Cheriz: My life with Cystic Fibrosis

I'm a hugging type person. In fact, I have a couple friends that aren't big on hugs or any kind of public displays of affection. However, they usually bring the bubble barrier (as I call it) down to hug me good bye. I'm also super extroverted and LOVE to hang out with friends.... Therefore, not being able to see my friends (with CF) in person drive me nuts. A lot of people (in society) don't realize that people with CF cannot be around each other. This is due to the bacteria we colonize in our lungs. It doesn't affect non-CF individuals. But, as we come in close proximity with other people with CF those bugs spread and continue to damage our lungs faster. We fight off the bacteria with antibiotics, but when I was younger we didn't have many options for antibiotics (especially nebulized). This meant we could not eradicate the bacteria and were stuck with it in our lungs for the rest of our life. The more bacteria we culture, the faster our lungs are damaged, t...

Typically, I've been very stable as an adult. I'm very lucky for that stability. However, 2018 started out nowhere near where I wanted. I was admitted mid March for a virus. We aren't sure what virus I had (every test came back negative). However, I had a consistent fever over 102 the entire 5 days I was in the hospital. Once I was fever free for 24 hours and I started eating better, they let me return home. I was excited to leave and get back to my everyday life. But, considering now a month later (and re-admitted), I think I should of just done a two week tune-up last month while I was in already. Live and learn. As you know (I'm sure) I have been trying to get my lung function back up to around 55-60%. 60% is my goal and it really is the highest my lung function can get, due to all the permanent scarring. I started Orkambi in October 2016. At that time it was the newest gene-modifier drug on market for CF patients (with my mutations).  After going on Orkambi I droppe...

I haven't really felt up to blogging or doing anything since I got sick. I have been having a few breathing problems and more asthma attacks the last two weeks. I had my regular Cystic Fibrosis Clinic check up on Tuesday of last week. I knew it wouldn't be great, but I had no idea how bad it really was... At clinic we found out my lung function dropped majorly from my "baseline" of 58% to around 38%.  My Respiratory therapist responded "Dang, that's transplant range, we gotta get those numbers up." It's scary to think one sickness, one medication and my lung function can go from 58% to 30s. Where I struggle to breathe and could be listed for a transplant. But I have faith, hope, and trust. This is why. My doctor(s) at the CF Center are very proactive! My doctor told me I had a couple options: 1. be admitted 2. Stop Orkambi 3. Keep doing Orkambi, but switch up some of my meds at home and call in a few days for update Of course... I decided...

In my last post I talked about how exciting it was to be finally check marking some boxes off my to-do list! My eyes are still healing really well. I only get an occasional headache if I try to read or stare at a screen to long. I know it will take time, but I struggle with patience.  Shocker. I know. Well, one thing I have to be REALLY patient about was getting Orkambi! As you know this was something Dr. B was contemplating putting me on back in March. Then I switched CF Centers (Read about why in THIS POST ) and my new Doctor thought I should definitely try it. Orkambi is a gene-targeting med that tries to correct the defective mutation (DF508) and just came to market in July of 2015, but it has bit a price tag attached. Orkambi costs a quarter million dollars per year. Which means if I take the drug say til I'm 71 (its an easier number for math reasons) insurance will have spent 10 million dollars alone on this one medication for me. So you can see why it is a bit of a struggl...

Big News, Big Times, Big Change: I had my post hospital Doctor's appointment on April 21st and it went well. I posted a video on my CF facebook page. My weight is back on track, heading up. Also, my lung function was around 55%, so that's almost my baseline. I got to take my needle out that night. OH BOY!! I loved being needle free! Whoohoo no more IVs!!!!! In the last week I have been super busy with a few different things. 1. Work. I have a new client AND I had all my post licensing webinars & exams this last week! 2. More CF Foundation Volunteer work. I held a presentation in my home town, where I discussed CF, the different mutations & severity of classes, as well as fundraising for great strides. I talked about the history of the CF foundation, care centers, and more about our local IL CF fundraisers. Overall, it wasn't a big turn out, so I was a little bummed. My hometown has always supported my CF cause, so it was disappointing not to have a good numb...

Weekend Luck: Doctor's offices are open Monday through Friday. Yet I always start feeling sick on Fridays. Luckily, I don't get "sick" too often. Which is pretty lucky considering my pathetic immune system. My immune system can't fight anything and even a simple cold could become more serious and life threatening. For example:  I had to break down and call Doc's after office hours (this morning) to figure out what to do, thanks to a simple cold sore, AGAIN! Yep, you heard me right. Again, thanks to a cold sore. Not sure if you remember in 2013. In 2013:  I had a cold sore that turned my  mouth and lips full of cankers, sore, and lesions. My jaw/mouth was so swollen that I couldn;t talk (which is really funny if you know me, since I gab all the time) or barely swallow liquid. My throat and tongue covered in bacteria and fungi (my tongue was covered in pure white - it was thrush). My fever reached 104 and my pulsox dropped into 80s, Andrew took me to...

