Monday, August 25, 2014

Minding My Own Business

So, I was just trudging along...  I had been minding my own business, doing exactly what was expected of me.  I had a tough summer after needing to have abdominal surgery, again, for something totally unrelated to CF.  Yet, I was under the false assumption that my CF was neither under control, nor flaring.  It was just there.  Every few months I added an oral antibiotic to my normal treatment regimen, but for 2 years I was feeling like I had things under control.

I set up an appointment to see Dr. Olivier at the National Institute of Health in Maryland for late July.  I made the appointment months in advance and it was just supposed to be an opportunity for me to "check-in" with the Mycobacterium guru.  For the past 4 1/2 years I have been steadily battling first mycobacterium abscessus and then mycobacterium avium complex (MAC).  MAC is the lung disease that patients with AIDS typically die from...it's rare and difficult to treat.  People with CF are susceptible as well.  I took 4 antibiotics for 3 years to combat the MAC and even though I was never able to completely eradicate it, I thought I had knocked it into it's place.

It turns out I was wrong.  The MAC is back...  My CT scan from NIH showed new infiltrates and nodules in my upper lobes.  Basically, the MAC is eating it's way around my lungs.  I'm so stinkin' frustrated.  I worked so, so hard.  All of those pills, the IV's, the precautions...none of it worked this time.  I have to start all over again.  I'm currently back on 4 antibiotics, I may eventually do a course of IV's and there's a possibility I will need to spend 3 days in the ICU being desensitized to an antibiotic I'm allergic to, but need to take.   I have an appointment with my local team on Thursday.  We'll figure this out.  It's certainly not the end of the world.  It just gets a bit exhausting...

One a positive note, I turned 39 last week...who would've thunk.  Every year is such a blessing!  I also sent my kiddos back to school last week.  I haven't written in this blog for a long, long time.  These little guys have grown a lot since then!  Emma started middle school (6th grade) and Jake started 3rd grade.



Tuesday, May 21, 2013

A Little Pill...

Just a quick little post to ask for any little  prayers, good vibes or positive juju you can spare.  I'm heading to Pittsburg tomorrow to be screened for the Kalydeco clinical trial.  I really want to get accepted for this trial.  Any number of things could make me ineligible, but I'm thinking positively. 



I have a few friends with my gene combo on Kalydeco and it has changed their lives!  I want to feel better, I want to breathe better, I want to cough less, I want to have more energy.  This pill has done it for them! 

I tried to see if my insurance would cover the Kalydeco off label for me, but they didn't like the $307,000 per year price tag.  So, I'm going to try to get on it by going the research route.  The other thing I'm hoping is that if I get accepted that I don't get placebo for the first 6 months. 

That's a lot of hopes and wishes for one post!

Tuesday, April 9, 2013

A Pain in the...

RIBS!!!  Ugh, I have a pain in my ribs.

Last time I wrote I was contemplating my treatment plan.  The clinical trial medication I was taking did not seem to be working as I was still culturing MAC.   I had been hacking so intensely that that tears would stream down my face and I would gag and nearly puke.  Joy...pure joy!  After getting tons of fabulous advice from you, along with a lot of contemplation I decided to stop the clinical trial drug.  Since then my coughing spells have decreased, but not completely ceased.

A few days ago I developed really intense pain on the right side of my chest.  This was like, "Holy Crap!  Why did somebody just stab me with a hot knife!" kind of pain.  Apparently, when you have CF and you report this to your Pulmonologist he promptly makes you haul your butt in for a CT scan complete with IV contrast.  That happened yesterday.  We had to rule out a bunch of scary sounding stuff like pulmonary embolisms, pleural effusion, pleurisy and such.  I have had pleurisy (inflammation/infection of the lining that surrounds the lungs) and I was convinced that this is what I was dealing with.  However, the CT was clear of any of these major complications, even though my left upper lobe is ugly.  Hugh sigh of relief.  However, this means I'm dealing with a rib injury.  I very likely tore the cartilage between my ribs.  I did this about 15 years ago in the same area, so I've probably re injured it.  Ouchie!!!

Not much can be done.   Pain is pain is pain is pain.  I can deal with pain.  The most concerning thing is that I can't cough, do my Vest, work out or do any kind of airway clearance until the intensity of the pain dies down.  I want to cough.  I really do.  I need to cough.  I just can't cough right now.  My impulse to avoid the pain outweighs my impulse to cough.

So this is one of those fun little "extra" things we experience with CF.  I know others of you have experienced this type of thing.  What have you done for airway clearance when this happens.  Has anyone tried a rib belt or binding the ribs?  Does it work?  Any tips or tricks?  Right now it's just 800mg of Motrin 3 times daily and rest.

Tuesday, March 19, 2013

Quandary

I've been  mulling something over all day, so I have decided to ask my blog readers for feedback.

