From time to time we invite guest bloggers who are part of the Emily’s Entourage community to contribute their unique voices to our blog. In honor of May being Cystic Fibrosis (CF) Awareness Month, we would like to showcase individuals directly affected by this disease in our #CFStrong series. Today’s guest is Jack Goodwin, the boyfriend of Mallory Smith, the author of Salt in my Soul: An Unfinished Life who tragically passed away in the fall of 2017. Mallory also had one nonsense mutation of CF. Hear Jack’s perspective of the importance of breakthroughs for nonsense mutations, as he recounts his loving relationship with Mallory.
I’m one of the lucky ones to be born without a nonsense CF (CF) gene mutation. In fact, I was lucky to be born without a CF gene mutation at all. Until a few years ago, I had never heard of CF, let alone met a patient. CF had no meaning to me then. That is, until I met and quickly fell in love with a beautiful young woman named Mallory Smith who had both: one common CF gene mutation, and one nonsense one. At that point, CF gene mutations took on a whole new meaning, since loving Mallory made me feel like the luckiest man alive—but having a girlfriend with deadly mutations threatened our future.
Tragically, Mallory passed away in the fall of 2017. Memories of our earliest flirtations seemed so very distant at the time. We had grown so much as a couple since we first met. As our relationship grew, so too did the fear that having CF posed. Mallory and I met through mutual connections at Stanford University. I was on campus working on my Master’s degree, arriving after Mallory had graduated from Stanford in 2014, Phi Beta Kappa. When we met, Mal had a chest port, the only visual betrayal of her declining health. Her vibrant spirit is what I noticed first, a spirit she had until she drew her last breath. Our first date went wildly well, as we spent the day surfing in the Pacific Ocean, basking in the sun and eating yummy food. I kissed her for the first time after we had gotten out of the waves and remember it being a little salty. Little did I know what I was getting myself into.
As we grew closer, and fell more in love, I would diligently study to keep up with the STEEP learning curve that was (attempting to) learn what it means to live with CF. As I was learning, she was writing—something I came to learn she’d done diligently for 10 years. It was devastating to lose Mallory but I’m so grateful that she documented her life with such exquisite detail. Random House published Salt in My Soul: An Unfinished Life by Mallory Smith in March, calling the book life-changing and the press has been phenomenal. Not only has it helped me with my grief but it’s helping so many others and Mallory’s family is donating all the profits—most to phage therapy—a viable option for treating superbugs. In promoting the book I’ve had the great honor and joy of meeting other CF patients, loved ones left behind, and inspiring individuals like Emily Kramer-Golinkoff, who is working hard with Emily’s Entourage to fund much-needed research accelerating breakthroughs for CF nonsense mutations.
As a scientist/engineer, I’m fascinated by the medical science of CF and rare genetic mutations. Mallory had the Delta F508 allele along with G542X. The first mutation was common. The second—referred to as a nonsense mutation—was not. As Mallory’s health started to show signs of weakening towards the end of her high school years, she and her family decided to accept a spot in the phase 3 trial drug called Ataluren, a CFTR modulator. She went hospital-free for more than a year and gained 20 pounds in the process. Mal would find out years later that she was indeed on the trial drug and not a placebo. As with most CF patients, Mallory would continue to struggle to gain weight because the mucus causes malabsorption, but for the 15 months that she was on the trial drug, there was hope for a healthy, happy future. But when Mallory got very sick, her Stanford doctors blamed the drug and made her leave the trial.
Her college years brought spurts of freedom, tempered by long hospitalizations and a rapid decline in lung function. I met Mallory when her lung function was less than 50% and soon after talk of transplant began as it hovered in the low 40’s. With only one center in the country willing to list Mallory—and knowing that transplant was fraught with problems—we spent time wishing that there were other options available for patients with nonsense gene mutations. Mallory’s case was risky, and everyone understood that. But there was no other option for her, so she bravely endured the wait. Seven months after being listed, Mallory became the recipient of donor lungs—a gift she was so very lucky to receive.
One perfect memory from those days is quite simple: when we went on a walk under the falling leaves
of the chilling autumn air, without oxygen, not needing to stop.
Recovery would prove to be the most challenging hurtle Mallory faced, but with fierce determination and staunch effort, Mallory recovered ahead of schedule. One perfect memory from those days is quite simple: when we went on a walk under the falling leaves of the chilling autumn air, without oxygen, not needing to stop. Our joy was palpable as we started to dream of healthier, happier days together. We started to picture a future together. Then, Mallory landed back in the hospital with necrotizing pneumonia caused by her superbug. The antibiotics weren’t able to stop the progression of infection and three weeks to the day after she was admitted she passed peacefully surrounded by her father, mother, brother, aunt and me…each of us holding onto her.
As much as I miss Mal, and as much as I wish she were still alive now to enjoy life by my side, I realize the battle to treat and cure CF is not over. There are still thousands of CF patients in the world, some of whom I’ve come to meet and know, who are still fighting for their life. Since Mal has passed, many patients have seen improvement in their health because of new drugs. That thought is bittersweet as I’m happy for them but mourning the loss our our beloved Mallory. But there’s a subset of the CF population that still hasn’t seen benefited from new treatments because of nonsense CF mutations so the fight for a cure must continue. There are still many with CF who have nonsense gene mutations desperate for the chance of longer, healthier, happier lives. Mal wanted desperately to live a long life, raise children on the beach and pursue a career. I shared her hopes.
In her memory—and because I’ve come to care about other CF patients—I will continue to fight for better treatments, work towards a cure, and share Mallory’s insights so that people will understand what it means to live with this dreaded disease.
Mallory was a cherished friend of Emily’s, to read a guest blog post she wrote in 2016 about the challenges in friendship CF patients face due to cross infection policies please click here. To purchase Salt in my Soul: An Unfinished Life, please click here.