A Long Overdue Update: A birthday celebration, toddler life, home IV antibiotics, and a hospital admission

Phew, it’s been awhile. Hi there!

I thought I would quickly write an update for Fenn and I while our one-year-old little boy took a nap this afternoon. There were some big moments/changes/challenges this summer both for Fenn and I, but I guess that comes with the territory of toddlerhood and motherhood with CF.

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Fenn
Age: 13 months
Weight: 22 pounds
Heights: 31 inches
Words: mama, dada, kitty, ball, hi, bye-bye, night-night, hot, shoe, ba-ba

Fenn turned one-year-old in late August! I can’t believe he is a toddler now! Let me tell you, that little guy had so much fun at his “Growing Up, Up, Up” hot air balloon first birthday party. He tasted cake for the first time and knew exactly what to do with it. He also loved helping us open all his presents and being the center of attention. Even though he’s just a wee little one, he totally understood the party was for him and that he was special and so important to everyone. We loved having our friends and family celebrate our sweet boy and all the joy he has added to this past year.

Fenn has been walking for a couple months now and is quite good at getting around. He loves to do laps in our house and climb up and down the stairs (it gives me a heartache every time, but we do it). He is CONSTANTLY on the move and I am exhausted 99% of the time. We have been working on adding more words to his vocabulary with the addition of baby sign language. I am surprised daily how quickly he picks up on language. We have a talker! It’s fascinating to watch him understand direction and learn to communicate with the world around him, and with us. I love hearing him say, “Mama” and then watching intently for him to motion to what he needs. I know I say this during every new phase, but I am really enjoying this age watching him learn and grow so quickly.

At the moment, Fenn really enjoys reading and looking through books, whether that’s solo or with anyone who is willing to sit read with him. He enjoys listening to songs, will do a little dance, and loves any toys that he can bang together to make noise (hmmmm, our kitchen pots and pans). His other favorite toys include stacking cups, cars, and balls. He loves to climb on everything and can get a little naughty when he wants to. He loves all different kinds of fruit, chicken, various veggies, and puffs at the moment. We are also still breastfeeding three times a day. He is always, always hungry.

Fenn is very social. I’m talking wants-to-be-friends-with-EVERYONE social. He loves going to story time and interacting with the other kids and adults. He loves being around people. He loves giving hugs and playing with strangers. He is definitely a high energy extrovert, meaning I spent 99% of the summer chasing him around. Lord, give me strength, please!

Last week, we had a very scary choking incident with Fenn. He was snacking on watermelon (which he has had 100 times before) and put too many pieces in his mouth without me noticing. He began to choke, and I quickly realized I needed to sweep his mouth to remove the pieces and then use the baby Heimlich maneuver. He coughed the rest out, but was very unsettled, drooling, and acting as if he was in pain. When Peter got home about 30 minutes later, we took Fenn into the ER because my mama intuition told me something wasn’t right.

In the ER, It took a long time to calm him down, and it was the most anxious and helpless I have ever felt as a mother thus far. He had an xray and Tylenol, and then finally settled from pure exhaustion. Because of the serious nature of choking and possible aspiration, the ER staff thought it was best to be transferred him in the ambulance to the main Rainbow’s ER for more tests and evaluation. Right before we got in the ambulance, Fenn puked on me and seemed to be better and in no pain. I think whatever was stuck in his esophagus had dislodged. We were transferred downtown, and our recovered Fenny-Boy had a blast in the ambulance–smiling, gabbing, ripping off his pulse ox, and showing off for the paramedics :|. I was just happy he was acting himself again after the scary non-stop crying and obvious discomfort. I was just happy my baby was back. Once we were there, the Rainbow’s staff checked him over seeing he was obviously improved and said we could go home after nursing him.

Thankfully, Fenn is ok and recovered quickly from the very traumatic experience. I am just thankful I knew baby Heimlich and CPR because I NEVER thought I would ever need to use such training. Everyone is healthy and happy now, but the Lord protected us throughout that night by keeping Peter and I calm and attentive and encouraging Fenn to be a good patient.

