Symdeko, here we come!

Update: In January, Lance and I found out that Crosby is included in the group the FDA approved at the end of 2020 for the gene modulator Symdeko for certain rare CFTR mutations. We were in shock because while we have celebrated each and every drug approval, having rare mutations meant that up until now, Crosby was in the 10% of persons with CF that do not qualify for any of the ground-breaking drugs (Kaledeco, Orkambi, Symdeko, and Trikafta) that have been developed by the CF Foundation.

The new FDA approval only affected around 300 people and Crosby was one of them! If you have supported us through the Cystic Fibrosis Foundation in the past 7 years, THANK YOU. This is what we have been waiting for and your donations helped make this happen. With each drug approval, I would get emails or texts asking me if they benefited Crosby and I always had to say no, he was not included. But now, it’s HIS TURN.

The gene modulator medications for cystic fibrosis are hailed as the greatest story in medicine today! They are literally transforming a genetic disease. These drugs are not a treatment for the symptoms cystic fibrosis causes, but are actual protein CORRECTORS. It is not a cure, but it is as close as they have have ever gotten.  

To give a brief description on how it works, Crosby’s salt chlorides have never been able to come to his cells’ surfaces because they were blocked by two gene mutations that screw up the salt and water content in his body. This causes the mucus to thicken in his lungs and digestive system. Gene modulators help the chlorides to come to the surface and virtually correct this. If you want to learn more about how Symdeko works, here is a great video: https://www.symdeko.com/how-symdeko-works

While we are so excited for Crosby to be eligible for Symdeko, deciding to go ahead and have him take it was a tough decision. Since insurance was a 3-month battle, that left a lot of time to research and weigh out the choice. Reading over the good and the bad has caused a mix of emotions for me.

There are side effects: Abdominal cramping, elevated liver enzymes, and eye issues to name a few. One of my fears is that he won't be able to continue the medication because of these side effects. I don’t want Crosby to endure anymore pain in our pursuit of quality and quantity of life. He is not too happy about the required blood tests every 3 months to make sure his liver function is cooperating with the Symdeko. So I’m pretty anxious. This medicine will change his body at a cellular level to work in a way that it never has. That is a mighty decision to have make for someone else, and not one we've taken lightly. As his parents, we want to protect Crosby but also not keep him from something that could be beneficial for him.

All that said, we decided to start and I am excited to say that on May 1^st, the first day of Cystic Fibrosis Awareness Month, Crosby took his first dose of Symdeko.

These gene modulators are giving those with CF a new chance at life. I am so thankful these medicines were developed during his lifetime. We are hopeful that Crosby’s life will change for the better. The worries that I have had since the moment that he was diagnosed with CF have lessened. All the negative things I thought he would have to experience as he gets older might not be a possibility anymore. I feel new hope for him and everyone with CF.

The Cystic Fibrosis Foundation has promised that this is just the beginning. Kaledeco, Orkambi, Symdeko, and Trikafta will not be the last medical advancements for CF. Things can and will only get better.

I am so very grateful to Vertex, the Cystic Fibrosis Foundation and everyone who made it possible for Crosby to get Symdeko. My heart aches for the CF warriors who lost their lives to cystic fibrosis before they ever had a chance to take these medicines. I continue to hold on to hope for those who still aren’t eligible (even though the gap is narrowing.) My promise is that I will continue to fight for and support the CF Foundation until all people with CF have the transformative medicines they need… Until It’s Done!

Great Strides is on hold again for 2021 due to COVID-19 and needing to protect the CF community. BUT! We are still fundraising for the CF Foundation to keep the momentum going and you can visit our page here: THE CROSBY SHOW

Love,

Lance, Carmen, and Crosby

CF Awareness Month 2020

Every May, we celebrate Cystic Fibrosis Awareness month and pandemic or not, May 2020 will be no different! Last year, Lance, Crosby and I were honored to represent the CF community at the Arkansas Capitol where Senator Missy Irvin sponsored a resolution declaring May as Cystic Fibrosis Awareness month.

We encourage you to take the time this month to learn something you did not already know about CF or share what you do know about CF and your buddy Crosby with someone else.

