U.S. Food and Drug Administration Approves Cayston(R) (Aztreonam for Inhalation Solution) for the Improvement of Respiratory Symptoms in Cystic Fibrosis Patients with Pseudomonas Aeruginosa

http://www.gilead.com/pr_1393831

For all my buddies who couldn’t qualify for the study or the ‘compassionate care’ program, it’s your time now!!!

(now for the fight for insurance coverage… fun fun!)

Hi y’all!

I survived the swine flu. I honestly have no idea how I did though. It was horrendous. I was so weak, I couldn’t even lift a bottle of Poweraide Zero to my lips and needed a straw. Since then it has been hard to get back on my feet again.  I’ve had two rounds of IV antibiotics with about 3 or 4 weeks in between. I’ve never needed IVs so close together before. If any of you cfers are debating on getting the shot, my advice is to GO GET IT!  :)  Just make sure it’s the shot and not the nose spray.

Slowly I will be getting back in the swing of things, more blog posts, replying to email, updating the format of the site like I promised long ago.

More to come!!!

Here’s the comment:

Dear Salty:

I think you’re being a little unfair to the FDA. They made a decision the CF community didn’t like — but that doesn’t mean they’ve been unethical as you suggest. And “foot dragging” would have meant no decision at all.

Still, I share your irritation because nobody — not even the CF Foundation — seems inclined to to explain exactly why the FDA decided that further clinical study was necessary. It always gets my dander up when the experts won’t let the regular folks in on the secrets, especially when their work is of such importance to CF patients like yourself.

The decision about which you complain was actually made last September and, as best I can tell, did not result in all access to inhaled astreonam being cut off. From the CFF web site:

“Because the FDA did not raise any safety issues related to the drug in their ruling, Gilead [the manufacturer] will continue to provide early access to aztreonam lysine for inhalation for those CF patients with limited treatment options and a serious risk of disease progression. Individuals with CF should contact their care centers to determine their eligibility to participate in the EAP.”

Presumably you’ve done that . . .?

My reply:

Although I appreciate your comment, it appears you aren’t completely up to speed on the study I speak of. The FDA has made clear their reasoning… the new board is not up to speed (pardon the pun) with the needs of cystic fibrosis patients, nor understands the need of getting drugs to market quickly to help save people’s lives.  As far as “foot dragging” I don’t know exactly how to address that.  The drug was up for review, they asked for more study results, Gilead studied, got more results, appealed the decision, the FDA now asks for another study, which will be reviewed at yet a later date… despite ”the FDA did not raise any safety issues related to the drug in their ruling” as you quoted.  I don’t know if there are better words than to say they are dragging their feet …as I have stated before, numbers are my game, not words so I apologise for my lack of vocabulary. …Yet this entire time  If there are no safety issues are related to the drug, then why on earth are they not approving it? I just don’t get it, and I guess that is what I was trying to describe in my previous post.

The inability to receive proper treatment for one month, may shave YEARS off a cystic’s life, no exaggeration there my dear. Speed means lives being saved. An ounce of prevention is worth a pound of cure my mother always told me. A good solution now is better than the best solution ten years from now.

For example, the year previous to my start on inhaled aztreonam I suffered horribly from lung infections, three rounds of IV antibiotics, at least three weeks in each round. And guess what drug I was infusing in my veins????? – AZTREONAM! Now if I happened to be lucky enough to have enrolled in the expanded access program at that time, I could have saved my insurance company quite a bit of money for the hospitalization, picc line placement, home nursing… the list goes on and on. Plus my employer would have benefited, not only from my ability to work instead of being sick (considering I was sick for weeks prior to each IV treatment), but maybe it would have kept their insurance premiums from going up astronomically (but that’s just speculation).  Not to mention that since starting the study I have not had a single round of IVs, and my need for oral antibiotics (which are pretty much useless to me anymore) has almost deminished.  I have yet to miss a day of work since starting on this program due to lung issues.

An excellent description of why the FDA has not approved the use of inhaled aztreonam, and has denied appeals since September 2007, is spelled out here by the Cystic Fibrosis Research Institute.  I found it with a simple google search, yes, I do my homework. APPEAL TO FDA FOR APPROVAL OF INHALED AZTREONAM TALKING POINTS FOR ADVOCACY (pdf file)

Perhaps the CFF has been keeping the reasoning quiet as to not step on the FDA’s new board member’s toes? You really don’t want to go pissing off someone saying they aren’t knowledgeable of a topic when they have the authority of denying what you are desperately trying to get approved.  Especially when people’s lives are at stake. -makes sense to me.