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Age: 28
Weight: 106
Lung function: 59%
Words: not as many as Fenn

Personally, it’s been a very challenging summer for me in terms of lung function and health. I started the summer off with two weeks of home IV antibiotics due to feeling tired and short of breath, and eight weeks later I was back on IVs again. IVs were not what I wanted to spend my summer doing but it happened and we made it through feeling better and stronger.

Prior to the most recent round, I felt “ok” but my lung function took a big hit. At my appointment, I blew a 48% and then eventually working my way up to a 53%. I knew I couldn’t sit with those numbers below my baseline and risk a permanent decline, and so I discouragingly made the decision so start three different home IVs again only eight weeks after the first round. I was discouraged. I was sad. I cried on the way home and had to mentally prepare myself for all the work and flexibility IVs demand from our family. I was doing everything right and yet somehow, I still ended up back in that “place”. That’s one of the most exhausting and disheartening parts of living with cystic fibrosis, and it can feel very emotionally painful sometimes. All my efforts to keep myself healthy weren’t enough, I needed to spend this summer with some extra help, and that’s the reality of CF.

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David Blakeslee Photography

I slowly started to notice minimal improvement on IVs, but didn’t feel I was improving, both symptomatically and clinically, as fast as I usually do. That’s when around 5 weeks in to my second course of IVs I started to feel as if I caught the stomach flu due to horrible stomach cramping and no appetite. I waited a couple days, hoping it would pass, but when the pain got more intense and I was obviously dehydrated (we were concerned for my kidneys because of the IV antibiotics), so my mom joined me on a trip to the ER. A CT scan confirmed what I feared; I had a complete bowel obstruction in my small bowel just below my stomach. DUH DUH DUH.

In the ER, they place an NG tube (a tube that goes through your nose down into your stomach) and suctioned the contents of my stomach to give me some relief. I received a bunch of IV fluids, nausea meds, and I felt much, much better. Then, I was transferred via ambulance to the main campus of UH and immediately admitted.

Over the next couple days, I was on bowel rest, nothing by mouth (not even ice chips, uhhhhh), and intermittent suction through my NG in hopes the release of pressure would relax my digestive tract. After I was suctioned dry, we then tried various solutions through my NG tube to try and dislodge the blockage. No luck there. We weren’t sure what was causing the blockage: old mucus, scarring from my appendectomy, or poop (of course). Bowel obstructions are very common in CF, but my blockage was in an uncommon location and so both the surgical and CF teams were a bit stumped at first.

Truthfully, I do not wish a bowel obstruction on anyone. Being filled with liquid when your body can’t digest and remove it, is simply horrible. At one point, my stomach was contracting so badly, trying in effort to move the blockage and solutions and my NG tube had clogged, the surgical resident had to replace it with the largest tube they could fit. Talk about an intense nose hose! My eyes watered like crazy! Thankfully, the first placement was successful, my stomach was suctioned once again, and I enjoyed a couple rounds of morphine after because the consistent stomach cramping was unbearable. I would equate the stomach cramping equal to my drug-free labor. I am as serious as a bowel obstruction, here. I am not someone who takes narcotics lightly, since I have a very high pain threshold, so you can imagine what this experience was like.

After a couple days of no good news, I was scheduled for surgery the following day (something we were desperately trying to avoid). Abdominal surgery is tough to recover from when you cough and have to do airway clearance. Also, one bowel obstruction surgery greatly increases your risk of another bowel obstruction. We were out of time and options. Until that afternoon, my CF doctor burst into my room and said, “If we go to radiology now, I bribed a guy with champagne to do a procedure on you.”

“Cool, lets go,” I said. I blindly got in her wheelchair and off we went.

I’ll leave it up to your imagination of what they did to me. But, I can tell you it included the head of radiology, a very large tube connected to a gallon of liquid, an x-ray machine, and an inversion table….

BUT, IT WORKED!

