Tag Archives: CF awareness month

IV time again

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Welp, I made it 4 months and 20 days since I was last admitted to the hospital. Which means it’s been 4 months and 8 days since my last round of IVs ended. I have been extra out of breath the last week and a half, give or take. Walking and bending over are making me more short of breath right now and it’s taking longer to recover and get my breathing back under control. I did lung functions on my home spirometer and they were down 5%. In March at my clinic appointment my FEV1 was 35%. Yesterday my FEV1 was 30%. I sent an email to my nurse yesterday with a copy of home spirometry results and asked for IVs and a prednisone taper. I hate admitting defeat and waving the white flag of surrender but it’s the necessary evil. I will be doing IV cefapime (IV push over 10 minutes) every 8 hours for 11 days. My prednisone taper will be 60mg for 3 days, 40mg for 3 days, 20mg for 7 days and 10mg for 7 days. We are hoping that by dragging out the end of the prednisone taper it will give a good burst at the beginning and then keep it in my system for longer to hopefully open my lungs up better. Prednisone hasn’t been working like it used to. Prednisone is to help combat the inflammation in my lungs and airways. I also have bad asthma on top of crappy CF lungs so it’s a double whammy. My lungs are very scared from CF disease progression and inflamed from asthma. Unfortunately you can’t reverse that damage so I have to work with what I have.

I’m only doing 11 days of IVs because we will be going on vacation very soon so I’m only able to do a shortened round of IVs and hope for the best. If I’m not feeling any better when we get back from vacation, I will call my doctor and we will work out a treatment plan. I’ve traveled with IVs before and it’s a pain in the butt so we’re squeezing in 11 days so I don’t have to travel with them. My IV supplies and antibiotics will be delivered this evening. Tomorrow morning I go to get my port accessed and labs drawn. While I’m at the hospital I will get a chest X-ray as well just to make sure I’m not dealing with anything extra since I have had some lung pain. I’ll go back to the hospital to get my port reaccessed (the needle has to be changed every week while accessed for IVs) and more labs next Tuesday. Hopefully this does the trick so I can enjoy our vacation. If not, I’ll push on through like always and have all the fun while we are gone and then come home and get going on another round of IVs most likely.

Nothing like ending CF Awareness month with a round of IV antibiotics. haha Although, I’m always aware of CF in my life and it’s inconvenience but that’s ok, it’s my life and I’m thankful to have the life I have. I’ll update next week after I do my home lung functions.

Until next time…

Depression on a rainy day

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Depression on a rainy day is no fun.  Depression itself is no fun to live with.  Depression on a holiday where we honor the men and women who paid the ultimate sacrifice for our country is not easy either.  Reading/hearing all the stories of the fallen heroes is hard when you are not having a good mental day.  I got teary eyed (as usual) as the XM radio’s “The Highway” played “Taps” to honor those killed in action.  This weekend has been hard one for me and I don’t really know why.   Continue reading →