Hit a Snag: Well, unfortunately we cancelled my doc's appointment for today. So I do not have an update on my lung function for you. I know, sounds crazy to cancel right? Actually, it was the smart thing to do. Considering our plan was for me to take prednisone for 6-7 days and do a Collistin Nebulizer treatment 3 x a day didn't work out too well. I got the prednisone filled right away, I had the pharmacy and Doc office receive and set up my new nebulizer on Monday/ Tuesday. However, the insurance needed a prior authorization and  they have a standard 15 days to decide if I get the new nebulizer. Since I'm already taking Cayston this month (another antibiotic nebulizer) they will prolly (yes, I always say & type prolly) deny it and we will have to fight it, etc. This is why it's hard to get lung function back after being sick. It can take so long to get the meds and treatments that we have twice as much work once we do, and sometimes its already permanently dama...

July 2nd, What a Day: First, I must tell everyone that it's my mom's birthday! That's right my amazing mother is celebrating her birthday today. As you may know... I'm very much attached to my mother, probably too much. Not gonna lie. I call or text her at least 5 times a day. Judge me all you want. But she ain't you're stereotypical mom. She never tells me what to do, she never judges, she is more of a best friend than a parent at this point. Granted I still call her for an awesome casserole dish recipe, or advice on something I'm doing for the first time, for the most part, she is just someone I like to chat with and share lots with. Plus, she has been helping me with  wedding & CF walk stuff. Here is a post I wrote about my mom on Mother's Day that explains EXACTLY how amazing she is  right here ! SECONDLY, she got a pretty amazing Birthday present. Today is day that Vertex's Orkambi was FDA approval for patient use. What does this mean? W...

The Weekend Cancelled: Yesterday was my 30th Birthday. Crazy huh, to think 30 years... dang. I kinda love it. However, it didn't end up quite like I had planned it. I had been having Arthritis problems in the last few weeks ( as you may have read in my last post ). I didn't even realize I was sick until it hit me all of the sudden. I had temps for two weeks around 99.5, but that's common with my arthritis. So it didn't occur to me until later Thursday Night... I spiked a 102.2 fever, was coughing a ton, my lungs felt really tight & crappy. My breathing was faster. I ended up calling Doc B on Friday morning. He told me I needed to stay home & rest, drink plenty of fluids, and he put me on Augmentin & TamiFlu (to be safe). Saturday & Sunday I just got worse with my temperature not going down with tylenol or ibuprofin. I had to cancel my plans to go to the Bridal Expo, cancel my Bday Party, and we couldn't go home to see our families on Easter.  M...

Follow up: First of all, look Andrew & I are making banana bread. It's either to celebrate the good news of the purple machine arrival or console me for not getting it.... We'll see. And yes, for those of you who know Andrew went to culinary school. This WAS a joint effort. haha, I cracked the eggs & put the banana in..... and we used our awesome Christmas present (Love our KitchenAid). If you aren't quite sure what I'm talking about... a pretty purple machine? It's been the topic of conversation on my Facebook page called " Cheriz: Living with Cystic Fibrosis "  (consider "lliking it" to keep updated on everything) and I've also talked about it a little bit on my blog over the last 9-10 months. See, It's about getting a new Cayston Machine.  Cayston is one of the nebulized antibiotics we (CFers) can take to keep infections & the bacteria in our lungs at bay. For example: 80% of CF patients have psuedomonas aeruginosa in ...

Juggling the Medical Stuff: I'm glad my life has been busy, since being busy keeps me focused. Plus, I love it , which most of you probably already know. I just prefer to be busy with more of the non-medical stuff  rather than medical crap. I feel like I'm a pro at Juggling life and medical problems.  For example: I'm getting married this August and I am planning the Great Strides Walk in Princeton (June 27th). I LOVE planning events, I'm super organized person (to a fault sometimes).  I wanted to make sure to plan the  wedding in Jan/Feb, so I could focus on the walk in April/May. Both events mean a lot to me and I wanted to make sure to plan each the best that I coud. And I'm pretty excited that I'm turning 30 in about 1.5 months!!!! How exciting is that???  For a long time I thought I'd never get the chance tobe 30 years old, or marry, or grow!  But I am, thank you to new research!  My lung function is around 52% which is okay, but it is at the ...

