As cystic fibrosis awareness month comes to an end, I’ve been trying and trying to come up with something to share.
There’s no lack of thoughts or words, I just can’t seem to get them organized with everything going on in our offline life with CF. This post is all I can muster, and I’m going to share some links that I’d love for you to check out.
I realize that I’ve skipped some context, blog-continuity-wise, so… sorry? But in a nutshell, our little Joanna was diagnosed with cystic fibrosis shortly after birth through the newborn screening (aka the heel poke).
Anyway, I’ve recently gone through a new wave of things sinking in for me about CF, the gradual realizations that I’m guessing come up for family and patients when a lifelong disease is involved. This time my realizations have revolved around: 1) the reality of being a special needs parent, 2) the persistent, complicated nature of cystic fibrosis when it comes to related medical issues and 3) navigating life with others as the pandemic recedes but our girl is still just as much at risk from not only Covid-19, but all the garden variety pathogens we will encounter in the world and haven’t had to worry about for most of her life because we were staying home.
That’s all heavy, but life is also very good! Joanna is a bright, curious, funny, smart, sweet, kind little girl who is growing in amazing ways. Just over the past week she has become a TALKER, imitating most of the words we say to her and using an ever-growing list of her own words. (And of course, “no” is one of them. 😆) We have a LOT of fun and are excited about every new stage when we get to see more of who our girl is growing up to be.
Joanna is so strong and becoming a real champ with all of this CF business, knocking back meds and participating in her care as much as a toddler can. We have graduated from having to hold her and her nebulizer during treatments, to her sitting in a chair by herself– what?!? We are also looking forward to her turning 2 this fall, because then we can start talking about an exciting possibility: modulator drugs that work to repair the hallmark cellular dysfunction of cystic fibrosis. Joanna will be eligible for one of these drugs based on her age and having the most common CFTR mutation, but they’ll have to do a little more testing to make sure the risks are worth the benefits. Still, we’re excited that she’s part of the generation of CFers who can start these drugs at a younger age and hopefully avoid/lessen some of the effects of the disease over time.
But please don’t miss this: for all her adorableness, for all the fun things she can do, she does have a disease— one that currently has no cure. The invisible nature of CF can be a blessing but also a curse, because it makes it easy to forget how vulnerable she is and how different life is for us.
Please remember these things about our family: First, we will always be dealing with something related to CF; it doesn’t let up, so we can’t either. It may “just” be the twice-daily treatments or it may be more, like extra appointments, a hospitalization or complicated insurance issues… but there are ZERO days when we can forget about it. Second, germs are our mortal enemy. We are still figuring out how to do life with this in mind.
We have also been in survival mode for the past few months, as Joanna’s reflux and vomiting mysteriously ramped up in March, and we sometimes see as many as 7 episodes of projectile vomiting in one day. Sometimes it’s like a little baby spit-up and other times it’s like a pint-sized volcano. We are working on it with her team, and thankfully she has not lost weight during this time, but it’s got us all pretty wiped out. (This may not be directly related to CF, but GI problems are very common for CFers, so even if it’s “unrelated,” CF still plays a role.)
As we talk with other CF caregivers, we’re hearing that the first five years can be the hardest. Kiddos grow and change so much during that time, and as parents we are finding our footing and processing the realities of a disease like this. So we’ll be the first to tell you that we need the support of our community.
Please feel free to ask us questions about CF. I didn’t know ANYthing about it when we first got Joanna’s diagnosis, so I can imagine many of the people we know are in the same boat we were 19 months ago. An easy way to ease our burden just a little is to understand more about what a CF diagnosis means for an individual and their family. Here’s a basic overview from the CF Foundation.
If you’d like to know what to do for a CF family (or really anyone with a chronic illness or special needs), here are my suggestions:
1- Ask questions, listen, empathize, then get creative about helping. Sometimes I can articulate what I need, but when I’m up to my neck in laundry and feelings and research and phone calls and questions for medical professionals, I do not have the capacity to come up with ideas for how someone could help me. I need people who know enough about my life to make an educated guess. Good starting points (for myself or others you know) would be the basic stuff anyone can do: cleaning, cooking, and errands. Super bonus points if you have the time and heart to learn some of the actual caregiving so there is someone else who can occasionally do a treatment or a feeding. We have had friends do laundry, cleaning, and pharmacy pickups for us and it is such a blessing. Prayer is wonderful, too. 🙂 This post does a good job of describing the CF caregiving experience.
