Sunday, March 26, 2017

Staying Alive: A Daily Struggle

Are you a mom, wife, husband, dad, brother, employee, volunteer? What do you do every single day? What does your life look like?

I have a wonderful live that I want to keep enjoying for many more years to come. But, keeping up with everything I do can be difficult. Some CF patients do more than me (they might be on oxygen, on a Transplant list, take more medications), each case is different. I think that is the hardest part for the public to understand. I think people see me working as a Real Estate Broker, doing volunteer work, going to church, grocery shopping and forget how much of my CF is "behind the scenes." This post may seem dramatic, but I want everyone to FULLY understand what it is like to have Cystic Fibrosis and WHY we need to cure it!!

Here are the things I do to remain healthy:

  •  I keep my weight up (eat a ton of calories a day around 4,000 a day. Since Orkambi I don't have to force myself to eat, because I'm always hungry. I take around 15 enzymes a day to help digest all my food).
  • I take 5 vitamins a day (including over 50,000 units of Vitamin D per week), still they always seem to be a little low.
  • take zithromax (the only oral antibiotic I'm not allergic too). I take zithromax (AKA: z-pack) 3 x a week. This help keeps the bacteria (CF germs) in my lungs under control and helps with inflammation
  • I take two inhalers daily. Spiriva & Advair (the 2nd is a steroid) and both help with the "asthma type" component to my CF.
  • I do at least 9 breathing treatments (nebs) a day. I do 3 Abluterol, 2 hyperontic salines, 1 pulmozyme, and then my antibiotic nebulizers (3 Cayston or 2 Colistin or 2 Tobi). Lately I have been trying to do 4 albuterols a day (like I do in the hospital). I have to switch up the anitbiotic nebs every month to keep my CF bacteria from becoming resistant to it. However, in the last 3 months we found out my Psuedo is resistant to Cayston. Which means I'm switching just between Tobi & Colistin now. Each neb takes an average of 10 minutes to breathe in. They take 10 minutes to wash properly after each use (Then I sterilize in Wabi for an hour- kills psuedo/other germs in the water). That's 2 hours of my day just for nebs!
  • I do 2 vest (chest percussion) treatments a day. I wear a vest for a 30 minutes a time. It shakes me, helping the gunk loosen off the lung walls (allowing me to cough it out & breathe better). I struggle with this (due to my small airways- we think from being 12 weeks premature).
  • drink nutritional supplements. I try to get my calories by food (avocados especially, yum!). But I usually drink at least 1 ensure a day (2 if I'm on-the-go).
  • I'm also taking Orkambi (since October 2016). It's a gene-targeting drug. It helps make the CF defective gene work about 20% more correctly. It's a not a cure, but a step in the right direction. Orkambi has to be taken 12 hours (9am&9pm) apart and with 20 grams of fat. It makes me very hungry all the time (doubling our grocery bill per month).
  • Once a month I get my mediport flushed (with a needle and saline) to maintain the flow (to keep it from clogging). A Mediport is an automatic IV line. It directly channels into a major artery in my neck for IVs. We can not draw blood off mine (due to another medical problem I have). I had it put in at age 12 after exhausting all options (Ivs in between my toes, legs, arms, hands, even on my head once). I have had a few regular IVs since. typically it's 4-6 tries before getting an IV started. 
  • Blood draws, labs, tests, scans, are very routine. At least one per every other month (almost every month).  I have 1 good vein left, I get all my blood draws off that one spot. In a hospital after 2+ draws per day its start to build up some scar tissue though, so they baby it more.
  • Doctors visits. I have been going to clinic typically every other month for the last couple years ( I have been struggling more for the last few years).
  • Working-out is what I struggle the most with. I walk 5,000 steps per day and I have been doing a PiYo class (30 minutes) 1x week. It's not enough, but it's all I can do right now. 
Having 3 pharmacies, and 20 prescriptions per month is a lot. Adding in the 2-3 hospital stays per year. I typically stay in a hospital for 3 reasons:
  1.  I'm having surgery: I have had 4 bowel resection/intestinal blockage surgeries. I've had 5 hernias, 2 mediports, 2 stomach feeding tubes, surgery on both my eyes, and wisdom teeth (if it counts as a surgery). I've had a bronchoscopy, endoscopy, colonoscopy, and a camera inserted into my groan (run up through an artery... That one was my favorite. I was awake, it felt like a
    worm, I got to watch it all on the camera, so freaking cool! I'm hoping after my eye surgery in the fall I'm done for a while.
  2.  I have pneumonia, lung infection, a virus, or even the common cold. Yes, a cold can turn into something real nasty very quickly for someone with CF.
  3. My clinic results are very low or something may be going on and we aren't sure what. 
For example: In November I was admitted due to my lung functon dropping to 32% -we thought it was due to my starting Orkambi. But since my baseline from 2007-2016 was between 50-55% we knew something was going on. 

