Putting Faces to the Disease: An Interview with Three Cystic Fibrosis Patients
Every May, the cystic fibrosis (CF) community comes together for CF Awareness Month. The resilience CF patients demonstrate in the face of their disease is awe-inspiring. I had the opportunity to speak with three individuals who shared some of their CF journey with us – Molly Baker, 39, of Iowa City, IA; Jessica McDearman, 33, of Memphis, TN ; and Sydney Reichart, 21, of Saratoga Springs, NY. Here’s what they had to say about the impact of diagnosis, managing therapy, advice they’d offer others living with CF, and the importance of the specialty pharmacist in managing their disease.
The Impact of Diagnosis
Can you take us through your cystic fibrosis (CF) diagnosis?
Jessica: Shortly after I was born, my mother noticed I was unable to keep milk down after nursing. I was listless and too weak to cry. My pediatrician noticed my protruding abdomen and sent me straight to the local children’s hospital. I was five days old when I was rushed into emergency surgery for meconium ileus (bowel obstruction). My parents were told to prepare for me to not make it through the night. Upon discharge, I started taking digestive enzymes through my bottles. It took me seven months to gain a mere two pounds. My diagnosis for CF was made “official” after having a sweat test. With no family history or knowledge of CF, my diagnosis was a shock to my parents. Their instincts and the knowledge of my doctors saved my life. Life with CF is all I have ever known.
What impact does CF have on your daily life?
Molly: CF impacts my daily life greatly. Because my sticky mucus attracts bacteria, I have chronic infections in my lungs and sinuses which will never be eradicated. This makes it difficult for me to breathe and get a full breath, so I am on oxygen. CF has also depleted the use of my pancreas, so I have CF-related diabetes and take many shots of insulin daily to manage. Diabetes caused me to lose feeling in my feet, putting me at risk for developing foot ulcers. I have been in a cast since November 2021 after having one toe amputated. I am working hard to prevent that from happening again. I do hours of breathing treatments each day. Exercise is very beneficial to those with CF, so I go to the hospital several times a week to exercise on oxygen, while having my heart rate monitored. Not a day goes by when I don’t think of CF. It affects every decision and even impacts how I live and how hard I love.
Jessica: While I don’t let my illness hold me back, CF is at the heart of every decision I make. Living with CF requires me to go to battle daily. As a mother, I have an even greater responsibility and motivation to fight for my health. My days consist of countless pills, several breathing treatments and physical therapy. Depending on the day, I could be dealing with fatigue, fevers from flaring lung infections, seemingly endless coughing and hospital stays. The threat of an exacerbation hovers over my days, and I do my best to keep infections at bay by staying on top of my routine. Along with the physical burdens of CF, there are mental and emotional aspects to living with an invisible chronic illness. The weight of navigating CF is heavy and can take its toll quickly. There are days when the best I can do is sit on the sidelines while my little girls play. There are days when the medical bills, calls to pharmacies and appointments with doctors are overwhelming. The worry of being a burden on my family and not living to see my daughters grow up is always the big elephant in the room. Despite all of this, every day is a new opportunity to find the balance between struggle and beauty.
Managing Therapy
Do you feel your medications and treatments help improve your quality of life?
Sydney: My treatment has made more than just a noticeable difference in the quality of my life – the difference is life-changing. In short, Trikafta saved my life. I was granted compassionate use of Trikafta prior to its FDA approval in October 2019. I truly could not imagine ever going back to the way my body felt prior to taking it. Before Trikafta, I struggled to get out of bed and do simple tasks like showering and brushing my teeth. Now, I am able to do things I love again like skiing and taking my dog for hikes. I was able to move out on my own for the first time in my life and work full time as a hair stylist and makeup artist. I am also a salon owner. None of this would have ever happened if I were not on Trikafta.
What would you tell someone who may be scared to begin CF therapies?
Molly: If someone were scared to take their CF therapies, I would tell them to explore why they are scared. Admittedly, I was scared to start taking Trikafta. I worried that I, or that expectations of my life, would change in a negative way. Then I realized it could also change things for the better, which is the beautiful thing about advancements and new therapies. I’m glad I listened to my doctors and gave this drug a chance to work! Oftentimes, I think we fear being told a medication will work and then it doesn't end up helping, which happened to me a couple of times. However, to me, it was worth another shot, and I couldn't throw away my chance.
The Role of the Specialty Pharmacist
How can a specialty pharmacist help CF patients stay on therapy?
All: Patients with CF are no strangers to the balancing act of living with a chronic illness. Days are filled with endless medications and treatments, calls to pharmacies, appointments with doctors, along with our “normal” responsibilities as students, working adults, and parents.
Specialty pharmacists can:
• Offer different ways to reach out: phone, text, e-mail
• Provide refill reminders and medication delivery
• Ensure medication prior authorizations are completed in a timely manner
• Offer financial assistance support
• Make sure medications are being taken properly and on time, and instructions are understood
• Simply be there to help in any way they are able.
What should pharmacy staff know when talking to CF patients?
Jessica: Patients with CF are typically very knowledgeable about the medications we take and how ordering works. We consider ourselves to be professional patients and have accepted the huge role we play in our own health. It’s important for pharmacy staff to know how vital it is for CF patients to stay consistent with medications. Missed treatments can quickly lead to infection and loss of lung function. Because we are juggling so many different medications, often from different pharmacies, having an understanding staff can make all the difference in the world for us.
Sydney: Ask if they are getting their prescriptions on time and if they are able to afford out-of-pocket expenses. Ask if there is anything the pharmacy can improve to make the patient’s life a little easier! And always be sure to ask patients if they feel they are receiving the best care from their pharmacy.
What would you tell someone living with CF?
Sydney: The first thing I would say is to relax for a moment and know that CF is NOT a death sentence. With all of the incredible advances science has made in the past five years alone, I fully expect CF patients to be able to grow old and live long, healthy lives.
There are also many resources for patients and family members:
• Your CF care team, including specialty pharmacists, should be your biggest advocates and supporters to help you through every curve ball CF throws at you.
• Social media groups often include amazing networks of adults with CF who help each other advocate as well as empathize with the struggles we face daily – something you can only get from someone in the same boat as you.
• Online resources and websites, such as CFF.org.
While having a chronic illness like CF is far from sunshine and roses, it has truly given me a beautiful outlook on life, and a close group of people who have something unique and personal in common.
What would you tell other moms with CF raising young children?
Jessica: The best advice I can offer is to put the blinders on. In this day and age, no one is anonymous anymore. Parenting with a chronic illness is not glamorous, nor is it what society deems “normal.” Forget the hustle and rest when you need to. Forget your pride and ask for help when you need it. We are so accustomed to taking care of everyone else, but we must put on our own oxygen masks first. Embrace the mundane, in-between moments – the moments no one posts on social media. This is where the magic happens if you’ll only slow down, put on the blinders, and begin to fall in love with your beautiful life.
Resources:
Understanding Cystic Fibrosis – AllianceRx Walgreens Pharmacy patient condition guide
¿Qué es la fibrosis quística? – AllianceRx Walgreens Pharmacy patient condition guide | Spanish
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