#31daysofCF

Happy, happy June! The month of May, or CF awareness month, is long gone by now but I saw the #31daysofcf challenge on Instagram in early May and liked the thought of it. Each day you are to cover a different topic in the realm of CF and I thought it very informational. (Also, you’re very welcome I saved you from enduring 31 pictures of CF on my Instagram account last month- that’s just too much. I need more cat pictures, I think.)

Below are pictures from the CFF Great Strides Walk at the Cleveland Zoo. The biggest thank you to those who purchased a t-shirt, joined us in walking on that very cold day, or donated to our team. You made it a very happy and humbling month!

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1. Diagnosis: I was diagnosed at age 4, which is slightly unusual (2/3 of CFers are diagnosed by age 1). I had no respiratory symptoms but suffered terrible stomachaches, stomach distention, and poor weight gain. The doctors were really clueless and frankly, didn’t believe anything was wrong. A very persistent doctor drove my mom to a nearby hospital and a sweat test showed I had CF on August 4, 1995.

2. First year- worst year: I don’t remember much of the first year because I was a 5 year old and as happy as a clam. I do remember my sweat test and my favorite nurse (which I still see to this day) explaining my pancreatic enzymes could taste like strawberries in my imagination. My mom probably has a different version of the first year: I had just been diagnosed and shortly after, my father was diagnosed with terminal pancreatic cancer. I am sure for my mom this was the most challenging and heartbreaking year of her life. She was and is the best caretaker in all realms of her life.

3. Medication $$$: Medication costs with CF (any chronic disease really) are astronomical. Orkambi, the first medication to treat the cause of CF, retails at $259,000 year- not even kidding. Pulmozyme, an inhaled medication I take twice a day, is $72,000 a year. Of course, I have primary insurance that covers a large portion of these costs but even the co-pays per a month would bankrupt you when you take 18 different medications a day. I am VERY, VERY, VERY thankful I qualify for an adult CF program from the State of Ohio which covers the remaining cost of my medication after my primary insurance. Hospital stays, doctor visit copays, clinical tests, and home IVs are a completely different story. It ain’t cheap being sick, ya’ll.

4. Illness + hospital admissions: I have lost count of my total admissions over the years, but my first admission was at age 11 for IV antibiotics (which is pretty dang good by CF standards). My longest stay was 41 days in high school. In college, I started “dorm” IVs so that I could continue going to school and reduce my hospital stay to only a weekend. Last year I was on IVs 5 times, which has been my worst year yet. Thankfully, I now bypass the hospital completely and begin IVs at home because my doctor is a rock star and that’s where I am most comfortable.

5. Diet: High-fat, high-protein, high-salt and high-calorie. This is why I am always eating :D.

6. Clinic: I have been going to the same clinic at Rainbow Babies & Children’s Hospital in Cleveland since my diagnosis and love the staff there. I could not say better things about my clinic. I even serve on their CF Quality Improvement Team as their only patient representative.

7. School: I am thankful that God has blessed me with intelligence and quick comprehension. In 4th grade, I missed 114 days of school due to CF and still don’t know how to multiply fractions. I had a tutor some years to instruct me when I was absent for extended periods of time, but I made it to Case Western and graduated with a Bachelors in Anthropology, Minor in Psychology and a Certificate in Global Health (still in the mix of getting my masters), so I did all right, I think?

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8. Non-compliance: Middle school and early high school were my non-compliant years. I didn’t physically feel like I needed my treatments and I was a snotty teenager. My mom had it rough for a while and we fought a bit before I got my head out of my butt.

9. Greatest accomplishment-biggest challenge: This is a tough one for me. I think my greatest accomplishment is just being genuinely joyful. The Lord has blessed me with consistent happiness despite everything, and I am grateful I wake up feeling that way most days. Lots of CFers (people in general, too) have anxiety and depression, and thankfully, I don’t suffer from either of those. My biggest challenge has been reminding myself that I need to stop comparing my life to the lives of my normal peers. It’s ok I don’t work full-time in a career, have a 401k, and I hop in bed at 8pm most nights. My life looks a little different and comparison is the thief of joy.

10. Pros + cons: Pros- Unique perspective, gratitude, faith, empathy, focus, strength, relationship with my mom and sharing my experiences. Cons- fatigue, pain, coughing, mucus, limiting my ability to work, feeling dependent on medication and others, and the financial burden.

11. P words: PICC line, port, pills, physicians, poop, pseudomonas- that’s all I have.

12. Cracking point: My cracking point was probably last fall. I was tired all the time, not sleeping, started Orkambi, suffering from chronic pain, and felt pretty horrible but my lung function was pretty good. Working full time was exhausting me and I had little energy to enjoy life otherwise. It was very confusing and I felt very burdened trying to be “normal”. Now, I feel much, much better after finding a better balance!

