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! 

2 comments:

  1. You are a warrior, and I am in awe of your dedication to your health. I am a mom of a cf patient, and I'm inspired by your adult life.

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    1. Thank you for saying that. I must admit my own mother taught me most of what I know. There is hope, just some work that comes along with it. Thanks for reading my blog and I am glad you enjoyed my post!

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