CF Awareness Month

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1938 Dorothy Andersen, M.D., writes the first comprehensive medical report on cystic fibrosis.
1953 During a heat wave in New York City, Paul di Sant’Agnese, M.D., and others connect the extra loss of salt by people with CF to the disease’s underlying cellular problem.
1955 The Cystic Fibrosis Foundation becomes incorporated as the National CF Research Foundation and awards the first research grants to Dorothy Andersen, M.D., Paul di Sant’Agnese, M.D., and Harry Shwachman, M.D.
1961 The Foundation establishes the accredited care center network by creating two centers devoted to treating CF.
1962 The CF predicted median survival age reaches 10 years.
1962 A total of 30 Foundation-accredited care centers are in operation.
1964 To investigate CF at the cellular level and find answers about this complex disease, the first basic science committee is established.
1966 Patient Registry launched to collect health information of patients seen at Foundation-accredited care centers.
1978 The number of Foundation-accredited care centers totals more than 100.
1982 The Research Development Program, a network of research centers at leading universities and medical schools nationwide, is established.
1989 A team of Foundation-supported scientists discovers the defective CF gene and its protein product (CFTR), opening the door to understanding the disease at its most basic level.
1990 CF researchers achieve “proof of concept” that gene therapy (in the laboratory) is possible.
1993 The U.S. Food and Drug Administration (FDA) approves dornase alfa (Pulmozyme®), which is proven to thin the thick mucus in the lungs and is the first drug developed specifically for CF.
1997 The Foundation establishes the Therapeutics Development Program.
1997 The FDA approves inhaled tobramycin (TOBI®), the first aerosolized antibiotic designed for CF, which is proven to reduce hospital stays and improve lung function.
1998 Specialized clinical research centers are designated as the Foundation’s Therapeutics Development Network.
2000 Cystic Fibrosis Foundation Therapeutics Inc. (CFFT), a nonprofit research affiliate of the Foundation, is established to govern drug discovery and development efforts.
2000 Foundation-supported scientists map the entire genetic structure of the most common cause of CF lung infections, the bacteria Pseudomonas aeruginosa.
2002 A CFFT-supported study shows the antibiotic azithromycin improves CF lung health.
2003 CFFT-supported scientists at Structural GenomiX Inc., determine the three-dimensional structure of a portion of the CFTR protein, opening the door to more drug discovery opportunities.
2004 CFFT-supported studies in Australia and at the University of North Carolina show that inhaled hypertonic saline helps clear CF mucus and improve lung health. It becomes a therapeutic option.
2006 Ivacaftor (formerly VX-770), an oral drug in development that targets the faulty CFTR protein, enters clinical trials. Ivacaftor is designed to open chloride channels that do not function correctly in people with the disease.
2008 Phase 2 studies of ivacaftor in people with the G551D mutation of CF show unprecedented improvements in key signs of the disease. The studies achieve “proof of concept” that it is possible to treat the root cause of CF.
2010 The FDA approves a new antibiotic, aztreonam for inhalation solution (Cayston®), to treat CF lung infections. The drug offers an alternative for people with CF who battle recurrent infections and develop resistance to existing antibiotics.
2012 The FDA approves ivacaftor (Kalydeco®) for people with the G551D mutation of CF ages 6 and older. The drug is the first to address the underlying cause of CF and opens exciting new doors to research and development that may lead to a cure for all people living with the disease.
2014 The FDA approves ivacaftor as a single therapy to treat people ages 6 and older with one of nine other rare CF mutations in addition to G551D, and later extends approval to children ages 2 to 5 with any of these 10 mutations — representing about 8 percent of the U.S. CF population.
2015 The FDA approves the lumacaftor/ivacaftor combination drug (Orkambi®) for people with CF ages 12 and older who have two copies of the most common CF mutation, F508del — representing about a third of those with CF in the United States.
2016 The FDA approves lumacaftor/ivacaftor (Orkambi®) for children with CF ages 6 to 11 who have two copies of the F508del mutation. The decision means that about 2,400 additional children in the U.S. are eligible to receive the drug, bringing the total number of those eligible for the treatment in the U.S. to nearly 11,000.
2017 Two Phase 3 clinical trials of tezacaftor (VX-661) in combination with ivacaftor (Kalydeco®) demonstrate positive results not only for people with two copies of the F508del mutation, but also for those who have one F508del mutation and a second mutation that results in residual function.
2017 The FDA expands the use of ivacaftor (Kalydeco®) to people ages 2 and older who have at least one of 23 residual function mutations in the CFTR gene. The FDA’s consideration of laboratory evidence coupled with clinical data to address the needs of people with CF who have less common mutations is an important step forward for the CF community.
2018 The FDA approves tezacaftor/ivacaftor (Symdeko™) for individuals with two copies of the most common cystic fibrosis mutation, F508del, as well as for individuals who have a single copy of one of 26 specified mutations — regardless of their other mutation. This approval paves the way for new, more effective triple combination therapies (treatments consisting of three different modulators, including tezacaftor), which are being tested in clinical trials.
2018 The Foundation maintains a robust pipeline of potential therapies that target the disease from every angle. The more drugs in the pipeline, the greater the odds of producing successful therapies and a cure for all people with CF.