To Do a Bucket List for Cystic Fibrosis: Lung function is one never ending battle we face. We can do all our meds, be compliant, and try really hard, then still see a decrease in lung function. We never know where we will pick up some bacteria or bug that will undo the work we have done! Its exhausting to think I do 9-11 breathing treatments and over 3 hours of therapies a day and see a decrease. I hope someday this will change. I had Clinic (regular CF appointment) on Monday. My mom went with me to keep me company. I live an hour and 45 minutes from my Doc office, so it can make for a long day. This appointment was faster than most (lately I've had Trial appointments at the same time). My lung function (FEV1) was 52% by my Doctor's standards. Normal (non-CF lungs) tend to be between 90-120%. I'm okay with 52, but I wouldn't say I'm happy about it. My lung function has been pretty stable in the 50s lately. BUT, my goal is to always keep it above 50% & stri...

Just a Lil Update: If you haven't read my last blog, you may want to do that so you know what I'm talking about ( read it here!) .  As you know I had a lot going on last week with the clinical trial, picnic, and then getting sick. Monday I called Doc and got some oral antibiotics prescribed. I'm now on (month's worth of) Cipro and doing a two week round of inhaled (neb) Tobi. As you know I've always done Tobi my whole life on and off every other month switching it out for Cayston. Since doing this clinical trial a month and half ago I've been off antiobitic nebs to test the effectiveness of the new drug. Now, it just so happens I had been doing great since off the nebs. Even on Thursday of last week I had a high lung function that hasn't dropped at all soince been taking off the nebs. But unfortunately, I caught something over the weekend and got sick. Of course the first thing that comes to my mind is "Oh no, I hope this doesn't hurt the trial...

Stomach Issues, Gee, Wonder Why: Let me start by stating that, I take a lot of pills. Its not a big deal and all of them are very important. I take vitamins, oral antibiotics, some oral steriods, pills for acid reflux, pills for arthritis, and then enzymes every time I eat.  But lots of people don't realize how crucial those pills are to me (well, to us CFers) I take 5-6 enzymes with meals and 3-4 with snacks (so 30 enzymes, and 10 other random pills a day). I have been good about taking them. I hate that Cystic Fibrosis affects my digestive system so much. If I can't digest anything, I won't have the nutrients I need to keep healthy, or be able to digest the food to keep weight on. Over the years, the enzymes have gotten better and help us absorb more. I've been on Pancreas, Creon, and now have been on Zenpep for the last couples years. I love my Zenpep ( read the RX list for Zenpep here ) However, the enzymes weren't effective en...

CF Climb, Get Some Motivatin Going and Figuring Out What To Do: So this last month has been crazy, in -out- in- out of the hospital. Glad to be home finally, AGAIN! lol. I'm doing a lot better, just have to take it easy with what I eat for a week. I'm more concered about school/effects on my health:  I'm unsure what this will do to my classes. As of right now, my school is telling me I need to drop 2-3 of my Graduate Classes, and they want to me to go to school part time. Making my degree a 3 year instead of a 2 year program. I'm just not sure If I can give that much time to it. Which sucks, since I'm getting all A's and I love going to class and doing research! I have goals, plans, things I want to do. Being in school for 3 more years is not part of the plan...It is just frustrating, because while I'm in school ( If I'm working too) I struggle medically. Regardless if school is part time or full time, we still have to committ 2 years of working ...

Chicago Weekend: I requested off from both my jobs, I don't have any play practices, etc; And I'm going to Chicago this weekend! I've been looking forward to the trip ever since Christmas! It was part the of the Christmas present Andrew gave me! I even got to sleep until 9:00am this morning! Now, I'm just doing my meds and packing my bag for Chicago. I've been doing as much as I can medically since my Clinic Thurs. I was so disappointed in myself. I can't afford to let my numbers drop like that. Not if I want them to increase more. Things I need to start doing EVERYDAY: 10 nebulizers 1 hour of my vest (once I get my new one) eat at least 2,500 calories sleep at least 6 hours take all my pills do my inhalers exercise/lift/jog (at least one of them) take at least 1,050 calories through my stomach feeding at night. If I can fit this into my schedule everyday, then I will be doing awesome! If I stick to this, then I can have a rewarding clinic on May ...

No IVs for me, thanks! First off, Let's clap, BECAUSE it has been 2.5 years since I have needed antibiotics, oral or IV (other than my bi-weekly dose of zithromax). Literally, I have not been ill enough to see my doctor in 2.5 YEARS! That's amazing. I used to get really sick every couple months, but thank goodness, that hasn't been the case. Although I have been working my butt off these past two years to get healthier, guess it is working. BUT... I was really nervous Dr. B would throw me in the hospital because I've beeen sick for a week and lost quite a bit of weight. However, he is having me take Cipro instead! I'm taking the usual (for me that is) 1,500 mg daily for two weeks! If I'm not feeling better & eating better by Saturday, he is going to add prednisone;Which I have never taken before. My mother was on an extremely large dose after she went through Chemo for a while and seeing the side effects kinda freaked me out a bit, but my dose will be s...