new iMAC and pink vest!

Study drug visit, IV antibiotics, and Bone Density - OH MY!

So yesterday I headed into the city for another Vertex study drug visit. The visit consisted of PFT's, blood work, EKG, a sweat test then I was given a meal and 30 minutes later I took the Vertex pill. I've been sick for a few weeks now with this lingering cough and I'm on vacation from work for a week so my doctor and I decided to start home IV's. I'm definitely not sick enough where I need to be hospitalized. This is more of a "tune-up" I would say. So my sister accessed my port the other day and last night I began what I hope is only two weeks of IV meds. Here's to beating the change of weather and staying healthy!!

Yesterday after the study visit was over, I stayed and talked to my doctor. There are a bunch of new things that I am going to begin in hopes to improve my lung function and over all well being. For starters, I am going to start acupuncture again and make sure that I make it there every week so I can feel the full effect. I started last year - but my insurance at the time didn't cover it - and to be honest I couldn't afford what it cost plus the hassle of getting into the city every week. I did feel it helped though, so I am going to give it another go! My doctors offices are finally moving across town into a new building and she said there's an amazing gym there and a fabulous pulmonary rehab that I would probably benefit greatly from. I am going to try and schedule both acupuncture and pulmomary rehab on the same day so I can just make a day of it in the city once a week!

Next week I am finally going for a bone density scan. I regret that I haven't gone earlier because I really feel that I have some issues now. I have random bouts where my hands and feet just lock up. If I'm holding something tiny, such as a pen my hand usually drops it. Whatever this is - I want to get to the bottom of it - because it hurts like a bitch when it happens and it can really affect my career.

Oh and I ordered the new washable vest, in PINK of course! Can't wait til it's here - I'll put up some pictures!

Did I just jinx myself?

I find it rather odd that today I am on the phone with my doctor discussing my very gross, thick, green mucus when just about two weeks ago I was posting about how good I was feeling lately. I'm not superstitious by any means - but maybe I did jinx myself. My 02 levels are down again - 90% on room air and my heart beat is fast (138bpm) and my cough is, well it's productive and definitely needs some high potency medications.

I am actually on vacation from work starting tomorrow for an entire week. I guess it's good that I am going to nip this in the bud and not have to take anymore time off from work. I also feel like maybe someone is playing a trick on me. Like they knew I had a weeks vacation coming up - so instead of letting me just relax and enjoy - they had to make my lungs revolt so that I have to pay very special attention to them while on vacation.

Feeling crappy

In less than two weeks I've gone from feeling pretty decent to feeling god awful. I am trying my hardest to not let this turn into something but deep down I feel it's time for IV's once again. Oral antibiotics seem to do absolutely crap for me lately. I hate the fact that I know at certain points, when it's more than a little cold and that my secretions are getting thicker and darker that IV antibiotics are the ONLY thing that are going to make me feel better. I have been drinking so much water that I literally run to the bathroom every hour. Trying to flush whatever this is out of my system just isn't working. I added an extra 10-15 minutes of using the percussor (which I dug out of the closet, it hasn't been used in years) after my vest therapy.

As far as medicine compliance goes I have to say I am extremelyyyy vigilent. I make sure that my chest PT is done, my nebs are done and all of my pills are taken. There are however two things that I am still struggling with.

One is exercise. I hate exercising. I really do. I have read SO many blogs and articles about how exercising is the best thing for CF and yet still I am reluctant to make it my priority. I know I have to add it in and be commited - but I don't know how someone goes from having trouble walking up a flight or two of stairs to becoming a full fledged gym rat. I WANT to be that gym rat - I just don't know how to get to point A to point B.

