behavioural issues caused by her hard first years. Emilie explained,
“Part of what goes with the medical trauma is this heightened alert all the time. It’s a lot of behavioral stuff which is a lot of work with a to differentiate between what is a toddler thing and what is a medical thing.”
“We always have hand sanitizer at the front door and we ask everyone who comes in regardless to do that. We won’t let people around when they’re sick, and we’re much more cognizant of if students in her brothers class are sick. We would keep him home from school so they wouldn’t bring it back. She can’t go to a lot of public places to play, swimming and all that.”
Coronavirus and Immunosupression
“When we found out the cases were coming up, we cancelled everything.”
She explained how she saw coronavirus differently because of this, and how hard its been to be so isolated from everyone, but it was a measure they had to take. She explained,
“We have been very vocal with everyone we know that this social distancing isn’t a joke. You don’t know how you’re affecting someone else’s life if you’re unknowningly carrying the virus and how could effect people that you know.”
While they did so much to keep the house clean and to distance themselves from the public, at times, there is no choice. While they switched her weekly therapy sessions to zoom and cancelled any voluntary appointments, she has an upcoming lab work appointment that she has to have done. Emily explained,
“It’s always anxiety-inducing to have to walk into a hospital with an immuno-suppresent child, but today it’s terrifying. So this is the ‘stay at home thing’ right now- because my kid can’t, she just can’t. We are trying, but we just can’t avoid everything.”
Community Support
The family has felt the care of the community who supported them when they needed it most. Emily described how when Kennedie was in the hospital, her sister gave up her last week of maternity leave to assist her, her mother was there at the drop of the hat, and friends and neighbors would take care of her boys and drop off food. She explained how caring and considerate her two young sons have been, one of whom is the twin of Kennedie, helping her with therapies and treating her gently. Emily said,
“A lot of people gave up a lot to help us, and we’re trying our best to help people realize that anything can happen at any point. You just have to put your pride aside and accept the help and ask for help. It’s been a really humbling experience. I’m really grateful for that.”
“Nobody is exempt from getting this and taking it to somebody it could be fatal for. It’s going to change your world somehow, and if there’s anything you can do to save another person or save yourself, then just do it.”
