Cystic Fibrosis re-entered my life December 17th, 2001 when my youngest daughter Anna was born with a bowel blockage known as meconium ileus . This is a direct factor from a genetic defect in the salt chloride channel. Surgery was required but failed leaving behind a brewing infection yet to be discovered. At just six days old, she found herself in the OR in a dire way with a foot of her small bowel removed and an ileostomy placed. In that moment, my mind understood the severity of this disease but I needed time to collect my emotions. A few weeks later all tests confirmed she indeed has cystic fibrosis. When the fight or flight dust settled, my family chose fight, diving straight in to raise money and spread awareness. Anna has returned to the OR for the same GI surgeries four times since leaving the NICU.

Nearly four years later to the day, her brother Will was born with cystic fibrosis. With him, he brought a new set of cards as only siblings can truly complement one another. He has shown us the other side of the spectrum, primarily the struggle to breathe.

We have found care at centers in Peoria, IL 2001-09, The University of Iowa 2009-16, back to Peoria, IL 2016-19, speciality consults at the University of Michigan and Colorado Children’s , and now St. Louis Children’s Hospital.

In late 2016, Will cultured non tuberculosis mycobacterium (NTM) causing care to be transferred back to our home in Peoria, IL. Shortly thereafter, Will began frequent admissions for treatment of the NTM. Over the next two years, his hospitalizations grew closer together and more severe, including Intensive Care stays, as the NTM fought for real estate inside the lung. Treading water, we took referral direction for treatment from the University of Michigan, National Jewish in Colorado, and Colorado Children’s Hospital. These experts complied an ongoing plan making changes when needed. After continuous iv antibiotic therapy lasting over 18 months, his lung function dropped from 77% to 24%, he was hovering around 72 pounds, and he could no longer attend school. An oxygen machine, cranked up to 4L, was required day and night to help Will through his daily activities. Treatment was considered failed leaving transplant as the next viable option.

As the Peoria CF team and I researched options, we affirmed St. Louis to be the best center for our family. The first week of February, Will was evaluated by the Lung Transplant Team in St. Louis. After a week of tests and other measurements, per the norm a follow up meeting was set one week out. Given the severity of his case, more time was requested and Will was given a to do list. The surgeon tasked him gaining and maintaining 15-19 pounds to give his body the best chance of sustaining transplant. The Infectious Disease doctor needed his NTM levels to drastically reduce.

After a long pause, on March 22nd the St. Louis Team accepted Will into the transplant program. It took another month to gather family to repeatedly run through the course of actions ahead, gain the necessary support from my employer, and secure a new temporary home for Will and I. On Easter Sunday we relocated with a well prepared plan. After being seen in clinic the next day, Will encountered another setback. Very rarely can CF, especially at end stage, have its voice silenced. The NTM levels had grown out of hand . While inpatient over the next two weeks, an intense plan for reduction was modified, including iv antibiotic therapies nine times a day. On May 17th, Will was officially placed on the UNOS list for double lung transplant. Three weeks later he was successfully transplanted and continues to crush the recovery expectations.

Surviving the first year is critical while adjusting to the new set of treatment. We remain in the St. Louis area through the first week of September as the required milestones are met. During that time, he remains on the same iv schedule giving him the chance for the best recovery imaginable.

Few can understand the perspective of extremely difficult to be away from the people, dogs, things, and home you love. Even fewer are given the chance to understand the beauty of organ donation.

Anna has continued her ongoing battle with NTM. Research is close that may give her a different opportunity before it progresses to the extent of her brother’s.

For years, I fought hard to not let the disease of Cystic Fibrosis define who I am. That was the wrong mainframe. This is my life. These are the lives of my children. I am CF.

CF=Changed Forever.