2- Respect our boundaries around germs. We are trying to develop routines around this and are still figuring it out, but simple things like washing/sanitizing your hands (and your kids’!) and staying home when you’re sick go a long way for us. We’ve already had a few friends tell their children that they need to be careful about germs with Joanna; that’s looked different during her different stages (i.e infant, crawler, walker) but I have so appreciated their taking that on so I can relax just a little bit when we’re out and about, which we hope can be a more frequent occurrence this year. Here’s a short piece about a CF patient’s perspective on cold & flu season. Hygiene, masks and distance have been part of the CF vernacular since well before the Covid-19 pandemic, and some of those things will remain a part of our lives forever.
3- Support the cause. We do not yet have the energy to organize walks or other fundraisers like that, and admittedly my energy for advocacy is often spent in the adoption realm, but if you feel led, there are some great organizations that help our family in amazing ways (as well as doing excellent work in advocacy and research).
The CF Foundation helps us tremendously through their free Compass program for CF patients & caregivers. I can call any time and speak with a case worker who can answer my questions about… well, a lot of things, but it’s always insurance! They also provide legal assistance but we haven’t had to use that service yet. I’m convinced our first case worker was an angel sent to earth to hold my hand (virtually) and teach me YEARS worth of knowledge about navigating insurance. I can call or email Compass to ask about equipment, coverage, preauthorizations, etc. They do the legwork and get back to me either saying they took care of the issue for me, or that they will call the pharmacy or insurance company with me to figure it out, or give me a detailed list of steps for what I need to do. They provide valuable peace of mind and save me hours every month. The CF Foundation is also involved in research, advocacy, support, and accrediting CF care centers (including Joanna’s clinic here in Houston). They do so much more than I can say here, and probably more than I know. (Our local chapter is Houston/Gulf Coast)
The Cystic Fibrosis Research Institute (CFRI) has provided me with a virtual community of moms as well as mental health support in the form of a counseling grant. I was able to attend their virtual moms’ retreat at the beginning of this month, and it was such a lovely experience to hear from other women who know what I’m going through. I heaved several sighs of relief hearing them talk about daily treatments, worries and fears, triumphs, and the expectation adjustments we all have to make (like using screen time to distract our children into eating more and doing their treatments!). I’m able to keep in touch with these women to ask questions and give and receive support. CFRI does a lot with research and advocacy, too. For example: there is a LONG way to go with racial/ethnic equality in the diagnosis and treatment of cystic fibrosis, which was once thought of as only occurring in people of European descent. This has resulted in delayed or missed diagnosis for people of other ethnic backgrounds, and will require a multi-faceted approach to bring about equal access to testing and treatment. Ethnicity aside, there is also a great variety of CF mutations, some of which are extremely rare and do not qualify for life-saving drugs. So yes, there are a few modulators that work very well for the majority of the CF population, but there are still many patients who cannot benefit from these drugs.
It’s not CF-specific, but The Healthwell Foundation has been a lifesaver for our family. They provide grants to cover out of pocket medical expenses for people with a number of different chronic conditions, including CF. It’s interesting, because the front page of their site mentions “underinsured individuals,” which I wouldn’t have identified with because our insurance has been pretty good and seemed like a normal plan to me. However, because of some factors like higher deductibles and copay accumulator programs, we do fall into that category for the purposes of receiving a grant (yet another advocacy issue!). With the majority of her meds and equipment that specifically treat CF symptoms, Joanna’s Healthwell grant can be billed as secondary insurance, saving us thousands of dollars. Fun fact: Joanna’s individual insurance deductible was met by the beginning of March this year. The cost of her digestive enzymes and one inhaled medication– just two items– could meet a deductible within three months.
Well, I think I’m spent. Thank you so much for reading about our family’s experience with CF.
If you have questions, or are a fellow CF patient or caregiver and want to connect, please don’t hesitate to reach out! Comment here, or find me on Instagram @deep_delightful_life. That account has been mostly adoption-related, but as I start to come out of my new parenthood/CF/Covid haze, I’m finding more capacity to engage with the CF part of our story.