Hello? Hello! Anybody out there?

[peeping my head up out of the ground]

Wow.  I just realized that my last post was on July 29, 2011.  I honestly didn't realize I'd been gone that long.  You know what they say about having good intentions - I've been meaning to put pen to paper (or hand to keyboard) for quite a while but I have let time escape me.

I think most of my family and friends have kept up with me on Facebook, but I needed to write a longer post to write the update I've promised to so many.  I'm home from the hospital, but still not functioning at 100%.  I was in the hospital for 28 days in December (the second longest stay I've ever had), for a visit that just didn't go as planned.  Here's a quick overview of what's happened.

• November 10th - Clinic visit.  PFTs were down and probably worthy of IV meds, but I wanted to try and "plan" my next admission so that it fell between Thanksgiving and Christmas.  My doctor and I made an agreement that I would be seen in two weeks and do a 14 day course of Avelox. He made me agree to an admission if my next FEV1 was less than 45%.

• November 22nd - Clinic visit with my nurse practitioner.  An odd day, because my PFTs were actually higher than expected, but I sounded like a freight train was rumbling through my chest.  I "presented" as being sick, so my NP wanted to admit me.  Once again, I begged and pleaded my way to holding the admission until after Thanksgiving.  I was already scheduled to be off the remainder of the week (Wednesday, Turkey Day, and Black Friday), so I would be able to rest and finish out the 2nd course of antibiotics that my ENT had prescribed.  "Patient reluctantly agreed to admission on Monday" (as my nurse stated in my progress notes).

• November 28th - Showed up (suitcase in hand) and prepared myself for 21 fun-filled days of doctors and antibiotics.  For all intents and purposes this was supposed to be a "routine" visit.  Through many years of trial and error, I have found that 21 days of antibiotics gives me my best outcome - bringing any infection under control and giving me a boost to stay out of the hospital for the next ten or twelve weeks.

Let the fun begin...

At my first clinic visit in November I talked to my doctor about the hip and leg pain that I had been experiencing since September.  As it had been getting progressively worse, he agreed to order a hip x-ray and MRI to see if we could find the cause of the problem.  The results:  two compressed discs in my lower back (in between L4 & L5 and L5 & S1) that are strategically positioned to sit right on the nerve root.  This explained the sharp, stabbing pain I was feeling in my hip that radiated down my leg and the numbness/tingling sensation in my foot.

By the time the x-ray and MRI were ordered, my pain had worsened significantly - bringing me to tears, barely able to walk from my bed to the bathroom.  While my normal "drug of choice" for pain is Tylenol or Advil, I was to the point of begging for something stronger.

[Cue massive drama and theatrics] .  Consults had been made with Neurosurgery and Anesthesiology (the god-like creatures who deign to treat the pain in mere mortals held captive at UAB).  To make a long story short, there was a difference in opinion in how I was to be treated.  Of course, those differences tend to happen when the resident assigned to the case fails to read the MRI results before concluding that said patient is merely "drug-seeking" and states there is no evidence of why she would be in pain. Interesting theory, considering both the radiologist and neurosurgeon had identified two compressed discs in my lower back that are sitting directly on the nerve root.  That explain exactly why I was in excruciating pain.

I was, of course, just the slightest bit pissed off that a resident I had never laid eyes upon was standing in my room accusing me of a drug problem.  I kindly showed him the way out and proceeded to raise holy hell with my pulmonary doctor and NP.  I am very fortunate to have an outstanding relationship with my NP, and although the attending Dr. that was seeing patients in December may not know me that well, he knew enough to be able to rely on my nurse's assessment of the situation.

In the end, my pain was managed by my pulmonary team.  I was discharged with a stack of prescriptions and referrals to both the Pain Clinic and Neurosurgery.  The plan-of-care recommended trying a steroid dose pack (which was done in the hospital, but with no benefit).  Up next in the play book are an epidural block and/or surgery.

On the Pulmonary front, things went from bad to worse.  My pulmonary functions declined throughout my hospital visit rather than improving as they normally would.  Overall, I never felt that I was truly "getting well" - on Day 21, I felt as bad if not worse as when I was admitted.  On Day 24 (the Friday before Christmas), my PFTs were extremely poor (FEV1 = 25%), which kind of sent me into a downward spiral.  To be perfectly honest, I am normally one of those people that can just put on a happy face, even if I am upset or frustrated.  After such a long and intense stay, though, I lost my cool.

I ended up completing 28 days of antibiotics and received a Solumedrol kick in the ass (high octane steroids) my last weekend there.  Unfortunately I was in the hospital over Christmas, but was able to go home the day after.  The hospital is never where you want to spend the holidays, but we made a go of it.  I dressed up in my Christmas PJs (a gift from my mother) and my family came to visit. My aunt made her famous fried chicken nuggets, so I enjoyed a good meal that day (Thanks, Cheryl!).

I've been home for two (almost three) weeks now and recovery has been slow.  When I was younger, I was always raring to go the day after I got home.  The older I get, the longer that seems to take.  I would say that, on average, it now takes me almost a full week (at least four or five days) to get myself back in the swing of things.  The negative side of receiving the steroid boost at the very end of my hospital visit meant that I had to come home on Prednisone.  And although my lungs appreciate the effects of the steroids, the rest of my body does not.  My taper runs for two more weeks.  This means two more weeks of insomnia offset by moments of pred-mania.  I am also suffering from a severe case of pred-munchies and cravings of the weirdest sort (Arby's mozzarella cheese sticks and a vanilla milkshake, anybody?).

So, where are we now?