The second thing I need to get more serious with is insulin. I take my standing dose of insulin every day but I sometimes forget to cover my carbs, especially if I am out eating and not in the comfort of my own home. I was diagnosed with CFRD when I graduated college in 2006. For the first three years I didn't have any complications. I took an oral hypoglycemic pill before meals and that maintained my blood sugars at regular levels. The last year however it's gotten worse. For the first time in my entire CF life I had trouble with my weight. I lost about 15 pounds in a short amount of time. I was started on insulin and was very good with it at first. I even bought a journal where you wrote down your sugars in the morning, before meals, 2 hours post meals, before exercise and before bed. My problem now is that I feel even when I count my carbs and take the exact dose that I need 1 unit per 15g of carbs, I sometimes get really hypoglycemic after. And that my friend, is the WORST feeling ever. I get so shaky and dizzy and just feel like I have no control over my body. I absolutely HATE that feeling. I've actually talked to my doctor about this and we discussed possibly getting an insulin pump. Apparently some insurances don't cover it but luckily mine does. So I have the number of this endocrinologist and I am going to give it a shot. It's not that I want another medical device to scream, "Hey, i'm sick!" but if this pump infact does make it easier to not get those dreaded low sugars and I don't actually have to stick myself with insulin multiple times during the day - I figure why not. It can only help. If I don't like it, I can always say no thanks - I'm done. So we shall see!

Fundraiser in MY honor!

This blog is actually a little late but it is definitely something that deserves recognition. Everyone knows the past two years have been hard on me healthwise. I had to stop working for an entire year because my hospitalizations were so frequent and I just couldn't seem to stay healthy for a long enough period in between stays to actually start a full-time job again. I helped out at Erin's Isle (the restaurant I worked at since I was 14) for some extra cash - since my parents were pretty much supporting me. In that time period, I stopped work, moved out of my apartment and back home and spent most of my free time either in the hospital or at home doing IV medications. I unfortunately still had bills to pay, a car payment, student loans etc. My parents and family helped me out as much as they could - but let's face it, the economy has been tough on everyone lately. Joey decided to put together a fundraiser at Erin's Isle where all the tips that he made at the bar that night would go directly to me to help me out with my financial matters. He not only made a ton of tips but he also sold 50/50 raffles to increase profits. The night was a hugeeeee success. So many people showed up that night (I wasn't there because it was a surprise originally). The next morning Joey showed me the results of the night and I was completely floored at the generosity and the willingness of people who truly love me by the support that was given. Thanks to that night - and a little loan from my amazing boyfriend I will be officially done with paying thing off in less than 6 months. The stress that has been lifted from my shoulders is amazing. I am truly the luckiest girl in the world to have a boyfriend as amazing as Joey and a fabulous family, both mine and Joey's!

Hello Fall ..

I actually don't mind the fall weather. I LOVE brining out the UGGs again and comfy sweaters. The thing I hate is all the yucky viruses going around. It seems like everyone has sore throats, is sneezing and coughing non-stop. I am always very cautious and good with hand washing - but this time of the year I am simply OCD about it. Working in a hospital I'd have to say I wash my hands at LEAST every 5 minutes or so. That can make for very dry, yucky hands - thank goodness for hand lotion! I honeslty cannot believe it's already November. Time has flown by so fast it's crazy!

I took a week off from work next week - not to go away - but simply to relax and get my room organized. I think it's time to do a major clean up. I also want to make room for a desk so that when I start back to school I am not crouching down at this little lap desk that I now have my laptop on. For someone who's prone to bone problems later on life - it's probably not the best set up right now!

Here's to the fall and the winter - let the damage not be so bad on my lungs this year! I might just have to buy a pretty new scarf, hat and gloves to keep the cold air out of my bagpipes!

I have been feeling really good lately. I still get random bursts where I am really short of breath but overall I feel like I am living a normal life again! Work is great! It's been very busy in the NICU lately and I really feel like I am starting to get the hang of things. I am learning new things day by day and enjoying each and every moment. I love that I am able to help not only the babies but also their anxious and worried parents. Being a nurse is everything I have always imagined and I am so happy that I did not give up on my goals! I am actually looking into starting school again to get my Masters degree! I already sent the application in - now I just have to send all of my transcripts in (since I took summer classes at a bunch of different schools over the years). I get tuition reimbursement with my job and the program I am looking into is actually not expensive at all because its a city school! I would actually end up almost getting my masters for free! I am applying to specialize in parent-child nursing. My ultimate goal is to remain working in the NICU and start teaching at a nursing school or university on a part-time basis! Ever since I started nursing school I always envisioned myself teaching a nursing class or even clinicals in the future!

Cough

Dear Lungs,
Please stop waking me up in the middle of the night. You are loud and obnoxious and you aren't even productive, so you are essentially doing me NO favors. My bronchoscopy showed nothing of importance and I am NOT ready to go back on IV's - so please just leave as quickly as you came!
Please & Thank You,
Katy =)

Nebulizers/Compressors

Okay so I have noticed that a lot of CF patients use a variety of different nebulizer systems and nebulizer cups.