Not just anyone can be on the expanded access program (link to details on ClinicalTrials.gov), unfortunately. There are 115 CFF approved care centers and only 65 are participating in the study. Also the study is limited to those who have a lung function with an FEV1 LESS than 50%.  Hence, the Expanded Access Program is not open to those with a lung function above 50%, which rules out most children, adolescents and young adults who definitely could use this drug as a preventative measure, allowing them to keep their highly valued high lung function until a cure comes along.  This program does not account for other people who may not have cf, but still culture pseudomonas, or other bugs that aztreonam could possibly treat.

Yes, I am on this expanded access program. I am getting my inhaled aztreonam for free.  If I didn’t think it could help others, I sure as hell wouldn’t be wishing the FDA would hurry up and approve this drug because then I’d have to open up my wallet.  BUT, out of the goodness in my heart, I wish others could get on this drug, the ones who do not qualify to be in the study, because I know for a fact it has extended my life in years, and improved my quality of life greatly.  I wish the same for my co-cystics, my transplanted buddies, and others who must deal with the nasty vicious Pseudomonas Aeruginosa maybe due to severe lung disease like COPD, PCD, and the list goes on.

I must say again, I just don’t “get it”.

I am becoming a better and better judge of the carb count of foods.  I plan on sharing some of my carb counting tricks with you soon. Also, I now always have some Smarties on hand if I go low.  Even my friends have started carrying Smarties for me when we are out.

Just because I am learning more, and having minor success at it, doesn’t mean I still struggle with this stupid diabetes every moment of every day.  I guess you may say that I am a little more comfortable in these new diabetic shoes (not literally, even though croc’s does have some “stylish” shoes specially for diabetics, hehe).  As of this afternoon I have had two icky low blood sugar levels.  One before lunch, 64, and another low at 3pm, 69.  I didn’t feel the 64 at all until I tested. But the 69? Wowza! I felt like I was on a boat lost in the ocean. The world was going round and round. Luckily I was sitting down and had 3 rolls of Smarties within reach.  I guess it is completely normal for people’s blood sugars to drop, even down into the 50’s sometimes, but it is when you start feeling the symptoms, it is time to grab the closest sugar source.  I don’t know how true that is, but for those of us with CFRD it kind of makes sense, but not so much for those with Type 1.  That is just one more difference the two different “types” have.

I do have to admit I have fallen off my healthy eating bandwagon.  I still search for high fructose free foods, but I admit I have been drinking diet soda, which makes me crave those super sweet sugary foods, ahem… cherry sours, junior mints, tootsie rolls… YUM! But UCK! It’s been giving me these highs and lows, thanks to that evil sluggish insulin and the speed the pure sugar hits my blood stream. Not good.  BUT… over all I think I am doing a lot better than I used to because I test my sugar level MUCH more frequently. This way I know where I am at and if I need to take extra insulin I do, and if I need to dial it back I can. Testing testing testing!

I am not exactly sure why I had the lows today.  For a change knew exactly how many carbs I ate at lunch, I actually bolused a little less than I needed since I had a long walk back to work. So all logic said I would have been a little on the high side after lunch, but nooooope!  I have gained a bit of weight recently  (yeaaa me!) about 20 pounds (since September), and thanks to inhaled Aztreonam (an antibiotic I’m in a study for right now) keeping my lungs healthy, I think my insulin needs have changed.  I am planning over the weekend to really buckle down and count carbs to the exact amount I can, keeping track of my blood sugars probably 7 times a day (morning and before and 2 hours after each meal) just to see where my body is going and what it is doing.  I’ll give my numbers to my doc and she will be able to give me some advice on what changes I may need to be making with my insulin levels.  Crossing my fingers that I have the willpower to do this!

P.s. I’m thinking of jumping on the Vlogging (or is it Vloging?) band wagon. My laptop camera is worthless, especially if I am on the run… does anyone have advice of what kind of tiny portable video camera to get?  What would give me the best bang for my buck?
Thanks bunches!  Hope you all are doing well!