Well, if you've made it this far, then I applaud you!  Because my PFTs were so low when I left the hospital, my doctors wanted to see me in clinic in two weeks rather than the usual four-week followup.  I went to clinic on Monday.  My PFTs were up some from my discharge (FEV1 = 34%), but not at all close to my "well" standards.  Generally speaking, if I blew a 34%, I would be admitted, but since we had just finished 28 days of strong antibiotics, my Dr. and I agreed that adding another seven or fourteen days would not be helpful.  So, fortunately, I was able to hold off on being re-admitted to the hospital.

Right now, our goal is to find out what is causing my PFTs to drop so suddenly and not bounce back like they usually do.  My Dr. wanted to check for ABPA (Allergic Bronchopulmonary Aspergillosis), which is a fungus that can wreak havoc in CF lungs.  Technically, I have not met the criteria for diagnosis (having a serum IgE greater than 1000), but previous studies have shown that I have had an elevated IgE for quite some time (in the 700-800 range).  Because I have a history of ABPA (in high school), my Dr. wanted to order a special test (which is, of course, expensive) that is more sensitive and may be able to determine if ABPA is causing my current issues.  If it is, then the plan is to treat with anti-fungals and steroids.  Honestly, although I do want to find out what is wrong, I really don't want it to be ABPA because I'm not sure I can stand to be on steroids for much longer.

If the test for ABPA is negative, then my Dr. wants to do a bronch to take a peek at what is going on inside my lungs.  Right now, this is currently scheduled for February 6th.  I'll also have a clinic appointment the same day so that we can discuss the results of the bronch.

Finally, we discussed the possibility of a referral over to the transplant clinic.  The appointment would be more of a "meet & greet" (although I know most of the doctors because they rotate in and out of our CF clinic).  We would discuss the possibility of beginning the evaluation process.  It does not necessarily mean that I would be listed right now (or any time in the near future), but if I get the tests done, they will be good for two years.  Although I'm OK with the eventual possibility of a transplant, I'm not quite ready to jump the gun.  However, once a Girl Scout, always a Girl Scout, so I will at least look through the window of that future possibility and "Be Prepared."

I think that about sums up everything that has been going on for the past month and a half.  I will try and do a better job of updating my blog with the latest information. 

And now I'm off to do something FAR more interesting -- getting the hair did! Catch y'all on the flip side.

New medical FICO score sparks controversy, questions

See the original article here.

Within the next 12 months, whether you like it or not, about 10 million Americans are expected to be scored -- much like a credit score -- on how likely they are to fill a prescription and take all the pills the doctor ordered, on schedule.

FICO , creator of the widely used credit score that predicts whether you'll borrow responsibly, is now rolling out its new Medication Adherence Score.

FICO based its score on a formula that predicts whether you will take your prescription drugs. FICO says that since correct use of medication is important for patients, medical providers, insurers and pharmaceutical companies, the Medication Adherence Score will help achieve that goal. They predict it will improve therapy effectiveness and reduce health care costs.

The company says those who score low can be targeted for extra reminders and educational efforts, with the goal of making patients more likely to complete their prescribed regimens.

"It's very important to identify those people who may need that additional education and that additional help," says Dave Shellenberger, senior principal consultant in FICO's health care division. 

Critics aren't so sure. Since the score uses information on patients' employment, homeownership and living situations, they say that in the current economic environment, the Medication Adherence Score may unfairly target people who have found themselves in challenging financial situations. It could also, they say, open the door for a new way for insurance companies to charge different prices for different scores. This happened in recent years to consumers buying auto insurance: The cost of those policies are now likely based on an auto insurance credit score .

What's in the score?
FICO launched the Medication Adherence Score on June 23, 2011. To create the scoring algorithm, FICO looked at data from a random sample of several million anonymous patients provided by a large pharmacy benefits manager. FICO observed patients who filled (or refilled) their prescriptions and patients who failed to, with the company then identifying those variables that best predicted medication adherence. For the Medication Adherence Score, those variables include age, gender, family size and asset information -- such as the likelihood of car ownership -- data also used by direct marketing companies. FICO says that with only a patient's name and address, it can pull the remainder of the necessary information from publicly available sources. 

The scores range from 1 to 500. "The higher the score, the higher the likelihood a patient will be adherent to a drug regimen," FICO's Shellenberger says. FICO says patients who score 400 or higher are likely to take medication as prescribed, while those who score below 200 are at high risk of not taking medication. According to FICO, patients who earn a low score may receive a medication reminder in the form of an email, letter or phone call from their doctor -- tactics that would be too costly to apply across the board.
FICO says those who wouldn't want to receive notices should contact their health care providers and ask about their opt-out policies. High scorers who appear likely to take all their meds probably won't be contacted. 

FICO: No insurance implications
What won't happen, FICO says, is your insurance taking a hit because of a low score. "No decision is being made as to whether someone is getting access to health care," says Shellenberger. That's because companies can't use the Medication Adherence Score when deciding whether to provide health insurance and how much to charge for it. "The score was not designed, tested or validated to serve as an underwriting tool. And, underwriting is not an allowable use of the score under the terms of our client contracts," Shellenberger says.

FICO declined a request for a copy of the contract. FICO says consumers can find out if they have scores by asking their health care providers. 

Age, gender taken into account
Who's likely to score low? Shellenberger notes that older patients are typically better about taking their medication than younger patients -- until those older patients begin to reach their 80s and 90s -- and men typically are a little better about taking medication than women. "One hypothesis is that women tend to be caregivers and take care of others rather than care for themselves to the extent that they should," he says.  

It's against the law for FICO to factor some information into its more-familiar credit scoring formula. It's forbidden from considering, for example, your race, religion, national origin or sex. In addition, it voluntarily does not consider your age or salary. But gender and age are two factors in the Medical Adherence Score. FICO says that's because the score only results in communication with a patient and only uses "publicly available third-party data sources, such as the U.S. Census," Shellenberger says. None of your personal credit information -- the data used to determine your FICO credit score, which is regulated by the Fair Credit Reporting Act -- is used in the medical score, he says.

