October CF Clinic update

Today was a good day and a good clinic day at that.  First stop is always the scale, everyones favorite thing.  I have been keeping my weight up which is a good thing medically speaking but not so good in the clothes department.  I have gained 12 pounds since January, 4 of which have been since last month. There is a lot of yoga, sweatpants and other workout gear being worn around here.  Mind you, there is no “working out” going on here except shoving myself into my skinny jeans/non stretchy shorts and “fitted” tank tops. Of course there is the real exercise going on at Physical Therapy for my shoulder, which is getting much better by the way, but that is it.  hahaha

Keeping my weight up is very important because when I get sick I burn a lot of calories breathing, coughing and fighting infections.  I need a reserve tank in the weight department but it is still hard to mentally process when you weight fluctuates so frequently.  Yes, I’m skinny and I know I’m skinny.  No you don’t have to remind me I’m skinny and that you wish you “had my problem.”  Trust me, you don’t want my problem.  Lots of ups and downs in the gut area which are not fun.

All my vitals (i.e.; blood pressure, O2 sat, respirations, chest sounds, etc) were all good.  Next stop, PFTs (pulmonary function test).  Today my FEV1 was 1.58/53% and my FVC was 2.88/81% (of predicted).  Last month on September 10th my FEV1 was 1.42/48% and my FVC was 2.71/76% (of predicted).  I know I throw out these numbers all the time and they can be confusing.  The FEV1 numbers are those that we most keep an eye on.  We also pay attention to the FVC numbers.

Here is a little help with all of these pulmonary function test terms:

  • FVC (Forced Vital Capacity) — This is the total volume of air expired after a full inspiration. Patients with obstructive lung disease usually have a normal or only slightly decreased vital capacity. Patients with restrictive lung disease have a decreased vital capacity.
  • FEV1 (Forced Expiratory Volume in 1 Second) — This is the volume of air expired in the first second during maximal expiratory effort. The FEV1 is reduced in both obstructive and restrictive lung disease. The FEV1 is reduced in obstructive lung disease because of increased airway resistance. It is reduced in restrictive lung disease because of the low vital capacity. 
  • FEV1/FVC — This is the percentage of the vital capacity which is expired in the first second of maximal expiration. In healthy patients the FEV1/FVC is usually around 70%. In patients with obstructive lung disease FEV1/FVC decreases and can be as low as 20-30% in severe obstructive airway disease. Restrictive disorders have a near normal FEV1/FVC.
  • FEF25-75% (Forced Midexpiratory Flow Rate) — This is the average rate of airflow during the midportion of the forced vital capacity. This is reduced in both obstructive and restrictive disorders.

As you can see my numbers (FEV1) are up a tad which is enough for me to be happy about it.  I’ll let you in on a little secret.  I am on day 9 of a 13 day Prednisone taper.  I started the taper with 60mg for 3 days, 40mg for 3 days, 20mg for 3 days, finally 10mg for 4 days.  Prednisone is a drug/medicine that can work wonders and open your airways, bring down inflammation and help calm bronco spasms.  With those medical wonders comes the not so pleasant side effects.  Some being; anxiousness, insomnia, food cravings which brings on lots of eating i.e. gaining weight and puffy face, irritability, what I like to call “prednisone brain” = lack of memory, which my memory already isn’t great.  If you see me searching for a word and get tired of waiting, just make one up and move on. hahaha.  So far the prednisone taper has been without any major issues which is pretty special because it is also “that time of the month.” Speaking of periods, I am trying a birth control pill (skipping placebo week, continuing on to next pack) to stop my period in hopes that it will keep my hormones level and not effect the frequency and fluctuation of my lungs exacerbations.  The poor men in the house.  All is good and for that I am thankful.  Remember, I am the only female in this house so it can be challenging.  I am surrounded by my 2 human guys and our 2 furry boys.  The only extra estrogen is in the house when our daughter/my stepdaughter visits which is coming up soon and we can’t wait!

Asthma side of things:  We discussed what new asthma inhaler to try because some have caused issues in the past.  Most of the powder based inhalers (like Spireva, Tudorza, Atrovent pod, etc)  seem to cause more irritation which in turn makes me have lung bleeds.  We settled on Dulera (100 mcg/5 mcg) 2 puffs twice a day.  DULERA is an FDA-approved inhaler that combines 2 medicines that treat asthma. DULERA contains an inhaled corticosteroid called mometasone furoate and a long-acting beta2-agonist medicine (LABA) called formoterol fumarate dihydrate.  One medicine in DULERA helps to decrease the swelling (inflammation) in the lungs that can cause asthma symptoms. The other medicine in DULERA helps to relax the muscles in the airways. This helps to prevent symptoms like wheezing and shortness of breath that can happen when asthma makes the airways close up (constriction).

Orkambi:  Disclaimer: a full dose of Orkambi is 2 pills in the AM and 2 pills in the PM.  We discussed an alternative way to get started on Orkambi in hopes that I wouldn’t have the significant shortness of breath and tightness like before.  I will take 1 pill in the AM for 3 days and see how I am doing on that.  If I feel like I can add another dose I will add another single dose pill in the PM.  I will stay at that for 1-2 weeks. Then hope to be at full dose of 2 pills in the AM and 2 pills in the PM, full prescription dosing.

I didn’t get away from there without a lab form for the lovely 2 hour GTT (Glucose Tolerance Test) for blood sugars.  It is not one of my favorite tests to take.  It’s not a fun one.  I will be seen back in clinic in 2 months to see how I am on the Dulera.  Here’s to hoping it will do the trick.  This asthma of mine has us on our toes for sure.

Until next time…

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