13. The first time I heard that big word (or phrase): “pregnancy in CF”. I remember being 13 and getting “the talk” from my peds CF doctor. Back in 2003, he said having a baby when you have CF is difficult but some women do it. You have to work for the opportunity to be pregnant and after that it was more of an “if” than a “when”. Other options included using a surrogate or adoption to preserve health, otherwise planning a pregnancy was important. That stuck with me forever because up until then I didn’t realize my disease would affect my ability to experience typical milestones.

14. Out of the mouth: “You’re so little.” “You eat a lot.” “Are you sick again?” “Do you need water/cough drop?” “Where have you been?” “You’re still hungry?”

15. Organ donation: Donate your organs, it’s really as simple as that. Double lung transplants, kidney transplants, and liver transplants are fairly common in end-stage CF. So many people pass away while waiting on the list due to organ shortages. Make your passing a time filled with hope and thankfulness for another suffering family. P.S. I am an organ donor as well!

16. What is that?: I hear this most often when someone sees my Vest for the first time or when I am in a bathing suit and my port is visible. Sometimes it’s just a puzzled side-eye look, but don’t think I don’t know what you are looking at.

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17. My support system: My mama, Peter, Sybil (of course), my siblings and cousins, and special friends in the CF community.

18. Hygiene: Germs are dangerous to CFers. When we catch the common cold, the chronic infections and the cold virus battle for resources in our bodies and can exacerbate our CF and become really serious. I don’t live like a germaphobe but many CFers do in order to stay healthy and out of the hospital. Wash your hands, cover your cough with your elbow, and get your flu vaccine, please.

19. Smoking: Ugh, smoking. Of course, I hate it. On one side, I don’t understand why you would choose to damage a perfectly good organ. But on the other side, I recognize the control addiction can have on someone, even someone who loves you and sees you struggling to breathe, and so I don’t really get upset when I see my family members smoking. Everyone needs grace and I have the opportunity to give it to them.

20. Family: I am the only one with CF in my family but we are unsure how many members are carriers. Within my family, two brothers married two sisters causing a group of double cousins who will also especially need to be tested before they have children, along with my siblings.

21. CF friendship: The CF community is crazy connected. Mostly, people talk online since we are encouraged not to be around each other due to infection control guidelines. It’s really hard not to be able to hang out in person with the only people in the world who “just get it”. I have bended the rules a bit this year and I don’t regret it!

22. The life of a CFer: Managing CF is a full time job. I do my Vest around 14 hours a week, treatments around 20 hours a week, cleaning and managing equipment around 4 hours a week. I take ~35 pills a day and continually watch my blood sugar. Add all this on top your average life of working full time (I work part-time now), managing a household, and having time and energy for a social life…. It’s almost impossible.

23. 5 minutes of fame: Salty Girls, working on The Salty Life Magazine, getting recognized in a townie bar for this blog- so funny!

24. Biggest wish: My biggest wish is to carry a pregnancy, be an exceptional mom, and have a nice garden to sit in with Peter. Underwhelmed by that wish, haha?

25. Biggest fear: My biggest fear, both on a micro and macro level, is missing out. I have extreme FOMO when my family hangs out without me because I am busy, too tired to do anything, or stuck in the hospital. I can’t handle it. I also fear I will miss out on life- pregnancy, raising kids, reaching milestones, or even silly family parties after I am gone. Also, that one day they will remove Friends from Netflix.

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26. A legacy like no other: I want to leave a legacy of empathy. CF has taught me so much about other people’s pain and I feel like I have an ability to experience their pain with them. I think bearing witness to someone’s struggle is one of the most important things you can do to support them.

27. My wish for CF: A cure for the people who feel like giving up, for the parents with children with CF, and for the young adults struggling to make really difficult decisions at such a young age.

28. Remembering those who lost their battle: This past year I signed onto facebook and saw too many posts about CFers passing away. It was a tough year for our community. Each passing is just as sad as the next, and I am always so heartbroken for their families they leave behind. I never talk about the life expectancy of CF on my blog, mainly because it’s not something I choose to focus on on a daily basis. But the reality is, expecting to live only to your early 40s is not good enough. People fight so boldy with this disease, and I always take a moment to remember them when I feel I am taking advantage of my own health. I am so very blessed to be where I am.

29. Fundraising: Fundraising is so important since CF research receives almost no federal funding. Thank you to everyone who supported our fundraising endeavor by purchasing a Flowerlungs t-shirt or joining us on the Great Strides Walk! Your support is so very appreciated and humbling and you are making a difference!

