43 Thoughts on Making It the FIRST 43 Years with CF

by Katie Richardson

Originally posted here on CysticLife.org

1. Remember your sweat test at age 3 1/2 . . . . something strange being on your forehead
2. Remember your first chest x-ray . . . cold table, green walls, crying
3. Remember your first enzymes . . . Viokase, three of them, in grape jelly on a spoon, mom and dad standing across the kitchen watching.
4. Chew enzymes instead of swallow them . . . never eat grape jelly again (can't even stand the smell of it . . .)
5. Quickly learn at age 3 1/2 that swallowing the pills is a much better idea
6. Only have to go to the CF doctor once a year in the 70s and 80s(can you believe it?)
7. Have your friends ask you why your thumbs look like cobras
8. Have your friends ask you if you smashed your thumbs with a hammer
9. Do bowl-a-thons, swim-a-thons for CF
10. Join the swim team even though your mom's not sure you can handle it
11. Win first place ribbons on swim team
12. Play soccer, make the varsity in high school
13. Watch your sister start having to go in for tune ups
14. Get pissed because everyone is always asking how your sister is doing . . .
15. What about me? I have CF, too . . .oh yeah, I'm the "lucky" one
16. Become fiercely independent . . . as much as I love my sister and we are great buddies, hate all the attention she gets, so decide to just be me.
17. Sob when cousin dies from CF at 14 in 1984 . . . first time really think about people dying from CF
18. Ask doctor if I should even go to college since I have CF and will probably die anyway
19. Praise God for having a doctor who when I was 16 told me YES, go to college as you have NO IDEA what the future holds
20. Go to college 500 miles from home
21. Watch "Alex, Life of a Child" with college friends . . . sobbing, explain to them that is the disease you have.
22. Vow never to read or watch "Alex, Life of a Child" again . . . because that was NOT me (and never do)
23. Interview for internship at national office of CFF
24. Do not get chosen for job in Washington, DC at CFF, instead intern at local CF chapter
25.. Graduate in 4 years, and take job working for college sorority
26. Praise God for not getting chosen to intern at national CFF when learn that CFers living in group apartments doing the internship started getting sick . . . (first emergence of cepacia and realization that CFers can pass bugs to each other in the late 1980s)
27. Hate sisters supposed friends for not including her while her disease was progressing when they were going out
28. Decide to go to law school
29. Spend summer living with sister in St. Louis while she's waiting for lung transplant
30. Decide her friends are not so bad after all because they come visit her and call while she's in St. Louis
31. Drive the 5 hour drive from Kansas City to St. Louis in 3 hours when sister gets call that lungs are available so dad I and I can join mom as surgery begins (thankfully we made it safely)
32. Meet future husband. Don't tell him about CF
33. Break up with future husband, get back together and tell him right away about CF
34. Learn that he already knew and had researched it and was okay with it
35. Get engaged, have mom panic that future husband really doesn't understand CF
36. In the course of 15 months from May 1993 to August 1994, graduate from law school, get engaged, move to Washington, DC to begin legal career, sister dies (too hard to balance lymphoma and rejection - no regrets, she had two great years), get married, find out pregnant with first child. (too many life changes in too short of time . . .)
37. Successfully have one, two then three healthy boys . . . and just live life
38. Realize that I've followed the "median age" as it has risen for most of my life . . . and actually surpassed it; decide to do clinical trials because if not me, then who? If I've made it this far, I must do my part to ensure others do, too
39. Vow to make it to 45, then 50, then 55, then 60, etc . . . one day at a time
40. Celebrate never having been in the hospital because of my CF
41. Celebrate never having iv antibiotics because of my CF
42. Vow to continue proving everyone wrong when it comes to expectations for those with CF
43. Toast myself on this Friday the 13th, the 43rd anniversary of my birth, that indeed, it is a lucky day for me . . .

Fundraising Video with the Long-Haired Man

I ran across this video with me rocking the long hair and I wanted to post it again. It turned out to be a very successful event! I love doing this kind of stuff :)

I hear the camera adds 100 lbs. Did you see that noggin take up the whole screen?