When admitted I am on many medications. I'm always usually on 2 IV antibiotics: Tobramyacin (which comes with bloodwork to keep an eye on my levels) and Zosyn or Meropenem. I'm allergic to Keflex, Bactrim, and the Levaquin/Cipro family. Plus, my psuedo is resistant to Cayston/Aztreonam. So it gets a little tricky for us once our bodies start to become resistant. I'm surprised and lucky I even have options at my age with all my allergies.

Even with doing everything we are supossed to, still sickness happens, refill gets missed, insurance won't pay for a med, etc. Our health sadly suffers each time. I have been 32%-48% lung function since April 2016.  Meaning my baseline technically in the last year has been 40%. I refuse to admit it and still hope it was just a bad year. I want my lungs to get back to 55%. Only time will tell. The lower they go and longer they are low the harder it is to recover lost lung function (permanent damage and scarring happens).

Everyday I have meds to take, nebs to do, vests to do, nebs to clean, food to eat, chores to do, errands to run, pharmacies to refill meds, meds to pick up, a career to handle, and house to clean. I feel as if every single minute of my day is busy with things I have to do. I have always had this kind of schedule, so I have learned to deal with it. My family & friends always understand when I just don't have time to travel to see them or have to do meds at their home.

Right now it just seems it is a bit more difficult. Insurance problems with Colistin  combined with the weird weather has dropped my lungs to 37%. Hoping April 11th I will get good news and be back up somewhat (enough especially to do the clinical trial that is coming up). 

This is what life is like in my shoes. Please help us cure CF. Spread Awareness, Share our Stories, or even donate to the cause!

Thank you for reading and following my Blog & CF FB page! 

Sunday, March 12, 2017

Our Local Chapter & Awards Night

Last night was the CF Foundation Peoria Chapter's Volunteer Appreciation Dinner & Awards Night. The Peoria Chapter hosts this every year to say a BIG Thank you to all the volunteers & give out 3-4 awards to individuals/Teams.

  Last year I attended as a guest/ award recipient. My parents and Andrew went with me to celebrate. I was very honored to receive my award last year "Outstanding Great Strides Partner for 2015." This year I was excited that Andrew was asked to be the evening's MC. He did a fabulous job! I'm so proud of him. I was the tech person for the evening & ran all the speakers slideshows & video content. The evening went very smoothly and the night was a huge success!

Peoria Chapter only has one staff member, Kellie. Many Chapters have different staff members for different events. Kellie oversees Peoria, Princeton, Bloomington, Champaign, Tuscola Great Strides Walks, the Peoria Suzana Lee Golf Outing, the Peoria Gala, and helps out with other secondary non-foundation sponsored events. This would include Red Dog Festival in Canton and the IVC High School lunch auction. So that is between 7-10 events a year she coordinates or help with. So last night is VERY important to the Chapter, without the dedication of volunteers, she couldn't do it all by herself.

(Andrew & I before the event started, before I put on my mask (and the other CF patients arrived).

I founded and decided to start the Great Strides walk. 3 other ladies in Princeton really stepped up and helped me out! Kellie has 3 events in June each year and Princeton really does manage itself (almost). Thanks to all the volunteers I have & people that always donate our food (Subway-Henry,IL), music (T Entertainment), activities like face painting, balloons!

 In the last few years I have really gotten to know Kellie. I don't consider her an acquaintance, I consider her a friend, so I enjoy planning events with her & helping out whenever I can. I became responsible for the food & entertainment at Peoria & Champaign walks the last few years, because I have contacts for those items. Andrew works at HyVee. The director of that HyVee donates all the water for the walk, and only charges us "at cost" for the sandwiches. He also donates auctions lunches for 10 people for the IVC lunch auction. I started getting involved in the Golf Outing & IVC High School lunch Auction last year & I will be the featured speaker at the Red Dog event this year. You may think that is a lot, BUT we have other individuals that volunteer for more than one event & really stepped up to chair certain events besides me.

I'm constantly amazed by the support the Peoria Community gives to the CF Foundation. We have an excellent CF Foundation Chapter ran by Kellie and an amazing CF Center (with a Research Coordinating Center). Our new Director of the CF Center in Peoria, Dr. Kharellah made a presentation to discuss the advancements of the CF Centers & how our local OSF center compares to other hospitals for the awards night. I love his passion for constantly striving to improve out standards to make us one of the top 10% of centers in the nation! Our CF Center Nursing Staff attended the Volunteer night too. Our CF Center Staff fully supports the CF Foundation & each CF patient! I love they encourage to do the best we can! 

(Picture: I'm with my 2 CF Nurses (I've had since I was little, LOVE Sara & Marie! )
Congrats to all the award recipients for 2016, you deserve it and without YOU our Chapter wouldn't be growing! Thank you to everyone who walks with me at one of the Great Strides Walks or donates to our cause, it means the world to me. Thank you for understanding March-June are my crazy months with CF events and putting up with my constant posts about my mission to CURE CF! But, we all know you want to cure it too! 