Thank goodness it worked! And thank goodness, I have a doctor who is willing to hand deliver me to radiology and be the absolute best advocate she can be for her patients. She promised me I would get home to my baby, and she delivered. I feel very blessed I am under her care. I left a couple days later, with all my bowels in tact and a strict order to never eat Pirate’s Booty White Cheddar Popcorn again. That’s right, Pirate’s Booty betrayed me. We saw the evidence with our own eyes. (No this is no sponsored. Please drink adequate water and consume at your own risk.)

Even though it was a difficult hospital stay, the worst part was that my admission was so abrupt and unexpected; we didn’t have a plan for Fenn. Thankfully, our families rallied together and he was cared for and even came to visit twice while I was in the hospital for a week. I missed him so much and felt very anxious about being away from him. I continued another two weeks of IVs once I was home and started to work on gaining the 8 lb. I lost back. After seven weeks of IVs, my lung function went back up to a 59% and we let my body rest. Overall, the summer was fun, joyful, and busy, and we came out both surviving an ambulance ride and and some challenging moments.

Thank you everyone who watched Fenn and cared for our family this long, difficult summer. Here’s to a healthier fall!

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#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

The Reality of Home IVs

“With your port, I forget you’re on IVs- that’s not fair ☹”

I received that text message a couple months ago during a conversation with my older sister, Jessica, who has a way of casually speaking in a manner that people never forget. Jessica talks and in return, we listen, remember, and bring up her wordy influence weeks later. We always joke in our family that she holds so much power in her words without even meaning to do so, and now, she has done it again.

Jess’s reply has floated around in my mind for some time now, and it returned to cognition two weeks ago when I accessed my port and began home IV antibiotics for the second time in nine weeks. Her words weren’t fully absorbed and interpreted at the time of reception and simultaneous iPhone ping, but I can make assumptions based on the context of our conversation and our relationship. My assumptions are as follows: a) because my port is located on my ribcage, covered by clothing, and more easily hidden, people often forget when I am on IV therapy and b) it isn’t fair because IVs require a significant dedication of time, persistence, and infirmity in the hopes of healing. Maybe, she meant it wasn’t fair because IV-laded Janeil deserves more hugs, more leniency, more “How are you feeling?”, more prayers, more flowers from Peter (hint), more rest, more understanding, etc- all the arbitrary gifts of inpatient care?

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Although, I try to avoid using the word “fair” in conjunction with my CF because no good comes from comparison and wishing to be displaced from your reality, I do think it is “fair”. I agree, IVs are a serious commitment and there should be a level of forgiveness towards the person injecting poison into their body multiple times a day, but home IVs are a privilege and the benefits outweigh the anonymity, in my opinion. It is a privilege to stay home, to continue working, and to live without the restraints of hospital walls. Receiving the same treatment that causes many patients to be stuck at Hotel Rainbow’s Babies & Children’s Hospital, Floor 7, for weeks, sometimes months, from the comfort of my own surroundings, allows me to feel free. Acting as my own head nurse, I decide when to hook-up, what to eat, where I can and cannot go, and who to see. I govern the controllable that inpatient care can unfortunately strip away from its guests.

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On home IVs, I am privileged to wake up next to my husband after a night tucked in our bed, instead of the resident asking me if I’m still coughing at 5:30am (yes- the answer is always yes). I receive a visit from my nurse twice a week, sometimes at my house or my work, to draw labs and monitor my kidneys against the force of the powerful drugs. During work, I flush, hook-up, flush, hook-up, flush, hook-up, flush, flush, and un-hook while little fingers pull my tubing. I answer calls from the homecare pharmacy and schedule delivery dates for bubbles of antibiotics to be dropped at my doorstep. I visit my doctor every week and decide if we should push on or call it quits without knowing if we succeeded in pacifying the infections. Weeks on home IVs resemble a compliance-marathon resulting in mounds of medical trash, aggregating co-pays, and days packed with responsibility. Sometimes, I feel poisoned and weary, but mostly I feel fortunate and comforted that I am able to live my days as if there wasn’t an intravenous line hanging from my ribs concealed by my cozy sweater.