I was just wondering what nebulizer system does everyone use. What cups do you use. And do you use different systems/cups with different medications?

For example I am on cayston - so I use the Altera nebulizer system and cups JUST for Cayston.
I use the Pari LC cups for the rest of my meds and I use the Pari ProNeb Ultra 2 compressor with those.

Vertex

Taken from the CFF.org website
"Vertex Pharmaceuticals is developing two oral compounds designed to treat the basic defect in Cystic Fibrosis - a faulty gene and its protein product, called CFTR.
VX-770, the most advanced of the two compounds, is known as a potentiator and is designed to allow CFTR located at the cell surface to function correctly.
Vertex is also developing VX-809, known as a corrector, which is designed to move defective CFTR to its proper place in the cell."

In January of this year my doctor's office called me and asked me if I wanted to participate in a new drug trial. It was for Vertex 770. They wanted a group of CF patients who were homozygous (carried two of the same genetic mutations) for DeltaF508. I was one of the few patients at my CF Center that was eiligble for this. I jumped at the chance! In order to qualify for the study you had to have a FEV1 of 40%. Luckily at enrollment date my FEV1 was 50%. I began the 16 week study. My first PFT which is the one that counts was only 38%. But since I had already enrolled in the study at 50%, I was still able to partake. It was a double blind study. That means that both myself and my doctors did not know whether I was receceving the actual drug or the placebo. The study entailed me keeping in touch with the doctors every two weeks. I either had to go into the office for a sweat test, multiple blood draws, pulmonary function tests and EKG's or follow up with a phone interview. The pill was a large blue pill that I had to take twice a day after consuming a high calorie meal. To this day I still am not sure whether I was on the placebo or actual drug but I am glad that I participated! At the end of the 16 weeks you were able to continue on to the second part of the trial if you had either a 10% increase in your FEV1 and/or a decrease in your sodium chloride level from the baseline sweat test that was drawn. I am not sure what my last sweat test result was but, I did have an increase in my PFTs. Day One (FEV1 - 38%). Week 16 (FEV1 - 43%) which calculated to be over a 10% increase.

In June I began the open label part of the Vertex 770 study. The study is going to go over a 96 week period. I am now officially getting the real drug. I still have to go to the doctors for visits but not as often as the first part. When I do go I still get blood draws, EKG's, PFTs, and sometimes a sweat test. I also have to go for liver function tests every two weeks. I got hospitalized at the end of June and my doctors made me stop taking Vertex for a little while because I was running fevers and they wanted to make sure that it didn't have anything to do with the drug. I began taking it again continuously at the end of August. It's been almost a month now of taking it on a regular basis and I really do feel like it is making my mucus thinner.

I am excited for the future of CF drugs. Vertex has two different compounds VX-770 and VX-809 and from what I have been reading - eventually they will be tested together. The two compounds together are supposed to have the best result. One is a corrector and one is a potentiator - so each compound has different effects based on your mutations and the effect it has on the CFTR protein. Research has actually shown that VX-809 has a better result in my genetic mutation (DDF508) - so I am really hoping that my CF Center will soon be testing this compound as well.

Health Renovation!

First let me start out by saying that life has been CRAZY since I last posted! I am still working as a NICU nurse and absolutely loving it! I have now been at my job for 6 months! The time has been flying by. It's really amazing how you don't mind working when it's doing something that you love.

This update is basically about getting myself as healthy as I possibly can be. I know that I have said this all before but after the last few months that I have had - I am more motivated and determined than ever to NOT let CF get in the way of my everyday life.

The past few months have been spent in and out of the hospital. I was in the ICU. Had a systemic infection from another PORT infection. I had fevers up to 108! I required oxygen 24/7 for almost two months straight. My oxygen levels went down to the low 80's with even the slightlest movement. After two back to back hospitalizations, being on oxygen 24/7, missing TONS of work, six long weeks of IV antibiotics, getting a THIRD port and losing a tremendous amount of weight - I want to physically do everything in my power to never get to that point again. Granted - the infection was probably something that I couldn't have prevented - but I wasn't in the best shape physically before the infection took place, so when it hit - my body had no idea what to do. It really took a toll out on everything. When I got home from the hospital I couldn't even make it up a flight of stairs without literally pulling myself up the banister. I was so scared, I honestly felt like I was starting to die. While I was in the hospital I had set up a meeting with my doctor, my mother and my sister because I wanted to ask her point blank if she thought I was getting progressively worse. My doctor, whom I trust very much, stressed that she really didn't have all the answers, but that she really felt I was just going through a rough patch and that I would start to turn around.