Whatever it includes, your own personal Medication Adherence Score is coming. FICO estimates that by the end of 2011, 2 million to 3 million patients will have been given a FICO Medication Adherence Score, with a total of 10 million patients expected to be scored during the next 12 months. As a patient, you probably won't be informed of your actual score, however. 

A very real problem ...
FICO and medical industry experts both say that medication noncompliance warranted the creation of the new scoring system. According to FICO's press release , medical industry estimates show that up to half of the 3.2 billion prescriptions in the United States annually aren't taken as prescribed, with nonadherence "cited as the primary cause for 10 percent of hospital admissions and 23 percent of nursing home admissions each year." It's also costly. According to a company fact sheet , the cost of medication nonadherence to the U.S. health care system totals an estimated $290 billion a year.

Pharmacists agree that nonadherence represents a challenge. "We recognize that adherence to medication has been a problem for 40 or 50 years. And the ability to identify people who would most benefit from interventions would provide pharmacists and others an advantage in their ability to target services to where they are needed the most. No one would argue about that," says Lucinda Maine, chief executive of the American Association of Colleges of Pharmacy (AACP) in Alexandria, Va.  

Patients are unreliable medicine-takers for a variety of reasons. "Some people don't understand the condition that they have. Some people might have high blood pressure but they're not experiencing symptoms every day," says Anne Burns, senior vice president of professional affairs with the American Pharmacists Association in Washington, D.C. Such a lack of symptoms could discourage patients from consistently taking their medication, causing their health problems to return.

As a result, "programs like this are starting to appear in the marketplace," Burns says.

... But is the score the answer?
While there's general agreement about the problem, not everyone thinks FICO has the solution. "There are many programs and assessments for patient adherence currently available and whether this algorithm is better than any others has yet to be seen," says Dr. Richard Perry, an assistant professor of pharmacy practice at Long Island University.  

Experts stress that medication adherence is a complex issue, with many factors -- some tied to a patient's finances, culture or attitude -- contributing to failures to take medicine.

For example, Dave deBronkart, also known as "e-Patient Dave," a personal health data rights advocate based in Nashua, N.H., says financial problems once kept him from buying a prescribed medication.

When deBronkart left his last full-time job -- but before his COBRA insurance kicked in to provide continued health benefits -- he went to refill his prescription for generic blood pressure medication. "I was very short on cash," deBronkart says. His prescription, which cost just $4 with insurance, nearly tripled in cost to $11. "All of a sudden, I don't have insurance and the price goes up," deBronkart says. Due to that jump in price, "I didn't buy it," he says, instead delaying the purchase until his COBRA took effect.  

Those types of situations may be increasingly common as more patients find themselves unemployed in the tough economy. That could mean low Medication Adherence Scores for otherwise responsible patients. "Apparently under this scoring model, you are at higher risk for not taking your medication and thus have a low score if you, among other things, don't have a job, don't own a home or a car and live alone," says Tena Friery, research director with the nonprofit consumer group Privacy Rights Clearinghouse.

Although some critics have voiced concerns over the Medication Adherence Score and possible privacy issues, other experts say that privacy isn't the problem. "Health plans already have information on what drugs you're on and which ones you went to the drugstore and picked up or got through mail order. The third step, the piece they don't know, is whether you took the drugs," says Deven McGraw, director of Health Privacy Project at the Center for Democracy & Technology.

"I think it's far less of a privacy concern than an age-old concern about how people can use health data to discriminate against people in the insurance marketplace or employment," McGraw says.

What's next?
Looking ahead, FICO says the Medication Adherence Score -- like any of its scoring models -- can be updated about every 18 to 24 months to make it work better. Just don't expect too many details when the company does. FICO guards all its algorithms closely as proprietary information. "I don't know whether they will ever allow researchers to look behind that green curtain of Oz to know what their analytic model is," says the AACP's Maine.  

And FICO also isn't identifying additional scoring formulas it could create to predict other areas of everyday life. If it were to do that, however, Privacy Rights Clearinghouse's Friery guesses that it could be some sort of "lifestyle" score. Since poor lifestyle choices can result in high health care costs, the score could be used to target individuals about health initiatives, such as weight loss or smoking cessation.

"Reports are that people of low income or those who live in certain areas are more likely to be overweight, to smoke, to be inactive. I assume that any future scoring, like the Medication Adherence Score, would not be based on an individual's past behavior but rather certain factors that would put them into the high-risk category," says Friery.

Although the "objective of lowering health care costs is certainly a good thing," Friery says that with such a score, consumers may receive unwanted phone calls or other contact about services that don't apply to them. "I can only hope that users of such scores would use caution in initiating these contacts. As a minimum, consumers should be given the right to opt out, to stop future contact, whether or not the targeted behavior applies to them," she says.  

Clinic Update

Today was my one-month post hospital clinic appointment and I'm happy to report that, overall, it was a fairly good visit.  My FEV1 has finally shown some improvement after being in the tank last time I was in the hospital and is near baseline.  I'm still hanging out around 44% lung capacity, which I'm happy with. Given that a few years ago I was struggling to keep it in the 30's (when I was battling back-to-back pneumonia infections -- SIX times in one year), I am always thrilled to see a higher #.

So the plan for now is to keep on keeping on.  My stomach issues are still rampant, but my GI doctors have been less than forthcoming in ways to fix or improve it and I'm just in for the long haul.  I have been on the Domperidone for over a month now and while I will say that while I see some improvement over the Reglan (which was doing absolutely nothing to treat my gastroparesis), it is still an everyday problem. In addition to the stomach issues, my doctors are having me checked for possible gallstones, so I have an abdominal ultrasound scheduled for Monday. I have been having sharp, stabbing pains in the area of my gall bladder every time I eat, particularly if I eat anything high-fat (which I typically try to avoid).