What the _____?: August 12th, 2010

A while ago we started a little contest on this blog in which I post a picture and whoever comes up with the most clever/funny/witty/etc caption gets put into a drawing for prizes at the end of the year. Still don't have everything figured out as of yet, but we do know a couple of things 1) There will be multiple prizes 2) I love the participation so far 3) If you think your submission is inappropriate for others to see, you can always email your answer directly to me at ronnie@cysticlife.org :) 4) I've literally LOLed all weeks so keep it coming!

This week's picture:

Alright, what do ya got!?!?

Last week's photo with the winning caption:

Winner: "We like your enthusiasm...and you can still join our band, but a beer bottle isn't "technically" a musical instrument!"

submitted by Rebecca

2nd: "WE will sing for your last red stripe"

submitted by Niki

3rd: "Holding this bottle should make me fit in....Do you think they will notice???!"

submitted by Ayishasmommy

Are Most CFers "Strong-Willed"?

I wanted to share part of an email that some good friends of mine posted on their fantabulous blog the other day. I'm sure most of you know the Olimb family, but if you don't, you're missing out. Rachel and Jeremy are two of the kindest and warm-hearted people I know and they have an awesome little fibro named Asher. You can follow their journey at asherolimb.com and I highly recommend that you do.

So here's the email that was on their blog describing a "strong-willed" child...

“Children who make decisions with intensity tend to be labeled “strong-willed.” At the end of the day, their parents feel as if they’ve been engaged in hand-to-hand combat—and that the child often wins at the parent’s expense! Most parents consider a strong will a negative personality trait because it often creates resistance and frustration in family life. Yet, in reality, it’s the strong-willed kids who are often better equipped to succeed, be creative, and face adversity.

Children with strong wills have the potential to become the next generation of leaders. They have their own ideas and plans. They know what they want. They’re persistent, confident, passionate, and determined to succeed at whatever they choose to do.

Leaders have an agenda, look for ways to incorporate others into their plans, and have a high need for control in life. Balanced with graciousness, leaders become a treasure because they make things happen, create organization out of chaos, and motivate people to action.

Unfortunately, it’s hard to raise a leader. These kids tend to have their own ways of doing things and like to tell other people (including their parents) what to do. A strong will keeps a child moving in a certain direction in spite of obstacles. Often these children need bigger barriers or tighter limits to teach them that those boundaries are firm.

Don’t be discouraged by the effort it takes to teach a strong-willed child which limits not to push. The strong-willed child accomplishes things in life, because the roadblocks that might hold others back are no match for this kid’s determination. Your job is to help him know the difference between obstacles to overcome and limits to live within.

A strong will can be an asset… as long as the heart is in the right place.”

Rachel actually sent me a message and said that she saw a lot of these qualities in Asher and was curious to know what I thought about my personality as a kid. I thought it none better to just go straight to the source and I emailed my mom about it. Her answer was short and sweet, but I think said all that it needed to:

Yes...you were a strong-willed child and the hardest part was teaching you "boundaries", "which limits not to push" and the "limits to live within." I lived in constant fear waiting for the next shoe to drop!

I found this pretty funny cause to be honest, I bet that if you would ask Mandi, she'd say that nothing much has changed. They say that most children develop their personality by 1st grade. I'm going to say that it's a pretty safe bet that I was no different.

So what do you think? Do most cysters and fibros you know exhibit "strong-willed" personalities?

The Perfect Way to End a Day...

Man, what a day! First though, I think we ought to start with the weekend that preceded it. This weekend was a blast. We had a successful event for CysticLife on Saturday (pictures here) and I'm so thankful that Fred Astaire Dance Studios would be willing to raise money for the CF community. The studio required nothing from us return and just asked that we enjoy the show. We were pleased to find out that they sold close to 250 tickets which was well over their average of 130 as compared to past years. It was extra special as my family drove up for the event and Mandi's mom happened to be in town as well. We were all able to hang out afterwards and grab a bite to eat (grandma included) which is definitely one of my favorite things to do.