30. Raising awareness: The past year has really been a time focused on raising awareness about CF for me personally. I get to live this special life and therefore, I have a responsibility to open up and educate those around me. To me, awareness brings understanding, understanding builds empathy, and empathy accelerates change.

31. Thank you: Biggest thank you for reading this and making it to day 31! I hope you learned a tid bit and continue to enjoy your month- whatever that looks like.

-J

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Holiday in the Sun

I come from a family of devoted vacationers. Like those before me, I thirst for a relaxing and equally exciting break from the hustle and bustle of modern living. For me a vacation is a period of time set aside to heal and rejuvenate my body, an opportunity to post better Instagram pictures (Kidding…or I am?), a warning to cease all calls with the insurance company and pharmacies (I know they’ll miss me), and a moment to just enjoy being alive with the people I love most.

My husband Peter and I just returned from a break with my family in sunny Florida. We had a great trip, and it’s bittersweet coming home and jumping back into the reality of our lives. All those phone calls I didn’t make were still waiting for me as we drove home, but I missed my cat so by the time we hit the Ohio state line I was eager for familiarity.

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Here’s my guide to vacationing:

p a c k i n g

Preparing for vacation with CF is an unique adventure to say the least. When packing, I usually gather all my medication and equipment first before packing any other items since they are the priority when space inevitably starts to run low. On this trip, I brought both my vests, two weeks of medication in a 7-Pack pill box, two nebulizer machines, six nebulizer cups, extra nebulizer tubing, a mini sharps container for needles and diabetes lancets, my glucometer and supplies, microwave sterilizer bags to clean my nebulizer cups, and the rest of my human things.

Am I too prepared? I understand it sounds a little extreme to bring two nebulizers, but I can’t explain the sheer panic that wells up in your body when your nebulizer dies while you’re out-of-state. Been there, done that. #neveragain

I wouldn’t consider myself a light packer. Yeah, I am pretty sure I am the complete opposite of a light packer. It comes with the territory of a chronic illness and someone who owns six bathing suits. It used to bother me that I could never spontaneously pick up and wanderlust my way through the world (I could but we would have to strap my vest to Peter’s back, and that’s just cruel and unusual punishment).

Now I realize, I still have the physical ability to leave as long as I bring my “baggage”, and that’s all that matters. You have to learn to adjust in life. For me, bringing all the necessities stings less when I remember my friends with CF on the transplant list not being able to venture beyond a 3-hour radius of the hospital. I am grateful for my freedom, as always.

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t r a v e l i n g

Since we decided to drive to Florida and would be stuck in the car for extended periods of time listening to the audio book, The Life We Bury, I had to do my treatments on the go. Using the cigarette lighter with the help of a power inverter to complete my nebulized drugs, I used my Afflovest which is battery powered for therapy. Each time before turning on the nebulizer, l silently prayed I wouldn’t blow up Peter’s car en route. I just know that wouldn’t be a good situation for our marriage. Also, the Afflovest looks like a throwback early 90s child life jacket, and I am positive the nearby cars thought I was sporting just that. My advice to myself: throw them the peace sign and crank up Justin Bieber. #belieber

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t r e a t m e n t s

One of my favorite times during vacation is sitting down each morning, coffee in hand, to do my treatments in the sun. Something about the change of scenery makes the entire process a little less daunting and mundane. I am always rushing during my normal routine, so the chance to leisurely complete my treatments surrounded by my family members seemed like a vacation in itself.

I warned my family prior to our trip that we would be completing one page each day from Start Where You Are: A Journal for Self-Exploration (Meera Lee Patel). Most members obliged during morning treatments, and it was so great to hear about everyone’s hopes for the future, what they valued most, and their perceptions of themselves. I enjoy learning about people in anyway I can, so I really love how this book allows you to ask the important questions.

t h r e a d s 

If we are being honest with ourselves, CF bellies are the absolute worst. I’ll never understand why scrawny arms and legs are partnered with obvious stomach distention in this disease. For all the coughing we do, we should be rewarded with rock hard abdominals. That’s not reality, though. Oh, well. To give myself a waist and the illusion of hips, I rely on wearing high-waisted bottoms while combing the beach or relaxing in the pool at the house. They are the absolute best when mixed with flash tattoos that even my grandma loved sporting on the beach. #buddahbelly

Just below my top on my rib sits my portacath as part of my tropical uniform. I know people probably stared and were left to puzzle what purpose the bionic bump serves. Truthfully, I am more indebted to that port for helping me than I will ever be to a stranger’s critical eye. It’s important for me to remember that. So, I wear my port with the utmost pride because beauty is not only skin deep.