If I know anything about maintaining my positive mental status and living with cystic fibrosis, it’s that I desire to feel free- to enjoy my days and accept the challenges of caring for myself with grace and coordination. Feeling untethered seems like a fair trade-off to me, even if onlookers are unable to see the invisible hospital band marking me as a patient. Thankfully, at this point, treatment administered at home works more effectively than when I am admitted. Until the day comes when inpatient care is the best option, I will enjoy my freedom for myself and all those living on Floor 7.

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I have toured New York City, visited the beach, celebrated my own bachelorette party and friend’s birthdays and weddings. I have attended sorority events, worshipped at church, presented college assignments, gone on dates, and enjoyed every holiday imaginable all while on home IVs. I can meet my dear friend, Alexandria, for coffee to catch up and chat about the time we once lived in Little Italy as the antibiotics, Zyvox, Tobramycin, and Cefepime, infuse into my bloodstream. I have the ability to live my life while simultaneously fighting the disease that threatens to take it away. This type of reality makes me smile and seems victoriously fair, don’tcha think?

Three cheers to home IVs, to modern medicine, to breathing, and mostly, to living!

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Health Update: Physical Therapy, Orkambi & IVs

This week was filled with appointments, decisions, and rearranging of medications.

On Monday, I graduated from physical therapy after eight weeks! Wahoo! How pumped am I to stop paying to workout early in the morning on my day off? The answer: So pumped. Really, PT was an excellent experience, and I am excited that it helped minimize the pain in my back from coughing and daily living. In PT, the therapists and I started with aligning and stretching my shoulder blades to achieve better posture by opening up the chest. I was taught different exercises to strengthen the shoulder, arm, and chest muscles that hold a lot of tension from coughing with the help of weights, bands, and foam rolls. Lastly, we worked on cough technique to better engage my abdominal muscles and keep my back straight, instead of contracting and rounding the shoulders on every cough. (CFers- bend forward at the hip instead of the middle back when coughing. You will feel like a new person!)

PT was also a humbling experience to say the least. In the midst of the coughing, sweating, and soreness, I recognized how simple movements and mindfulness of body placement could ultimately heal an aliment I never thought mendable. I am so thankful for the therapists’ expertise and advice. It felt wonderful to treat an issue with positive results and therefore, gain a sense of control in a body that sometimes feels wildly out of my control. Plus, there is nothing more humbling than a high school gentleman watching you do “pelvic tilts” on an exercise ball. Yes, it looked just as provocative and cringeworthy as it sounds, but I could only laugh at the ridiculousness. All in all, I highly recommend physical therapy to all my cysters and fibros out there and to anyone who is suffering from daily pain with little relief.

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On Wednesday, I headed to CF and CFRD (cystic fibrosis related diabetes) clinic with the wonderful company of my dear best friend and cousin, Cara. I told myself going in that I had no expectations as to what my PFT (pulmonary function test) or the plan of action would be. I have learned over the years to go into appointments with little expectation, that way I am able to focus on the positives of a bad appointment or celebrate the victories of a good appointment without feeling weighed down by disappointment. I knew I had been fighting a low-grade fever, increased cough, and fatigue (an on going problem for the past 5 months) but was unsure of what my doctor would say. My PFT was only down a bit- 65% and still one of the highest numbers I have seen in the last 12+ months. Those high numbers are very encouraging, and I still don’t believe this is reality when the respiratory therapist reads off the score as I catch my breath posttest.

The conversation between my doctor and I became increasingly complicated as we discussed my symptoms, life on Orkambi (a drug which targets the cause of CF), and the options available. In early December, I took myself off Orkambi for about a week (whoops) and realized how much better I felt without the drug. I had an inkling Orkambi was to blame for my level of fatigue, aches, tightness, and shortness of breath, even though the tests showed clinical improvements within my lungs. When Cara asked what I felt like, I told her, “Every morning, I wake up feeling like I lived another day in my sleep. There is no break in the fatigue.” Eventually, I was faced with a difficult decision: a) Stop Orkambi and risk losing lung function but feel better in the near future, or b) Continue Orkambi and keep my lung function but feel poorly as I do as a result of the side effects. What would you do if faced with this decision?

