Emily Ventura was already well aware of the risk of germs before coronavirus. She works as a nurse in the ICU and takes care of her 8-year-old, who is immunosuppressed. Her personal connection to the rare disease world began when her daughter, Cedar, was five months old and was diagnosed with progressive familial intrahepatic cholestasis, PFIC.
Since then, she’s learned to balance her work and her full-time motherhood, serving as her daughter’s primary caretaker and teacher. She co-founded the PFIC Network and served as president until this past March, where she shifted into an executive director role. She talked to Patient Worthy about how her family has always dealt with the high risk posed by germs, and how coronavirus has meant Emily and Cedar had to spend quarantine apart.
Progressive Familial Intrahepatic Cholestasis
Progressive familial intrahepatic cholestasis or PFIC is the name of a group of liver diseases caused by a defect that damages the transportation of bile from the liver. This usually causes symptoms before age two, in which patients tend to exhibit cholestasis, jaundice, failure to thrive, and a characteristic itching due to liver failure. Because patients tend to have difficulty absorbing vitamins, they often display a host of other issues due to vitamin deficiency including broken bones, difficulty with balance, and vision issues.
Cedar’s health promptly demanded attention- displaying symptoms in her first months. At 15 months she had surgery, and at five, she received a liver transplant. Emily said,
“We always struggled with the uncertainty with her diagnosis. It’s a very rare condition so there’s not a lot the doctors can tell you about what to expect. So you know, we struggled with that.”
Cedar’s transplant left her heavily immunocompromised. Her antibodies specifically cause her issues because they reject her liver. Emily said, “She’s doing fine as long as she’s on her immune suppression. Until we understand more about the disease and the antibodies and treatments are developed, she’ll be immunosuppressed to protect her liver.”
A Life With Immunosuppression
A life with immunosuppression is one that requires you to be extremely conscious of germs, since any illness becomes dangerously severe. Emily describes how living with immunosuppression is not unlike living under coronavirus safety restrictions. She said,
“Basically, the world is functioning how we function on a daily basis. If friends are sick, we ask they don’t come around, and we try to avoid large group functions. We also homeschool her while she remains immunosuppressed. we don’t have to do that, but it works for our life for the moment.”
Still, Cedar has a busy life. She does gymnastics, forestry, and other outdoor activities, since they live in the woods in rural Kentucky; but they try to keep them private if possible.
Working at the ICU and Immunosuppression
When it comes to Emily’s role in her daughter’s life, she’s basically supermom. When she’s not teaching her daughter and organizing her health needs, she is doing it for others in her work at the ICU. Emily explained the precautions she takes to protect her daughter. The minute that she comes home from a shift, she changes her clothes and gets right in the shower. She won’t touch Cedar until she’s sure that there are no germs, and this was her system even before the pandemic.
While on the job, Emily describes a rigorous routine of hand washing and precautions, and her attempts to avoid tending to the most at-risk patients for contagion. With the high-risk consequences any illness could spark for her daughter, she doesn’t take any chances though. She said that if she thinks there is even a chance she has been exposed, she maintains a safe distance from her daughter. While she acknowledges that it’s not the ideal situation, she knows that she has to be honest with herself about exposure to anything that could harm Cedar.
Emily also co-founded the PFIC Network in 2018, a grassroots organization that is still in its infancy. Her and two other moms began their advocacy a few years back, before growing their work into a whole network that links the community together and provide them with resources. She said,
“We advocate for our community and create awareness opportunities connected to medical advisory boards around the world so that we can get some more answers for our community and bring a patient voice to the research.”
While she stepped down from the presidency just before the mayhem of coronavirus began, she continues to be on the board as the executive director. She said the organization struggled at first to adapt to the difficult times of COVID, but luckily they had planned their second conference for 2021 so it didn’t change their goal as much. The lull didn’t last long though, Emily said that passion drives them, as they’re all parents. This passion has helped them to focus on their infrastructure in the case of another event as big as the pandemic.
… And Then Coronavirus
In the time of coronavirus though, taking care of her daughter while working in an ICU where patients are more likely to be carrying the extremely contagious virus became an impossibility. Emily explained that Cedar had to live with her father a few hours away. This meant schooling through Zoom, which she points out, her daughter has already mastered.
Once she was living on her own, she picked up extra shifts at the hospital since she wasn’t going to take care of her daughter in this time anyways. Her hospital in Louisville experienced a rush of need early in the pandemic in March, but as the high demand went down, her hospital had enough resources to let her take time off to self-quarantine for two weeks, before spending a week with her daughter. Her hospital has always been accommodating for that, and she said, “It was really appreciated- a good mental health break for me her father and her, because were used to being around each other everyday, so the time apart was hard.”
Cedar Adapting to the Pandemic Changes
Emily explained that while her and her husband are separating and have different work day schedules, in the end the experience was eye opening and they both adapted well. She acknowledged that they learned a lot about themselves and each other during this time.
Cedar adapted well to the shift though it was difficult, she notes, Cedar has already spent a lot of time in hospitals and adapting to disruptions to her routines. Still though, she misses her routine and social life. Emily said,
“It’s almost like she was starting to feel comfortable and her world got rocked again. But overall she’s doing okay and she’s gotten to spend lot of time with her dad which is great. She’s a really creative kid and she’s handling it really well.”
Re-Opening While The Virus Remains
While the country slowly re-opens, nothing will be changing for the Ventura’s. The quarantine rules existed pre-pandemic for them and will be even stricter as the contagious virus continues to spread. She said,
“Its nerve racking but [re-opening] is going to have to happen at some point. We just can’t drop our guard. Those rules are important to people like us. I hope people understand there are still people in the high risk population that can get sick, and I hope people are respectful of that when they go about their day to day. People like Cedar, and the older population and the high risk. Just keep them in mind while you’re doing your thing.”
Emily notes how grateful she is for her community who supported them through the tough times. She said without them, they really couldn’t operate. Friends dropped them groceries, helping her daughter and her father stay in a bubble, and provided emotional support for the stress. When I asked Emily where she gets her strength to deal with all of this, she said,
“Thats hard I don’t know. Oh wait. I do know. Its Cedar. She’s this incredible force, and I’m honored to be her mom. We all stay strong for her.”
She hopes the general population will walk away from this with empathy, and understand this is just what life is like for the immunocompromised. In order to keep the vulnerable population safe, everyone else has to follow rules of hand-washing, staying home when sick, and being mindful of who you’re around. Emily said, “That’s not a COVID thing, that’s an always thing. You never know who you’ll be around. The only way the vulnerable population can live is if we all follow those simple rules.”
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