I talked with my NP today about scheduling a tune-up in August because I want to be in tip-top shape for September when I go on a cruise.  Two of my BFFs, Angela & Brien are getting hitched and are doing a wedding cruise out of Mobile.  It's actually the same cruise I went on last year (to Progreso and Cozumel), but ANY cruise is a good cruise in my books.  I am definitely looking forward to some true vacation time (even though I qualify for four weeks of vacation with my employer, I typically only get one week and forfeit the rest when I am in the hospital).

And that's about all the updates I have for now. That, and the fact that my legs are numb from allowing my sixteen pound beast to sit in my lap while I've been typing this blog (love ya, Beau-ba-lo!).

Catch y'all on the flip side!

Updates, Updates, Updates

At the end of February, early March, I had the opportunity to travel to Orlando, FL for work.  I attended a conference (training) related to my job.  For four days, I studied the basics of Deposit & Lending Compliance.  I know that might not sound exciting to many of you, but YES, I actually enjoyed it.  I was familiar with most of the deposit regulations (Reg CC, Reg DD, Reg E, among others) but the lending session really kicked my butt (especially Reg Z & RESPA ... my brain was toast by the end of Day 2!)

While I was in Orlando, I also got to have a little bit of fun. (Don't tell my boss that!)

On Wednesday night, I met up with an old friend ... we haven't seen each other since 5th grade, when she moved back to Japan.  We'd kept in touch over the years via letters (yes, real ones) and then found each other on Facebook not too long ago.  When she saw my post about being in Orlando, she contacted me that night and we immediately decided that we had to get together.  We met up in Downtown Disney and enjoyed dinner at an Irish Pub (where we had meat pies ... savory & sweet pies for you Sweeney Todd fans). Later that night we went over to the Magic Kingdom where I got to do some shopping and we watched the fireworks show. All in all, it was a great night!

On my last night there (after the conference was over) I decided to catch the final performance of Wicked. I love, love, love this show and this was my second time seeing it.  Although the Bob Carr Performing Arts Center was a little smaller than I expected for a city the size of Orlando, it was a nice setting.  Even better, the tickets I scored were three rows back (which I had no idea when I purchased).  To say I was thrilled would be putting it lightly.

 

15 minutes til curtain! 

After a whirlwind week, I was glad to arrive home on Saturday. I was in kitteh withdrawal after a week in a hotel (Although I highly recommend the Homewood Suites in Maitland, FL if you are ever visiting the area, it was a little off the beaten path. However, it was very close to the office my training classes were being held in and the rooms and free food made it even better).

I was home for a couple of days when my body crashed and I had to be re-admitted to UAB Hospital.  So, on March 9, I checked in for sixteen fun-filled and exciting days.  Although it meant celebrating my birthday in the hospital, it wasn't a bad visit overall.  I am now officially back to work and back up to all of my old tricks!

Hello? Hello? Anybody out there?

It's been a while. My apologies for playing least in the sight the past few months, but I've had a serious lack of motivation to write - which is unusual for me. I hesitate to write when I have nothing new to report and (fortunately) life has been pretty routine lately (you know the drill - work, sleep, play, hospital {rinse & repeat}).

I survived another 20-day visit to Club Med in December and made it home just in time for the holidays. Spent lots of time with the fam, which was fabulous and started back to le job two days after Christmas. Since then I've been absorbed in work and trying to stay well.

Today was my one-month post-hospital clinic visit. Not exactly a stellar report, but it's been a pretty sub-par month for me. I haven't been Sick (in my world there's "Sick" (which equates to hospital time) and "sick" (which means I don't feel great, but not bad enough that I'm willing to do anything about it). I've had a stomach bug and some other infection that I couldn't really pinpoint (but I knew my body was fighting something because my lymph nodes were swollen to about the size of a nickel for about a week & a half).

So today, my FEV1 was down 7% from discharge, which was Not. Cool. The nurse threatened me with another round of IV abx but I managed to sweet talk my way into two weeks of oral abx (Levaquin & Bactrim).

It also appears that Cayston and I are going to have to part ways. After six days of SOB & wheezing and three days of hemoptysis, I decided to take a break. Determined to give it the old college try, I waited three days and decided to go one more round. Cayston = 2, Brandi = 0. Based on that, the docs recommended stopping the Cayston for now. If we have to bring it back into the arsenal later, we'll see if I can tolerate it then.

In other news, I'm very excited about an opportunity that I've received at work. Beginning Monday, I'll be shadowing a new manager and will begin learning how to do Compliance Monitoring (reviews). I'm very excited because this keeps me with the same team that I've been working with, but allows me to move outside the world of official complaints. Seeing as how I've been working with complaints in some form or fashion for the past seven years, I'm really looking forward to the change. It will also be a great opportunity to learn each regulation more in-depth which will be a huge bonus when I start studying for my CRCM (Certified Regulatory Compliance Manager) next year.

That's all for now, folks. Catch ya on the flip side!

A Breathtakingly Artistic Event

 

Save The Date!

Art Show & Silent Auction Benefiting Cystic Fibrosis Foundation

Location: Naked Art Gallery, 3831 Clairmont Avenue, Birmingham, AL 35222
Date: October 22, 2010
Time: 5:30-8:30 pm
Sponsored by: Naked Art Gallery, Silverton Cafe, Zoe's, and the historical Forest Park Village

This is our first event sponsored by our newly formed Young Professionals Board.  It was coordinated by Alexis McBrayer, who recently lost her husband to Cystic Fibrosis.  This fundraiser is in honor of David McBrayer and all the other wonderful people who have fought the battle against CF.