(oh! and I am a bit behind on my email… I haven’t forgotten you! I’ll get back with you very soon!)

side note: I have wondered since the extra glucose messes up the red blood cells, I wonder if it affects how oxygen, and how much of it, is carried through the blood stream (since it is also carried by the hemoglobin) which is kind of an issue for some of us cystics with messed up lungs.  I’m going to look more into that someday. But if I find that is true I am going to get even more pissed at the CF docs who put off CFRD testing (not mine luckily). *sigh*

Apparently a “normal” person would have an A1c level at about 5.0%.  My doctors suggest an A1c of 6.5% or a little lower, normal would be ideal, but that is darned hard to reach.  My docs find that above 6.5% it seems to really affect the CF lungs.  To me I really couldn’t understand what that number meant until I found a calculator online that could translate the number into something I was familiar seeing on my little meter tester thingy I use every day.  Here’s one I like, it goes both ways from A1c% to mg/dl.
http://www.accu-chek.com/us/rewrite/content/en_US/4.1.2:20/article/ACCM_general_article_2422.htm
Here’s a little chart on dLife:
http://www.dlife.com/dLife/do/ShowContent/blood_sugar_management/testing/a1c_conversion.html

154 mg/dl is the magic average number.  Personally, what I try to do is make sure I am at a safe, but lower number (preferably 85-95) through out the night since I am spending 8-9 hours at that level.  Recently with my Continuous Monitor I have found I am having a strange spike at 2:30-3am which means I am going to need to change my basal insulin rate at that time… but that is a whole different story.

Once last year I scored a 6.0% on my A1c test (yes, it went on the refrigerator)… I really doubt I am there again, it took a lot of hard work and discipline, but here is some tricks of how I did it…  Having a lower level for many hours while I am asleep made it easier to get to my goal A1c level.  When I spike at meals, I try to keep it under 200 mg/dl at the two hour mark by counting my carb intake as close as I can (IMPOSSIBLE when on evil evil prednisone), but I make corrections at the 2 hour mark if I have to (my pump figures out the calculation taking in consideration how much active insulin I already have in my system, which is why I am such an advocate of the pump, it does all the brainpower).  I have played with my numbers and found at my of 1 unit of insulin (which is an unbelievably small amount, it’s like a few drops!) for every 8 grams of carbs and my correction or sensitivity rate of 1 unit to 25 mg/dl (how much my bg number will drop with one unit of insulin), If I miss calculate my carb intake by 20 (two macroon cookies, who ever eats just two cookies?!) my blood sugar level will go up 62.5 points (mg/dl). HOLY!  That means I need to keep quite a close eye on things.  It never used to be that way when I was on shots, I was on a 1:15 ratio…. but then again, I was never in control of things on shots.

Back when I was out of control, my A1c level (at least the first one I really paid attention to) was 9.3%.  That number alone wasn’t enough to scare me.  But it should have.  The toll it took on my poor body was too much for it to handle and I have not escaped a few complications.  Nothing too scary, but annoying enough to get put on a few extra medications.  I wish I would have known then what I know now.

Just as I was starting to feel better from the freakin expensive medicine allergic reaction, my tonsils started to blow up again like one of those puffer fish. (geez! can’t I catch a break??!)  Back to the doctor I go.  Turns out I got ANOTHER weird bug I have never had before.  Luckily this one can be kicked by some cheep non-allergy inducing (fingers crossed) meds.  For an extra boost I agreed to do a boost of Prednisone (evil evil evil), which has given me some lovely roid rage, blood sugar numbers that go from normal to 450 in 30 minutes and a tummy monster that can not be tamed with no matter how much food I cram down.   I am afraid to say, go away bad juju, that I am starting to feel better FINALLY.

Holy smoke folks!  I never thought I would have this conversation with my docs ever.  Partially because I was reluctant to ask, because I didn’t want to get my hopes up, until I found that special someone to share my life with (yea, I am giving up on that for the time being, until time wears away some more horrible memories).  But my doc said some of the most wonderful things to me.  If I am able to get my lung function just a little bit better, which is very possible and should be easy to do, and keep my diabetes under control, my doc believes I could live a very long and healthy life, which may even include… having a FAMILY!  Oh my! She said the “f” word!  I never really considered this to be an option for me.  So hearing it makes my mind boggle!

