{Also, a friend shared this video with me, and it is one of the most amazing things I've seen. If you know me, you know I already have an affinity for the band OneRepublic, not only because I do like their music, but also because they're based in DENVER. :) Well who knew that one of their most beautiful songs would be set to a video with a child who has CF! And not just that, but they let this sweet teenager talk about his disease and fears, and you get to watch him do his vest treatments and nebulizer. You also get to watch him riding his bike through gorgeous Colorado scenery and make visits to our very own CF Center at the Children's Hospital here in Denver. Please watch this video for a look at someone living with CF. Nevermind that the lyrics are ridiculously beautiful and something that I pray for Avery everyday. I've watched it several times and cried the whole way through each time. Thank you OneRepublic for bringing awareness to this disease!}
***
Dear Friends and Family,
Our family wants to thank you SO much for all the love and care you've shown us in the past month. It would be a lie for us to say that it's been easy - in fact, it's been the hardest month of our lives. Cystic Fibrosis can be isolating for both the patient and the parents/family in many ways (much like other diseases), and at times we're finding that to be true. Unfortunately, CF patients are not supposed to come in contact with one another due to the high risk of dangerous bacteria being shared, so Avery will never have any friends with her same condition or lifestyle, which will be particularly isolating for her. I know for tweens and teens this can be especially difficult. But we are thankful to have spoken with other parents and read blogs/stories sent to us by others and know that we are not alone in this journey. We know there are resources for support.
We are in this for the LONG haul, and now we just move forward with good people and care around us and a strong God behind us.
We wanted to send out an email with some general information about what moving forward is going to look like for both Avery and our family (and you, as our friends and family!). #ittakesavillage :) Many of you have asked to know more about this disease and how it will present itself or change our daily lives, or how it may impact your relationship with her. I hope this email will be helpful for you, but please don't ever hesitate to ask us questions if you have them! We're always learning more and more, too.
For Avery, we don't know exactly what her path looks like as we haven't been through a sickness with her (and hopefully won't for a long time), nor what her lung function will look like as she grows. {More on the actual science of CF below} We do know that the particular form of CF that she has makes her unable to digest food on her own and that her pancreas will eventually shut down that function. We also know that the pancreas issues will likely lead to her getting a "special" version of diabetes at some point in life. For most patients, this will result in needing an insulin pump.
Here are a few other things we wanted to share:
* Cystic Fibrosis is NOT contagious. I know most of you know this, but we just want to be clear that this is a genetic disease and none of your kiddos will get sick because of Avery. She is not "fragile" nor mentally impaired, and we hope she's treated much like all her buddies and friends around her with the exception of being extra careful in keeping her from getting sick.
* Catching a cold or bacterial infection in her lungs is our #1 enemy. For this reason, we will be asking everyone in contact with her wash their hands regularly or use hand sanitizer. What can be harmless for most of us could be something deadly for her. And while asking strangers to use hand sanitizer is not something I'm looking forward to (I mean, we let Benjamin eat off the ground!), I know that it's a small thing that makes a big difference. We also ask that you let us know if you feel an illness coming on so we can perhaps reschedule our get-togethers. We've been encouraged to be diligent about this when we can.
* Avery's daily regimen currently consists of enzymes before each feeding, a special multivitamin (that she hates!), extra salt poured into her food, and twice daily chest physiotherapy. Near the age of four, Avery will adopt the additional lifelong routines that include inhaled drugs via a nebulizer (likely twice daily) and vest therapy that will replace our manual chest physiotherapy (likely twice daily for 30 min at a time). Each of these things will be adjusted during bouts with colds or bacterias, and if she can't get rid of bacteria on her own, she will likely be hospitalized until she can. We expect this to happen occasionally though hope it happens as little as possible. There are many other complications from the disease, but we hope to never face those though will with bravery should we have to.
* Avery will be visiting the CF Center at Children's Hospital about once a month for awhile and then will transition to once every 3 months (for the rest of her life). There they test her lung strength and take cultures to make sure she doesn't have any bad bacterias (among other things).
* Avery will need to be athletic! Physical activity helps keep the lungs healthy, so all CF patients are encouraged to be athletic (which is why you often hear about them being marathon runners!). Cardio activity is actually like a medicine/therapy for them! Matt is hoping Avery takes up a love for soccer like her brother already has :)
* Avery will always need to avoid smoke. For those that smoke, we ask that you don't smoke near her and try to not wear the same shirt around her that you've been smoking in (the smoke particles can still be dangerous). Again, this is related to keeping her lungs healthy.
* Avery will always be on a high-fat, high-calorie, high-salt diet! What?! That is the exact opposite of the diet needed by everyone else in our household :) But, due to the digestion issues caused by CF, this type of diet will help her grow and stay strong. So if you see her eating fatty, high-calorie, salty foods... well, don't judge us. That's doctors orders!
* The average life expectancy for people with CF has increased dramatically over the past decade. It currently sits in the 40s, and we hope that we don't even have to talk about shorter life expectancy issues someday.
* If you ever feel the desire to research CF, please only use www.cff.org as a credible source, otherwise you may find some pretty scary stuff that may or may not be reliable. This has been advised of us by our team at the CF Center.
{And now, the science of it all (skip this part if you don't need the nitty-gritty): Cystic Fibrosis is a life-threatening genetic disease that primarily affects the lungs and digestive system. An estimated 30,000 children and adults in the United States (70,000 worldwide) have CF, which technically makes it a "rare" disease. In people with CF, a defective gene and its protein product (called the CFTR protein) cause the body to produce unusually thick, sticky mucus that clogs the lungs and can lead to to life-threatening lung infections, and in many, the need for a lung transplant. There are a number of different gene mutations (over 1,900) that can cause CF and each affects the function of the CFTR protein in different ways and can result in different severity levels of symptoms - this means that each patient has a very unique version of the disease. Avery's two gene mutations are DF580 (the most common mutation that over 70% of people with CF have and generally presents itself in a less-severe form of CF) and W1282X (which occurs in about 5% of CF cases and is associated with more severe lung issues). We don't know exactly how this combination will present itself in terms of severity, though we know she will have to fight the symptoms of both mutations as opposed to patients with only one type of mutation. We have been encouraged that there are some drugs on the horizon that address these mutations specifically, and the CF Foundation continues to fund research that will hopefully transform the disease. Prayers are always needed that this happens!} There's a lot of information about CF and drugs in the pipeline at www.cff.org.
Thank you again for all of your continued love and support. We'll always need it through this journey. Again, we don't know what to expect for Avery's life, but we want to fill it with LOTS of love and wonderful experiences. Disney World, we're coming for you as soon as she's old enough! :)
With love,
Ali, Matt, Benjamin & Avery
No comments:
Post a Comment