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Ultimately, I felt stressed making this choice. I didn’t want to disappoint Peter or my family who felt an immense sense of hope as a result of Orkambi. I didn’t want to be the patient that discontinues a drug thousands of CF patients wish they had an opportunity to be on. More importantly, I didn’t want to risk the possibility of never gaining that lung function back. However, quality of life has always been my top priority, and we agreed that if I am not able to enjoy the benefits of Orkambi after five months because my days are overwhelming burdened by fatigue and aches, what’s the point? So, for the time being, Orkambi will be removed from my list of treatments and I’ll go without.

I do want to say how very thankful I am for the opportunity to take Orkambi for the past five months. It really is a fascinating (and ridiculously expensive) drug that allowed me to cough less, use less insulin, and obtain better lung function. I can’t believe we are at this point in the advancement of CF research, and it is a time filled with so much hope. My doctor ensured me that numerous innovative drugs are coming down the research pipeline, and one of those drugs may be the perfect fit for me in the future. For now, I can enjoy the days while I wait and know that I have tried all the options available to me with a peaceful heart. I am thankful for such a great and supportive care team who continually reminds me that how I feel is more important than a number on a screen.

Now, the goal is to “reset” my body to determine what my daily baseline looks like without the effects of the drug and after the help of IV antibiotics. I definitely didn’t expect to be on IVs again this soon since I last had them in October/November. With the fever, cough, and fatigue, they are warranted. Truthfully, I am relieved, though. I can’t wait to see who I am without the weight of Orkambi and with the help of IVs to nudge me towards a version of myself I almost forgot existed. Goodbye, fatigue and grumpiness!

So, here we go again- no Orkambi, on IVs, and working to feel the very best I can.
I am snuggled in my bed, and 2016 is certainly starting out with a bang.

Health Update

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Last week, I went to CF clinic and followed through the motions that have become second nature: check-in, put mask on, sanitize hands, get vitals and sit down to do pulmonary function tests (or PFTs). I wasn’t expecting much since I have had two colds and 21 days of oral antibiotics in the last six weeks and had prepared myself mentally for a hospital stay and IV antibiotics. After my first blow and subsequent coughing attack, I was told I blew a whopping fev1 of 71%! I don’t think I will ever experience that level of shock again in my lifetime and I want to publically apologize to my PFT lady who I told to “shut up”. I WAS SHOCKED. I also apologize for telling you, “not to joke with me like that”. After I regained my composure, I asked her to look through my records to see the last time I blew in the 70s, and she responded that it was more than two years ago because those numbers weren’t even recorded on the card. SHOCKED. Then the mental light bulb flicked on and I knew this was the doing of Orkambi. To go from 54% in July to 71% in October could only be the work of the recently developed revolutionary drug that treats the cause of CF and not just the symptoms. I also believe it is my dedication to compliancy and the work of all those people praying for me on a daily basis- I feel your support, thank you. I walked out to the waiting room to tell my mom who had come along and she jumped up to hug me while the secretaries and nurses all clapped and cheered. It was a TV sitcom moment and I teared up a little, of course.

My first question as my doctor walked into the exam room was, “Why do I feel so sick if my lung function is so high?” It is true that the last few months have been difficult. I am constantly battling fatigue and for the first time, I am admitting that I have chronic pain everyday. I haven’t felt like myself lately, but a much more stale and weathered version. Most nights when Peter gets home from work, he finds the cat and I in bed resting and exhausted from the day endured, forgoing the preparation of dinner. I have essentially become non-functional in the evenings due to pain and fatigue and we planned to remedy that. Our action plan included a referral to physical therapy for chronic pain due to coughing, to stretch and strengthen the muscles in my back and chest. That referral alone gave me hope that I would feel better soon. We had further labs drawn to make sure we weren’t over looking an obvious issue that could explain the fatigue. Also, I received my flu shot (PSA: Get your flu shot! You are not only protecting yourself but people like me who would become life-threateningly sick from the flu. Just do it, pretty please. Ok, preaching over.). My doctor also suggested a hefty round of IV antibiotics to placate whatever bugs were sucking my energy. She agreed that I am more than qualified to start IVs at home without being admitted to the hospital for 48 hours. THANK YOU! I always thought that 48-hour policy my hospital instated was ridiculous and I am glad she decided to step outside the box for her patient.

