Family and Friends:
Social distancing, staying away from those who are ill, sanitizing, that is the everyday world for the families that are connected to anyone with Cystic Fibrosis. This is normal daily life. All you would need to add is to try breathing thru a straw. That is their life. And now with the Coronavirus, they are more at risk than ever before.
We are happy to inform you that Sophia does NOT have the pseudomonas any longer. We are so thankful she did not have to go into the hospital. Savannah is healthy, but of course things could change in a heartbeat.
The Cystic Fibrosis Foundation has cancelled all activities and will set up a virtual Great Strides Walk. Going to my Great Strides website will have all the updates. As of now, the foundation is still in the planning stages. The only known information is that the date has changed from 02May to 16May.
Jackie has a virtual walk in the making also and updates to be posted. You could walk in your neighborhood while chatting with all of our national Florio walkers as well as Sophia and Savannah. I will keep you updated. I am excited about walking with everyone nationally since I can no longer be personally at your walk. If you are not from Wichita or Tuscon, and have not registered to walk, contact Jackie or me to be added. Since this is the 10th year we have walked, Jackie and Bryan are giving back to you....because of your support, whether it has been in prayer, physical support or financially. It is because of YOU the girls are as healthy as they are. THANK YOU.
Below are updates and programs that I would like share with you:
65 Roses - We are celebrating 65 years of working to find a cure for the dreaded disease - Cystic Fibrosis. Take a moment to read the story behind 65 Roses: (copy and paste the below link)
The 65 Roses Story
There is a 65 Rose Club where you can donate on a monthly basis. Consider joining us in the fight monthly. Copy and paste the below link:
The below link is to donate to the Great Strides Walk for 2020. I will keep in touch for future updates.
fightcf.cff.org/goto/gayleflorio2020 (copy and paste)
Now for the greatest news of 2019 and for the future of those with Cystic Fibrosis:
TRIKAFTA --- Another research breakthrough. Not a cure, but the documentaries from those with CF and their improvement in their quality of life is unbelievable. All the stories begin the same. Within days, the flim that has remained in their lungs for years and that has done damage, begins to be coughed up. Keep reading, it gets better. Let’s get a better understanding of exactly scientifically we are looking at.
Trikafta is a “triple-combination therapy” consisting of three different modulators -- tezacaftor/ivacaftor (which makeup Symdeko®) combined with elexacaftor. Modulators work by helping to fix defective CFTR protein. Trikafta has the potential to be significantly more effective than current modulators. It is available to anyone 12 and older. There is a clinical trial for 12 and under and should be available to Sophia and Savannah by the end of 2020. Sophia and Savannah have the combination of Delta F508 which 90% of those with CF have. They also have a rare mutation - G551D.
Clinical trials of Trikafta showed dramatic improvements in key measures of the disease.
People with two copies of the F508del mutation had a 10 percent increase in lung function compared to treatment with the modulator tezacaftor/ivacaftor (Symdeko®), and people with one copy of F508del had more than a 14 percent increase in lung function compared to placebo.
Participants also saw a significant increase in the assessment used to measure individuals' quality of life as well as sweat chloride.
Clinical trial data show that many people taking highly effective modulator therapy will have improvements in lung function and gastrointestinal (GI) issues. However, advanced damage that has already occurred in the lungs and other organs (such as the pancreas) generally cannot be reversed. Jackie and Bryan have been diligent in keeping the girls as healthy as possible. We know they have lung damage, but Kalydeco has prevented any future lung damage. They have been on Kalydeco since 2015. We are blessed and now with this new Trikafta, it will be another line of defense.
The following is a blog from our dear friend who has been on Trikafta. I know it is lengthy but oh so worth the read:
If you want to follow her journey:
Hi, my name is Michelle (you may call me Chelle) and welcome to my page! I’ve always loved to write. Ever since I was a little girl. I even enjoy sitting down, grabbing pen/paper and writing down all of my thoughts. Reflecting my thoughts in a journal. It’s healing. It’s releasing. It’s a way to remember life. I also love to write letters.
So, why am I doing this blog? What do I want to achieve? There are no achievements determined, only my deepest thanks to each of you for taking the time to read my ramblings and follow my adventures. I decided to blog to share my life experiences, the ups and downs and everything in between. I also want to share my journey since beginning the medication Trikafta. I have Cystic Fibrosis. I am 43 years old, and for the first time in my life, I am able to take a triple combination genetic modulator. It isn’t a cure. My lungs still are damaged after years of infections, coughing, mucus build-up. etc., with scarring and scar tissue. Trikafta will not reverse the damage. I am hoping for stability, maintaining and overall my quality of life improving. Maybe I will live past 50..? I’ve never felt this hopeful. Trikafta may be life changing for me. For all of us who can take it. I cannot wait for each of us living with CF to experience these new experiences.
Who knew the impact this medication would have on me in not even 3 months? I remember the first dose, the purge of mucus from my lungs and sinuses the first 24 hours. My energy level feeling like I could climb a mountain effortlessly. The issues of wanting to eat everything I see in sight. It resembles that “hangry” feeling when on prednisone. That actually still holds true.We are working on that unimaginable adjustment. line of salt crystals that used to form on my brow! The that Waking up and not feeling congested! Do you have any idea how amazing that is for me? To dance between exercise reps, during meds, while cooking, while in the shower, in the car, in the yard (sorry neighbors) or with my dogs (they love it). To dance and sing and not feel winded. My laugh! To laugh without following a series of gasping-for-air-wheezy- moments that literally scare everyone who may witness, thinking you might die. Not a bad way to go, really. She laughed herself to death. Being able to run without coughing makes me cry quite often. I smile through the joyful waterfall that falls onto my path with each step. Sometimes, I just can’t help it.
I have 44 years of damage that cannot be reversed. My hope has been to gain back to being at what is considered my “baseline.” This week, that wonderful number came back to me. I wanted to cry. I hugged my RT. I’ve gained 13% lung function while claiming my breaths again since starting Trikafta.The next time you realize you are surrounded by silence, stop and soak it in for a moment. Take a deep breath. Smile. Give thanks for your life.
Trikafa is giving us the beauty of quiet. It is a surreal feeling. It also is allowing us to hear more. To listen to life and all we’ve missed. I don’t know if I will ever get used to it. Reminder, Trikafta is not a cure. But it’s giving us more hope, more strength in our lungs and airways, and improving, on numerous certain levels, our quality of life. Leaving me speechless and full of wonderment for what may come. This is why we cannot stop giving so research continues to happen. We need life changing genetic modulators to be available to everyone living with CF. With all my heart, I want all of my CF friends to reveal in the glorious joy of this silence.
This is why your donation is still as important as ever. Researchers are reaching out to the CF Foundation to join the team to be a part of the team that finds a cure. I am not scientific, but the information that has been presented to us in layman’s terms, indicates that the future holds a cure sooner rather than later. This is because of your continued support. Again to donate:
Copy and paste and it will take you directly to my Great Strides Donation page.