The fun continued on Sunday when Nancy (mom-in-law), Josh (bro-in-law), Chrissy (sis-in-law) and Wongers came down to hang out and enjoy some homemade tacos. Mandi and I love hosting company. We actually had to run out and get some shower curtains as all of them stayed the night (except for Wong). Nancy was flying out the next morning and Josh/Chrissy wanted to see what the morning commute was like as they are thinking about moving to the area (don't even get me started about how excited I would be if that happened). Anyways, it made for a great time and I'm hoping that we have another hangout/sleepover/dinner party real soon!

Monday started as any typical Monday full of work with a little bit of work sprinkled in. I was actually looking forward to getting back to it as I worked less this weekend than I normally do. The real fun started at the end of our workday though. We were able to babysit the A-Clan, minus one member. We met my cousins, and their gaggle of kids, at a local park to hang out with them as they took Ella (oldest child and only daughter) to her first grade orientation. We were left with Levi, Noah and Luke. All are adorable and all are full of energy upon energy. The job was made simple in the fact that this park has a killer playground. The kids spent most of the time on the "monkey web" as they call it. It's this big contraption made up of 100's of ropes that form almost a pyramid shape that the boys (and I) had a fun time climbing. The park also has a dog park inside of it, so it was also fun to watch all of the dogs come and go. We only got to spend a couple of hours with them, but it was packed full of excitement and non-stop action. Which leads me to the best way to end the day...

...I'm actually writing this early Tuesday morning as I just woke up next to Mandi on the couch. We fell asleep snuggling on the couch at who knows what time. We couldn't keep our eyes open (which is rare for me) and before I knew it, it was the middle of the night and I still had to do my last treatment. That last treatment is currently shaking me as I sit here and I type this blog. I can look to my left and see my beautiful bride just laying here sound asleep. I can't believe how lucky I am to be able to see her every night before I close my eyes and then wake up with her every morning when they open.

My vest just ended and now I must be off to bed; it all starts over in the morning. If I'm luckily though, tomorrow will end just as today.

Martha Stewart Watch Out!

My mom has an eye for decorating. My parents' house looks like something straight out of a decorating magazine. My mom has this unbelievable ability to find the most beautiful items at unbelievably cheap prices. She'll find the coolest things at TJ Maxx, second-hand stores, you name it. And the best thing about my mom's ability to decorate, is her ability to find something cool, refashion it, and make it cooler. She can sew, build, create. It's amazing!!

...I, on the other hand, am a decorating IDIOT. I don't know what goes with what, where things go, how to find killer deals - nada. But lucky for me, I had my mom in town this last week to get me going on a decorating kick. She showed me that with just a few additions, you can make a big impact. So Ronnie and I added a few touches this week....check them out!

This is a Turkish Dough Riser I got from Razmataz and filled it with chick peas, split peas and kidney beans and candles.

Two veggie pictures from The Great Indoors

A GIANT fork from The Great Indoors- who doesn't need a giant fork, right?

An iron piece to hang on the oven hood from Hobby Lobby (like how I don't know what to even call it?!)

Used the same beans to fill the glass jars on the table - beans from Walmart.

Another iron thing, this time hung above the bed from Hobby Lobby

Clinical Trials Act

'IMPROVING ACCESS TO CLINICAL TRIALS ACT' PASSES U.S. SENATE IN VICTORY FOR CF ADVOCATES

The U.S. Senate last night passed the "Improving Access to Clinical Trials Act" (I-ACT), a bipartisan piece of legislation championed by the Cystic Fibrosis Foundation, its advocates and 120 other health advocacy organizations.

The legislation enables patients with rare diseases to participate in clinical trials without losing eligibility for public healthcare benefits.