Please join us!  In addition to the Art Show & Silent, Vero VanBlaere (of the Naked Art Gallery) has agreed to donate 10% of all sales during the event to the CF Foundation.

FSA Accounts - New Rules 2011 & 2013

I'm not sure how many others utilize the tax benefits of an FSA account, but be forewarned, things are about to change.

In 2013, the maximum contribution to your FSA account will be limited to $2500.00. This is going to be a huge drawback for me as I currently contribute $5000/yr to my FSA and that barely covers 1/2 of my medical expenses. $2500 will cover one hospital co-pay ($1725 for me) and about half of the expense for one order of prescription refills (a three month supply), which (obviously) I spend three or four times a year. Needless to say, this is going to be quite a hit on the budget for me.

Also, in 2011, if you use your FSA account to buy over-the-counter items such as Nyquil, Benadryl, Claritin, etc., you will have to provide a prescription from your doctor in order for it to be an approved FSA purchase. You can still buy these items out of pocket without a prescription. Read this IRS.gov article for more details.

These are two new regulations that are part of the Patient Protection and Affordable Care Act enacted by Obama that will greatly affect the lives of CF patients. BE INFORMED! You can read news releases and legal guidance from the IRS here.

Fiddle-Dee-Dee!

What a month it's been. I'm EXHAUSTED. And, I must admit, I am looking forward to a weekend of doing absolutely nothing. Since the end of June I've helped redecorate a kitchen, spent a long weekend in Virginia, hosted a pool party, and took a quick trip to Atlanta. Last week I spent the latter part of the week & the weekend with my family celebrating my cousin's graduation. In between, I've attended board meetings and birthday parties. And, lest I forget, I've worked at the real job. You know, the one that pays the bills.

I'm finishing up my first full month of Cayston. Great stuff. I'm almost three months out of a tune up (August 7) and still feeling decent. Not great, but decent. I truly am exhausted by the end of the day and typically go home and catch some zzz's before tackling any chores around the house. And, although the kittehs are enjoying the extra snuggle time, it's really cutting into the few hours I have each evening to get things accomplished.

About two weeks ago, we got an early start and headed over to the ATL.  We spent Saturday afternoon exploring Margaret Mitchell's apartment (affectionately referred to as "The Dump").  This is where she spent several years writing Gone With The Wind. Later that evening, we had a fabulous dinner at Nakato Japanese Restaurant and then journeyed over to the Fox Theatre to see Phantom of the Opera.

We had a few newbies with us, which is always fun.  I know that they enjoyed the show but I don't think they were quite as enthralled as my aunt, cousin & I are! According to my Playbill, this is the final season for the US Tour. It will still be showing in London, New York, and Las Vegas. What a tragedy! Actually, it just gives me that much more desire to get my tail over to England see a performance at Her Majesty's Theatre.

On Sunday we grabbed a quick breakfast at the hotel (cold cereal, anyone?) and then scurried over to the Georgia Aquarium. My favorite exhibit? The penguins, of course! Actually, all of the exhibits were fascinating and I really enjoyed seeing the beluga whales, the sea otters, and the sharks. They have a swim or dive program where you actually have the opportunity to swim with thousands of fish, sharks, and rays. It's a fairly shallow dive and I'm thinking about asking my Dr. if he thinks my lungs could handle it. I would love to learn how to scuba dive.

LA! That's all for now ... must get back to work so that I can pay the bills.

Oh, before I sign off, I'm ecstatic to hear that CysticGal finally got a new set of breathers.  At last report she was already off the vent and trying to talk.  Go Beth!!!

And the results are in ...

AMBRY results, that is.

My sweet doctor e-mailed me the results of my Ambry test today, even though he is technically still on vacation. I have a copy of the report which says:

DeltaF508 / p.S945L (with a 7T/9T variant)

"This mutation has been associated with severe pulmonary disease, elevated sweat chloride levels and variable pancreatic sufficiency."

I'm guessing that the first two charecteristics are typical to the DeltaF508 mutation and the last to the p.S945L.

I've been fortunate enough to have moderate pulmonary disease for the past several years and I've been pancreatic sufficient (for the most part - I have to take enzymes when I eat high fat / greasy foods).  I do have other unrelated stomach issues, mainly severe reflux and gastroparesis but that appears to be something that runs in my family.

Dr. C. said that he needed to do some homework because my second mutation is "very rare." He also mentioned that he believes it may be responsive to some of the potentiator drugs (VX770) and may also be related to some of the current research that he is doing, both of which he finds encouraging (and so do I!).

That's all the information I have for now.  After doing some digging on Google, I found several articles that mentioned the p.S945L mutation but none that gave good specific information about it.  A search of the Cystic Fibrosis Mutation Database didn't turn up much information either.

P.S. There is a small bitchy part of me that would like to say "suck it" to a doctor (who shall remain nameless) from my past that essentially told me I didn't have CF, just couldn't have CF and should never have been diagnosed as CF because I didn't fit the "textbook" definition. I guess I haven't been imagining things all these years.

Singulair - $20 Off Coupon Up For Grabs!!!

Anybody take Singulair? I have a coupon for $20 off a qualifying prescription that I won't get to use before it expires. Happy to pass it on to someone that can use it.

Just leave me a comment here or on Facebook ... he/she who asks shall receive!

UPDATE:  The coupon has been claimed.  If I get any others that I can't use, I'll share.

The Transition

My aunt, a nurse at Children's Health System, was recently working on her Magnet Recognition Program and the focus was on the transition from pediatric care to adult care.  She asked me to write a couple of paragraphs about my experience:

In 2002, I was 24 years old. That year, I faced an important transition in my life – not from childhood to adulthood, as one might imagine, but similar. I was next in the group of patients being transferred from pediatric care at The Children’s Hospital of Alabama to adult care at UAB Hospital.