As you have probably already guessed or heard, Great Strides is postponed due to COVID-19. The Arkansas Chapter of the CF Foundation has announced a new date for Little Rock Great Strides - October 3, 2020! Lance and I are choosing not to fundraise at this time, but please mark your calendars and plan to join us in October at the LR Zoo! 

Lance and I have had so many wonderful and thoughtful people reach out to us since the start of the pandemic – checking in, thinking about us, praying, etc. I cannot say thank you enough! This is an incredibly scary time - for everyone. But for those who have recognized the additional impact this has on a family with Cystic Fibrosis – thank you for seeing us and letting us know you have thought about us. It has meant so much. 

We have been asked (a lot) if we’re scared of the coronavirus. But the truth is, we have lived with the threat since Crosby was diagnosed with CF. Of course, the threat hasn’t always been COVID-19, but for the past 6.5 years we’ve had to take the same precautions that everyone else has just started taking the past 8 weeks. We wash our hands constantly, disinfect everything, social distance, avoid crowds, cancel trips and activities, and weigh every decision on what the impact could mean for Crosby’s health. When lock downs across the country first began, Lance and I both said how a lot of this reminds us of Crosby’s first year and how hard we worked to keep him isolated and give his lungs a chance to grow without being impacted by infection. 

So, have we been scared? Maybe a little. But that’s the life we live everyday having a child with CF. So many unknowns, so many rules and restrictions. Staying home when we want to go out, not having friends over, saying no when the tears in Crosby’s eyes are begging me to say yes. We have to approach every decision by thinking, are we doing the best that we can do? I don’t want something to happen and look back, regret a decision and then have to say, well I could’ve done better. When given the choice to follow the rules or potentially lose my Crosby, there really is no choice.  

I know we are all ready to get back to a more normal way of living. But as we start to go back to places and activities, please remember we need to still protect not only ourselves and families, but we also need to protect those most vulnerable. It may not mean much for you to get a virus, the flu or even a cold, but it could mean hospitalization, serious complications or even death for others. 

Please continue to take the time to wash your hands A LOT. Please stay home if you aren't feeling well and keep your kids home when they aren't well. And please please PLEASE wear a mask when you go out in public. Not because you are required to, but because of your love for your neighbor – the kind of love that God calls us to have for one another. Your mask protects others. Wearing a mask is simply just being considerate. No one is asking you to live this life forever. Please do the right thing. Remember, when this pandemic is over, social distancing and masks will still be the norm for some people:

Fear and Hope and Thank you

Fear.
I hate the word and what it represents for me.
There is a fear that has regularly stopped me in my tracks for the past 5+ years.

From the day Crosby was diagnosed with cystic fibrosis, I have had to live with the reality that one day, I may watch him lose his fight. The moment that I laid eyes on him, I could not fathom my life without him in it. And when he was 15 days old, I had to try and process the dark cloud of statistics that looms over a CF diagnosis. I have been forced to think about the fact that even if he is lucky enough to surpass his life expectancy, I may likely still spend countless days watching him suffer while I stand by, helpless. The statistics tell us that less than half of CF patients live into adulthood.

I think of all the pills taken and hours spent daily on treatments for Crosby to maintain health. I think of all that CF can bring as the disease progresses: CF related diabetes, feeding tubes, ports, bacteria cultures than can’t be fought off by antibiotics. I carry the heartbreaking reality that he will likely at times fight for his every breath. And it terrifies me to think about how he will process these things for himself mentally.

I am most scared that one day, he will be gone and I will be alone with regrets. I’m afraid I will regret I didn’t take more chances or make more sacrifices to make his wildest dreams come true. I’m afraid I will regret that more days and dollars were spent trying to do all we could to keep him healthy, rather than just spent living.

That same fear also manifests as guilt for even being fearful in the first place – because I do have hope! It is just that Crosby is my baby and it is so heartbreaking to not be able to take this burden from him. I know that CF or not, nothing is ever guaranteed in this life and the only way to live is not let the fear take hold and squeeze out the hope.

Thank you to everyone who hopes, prays and fights for a cure along beside us. As CF awareness month comes to an end, I’m reminded that we are not alone in our fears nor or in our hope. Thank you for for your support and thank you for loving Crosby.

Until. It’s. Done.

Carmen