A Resilient Spirit

Fighting to Stay Healthy for Her Family

A young girl sings in Spanish, her voice high and sweet. Mariana Valencia translates; “Don’t give up; keep fighting. If you’re fighting for you, you’re fighting for us.”

Mariana’s mother wrote the song to encourage those with cystic fibrosis (CF) to not give up. She knows all about the battle because two of her children had CF, Mariana and her younger brother, Guillermo. He passed away from the disease when he was only 22 years old.

CF is a genetic disease that results in thick and sticky mucus that clogs the passageways of the sinuses, respiratory system and digestive system. Germs and bacteria can easily get trapped in the thick mucus and cause infections. Repeated infections can damage the lungs and lead to respiratory failure. Less commonly, other organs, such as the liver, can fail.

 

A Rare Mutation

“Severe liver disease needing a transplant is uncommon with cystic fibrosis,” said Jennifer Taylor-Cousar, MD, Mariana’s CF specialist at National Jewish Health and Saint Joseph Hospital. “But certain CF mutations can make people predisposed to liver disease.”

When Mariana was 11 years old in 2000, she went into liver failure and needed a transplant. Her parents researched the best places for Mariana and found the pediatric CF program at Children’s Hospital Colorado and the adult CF program at National Jewish Health. Now 30, Mariana has traveled to Denver from Nogales, Arizona, for her care for over two decades.

At the time when the Valencias learned a liver was available for Mariana, her brother Guillermo wouldn’t let her leave the house until she promised to come back from the operation. “I realized I needed to come home because I had someone waiting for me,” Mariana said.

Ten years after Mariana’s successful transplant, Guillermo went into liver failure. He was placed on the transplant list, but was not strong enough to undergo the operation. Guillermo passed away in 2012. “For selfish reasons I want him here, but he lived his full life. And he’s no longer in any pain,” said Mariana. “I was lucky to have my brother growing up. I was never alone in my disease.”

Dr. Taylor-Cousar says it can be difficult being the sibling that survives, not only because of the loss, but because of the pressure to stay healthy. Mariana continues to fight hard to take care of her lungs.

 

Resilient Spirit

“There are some as sick as Mariana who would just give up, but Mariana is a fighter and she works really hard to continue to do both her therapies and enjoy her life and her time with her family. She has no intention at all of giving up,” said Dr. Taylor-Cousar. “Her resiliency and her positive outlook are two traits that make a huge difference in how people cope with their CF and their outcomes.”

Now, each time Mariana gets sick, she goes to Saint Joseph Hospital for treatment. The care team is able to manage her regimen, therapies, diet and exercise and administer high doses of medications to fight infections. It can be grueling, but Mariana is determined to keep her lungs functioning as well as possible.

“It’s no longer only about me,” said Mariana. “I’m fighting for my mom. I’m fighting for my dad. I’m fighting for my brother. I am fighting for more than just my life. And that is a huge component in recovering.”