As one of the first patients to transfer to UAB, we were truly the “guinea pigs." Our transition was difficult. Life at Children’s was good – our nurses cared for us not just as patients, but also as family.That happens when you grow up in the hospital – spending days and weeks in “Club Med” several times a year.We were treated as “VIP” – in short, we were spoiled. While we had to adhere to a treatment program, we were allowed to essentially do as we pleased. There was an activity room with games and movies to keep us busy and we were allowed to go out on “pass” – which allowed us a chance to return to real life (even if just for a couple of hours) in the midst of doctors, antibiotics, and respiratory treatments.

Life at UAB was different. We were little fish in the big sea of patients at UAB. The doctors and nurses didn’t know us, didn’t understand our routines, and were simply not used to caring for patients like us. We were expected to stay in our rooms. For insurance reasons we were no longer allowed to leave the hospital.  We had to be adults. Imagine our shock.

I know now that it wasn’t difficult just for the patients, though. Over the past eight years, we’ve faced many challenges and a steep learning curve, but we’ve done it together. Our doctors and nurses have grown as use to us as we have to them and they are now our “family” just as much as those from childhood.

What was your transition to adult care like? Were you transferring into a new adult program or one that was well established? I'd love to hear what other CF patients have encountered.

clinic and other assorted CF stuff

Four weeks. That's how long I've been out of Club Dread, and my, how time has flown. Not that I'm complaining.  In fact, I'm rather in the mood to celebrate.

Why?

Possibly because I blew the best #'s I've seen since 2006!

To be honest, it actually took me by surprise.  My sinuses have been jacked up for the past week or so and I felt like my #'s might reflect that. Happy to say I was WRONG. The docs attribute part of it to the fact that I'm finally getting my blood sugars under control.  As my body is expending less energy on keeping my sugars in check, it can spend more energy on breathing. I've also been on the Cayston for a month and I have no doubt it's also helped.

My next appointment was to participate in a CF study.  I can't remember the exact name of the study but it involved taking biopsies on tissue samples to test CFTR functioning.  The study has been ongoing because they were having problems recruiting people for the second part of the study.

I'd like to keep this family friendly but there's really no nice way to describe the procedure (flexible sigmoidoscopy), other than by saying it involves going in the out door.  Yes, the doctor will tell you "it's only 10 centimeters," but I can guarantee you that it is the longest 10 centimeters you can imagine.  Thanks the gods for Demerol and Versed. Although I can say that the whole experience wasn't that bad, there is NO WAY I would have done it without happy-I-don't-remember-anything drugs.

I'm sure you're wondering how exactly I let myself be talked into doing something like this.  It's certainly not something I volunteered for! However, the whole study happened to be headed up by one of my favorite childhood doctors and he dangled a prize in front of me that I couldn't turn down -- an AMBRY genetic test.

After spending the past six months fighting with Blue Cross about WHY I should be re-tested, it was time to go another route.  When Dr. C asked me what he could do to entice me into participating in the study, the little light bulb in my head flickered on.  I explained the problems I've been having with insurance approvals and we quickly came to an agreement that the research program would cover the costs of the genetic screening if I would participate in the study.  Too sweet of a deal to turn down, in my esteemed opinion.

Now I just have to find the patience to wait ANOTHER four weeks on the results. Argh!

CF Awareness Month

With May being CF Awareness month, I wanted to do my part and spread the love. If you have a minute, check out some of my favorite blog stops:

1.RunSickBoyRun - everybody knows and loves the famous Rondi! If you are in need of exercise inspiration, this is the place to go.

2.CysticGal & A Matter of Life and Breath - Need some pre-transplant perspective? This is it.

3.The Unknown Cystic - IT. IS. HYSTERICAL.

4.Welcome to Joshland! - Check out Josh and Moganko for some education, inspiration, and general silliness.




5.My CF Journey with God - This is my IRL friend Katey and simply put, she rocks. She's an inspirational hard worker and one of the nicest people you will ever meet. Katey is a wealth of knowledge about transplant. She had a living donor transplant six years ago and has gone on to graduate college and is (hopefully!) off to nursing school next.

Our Great Strides walk is this Saturday. I'll be there with bells on!

Catching Up

I have a bad habit of being quasi anti-social when I am in the hospital (of which I was for the better part of last month).  Although I wasn't terribly sick upon being admitted, I had been out of the hospital for a considerable length of time (for me).  In addition, a mild virus / cold decided to make its presence known around the time I was admitted precipitating yet another round of Prednisone.

Prednisone and I have a love / hate relationship, at best.  I started and finished this course with the usual range of side effects, including CF-related diabetes.  In the past, I've had to treat blood sugars while on Prednisone (with insulin) but my sugars tended to normalize once the course was complete.

Apparently my pancreas has decided that it doesn't want to participate in this little game any longer.  I'm now an official insulin-pen carrying, blood sugar checking member of the diabetes world.  Of course, CF-related diabetes is something that tends to be expected as Cystic Gal's age gracefully, but it wasn't something I planned on encountering anytime soon.

Speaking of planning, I'm fast discovering in the diabetic world that daily life requires some amount of planning, scheduling, and thinking ahead.  As living by a "schedule" is the bane of my existence, it's taken some time to adjust to this new lifestyle.  Of course, having experienced a few true diabetic "lows" over the past week has proved to be a great source of inspiration in getting with the program.

Along with "di-a-beet-us" (so said Wilford Brimley), I've been dealing with high blood pressure.  The prevailing opinion is that the high blood pressure is because of, and/or related to the diabetes and that with control of one will come control of the other.  But until it normalizes, I'm taking a small dose of blood pressure medicine once a day.