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h e a l i n g

Even though I had taken time off from work, adventured on kayaks, and spent most my days collecting freckles in the sun, the reality is that a vacation from CF just doesn’t exist. About half way through our trip, I felt my body beginning to break down and slow down. Due to breathing in the naturally salty air and too much mermaiding (probably), Wednesday night I started coughing up blood which is very normal in CF, but less normal for me. My mom, who can detect my unique cough in a grocery store during flu season, came into my room and suggested it was time to drink tea and rest. After a night in bed reading A Life in Men (Gina Frangello) and stopping my inhaled sodium chloride treatments for a couple days (CFers inhale salt water to restore moisture in our lungs and help clear them), I felt better and restored.

Slowing down and yielding to the healing process of CF, especially on vacation, can seem impossible. I suffer from an extreme case of FOMO (fear of missing out) with my family which causes me to push myself, sometimes beyond my limits. I am still learning to find the right balance between experiencing, managing, and healing in my little world of chronic disease. A holiday in the sun always serves as a good reminder to slow down and enjoy your time doing whatever you are doing. Embrace the JOMO (joy of missing out).

Thanks for a great trip, Florida! I miss my family, the dolphins, and early bird karaoke already. The restoration before the busy spring and summer is exactly what we needed.

Salty Girls Book

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All of last week, I eagerly waited for a very special package to show up on my door step. Each day that passed, I grew more excited and finally, Friday evening the anticipation was over and there it was: my copy of Salty Girls: The Women of Cystic Fibrosis! I spent the majority of Saturday morning, coffee in hand, reading the overwhelmingly honest and brave stories and admiring the beauty of all 77 women. Each woman photographed in this book has CF and has allowed the reader an intimate and vulnerable view of what true beauty and strength looks like. The photographs show smiles, sexiness and scars, while the stories read courage, perseverance and spunk. To my fellow Salty Girls, I am amazed by all of you.

I want to publicly thank Ian Pettigrew, a Canadian photographer. He is the mastermind of this grand movement and campaign for CF awareness. He also has cystic fibrosis himself and I can imagine how tired he is at this moment. Ian has traveled the country to take each photo and he designed, composed and shipped each book all by himself. He is such a hard working man and deserves praise for being a true catalyst for change. He stood up for us when the world told him not to do it and he allowed women with CF to have a voice. He gave us a moment to be strong, beautiful and flawless, all while owning our bodies and experiences. Thank you, Ian! You have forever changed the world of CF.

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Go buy a Salty Girls book and support the immense courage it took to do something never done before and display your body and vulnerability for the world to see.

http://thecfproject.bigcartel.com/product/saltygirls-the-book

A New Beginning

I did it.

I got my act together enough to make this blog happen. If you believe it or not, this blog has been a distant thought for almost 4 years. I continually put it off for when I was sicker, when I had more time, or when I was ready. A close friend was quick to remind me that the blog might never happen if I don’t begin now, in my present where I felt my voice was unnecessary at this stage. She was right. I am just now beginning to be vocal about the reality of my CF on social media after almost 20 years of “being a CFer”. Why? I really don’t know. I think it’s a number of things that have changed my perspective and given me the bravery to be honest and transparent.

First, Salty Girls has played a big part in this change. In April, I posed for a photo book about women fighting CF and the many challenges we face physically, mentally, and emotionally. It was my first time directly meeting other women with CF and knowing that we had similar scars, schedules, thoughts, fears, and never ending coughs. After the shoot, I got in the car with my husband; my heart felt full and I smiled, “They all coughed like me.” It sounds silly now, but that brief sense of community has changed me forever. I feel a duty to my fellow Salty Girls and to myself to educate people about our lifestyles and the bravery the world doesn’t see.

In addition, I am sicker despite my compliant efforts. In college, I did all my treatments, IVs, pills, etc., everything that someone with CF has to manage, but I was healthy enough to compartmentalize my disease and my other life. I lived in two separate worlds and I had enough energy to be present in both, while keeping them as distant as possible. That has changed. I have aged and my disease has gotten worse. Subsequently, my CF is everywhere now. It has grown into almost every aspect of my life and I would be stupid to continue to try to prune it, instead of embracing the change. I can’t eat, breathe, sleep, or move without CF being present. Basically, I can’t exist without recognizing my CF as an important aspect of my life. Every decision, every part of my day, and a very large part of my identity includes cystic fibrosis now.

And so, I am scared. I am scared to write about my life and my experiences with the belief that people might care. I want my friends, family, and strangers to see the reality of living with a brutal chronic disease, but more importantly I want you to see the joy that can persist in spite of pain. I want to put a face to that universal struggle of being happy in your present when things haven’t gone the way you planned. I believe with awareness comes understanding, followed by empathy and lastly, change. So, welcome.2015-10-13_blog-post