"We are one step closer to breaking down a serious barrier to participation in clinical trials, which one day could deliver a cure for cystic fibrosis," said Robert J. Beall, Ph.D., president and CEO of the Cystic Fibrosis Foundation. "This legislation represents an important opportunity for people with CF to take part in groundbreaking research that was previously out of their reach. We are elated that this bill has been approved by the Senate."

The legislation was introduced by Senator Ron Wyden, D-Ore., with Senators Chris Dodd, D-Conn., James Inhofe, R-Okla., Richard Shelby, R-Ala., Dick Durbin, D-Ill. as original co-sponsors and an additional 14 co-sponsors also signed on.

Current law prevents many people who receive Supplemental Security Income (SSI) from accepting research compensation because it makes them ineligible to receive government medical benefits. This penalty has stopped significant numbers of people with rare diseases from participating in clinical studies.

Following Senate approval, the bill now awaits consideration by the U.S. House of Representatives. Reps. Edward Markey, D-Mass., and Cliff Stearns, R-Fla., are leading the effort to pass the bill in the House. The legislation, HR 2866, is co-sponsored by 135 members.

Passage of this legislation is particularly important for people with CF, a rare genetic disease that affects 30,000 people in the United States. A limited patient population makes it challenging to find enough people to participate in research studies evaluating the effectiveness of promising new drugs.

Source: http://pr-canada.net/index.php?option=com_content&task=view&id=241611&Itemid=58

What's in a Google? WHERE

Here's something I started a couple of weeks ago on RSBR, cause as we all know, the weekends are slow around here. I call it "What's in a Google?". It's simple really; type one word into Google and see what the magic bot suggests for you. Once in a while, you can get some pretty funny suggestions. What's crazy, is I'm pretty sure that it means these are the most common searches.

Word: Where

Top suggested searches:

where's my refund

where do i vote

where the wild things are

where is chuck norris

where to vote in arizona

where's waldo

where the heart is

where to vote

where

where the red fern grows

I've highlighted my favorite. I often wonder what Chuck Norris is up to these days and where to find him. When I actually searched for Chuck Norris, I came up with this screen:

That's enough for me! I'll be going into hiding now :)

Flash Me Friday - "The Fridge"

There is officially no Flash Me Friday today, but I'll be making up for one of the two that I've missed leading up to this point. In late May, the Flash Me called for pictures of our refrigerator but I didn't get the memo until I was walking out the door for a family trip. To see the cold boxes of others, please click here. Our fridge is pretty typical, and in a perfect world, would be bigger, but such is life. I do the best I can to keep it clean and organized, but as you'll see in the pictures, this doesn't always happen :)

What you see is what you get...although the water and ice dispenser come in handy for late night drink runs :)

Can you spot the Pulmozyme?

From top to bottom (must haves): Eggs/Powerade, Diet Mountain Dew, Cup Cakes, Yogurt, Hamburger, Bacon, Pizza Sauce

From top to bottom (in terms of organization): Reese's Peanut Butter Cup, [empty], Frozen fruits/vegetables/cheese, Meats, Grains

Side fridge: Full of ice packs and coffee

Are you interested in participating? This brainchild of Jen over at The Groettum Family blog is a weekly series in which we have to take a picture of her choosing without any cleaning up whatsoever. If you want in on the action, please visit here for the "rules".

What the _____?: August 5th, 2010

A while ago we started a little contest on this blog in which I post a picture and whoever comes up with the most clever/funny/witty/etc caption gets put into a drawing for prizes at the end of the year. Still don't have everything figured out as of yet, but we do know a couple of things 1) There will be multiple prizes 2) I love the participation so far 3) If you think your submission is inappropriate for others to see, you can always email your answer directly to me at ronnie@cysticlife.org :) 4) I've literally LOLed all weeks so keep it coming!

This week's picture:

Alright, what do ya got!?!?