Happy CF awareness to me

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I have not been feeling good for about 3 or so week, maybe more.  I did the 2 weeks of IVs 2 weeks ago and wasn’t much better but that is probably due to the fact that my pseudomonas is multi drug resistant.  We are limited on what will “work” on the stubborn drug resistant bug (pseudomonas) in my lungs.  I made the dreaded call after the 4 days of 40mg of prednisone and no improvement.  I need something more therefore I had to call with all the I have coming up.  I have a busy 5 weeks ahead.  Next weekend we are going to the Zac Brown Band Concert, a bucket list item for myself.  Then I have a girls trip to the Gulf Shores the next week.  Then in 5 weeks we have our good friends wedding weekend. Continue reading →

CF Facts

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Cystic fibrosis (CF) is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:

  • clogs the lungs and leads to life-threatening lung infections; and
  • Obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

Continue reading →

CF Awareness Month begins

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May is Cystic Fibrosis Awareness month.  This is the month many CF Foundations do their Great Strides fundraising walks.  I am very aware of CF every day obviously.  Now is the time to educate others.  If you have questions about CF or what I have to do every day feel free to comment on this post and I will answer them. Continue reading →

It’s Parole time!

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I haven’t had any more hemoptysis (blood) since the second embolization.  Yay!!!  My groin is very sore where they went in for both embolisms. They went in on the right side for both embolisms.  My chest is a little sore. Since I’ve been laying in bed for the last 4 days, it doesn’t take much for me to ‘overdue’ it.  I washed my hair in the sink and it was just enough to make the pain in the groin more noticeable.  Also, I get short of breath more easily right now due to the embolisms. Basically, my lungs have had a bit of trauma over the last week.  Continue reading →

Coughing up blood is no good

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Well today didn’t turn out the way I planned at all!  I woke up around 3am coughing up blood. It was a few tablespoons or a little more.  It finally tapered off after about 30 min.  Back to sleep for me, not good sleep but some sleep.

Then my day started off with a fantastic visit with a great friend. Then it was time for all of my errands. First stop of weekly labs at UT.  I have to have labs 2-3 times a week when I’m on IV antibiotics to monitor my kidney levels, liver levels, blood counts, etc. Then it was on to my errands. First stop was Staples to pick up more Shannonigans flyers and donation letters! Then of course I had to stop by Sun Tan City to get tanning points because they went on sale today! They are for when I’m off of IVs…Hopefully that is sooner rather than later. Next stop, Target for a few things.

When leaving Sun Tan City I started coughing at 12:30pm, I started coughing up blood again. I drove to my next destination, Target, while coughing up blood.  I sat in the car coughing and hacking up blood for a good 30 minutes. It finally subsided enough to streaks so I could “run” (we all know I am not running anywhere) into Target to get 2 polo shirts for the teenager. He goes to his moms for the summer and they will be doing family pics so he needed something nicer.  Next up was a run through the bank, a stop at the pet store for dog food and then Hobby Lobby for a quick tour.  Did some window shopping and took pictures of what I liked for the teenagers room makeover this summer (it’s a surprise). His room has been the same for 5 plus years. I don’t usually talk about the kids for their privacy and out of respect for their mom. This has been a monumental month….the teenager turned 16 and is now street legal in his new car.  He is doing decent on his own so for.  The young adult child is going great in college and getting her first apartment.  She is growing up and adulting!!  We all are very proud of them both, myself,their dad and their mom.

On my way home I realized that I still needed to get a watering can for plants and to dilute the boys (dogs) pee so they will stop killing grass and plants by peeling on them. Hahaha

I got home and was taking care of all of office stuff and once again the blood mama pouring out!  This was at 3:30pm. I texted my husband and asked him to come home. I made the dreaded phone call to my nurse and she called back in about 8 minutes.  She said my doc wanted me to go to the ER and that he had already called all the people he needed to to let them know I was on my way and what was up.  He had already requested a bed for me and all.  He also had written down what he wanted them to do for/with me. All together I coughed up probably 3-5 ounces. Heck a little bit is enough for me to not be a fan of it.