I don’t know… maybe doc has been reading my blog and found out that I have been down in the dumps and wanted to lift my spirits about my future… who knows. (by the way, I don’t mind if you read my stuff docs and nurses.. if you are reading this)    But still.  A FAMILY is something that I had never seriously dreamed of.  Much like the people I have dated, I always invisioned myself to have the “expired” stamp on my forehead.  These people have done hell to part of my self-esteem, which you could probably read into in some of my recent posts.  Making me feel like I am less worthy than someone without my genetic mutations.  I look back to see my past wasted with tears caused by people who looked up CF on the web and deemed me lesser than thou.  They hurt me because they were scared of some outdated data.  They were scared I may die (well DUH!).  It is horrible.  And despite this happy news my doctor spoke to me, the tears I have spilled over the years over what others have told me… it is very sad to realize that this cycle will go on, people believing the internet over my word, or even my doctor’s word.  I cry more tears knowing that even though I have been given a blessing of sorts, I may not ever be able to have it just becuase of this damn internet.

What brings us together can also push us apart.

But she still said the “f” word! 

This past year has been super hard health wise, where I have passed a few unhappy milestones and set some disappointing records for myself.  But overall I have had no decline since this year started.  But I feel now more than ever my life slipping away, I know most of it is just mental.  Not helping are the loved ones in my life who some are not realizing that I have been having a tough time, most are living in denial.  Then there are others, mostly friends or people I have dated, who don’t have a clue what I go through and then turn their backs on me because I have had to cancel some plans because I simply did not have the energy to go out of the house or because I caught yet another one of their colds.  This year has definitely shown me what makes a true friend.  I am coming to terms that CF is a very lonely disease.

From those who I dated, it hits harder than ever, the words still echoing in my head, “I looked up CF on the internet….”  now I stop them in their tracks.  I know what comes next… blah blah blah… you are going to die… blah blah… I am just not strong enough to deal… blah blah… It scares me… blah blah blah.  HELLO?! Have you come to terms with YOUR OWN morbidity?!  They make me feel like a charity case.  Wasting their time until Ms. Perfect comes strolling along.  I have heard this story from almost every single person I have dated.  I used to try to plead my case, try to get them to understand that they are mortal too, that they have no idea what their own future could bring.  Treatments are coming out by the bucket-load to help prolong my life, so goodness knows what my future will hold.  But it always falls on deaf ears, so I no longer try.

Thanks to those who have attempted to point me to the social internet sites where others with CF can bitch, moan and complain about their ailments, I appreciate your attempt… but seriously.  Knowing that others are going through the same thing really doesn’t help that much.  I want what most “normal” (as if there is such a thing) 32 year old women want.  So far, there is nothing stopping me but kismet, which I am promptly growing tired of the lack there of.  I see my friends getting married, having babies, getting divorces,  getting re-married.  Am I ever going to be able to experience those things?…  I don’t understand why I am so… second-class? disposable? undesirable?

No wonder why people with chronic illnesses end up with depression.  Not as if dealing with the illness is hard enough, but having to deal with people who are so uncaring… or maybe they are just out of touch with reality.

Physically, I feel alright, my health is stable (even though I had some rough bumps in the road), but I constantly have people are pulling me down emotionally.  I do have bad health days, but I think it is because of my emotions more often now than before.  Being sad makes me feel sicker.  Taking care of myself is taking up more of my time, but it is only because I have more of a desire to stay healthy, live longer, it is my last hope of a somewhat “normal” life.  Even if I am the only one hoping.

For the past several months I have had a slight obsession with the show You Are What You Eat on the BBC.  On one episode the host, Gillian, describes how intestinal gas is formed in the average digestive system.  What she said (in my confusing words) is that if you eat your slow digesting proteins before your fast digesting carbs, the proteins act kind of like a stopper and the carbs sit, digested, rotting, fermenting, and producing gas.  This is how “normal” gas, (dare I say FART! hehe!), is made.  I have no idea if her theory is valid, but it kind of makes sense to me.

Now with CF, specifically in pancreatic insufficient folks like yours truly, the digestive tract is a bit slow thanks to the lack of water between the poo and the walls of the intestines (epithelial cells, CFTR malfunction, I’ll spare you the details for another post), this could cause a longer digestive process causing the fermenting fast acting foods to ferment further and make even more rotten stinky gas.  (oh so pretty, I feel pretty!).

No idea if I am on the right track here or not.

So I will share some things that I have changed recently in my life that has helped me with my gas issues… (except for when I cheat, then all bets are off):

• Drinking lots of water (I am never really good at that and would much rather drink coffee)

• Not eating red meat (no idea how this is related, but there seems to be a connection for me and my body and gas)
• Eating more fruits and veggies

• Taking Miralax when I am starting to feel a bit backed up and bloated.

Anyone else want to take a stab at the causes of gas?  Do you have some home remidies to prevent gas?  Go ahead, it is a funny topic so be as silly as you want!