So Thank you everyone!!!! 

Friday, March 3, 2017

Should I prepare for a Lung Transplant

I hear a lot of people say "I wish you could get a lung transplant" or "Can you get a lung transplant."   Which is fine and I completely understand. It does sound great.. new lungs right?!?!?! But I heard A LOT of those remarks over the last few months when I was struggling in the 30s for lung function.

I wanted to explain. No, I don't want a lung transplant (tx), well not until I have to look at them. And no, I cannot receive one yet, which is a GOOD thing! A double lung transplant is way of extending our life when we are running out of time, it is not a CURE. When someone is waiting for a transplant, they are holding their breath, just hoping to get a pair of lungs. To even be considered you normally have to be below or around 30%. So by that point daily life is fairly limited and difficult, but if you get too ill or drop too low, it you may not be eligible anymore. So I don't wish to ever get that low in lung function, I do not wish to struggle for breath anymore than I already do. When I was low 30s I couldn't even carry a grocery bag or walk around a store, daily chores were exhausting. I cannot imagine what it is like being in that range or lower every single day knowing it isn't going to get better.

Also, you have to think about all the risks too. While, I will never turn one down in the future, it is very complex. The CF Foundation has a great website full of information about transplants HERE!

But I am a numbers person. Yes, huge nerd. I loved my college stats & stats research classes! Here are some numbers: Most people to be eligible have technically less than 1 half of one lung working, terrifying huh. Once you do get listed it can be months, years before (if) you get the call for lungs!

While I'm beyond thankful to everyone who is an organ donor & happy to say over 200 people with CF received transplants in 2014, I do not wish to need one yet. In fact, I hope to never need one... because...  around 80% survive the surgery (so 20 out of 100 don't). Out of the 80 people who do survive the surgery, only 40 of those people will be alive after 5 years post transplant. So out of the 100 originally listed, only 40 are still alive in 5 years! Everyone is different, some friends have celebrated 10 year lung anniversaries already, while some of my friends were denied (Laura), did not receive lungs in time, didn't survive surgery, or their body couldn't fight the rejection of the organs(Alyssa). I'm just saying those numbers aren't good enough for me to want a transplant. My CF doctor discussed transplants a little with me this fall. He said my allergies to the Keflex, Bactrim, Levaquin families won't hurt my chances, but we would have to find ways to work around those, my pain med allergies & drug resistance. So it is tricky for people with CF, lots of things to consider.

It's a long and difficult road pre & post transplant. Andrew & I have discussed this in depth. Our goal is for me to never need one, but as you can see this fall (one small illness or change in meds) can drop my lung function so fast. It is hard to recover from that. So while we hope I don't need one, we are making sure we are prepared.

I have researched a couple hospitals that I would feel comfortable with (knowing their numbers) and even have friends that live near those cities. Andrew & I know there might be a day we have to pack up and move while I am waiting for lungs. We also know how expensive it is, over $800,000 for hospital, surgery, and medical related costs. This doesn't include transportation to hospital, gas, food, extra rehab. Plus, Andrew might not-working for periods of time, in order to care for me. We started a Health Savings Account (AKA transplant fund) this fall. His employer matches up to 1,000 a year. So we put slightly over 1,000 of his paycheck into it a year and his company matches. Now for more math.

One CF doc in the past explained to me in a good year I will lose 1-2 % and in a year I get sick (flu, or pneumonia) probably more like 5% (this was pertaining to me particularly, not everyone with CF). Now I currently have 45% and TX range is 30% so I have 15% of "wiggle room"  assuming I'm healthy every singe year (average 1.5% decrease per year) = 10 years I would need a TX, sooner if I get sick more often.  I have beaten those numbers, for example from 2010-2012 I gained lung function. Some years I maintain too. So we are working towards me not needing one, but planning just to be smart.

We put $1000 per year in the HSA & his employer matches: it means we will have $20,000 for transplant expenses in 10 years or 40,000 in 20 years. We also opted into a higher paying & higher benefit insurance through his employer. We pay more, but it has critical care pay-outs. For TX it was like an automatic $20,000 & $500 per travel expense (for 1 person).

I'm also making sure I gain more muscle & I'm trying to be as healthy as possible. I'd love to see my lung function rise up again, it takes dedication & time. So only time will tell. I wanted everyone to have a clear picture of what TX means & specifically how it affects me. I don't see it as my goal, I see it as a back-up plan. My goal is to keep raising money & see CF cured before I need a transplant!!!

Also, please consider being an organ donor, check out DonateLife website for more info & facts, for instance did you know "Just one organ, eye, and tissue donor can save up to 75 lives!