Last week's photo with the winning caption:

Winner: "I'm so desperate, even a one handed hooker will do"

submitted by Allison

2nd: "Unfortunately, she is the best he can do..."

submitted by Amara

3rd: "At least this 'woman' is solid and won't pop like my blow-up doll!"

submitted by Kati

Dancing Through The Decades w/ CysticLife

Here's the question: How many of you live in the Phoenix area AND have no plans this Saturday? Please, not everybody speak up at once :) Well, it's worth a shot to at least give you guys the info on this cool little painless fundraiser that's being held with all of the proceeds going to CysticLife. Here are the details:

August 7 · 2:00pm - 4:00pm

Tempe Center for the Arts Theater

700 W. Rio Salado Parkway

Tempe, AZ

Fred Astaire Arizona is proud to present “Dancing Through The Decades” – A benefit performance for CysticLife. Both Fred Astaire students & teachers will be dancing numbers and routines to music throughout the decades to the songs that have inspired us, made us laugh, or just want you want to dance. The show is filled with novelty numbers, ballroom numbers, and Latin numbers for the audiences enjoyment. We would LOVE to see you there!!

Not in Arizona? You can still purchase a ticket just to donate to the cause!

CLICK HERE TO BUY TICKETS:
http://tca.ticketforce.com/ordertickets.asp?p=815&backurl=default.asp

I would absolutely love to see you guys there and like the blurb said, if you can't come but would still like to buy a ticket, the money is going to a great organization. CysticLife's sole mission is to make an immediate impact in the lives of CFers and their families. Every dollar that we raise can help CL fulfill that mission.

Thanks guys!!

Another Day, Another Drug Study

Can I just say, I love me some drug studies! I'm pretty much on a schedule now of doing drug studies back-to-back-to-back and so on and I'm already plotting the next one I'd like to do. I figured though that I'd fill you guys in on the one that I started today. Actually, I misspoke, I sort of started it today. I went in to give blood and urine so they could establish a baseline to compare the rest of the data with. Friday is the big day in which I actually get to take one of two doses of the drug or a placebo. I of course hope I get the real deal, but hey, somebodies got to take the sugar pill right?

Ok, so what is this drug? Since I'm no medical mind and although I have a pretty good idea of what it is supposed to do according to my doc, I still won't attempt to answer. I will however let Clinical Trials Feeds give you their answer:

Official Title: “A Randomized, Double Blind, Parallel Group, Placebo Controlled 28 Day Study to Investigate the Safety, Tolerability and Pharmacodynamics of SB-656933 in Patients With Cystic Fibrosis”

Study Drug, SB-656933, is a selective CXCR2 antagonist in development as a novel, once-daily oral anti-inflammatory agent for the maintenance treatment of Cystic Fibrosis (CF) and Chronic Obstructive Pulmonary Disease (COPD). We want to find out if this experimental drug will help decrease inflammation and slow the progression of lung disease. This study compares how well different doses of the experimental study drug (SB-656933) control inflammation in patients with CF. Two doses of the study drug will be assessed against placebo to see which dose works best.

And how will they know if this stuff works? Well, we have to go to ClinicalTrials.gov for that:

Primary Outcome Measures:

• Safety and tolerability of SB-656933 in subjects with cystic fibrosis, including, adverse events, vital signs, clinical laboratory assessments (hematology, chemistry, urinalysis, and VB-1),electrocardiographic (ECG)parameters,and exacerbation of CF [ Time Frame: 28 days and followup ]
  

Secondary Outcome Measures:

• Sputum microbiology at 28 days as measured by bacterial colony counts of Pseudomonas aeruginosa and Staphylococcus aureus [ Time Frame: 28 days ]
  
• Induced sputum neutrophil number (cells/mL) and percentage (%) [ Time Frame: 28 days ]
  
• Induced sputum inflammatory markers: (e.g. but not limited to: NE, MPO, total protein). [ Time Frame: 28 days ]
  
• FEV1 and FVC [ Time Frame: 28 days and followup ]
  
• Serum and plasma markers of inflammation: (e.g. but not limited to: fibrinogen, CRP, CC-16, MMP8, MMP9, SP-D, CXCL8 (IL-8)) [ Time Frame: 28 days ]
  