Learning to manage diabetes in the hospital was much easier than doing so at home.  Rightfully so, when your food is delivered to you three times a day on silver plastic platters.  But I'm adjusting to being home.  It's been a process and I am trying to steadily improve my rather erratic eating habits and curb my consumption of the nectar of the gods (it's not my fault that I'm a good Southern girl who enjoys the occasional gallon of sweet tea).

Other than that, life is good and quickly returning to normal.  Here's to another round of good health bolstered by an extra dose of healthy eating.

Eva

There's another bright and shining star in the sky.

Breathe easy, Eva.  You will be missed.

Edit: I wanted to include this video about Eva's Legacy, which was shared by Nathan, Tricia, and Gwyneth Rose from Confessions of a CF Husband.

ENT Update

Yesterday I went back to UAB ... on the schedule, a CT scan and another appointment with ENT.

The verdict?  CT Scan was a hot mess. It's about what Dr. W. expected ... scar tissue and polyps. I'm going to start the Pulmicort flushes and he is going to try and revise some of the scar tissue in the office on March 9th. Not that I'm looking forward to having him dig around in my head for a few hours (it will be numbed with Pentacaine, which is like a super-duper Lidocaine), but it's better than going back to the OR. Anesthesia does not make the lungs happy.

I did not sign up for this.

In other news, I talked to C.Fred about starting Cayston.  She said there is still a study ongoing at UAB that I can possibly participate in.  Meaning that I won't have to wait for the actual release (even though that is only about two weeks away).  Yay!  I am more than willing to try ANYTHING that may keep me away from Club Med.

Still trying to appeal BCBS's denial of being genotyped.  I did find out that my company has no specific exclusions for genotyping, so it is simply a matter of convincing the insurance company that being genotyped is important for all CF patients ... it's value is not just in diagnostic or pre-pregnancy / newborn screening.

But, hey, if all I need to do is tell them that I'm thinking about getting knocked up, then it might be worth it.  Just jokes.

Feb 22, 2010: FDA Approves Release of Cayston(R)

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.

FOSTER CITY, Calif., Feb 22, 2010 (BUSINESS WIRE) -- Gilead Sciences, Inc. (Nasdaq:GILD) today announced that the U.S. Food and Drug Administration (FDA) has granted marketing approval for Cayston(R)(aztreonam for inhalation solution) as a treatment to improve respiratory symptoms in cystic fibrosis (CF) patients with Pseudomonas aeruginosa (P. aeruginosa). Read more.

This is the first new inhaled therapy approved for Cystic Fibrosis patients in more than 10 years!  FTW!!!

The Good, The Bad, and The Ugly

I was off work yesterday, nevertheless, it was a busy day.  From 7:30 AM to 2:30 PM, my day was full of doctors, waiting, tests, clinic, waiting, labs, and more waiting.

The Good
CF Clinic was grand!  After dashing from St. Vincent's over to UAB, I leisurely strolled (editor's note: ran my ass off) trying to get to the 4th floor in time for my pulmonary function tests.  I made it with two minutes to spare - checking in at 9:28 AM for my 9:30 AM appointment.

The Stats

FEV1 = 1.58 liters (55%)    Woot!

O2 Saturation: 91% on room air

With vital signs and PFT's out of the way, I sat down to wait.  Luckily, I didn't have to wait long and I enjoyed a few minutes catching up with the two Ashley's.  A few minutes after 10 AM, I got called back to see the team.

First up was Karen, our new nurse practitioner.  I met Karen when I was in the hospital in January and I must say, I was very impressed.  She has not worked with CF patients in the past, but she is eager to learn and wants to get to know each patient individually.  This is important in any case, but particularly when you are dealing with a disease like CF that affects each person differently.

Next up to bat was Dr. Y, one of the best doctors in our group (and no, I'm not just saying that because I know he reads my blog) ... he really is that good.  After all, he listened to me babble on and on about the incessant itching that I've been fighting since last Friday.

By incessant I mean non-stop, never-ending, all over my body itching.  For reasons unknown.  I've ruled out all the obvious causes - dry skin (I've been drenching myself in Aveeno twice a day); changes in diet; new medicines; change in detergents; fleas (the kittehs are inside only so this was unlikely, but worth looking into).  I've re-washed all my bedding, bathed in oatmeal, taken Benadryl, and finally - out of sheer desperation - took 20 mg of Prednisone on Thursday night.  I think Dr. Y realized I was serious when I copped to taking the Pred - he knows how I detest that "evil little miracle."

After ruling out any likely reactions to my medicines, the next step is to see the Dermatologist.  We tried to setup an appointment for yesterday afternoon, but the earliest available was next Thursday.  Dr. Y took pity on me and prescribed me something stronger than the Benadryl to try and keep the itching at bay until then.  I took my first dose last night and was able to make it through the night without scratching my arm or leg off.  Sitting here typing this post working, the itching is itching to make a comeback, but it's tolerable.

The next topic of conversation was genotyping.  I've been genotyped before (DeltaF508 + unknown), but that was when they only tested for 32 mutations, not the 1000+ that are tested for today.

Dr. Carla submitted a pre-authorization request to BCBS while I was in the hospital, but the claim was denied.  The response that I received from the Medical Review Board stated that "the patient already has a diagnosis of Cystic Fibrosis" (really? you're kidding!).  We are going to file an appeal - two new treatments that are currently undergoing testing (VX809 and VX770) are genotype specific.  While I don't think I have the genotype necessary to be part of these studies, it will be helpful to know.

Last (but certainly not least) was the subject of my port. When I was in the hospital last month, I had developed cellulitis in the area around my port.  We decided to take a "wait and see" approach hoping that with the number of abx that I was on and with some "alone time" after I got de-accessed that it would heal on its own.  Happy to report that it did!  The infection, inflammation, and tenderness are all completely gone.  We were able to access my port and flush with zero problems and I had a beautiful, bright red blood return.