I got a text from my awesome CF doctor today.  He told me that my latest culture came back positive MAC.  For 5 months I have been on a clinical trial for Arikace, an inhaled antibiotic to treat resistant MAC, in hopes of getting my MAC infection under control.  I have appointments, including cultures, monthly to monitor my progress.  In December and January my cultures were free of the MAC infection and I was hopeful that I finally found something that worked.  Today I found out that even if the Arikace was helping at one time, it is no longer doing the trick.  I wasn't surprised, because my lungs have been having a rough time for the past month or two.  My doctor said that I can go off of the Arikace if I choose.

He's my quandary...  I am 5 months into this 6 month clinical trial.  If I withdraw from the trial, none of my information will be able to be used in the final trial results.  I have spent many, many hours at appointments and doing the required treatments for this trial at home.  The coordinators at my hospital have spent countless hours offering this trial.  I am the only one at my center enrolled in this trial.  My lung function has been constantly declining for the past few months.  I have no idea if this is due to the study drug or just CF in general.

So, with one month left should I withdraw from the trial since it is no longer providing me benefit and MAY actually be making me worse.  Or should I stick it out.  I am lined up to start the Kalydeco trial in Pittsburgh 30 days after I finish the Arikace trial.  If I withdraw a month earlier, I will be able to start Kalydeco a month early.

I'm leaning toward seeing through my commitment with this current trial, just because I think it's important to follow through with commitments.  However, I feel like I might feel better physically faster if I quit.

What would you do and why?

Wednesday, February 27, 2013

The Good, the Bad and the Exciting!

I had my monthly CF clinical trial visit on Monday.  I went in feeling fair and expected to just  do my routine 4 hours of testing that I have come accustomed to since beginning this trial.  I left with a lung exacerbation that I didn't know I had, two new prescriptions and a head full of confusion.

My PFT's dropped 5% since last month and I had been down about 3% at my previous appointment.  I am down roughly 16% since last year at this time.  The numbers are definitely going in the wrong direction.   The doc put me on Avelox (antibiotic) and Spiriva.  Apparently, Avelox will do something that the other 4 antibiotics that I take hasn't been doing.  Three hundred dollars later I headed home with these new weapons in my arsenal.  What are you gonna do?!?!



At my appointment Dr. D and I discussed Kalydeco, again.   He really wants me on it.  My gene mutation DF508 & R117H is considered a gating mutation, just like the G551D mutation that Kalydeco is FDA approved to treat.  We discussed ways to convince the insurance company, then decided that I would just ignorantly take a prescription for Kalydeco to my pharmacy and see if I could sneak it through.   That plan was foiled when we learned that there is a huge paperwork process to apply to get the drug.  

Then Dr. D came up with the brilliant idea of seeing if they are still recruiting for the R117H Kalydeco study at another facility. I didn't qualify due to the my lung function being higher last year.  This year I would qualify...my decline may be a bit of a positive in this case.   We knew that the study is wrapping up, but it was worth a shot.  I would need a center that would agree to take me in May, since I need to finish this current clinical trial and allow 30 days for the current drug to flush out of my system.   I called the Vertex people and they referred me to www.clinicaltrials.gov for a list of study sites.  I called the closest one... Pittsburgh Children's Hospital.

Here's the exciting part!!!  They said that the study would be open through May and they would love to have me come be a part of it!  I'm keeping my fingers and toes crossed that nothing changes that would disqualify me for the study.  This is good stuff!  The best part is that if I get in on the study I can stay on the drug off label until it's FDA approved.  I won't have to fight with the insurance company.  I know I am blessed...but this makes me feel doubly blessed!  This feels like an answered prayer :-)


Friday, February 22, 2013

The Waiting Game

Hello World! It's been a while and I have no excuse, except for feeling a bit boring lately. A couple of readers have requested an update, so here goes...

My health is no better, but not tremendously worse. No huge complaints! I am a bit disappointed that my lung function is back to where it was before I started all of this ridiculous treatment for my Non-Tuberculosis Mycobacterium two and a half years ago. I'm also frustrated that I'm still culturing MAC, but I guess that's the nature of this beast sometimes. I just wish the LOADS of antibiotics that I have been taking for 2 1/2 years straight would have showed some tangible progress. I want clean cultures, Dammit!

In October, I started a clinical trial for the MAC. The trial involves replacing the inhaled Amikacin that I was taking twice daily for once daily inhaled Arikace. The Arikace is the same drug as Amikacin, but it is attached to a lipid that allows it to absorbed deeper and better that Amikacin. The other benefit is that I inhale the Arikace out of a Trio nebulizer, which makes treatment time about 12 minutes. My inhaled antibiotic treatment time decreased by about 48 minutes! I'm really liking the ease of taking the new drug. Another benefit of the clinical trial drug is that it is not supposed to cause the same hearing damage as the Amikacin. The bad news is that even if it's working and I prefer the new drug, once the trial is over in May I have no option to continue. I have to go off of it and wait for FDA approval (years) before I can access it again.