• Daily Respiratory Symptom Diary for Cystic Fibrosis (Self Reported Version (Exploratory)) [ Time Frame: 28 days ]
  
• Plasma SB-656933 concentrations and pharmacokinetic parameters including area under the plasma drug concentration vs time curve (AUC(0-24), AUC(0-infinity)),maximum observed plasma drug concentration (Cmax)and time to maximum observed plasma drug consent [ Time Frame: 28 days ]

So needless to say, I'm hoping this sucker works cause inflammation is definitely something I battle. I will keep you guys posted on how it's going and let's all hope that this can be another gun in our arsenal in the next handful of years. I guess I better go read the disclosure clause in the contract now :)

Slacker Monday

I know I know, I'm horrible because it's 11 am AZ time (meaning for many of you folks it's 2 pm) and I'm JUST getting a blog up.

As many of you know, I have been in San Fran all weekend for the CFRI conference. It was a BLAST. It was great getting to put faces to so many names and voices and to meet many people I didn't know before. I will admit, I have SEVERE small talk anxiety, so the thought of making small talk all weekend made my heart race and my palms sweaty. But as soon as the weekend was kicked off, I was chatting away sans racing heart and sweaty palms...everyone was so nice and comfortable that it made small talk bearable - even enjoyable!!

Some personal highlights - in pictures:

I finally met Lisa Greene, a CF mom and parenting guru in person after MONTHS of talking on the phone and working together!

I got to spend time with some great people in the CF world, some faces of Genentech, Novartis and Blooming Rose Foundation (BRF)

Some GREAT women - CF moms rock my socks.

A pair of FUN CF parents that I finally got to meet in person!

Grandma Bev has been around the CF community forever and runs CF Pharmacy in FL - she's a real spark plug and who I want to be when I'm grown up.

Kat from BRF again - on the right, Rose, a freakin' awesome cyster and it was soooo awesome meeting her in person after watching her over and over again on the Breathe video (in the middle is her BF)

This smiley lady is my hero - CF mom and incredible person. I'm lucky enough to see her quite often, but having her at the conference made it that much more enjoyable. She's a riot. (Can you spot Jerry Cahill?)

Finally after talking much on the phone and respecting all that she does, I got to meet Carroll Jenkins, CFRI executive director, in person.

More CF moms - These ladies are the best!

What's in a Google?

Here's something I started last week on RSBR, cause as we all know, the weekends are slow around here. I call it "What's in a Google?". It's simple really; type one word into Google and see what the magic bot suggests for you. Once in a while, you can get some pretty funny suggestions. What's crazy, is I'm pretty sure that it means these are the most common searches.

Word: How

Top suggested searches:

how to tie a tie

how to train your dragon

how to

how i met your mother

how to kiss

how to draw

how to destroy angels

how stuff works

how to boil eggs

how to write a resume

I've highlighted my favorite. How to destroy angels? I'd like to know where they're finding the angels that they're destroying. Maybe google could tell me that.

CFRI Day 1

The CFRI Conference kicked off yesterday and Mandi is there with CysticLife doing us proud. If you'd like to check out some pictures from day 1 please click here.

Enjoy!!

Flash Me Friday - "The Garage"

This week's addition of Flash Me Friday call for pictures of our garage (or wherever we decide to park our rides). Our garage is about as standard as they come except for the awesome built-ins that we were left with from the previous owner. I absolutely LOVE the extra storage, although I guess it would be nice to not have to squeeze our cars in the tighter space. Every time we get out of the car or truck we have to take extra caution to avoid any door dings. But, it's the first two-car garage I've ever, so I better stop my complaints :)

View as you pull in the driveway

Two peas in a pod

Extra storage

All that we have in our garage out of storage: bikes, extra tiles and A/C filters

Close-up of our preferred choice of transportation

Are you interested in also participating? This brainchild of Jen over at The Groettum Familyblog is going to be a weekly series in which we have to take a picture of her choosing without any cleaning up whatsoever. If you want in on the action, please visit here for the "rules".