“We Were Most Excited to Someday Not Take Pills and to Not Do Vest Therapies”: Her Lifetime With Cystic Fibrosis

As originally reported in Cystic Fibrosis Life Foundation, Martha has spent her 24 years of life with cystic fibrosis. She was born with the rare disease which means for a lifetime of infections, difficulty exercising, and mediatrions. At the time, there was no treatment options; she spent her childhood living with this knowledge.

Martha describes the hope of her family when she was six when they found out the news that there was progress to a real cure. Her and her brother, who was five years older at the time both suffered from symptoms of the incurable condition.  Their mother told them one day, they may live a life free of the disease. This was unimaginable to the siblings, they excitedly imagined it together on their bunk beds. Martha wrote,

“We were most excited to someday not take pills and to not do vest therapies. This meant sleeping over at grandma’s house without packing anything and that was considered a big win for us.”

Cystic Fibrosis

Cystic fibrosis is a rare disease that causes a buildup of mucus which blocks the respiratory and digestive systems due to a faulty gene that otherwise regulates salt, damaging these systems over time. Too much mucus gets in the way of normal functioning in patients: breathing, coughing, frequent infections, difficulty absorbing essential vitamins, salty skin, difficulty exercising and maintaining weight, and more. The disease varies in severity but is progressive. Most patients lose their lives eventually to lung related diseases in middle age.

Trikafta, An Answer to Hope

The day this cure came was much later than they hoped for. As a rare disease, it can be hard to obtain government funding. For cystic fibrosis, there was no government help, so all the money for research came from grass roots funding. In 2019, the first cystic fibrosis therapy was approved that was expected to have life changing effects for 90% of patients. The medication was seen as one of the biggest achievements in medicine. The rare disease is known to take patients lives in youth. It is a tough hand to be dealt. The new medication is a combination therapy of three drugs, and is seen to act faster and more effectively than anything previously available.

Martha describes her first weeks with the medications, immediately she felt a renewal of energy that reminded her of high school, she gained weight, and in days, felt the relief of her chronic cough.  She also describes how hard it is that her brother didn’t make it to see it too. She recounts times as kids that they discussed their fantasies about where they would go if they found a cure. Their mother told them could go anywhere in the world. The childrens limited experiences made them choose close to home- Martha wanted to go to Indiana, foundly recounting a recent road trip through the state. Her big brother wanted to go to Elvis’s hometown. She explained how important it was to have him to share the experience with her, when nobody else she know dealt with the same.

“He was the only person I knew growing up that also had CF. We sat with each other for hours each day during our vest treatments and waited for the other one to finish since we only had one machine, we chugged weight gain shakes together like it was a game, we went to every doctor appointment together which always ended up being an all day affair but with Ray it was fun because we usually were goofing off and laughing.”

So losing her role model and best CF companion before finally reaching the long anticipated day meant mixed emotions. She explained,

“This isn’t exactly how I envisioned it would be but out of respect for Ray and all those with CF who left this world too soon, I refuse to waste this gift when I have been given the opportunity to live a longer and healthier life.”

She also knows the struggles continues, and she continues to dedicate herself to finding answers. She concluded simply,

“There is still work to be done.”

 


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