It's now almost a month and a half since I was discharged from the hospital and I do believe my doctor was right. I no longer feel that I am losing control of my very existence. My PFT's are stable. My FVC on 9/14/10 was 63% and my FEV1 was 42%. In January my FEV1 was in the 60's. I KNOW that I can do better. I have been following a blog on here written by a fellow CF patient, Ronnie Sharpe. He has an amazing outlook on life and is extremely beneficial to anybody that has CF and is in a funk. His blogs, plus guest blogs that he features on his site have officially motivated me to really give my all to my health. Through exercise, many CF patients have turned their health around and increased their lung functions.

From all of the data that I have seen and written proof that exercise is the key to CF management - it's time I get serious about fitness! I have always been compliant with my medications. Unfortunately for someone with CF, that isn't always enough. I am going to start today - exercising seriously. Getting my body in the best possible shape will help me to be able to work at my job for a very, very long time. It will hopefully enable me to feel well enough to be able to realistically think about becoming a mother when the time comes. Most importantly, it will show CF who's boss!

I have a job finally! My DREAM job!! I am back in nursing and working at a hospital 10 minutes from my house. I am working in the Neonatal Intensive Care Unit (NICU). I ABSOLUTELY love it!!! I can see myself in this area for the rest of my career. If my health remains stable, I am actually thinking about going back to school in a few years to become a neonatal nurse practitioner!

Since February I have been taking a new study drug, Vertex 770. This pill taken twice a day is supposed to work at the cellular level, fixing the main defect in CF - causing symptons to disappear! I'm not sure whether I am on the real thing or the placebo. A month into taking the pill I wasn't feeling any changes and actually felt quite sick. My doctor started me on another round of IV antibiotics. Since then, I have been feeling MUCH better. It could be that the Vertex just took a little longer to take effect in my system, the IVs are whats done the trick, or possibly a combination of the two things helping each other out. Either way, I hope that I stay feeling the way I am feeling now for a LONG time!

Working full time again is definitely wearing me out - but I don't mind! When you are out of work for over a year because you were so sick that you just couldn't - working again is a blessing! I am exhausteddd, especially being on IV's right now. I wake up at 4:30 every morning to make sure that I have ample time to shower and get all of my meds and therapy done. When I get home, I still have two doses of meds to go - but knowing that I LOVE where I am working makes it bearable. As tired as I am waking up at the crack of dawn, I cannot wait to get into work and take care of adorable, innocent babies. Something about seeing what they go through (even though I go through enough of my own stuff), just makes the crap I deal with on an everyday basis, seem less important and not so bad.

This year I redid my youtube video for the CF walk. I am hoping that this year is another huge success! I really feel that with all of the new drugs out on the market now for people with CF, that things are going to be okay. I can't wait to turn 26 and then have a million more birthdays after that!!

2010 = MY year

So this year started out pretty good. I've had a few rough patches to get through but I am finally clear headed and know that things will work out in time. February 11th I am starting a new study drug that is actually supposed to revcrse the effects of CF at the cellular level. This is the most amazing thing that has been discovered thus far. It has already shown an average of a 10% increase in lung function and a decrease in sodium chloride level (this is what causes the mucus to get thick, sticky and cause infections). It's phase two of the study. There will be a group that gets the actual drug and a group that gets a placebo. I am PRAYING that I get the actual drug. Most studies there is a 50/50 chance of whether you will get the drug or not, this study is 4/5, so my chances are definitely better. In the meantime I am still looking for nursing jobs, I feel that it is time for me to get back into the workforce. I'm still bartending a few nights a week, but I MISS nursing so bad! I am looking to become an RN in a neonatal intensive care unit. I actually have an interview monday (so keep your fingers crossed). Monday I am also going to start scheduling in the gym into my daily planner. It's sooo hard to get myself there, but when I am there I feel good. My boyfriend has been waiting on me to get my ass in gear - so I am going to this week! I know that a consistent exercise routine will also help me feel great.