And so, here I am a few days into treatment and feeling the effects of the drugs. My weekend consisted of sleeping in my own bed, receiving psychiatric therapy from my cat, using hot packs to ease pain and only stomaching Chick Fil A and Mac & Cheese. I can’t help but feel happy and hopeful though amongst the achiness and coughing. I never, ever thought I would see the 70s again and that just shows that I have been dreaming too small and my faith too little. Time to celebrate with another round of breathing treatments and limitless episodes of Friends!

Processing Change

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I unpack my saline, heparin, needle, cap and dressing change kit and place it all on the table. My iPhone alarm tells me it’s port flush day; something that rolls around every four weeks or so. My port (or portacath) is located on my left ribcage and allows access to my bloodstream for blood draws or IV antibiotics whenever I need them. An internal septum was placed surgically and connected to a long tube that’s tunneled under the skin to a larger vessel in my heart. When my arm brushes against the obvious bump or I go to flush it and I see the inch and a half scar, I am reminded how much I love my port and how thankful I am for it. In all honesty though, it took me years to get here and a challenging internal process to be “ready” for it.

Flashback to a few years ago: entering the hospital felt routine at this point, my symptoms would worsen, my body would feel heavy and weak and I knew I needed IV antibiotics to give me a boost. I always felt at peace going into the hospital. Granted, I hate putting my life on hold, I hate relinquishing some of my independence, and I HATE hospital food, but I knew I could smile, negotiate a weekend stay and be shipped back to do home IVs. Subsequently, I would resume my role as nurse and my life would return back to normal to uninformed onlookers. Home IVs meant PICC lines, a semi-permanent IV tunneled through your arm to the same vessel in your heart but removed once treatment was over, and after 15+ PICCs my veins were scarred and occluded. The placements became slightly traumatic (that’s what you get when you refuse sedatives) and I developed some sort of anxiety equal to the amount of scar tissue hiding in my vessels. I will point out, I am generally not an anxious person (praise the Lord) and the only obstacle standing in the way of my happiness and peace during frequent hospital stays were PICC lines.

I knew it was time. It was time to take the next step in my care and that meant a portacath. The idea of having a port always weakened my soul and I was disappointed in myself. A port meant my body would be altered on the outside and I would physically look irregular. It meant I was really sick and my disease was in fact progressing. It meant I lost control and the force I battled to hold off everyday was gaining ground. It meant CF was winning and I was ultimately losing. Ports were for very sick cancer patients, certainly not for me.

Weeks turned into months, my doctors continually brought forth the option of a port and offers turned into dismissals. Slowly, greater clarity erupted and I saw through my vanity, stubbornness and shortsightedness. The Lord reminded me of content and faith and that with every season comes passing, but also new life. I was starting a new life and I owed it to my groom, myself and to the opportunities ahead of us to do everything in my power to best take care of me. I attended my bachelorette party with my last PICC and it was removed seven days before my wedding.

A year later, my husband comes to kiss me on the cheek and tells me I look beautiful as I go to care for the implanted device that has given me peace and a renewed sense of independence. It is a concealed weapon against my greedy disease and I am thankful for the continuous protection. The bump under my skin proves that sometimes the change we desperately try to hold off can alter our lives in the most positive and forward thinking way. I know that I will need a reminder of this lesson time and time again in this life as CF brings forth unwanted changed repeatedly, but I hope I will always look down to my ribcage and be comforted and reassured as I am now.

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