As you can tell, this appointment was a little more involved than the usual "How're you doing?" - "I'm fine." - "See ya in a month." conversation that I usually try to have.  Because of that, I was running a little late for my ENT appointment, which was scheduled at 11:15 AM.

The Bad
After finishing up in clinic, I high-tailed it upstairs to ENT clinic and checked in around 11:30 AM.

Where I proceeded to wait.  And wait.  And wait.

About 12:15 PM, the PCT called me back, took my vitals and set me up in Room 2.  Where I waited some more.  Finally around 12:30 PM, the nurse came in.  With a look of chagrin, she apologized profusely for the long wait. "No big deal," I said, "What happened?" "Well, umm ... you see ... mmmm, we kind of forgot you."  Wow.  OK.

Apparently word had not traveled from the front desk to the nurses that I arrived at 11:30 AM.  Dr. W. thought he'd seen everybody and went to lunch around noon.  She asked if I could wait til he returned at 1 PM and promised that I'd be the first patient he saw.  Even though I was mildly annoyed at being "forgotten," I know I'm not a VIP.  And I needed to have some lab work done, so I decided it was worth the wait.

Word to the wise - don't try to get labs drawn at Kirklin Clinic on your lunch hour.  Apparently, everybody and their brother had the same great idea.  I waited for about 25 minutes and called it quits.  I hopped on the next elevator up and headed back to ENT.  Fortunately, they didn't forget me this time.  =)

The Ugly
To be perfectly honest, ENT is never my favorite place to be.  Nobody in their right mind would enjoy having a 12-inch scope shoved up their nose.  I'd rather be strapped to a spinning wheel and have daggers thrown at me.  Fortunately, there's enough eye candy in the ENT office that the experience isn't a complete waste of time.

Even though my sinuses have been a little stuffy this past week, I honestly expected to hear the same pronouncement as last time -- "PERFECT."  But alas, it didn't happen quite the way I planned it.

My maxillary sinuses (the ones across your cheeks) still look great.  I flush my sinuses obsessively and it is well worth the time and effort.  He did clean out an inconsequential bit of gunk, but nothing to write home about.

And then he got to my frontal sinuses (the ones above your eyes).  Not so great.  Polyps and scar tissue that weren't present last time have decided to make themselves known.  GRRR.  It's frustrating because there is nothing I can do about it, it's "just a CF thing."

I'm going next week to have a repeat CT scan and then meeting with Dr. W. to discuss options.  I think the plan is to try and treat it with steroids (Pulmicort).  From what I understand, treating sinuses with Pulmicort is not an "approved" therapy (yet), so it has to be pre-authorized through Blue Cross before he can write me a script.

Just like that, my run of "perfect" sinuses was at an end.  With nothing more to be done (at least for now), my sinuses and I decided to head home after one more stop at the labs.  The crowd had finally thinned out and I was in and out in about five minutes.

Whew!  What a day.  I was glad to see it end (well, at least that part of it).  I was looking forward to the rest of my evening with family, friends and some excellent food.

Join the UAB Adult CF Team for Great Strides '10!

Please consider joining the UAB Adult CF Team for the annual Great Strides event in Birmingham, AL on May 22, 2010. Our focus for this year is to remind us that "Every Breath Is A Gift." GREAT STRIDES is the CF Foundation’s biggest fundraiser and provides us an opportunity to help make a difference in the lives of those with Cystic Fibrosis.

Advances continue to be made in finding a cure, but your help is needed now - more than ever - to help keep up the momentum of this life-saving research. As you may know, the CF Foundation receives no government funding. Therefore, the funds that we raise go directly to helping us find a cure.

Last year the UAB Adult CF Team raised over $6,300.

This year, I would love for you to walk with me and the other members of the UAB Adult CF Team, with a goal of raising at least $5,000 by May 22, 2010 in Birmingham, AL.

I know this year’s goal can be met with your help! Remember, every dollar that you raise is used efficiently and effectively. Each contribution is tax-deductible and goes directly toward supporting research and specialized care that improves the quality of life for those with CF.

If you would like to join the team, log on to my website. If you have any problems registering, please let me know and I will be happy to process your registration manually. You can reach me via e-mail at bankrgrl -at- gmail -dot- com. Once you are registered, the next step is the easiest - get out there and fund raise, fund raise, fund raise!

I look forward to seeing all of you who participated last year and hopefully a few new faces. Please feel free to share this e-mail with others. Help this be an outstanding year ~ let's start taking GREAT STRIDES to cure CF.

Eva

Please keep Eva and her family in your thoughts and prayers today. They need it now, more than ever.

The video below shares Eva's thoughts about what is to come. It is both dark and light, and shares some very serious content.

I have been quietly following Eva's blog for awhile now. Eva was diagnosed with chronic rejection following her double lung transplant two years ago. Through it all, she has shown a zest for life that not many people know, an appreciation for the beauty that surrounds her, and a fiery spirit (which goes well with her fiery hair).



Sadly, Eva is not expected to live much longer. She was re-listed for a second transplant, but the lungs have not come in time. As Eva reminds us,

the greatest thing you'll ever learn
is just to love
and be loved in return

There is an amazing documentary, 65_RedRoses, that shares Eva's personal journey and takes an unflinching look into Eva's life as she fought to beat the odds against Cystic Fibrosis and lung transplant. The film received several awards at the 2009 Vancouver International Film Festival.

I have not had the opportunity to see the film in its final cut, but just watching the short clips posted online reveals the beautiful, quirky, funny and inspiring person known to the world as Eva Markvoort.

Breathe easy, Eva.