This is the same frustration I'm feeling about Kalydeco. If you are not part of the CF world you may not know about the exciting new drug that has been discovered that basically corrects the malfunctioning CF genes. It was named as the Most Important New Drug of 2012 by Forbes Magazine. For some patients symptoms and progression of the disease have virtually reversed. Lives have changed! The catch...this drug is only FDA approved for 4% of the CF population. Only people with a certain genetic mutation have access at this point. I'm not included in that small group. The good news is that Kalydeco is being trialed in those with my genetic mutation ~ Delta F508 & R117H ~ and the trials have shown impressive improvements in some of those involved! I want this drug and I want it now (said in my best Veruca Salt voice). Unfortunately, I have to wait until this drug is FDA approved, as well. My doctor said this may be another 2-3 years.

So here's the deal...I am very slowly watching my lung function decline, but still holding my own. All while I'm waiting for clinical trials to progress and the government to give me access to these drugs that could rock my world. I asked my insurance company if I could go on Kalydeco off label, but they were not impressed with the $300,000 yearly price tag. I am going to appeal and fight for access to the drug, so we'll see. I feel like I'm desperately trying to save as much lung function I can before my sliver of hope arrives.

Other news in my world... I retired from my career. Whew! This was an extremely difficult and emotional decision for me to make. Trying to juggle a full-time job, 2 (sometimes 3) children, a husband and Cystic Fibrosis has always been chaotic. For many, many years I thrived on the chaos. It made me feel like I was able to do it all. This became more difficult after the NTM diagnosis 2 1/2 years ago when my treatment plan became much more complex. To be honest, I've been faking it for the past couple years. I have been exhausted to the core, sick and struggling. When something had to give, my health got put on the back burner. I knew that this couldn't continue. After a particularly exhausting business trip that left me with another lung exacerbation I decided to go on Short-Term Disability and explore my options. That was in early August. I never went back to my job. I retired...a bit earlier than I expected I would, but I knew it was time. I had the privilege of being with my company for 13 years. I really felt like I had done everything I wanted to do in my career up to this point. I didn't have aspirations to climb the ladder any higher. After 5 1/2 months of trying to determine if I should go back to work or not, I decided that now was the time to devote everything to my family and my health. I am very happy with my decision now, but, jeez, that was a stressful and difficult decision to make.

I guess I had more going on that I thought! I am going to try to post a bit more often. I've missed my readers...

Here's a little song that's been speaking to me lately.


Wednesday, August 1, 2012

Boiling Over

Warning!!! I may complain a little in this post. It's where my head is right now. If you are looking for happy thoughts you can skip ahead to the very last paragragh. Happy reading!

Do you ever feel overwhelmed to the point where you feel like you can't make good decisions? Do you ever feel like your running in place, and getting nowhere? Do you ever feel like you're trying to do everything for everyone, but you end up doing nothing well and ignoring your own needs?

Unfortunately, these feelings have been crowding my my life as of late... My surgery went well in May, but it's now two months post-surgery and my body feels broken. A few weeks ago I got the news that my MAC (Mycobacterium Avium Complex) is back. What sucks is that I never even got to a point where I could take a break from treatment. At this point I'm considered as having refractory MAC. This is such and evil and tricky (and acary) bug. The good news is that there is a new clinical trial (Arikace)starting within a month for people with Refractory MAC. I am first in line at my CF center to start this clinical trial. I'm hoping that this inhaled drug will beat this bug into submission.

(this is one day of antibiotics)

The bad news is that I have been feeling lousy. To say that I have been fatigued is an understatement. I had a clinic visit last week. I got put on another antibiotic. That means that I now take 5 antibiotics a day. For 2 years I have been taking 4 antibiotics a day, yet the infection is still brewing. How is that possible? I am now taking inhaled Amikacin, Azithromycin, Ethambutol, Doxycycline, and Moxifloxacin. I have tried the power of "positive thinking" to no avail. Will this infection ever succumb? Why am I so, so unbeleivably tired? Will my belly ever get used to the insult of all of these medications?

After feeling really, really lousy for several months, I finally decided that I had to take some time off of work to get my health in check. I need to focus on my treatments (all 4 hours a day of them) and this clinical trial. I have pushed and pushed and pushed through...for the past 2 years of this treatment. I have told myself that I can do anything I put my mind to. Lately, my body has decided to revolt. I have finally decided to listen to my body, instead of trying to convince myself that all is well. Growing older with a progressive disease can be really, really hard...

I turn 37 later this month! That's life expectancy for CF, isn't it? I'm looking very forward to blasting past "life expectancy"!