I got checked in and triaged quickly, then the wait was on for an ER room. Then off to chest CT for a scan with contrast (thank God for ‘Pollie’ my power port). The scan didn’t show any active bleeds at that moment. After the scan, I was whisked away to my room.  I am in the CVICU (cardio vascular ICU) for monitoring due to the massive blood espisodes.  I will hopefully see my CF doc tomorrow. I’m hoping it’s one of the duckling doc I know and like. It makes my life in here easier.  I am on IV Zosyn (just like I was at home every 6 hours), IV Colistin (every 12 hours IV push) and staying on the oral high dose Bactrim (1600mg three times a day).

I have no idea how long I will be in lock up. My husband seems to think it will be a short stay but we shall see.  I am NPO after midnight just in case they need to perform a procedure of some sort like a bronchoscopy or exploration in IR (Interventional Radiology).  I just stuffed my face with a cheese burger and Mac and cheese?

The plus is, I got all of my errands run. The negative is, I didn’t get all the phone calls Made, or the laundry done, or office stuff organized…. Oh and I was short of breath all day AND I coughed up blood 3 times. The day started off good even with the middle of the night blood situation. It was a beautiful day in East TN and now I’m in lock up! Ugh

REMINDERS:

5/24: Tomorrow is the Texas Hold’em tournament, silent auction and 50/50 raffle!!  It is at Tanners in Lenexa at 7pm.  Invite your friends even if it’s just to hang out.  It is being held in my moms memory and to raise funds for the Shannonigans Lung Fund    Texas Hold’em Tournament, silent auction and 50/50 Raffle

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6/5:   The deadline for Shannonigns Gear online sale!!!!  Please try to budget it in for your last paycheck of May. I REALLY don’t want anyone to miss out in this fabulous design. Http://design.goteez.com/Shannonigan_strong  Ready, set, shop……

Shannonigan Strong online sale

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9/17: 2nd Annual  Shannonigans Cornhole Tournament held at the Casual Pint-Northshore at 2pm. Lots of raffle items including a custom set of cornhole boards!  2 Annual Shannonigans Cornhole Tournament

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To make a direct donation to the Shannonigans Lung Fung, there are 3 ways:

http://www.gofund.me/shannonigans

http://www.paypal.me/shannonigans

Personal (non rubber) check made out to myself.

I am super tired. I’m off of here and I hope I didn’t forget anything.  I’ll update tomorrow when we have a news worthy update.

Until next time….

Our time in the ER and getting settled in my room. I look thrilled huh?

Oscar and Oscar II have arrived

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Today was an early start.  I had to be at the hospital for labs early because we ha plans to go to Dollywood the amusement park and wanted to be there when it opened at 10am.  So I made to the hospital for labs, stopped for gas on the way back and made it back home by 8:05 am.  We made it to Dollywood around 10:15 am, they opened at 10am.  Our tradition in our family is that we have a family day and/or night before one of us goes away for an extended period of time. My step-son is heading to his mom’s for the summer so that meant a family day was in store.  What I learned today was 1.) handicapped parking when at an amusement park is a life saver 2.) you don’t realize how much lung power you use while riding roller coasters until you are out of breath while riding one 3.) long lines for rides give you time to calm your breathing and regain control of it 4.) your inner thighs get sore from “holding on” and finally 5.) I was right when I said that I could see a lot of coughing from laughter in my day.  We sealed family day by playing a game of Sequence after dinner.  Continue reading →

Holy lung spasms batman!

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There’s nothing like being woken up by your lung/back spasming at 5am.  Luckily I had a few pain meds left from my kidney fiasco a few weeks ago.  They took the edge off of the pain.  I wasn’t able to take a deep breath without my lungs freaking out and spasming.  I had to take short little breaths as to not expand my lung very much.  It just takes your breath away when there is pain when you breathe.  I emailed my nurse and they had me go for an x-ray to rule out a collapsed lung (small or big).  It takes great talent taking in a deep breath for an x-ray when it hurts.  Add that to my list of super powers…haha.  The x-ray was fine.  So that means that it is just good ol pleuritic pain.  Continue reading →