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Friday, December 28, 2012

Probiotics and exacerbations

Other than the prevention/treatment of C Diff, looks like these buggers may help prevent exacerbations. 


Pediatr Pulmonol. 2010 Jun;45(6):536-40. doi: 10.1002/ppul.21138.

Probiotic supplementation affects pulmonary exacerbations in patients with cystic fibrosis: a pilot study.


Division of Pediatric Gastroenterology and Nutrition, Safra Children's Hospital, Tel-Hashomer, Israel. weissb@sheba.health.gov.il



Probiotics reduce intestinal inflammation in, and Lactobacillus GG (LGG) reduces pulmonary exacerbation rate cystic fibrosis (CF) patients. We intended to determine the effect of a mixed probiotic preparation on pulmonary exacerbations and inflammatory characteristics of the sputum in CF patients.


A prospective pilot study of 10 CF patients with mild-moderate lung disease and Pseudomonas aeruginosa colonization, treated with probiotics for 6 months. Pulmonary function tests (PFT's), sputum cultures with semi-quantitative bacterial analysis, and sputum neutrophil count and interleukin-8 (IL-8) levels were compared to pre-treatment and post-treatment values. The rate of pulmonary exacerbations was compared to 2 years prior to the study.


The exacerbation rate was significantly reduced in comparison to the previous 2 years and to 6 months post-treatment (P = 0.002). PFT's have not changed at the end of treatment and during 6 months post-treatment. No change in sputum bacteria, neutrophil count, and IL-8 levels was observed.


Probiotics reduce pulmonary exacerbations rate in patients with CF. Probiotics may have a preventive potential for pulmonary deterioration in CF patients.
 [PubMed - indexed for MEDLINE]


Wednesday, December 26, 2012

Well, well, well, look what we have here

Years and year ago, when I was purporting the benefits of the eFlow (loooooong before Cayston was approved.... check out my posts from 6+ years ago), I had a really hard time finding internet links for the eFlow studies that had been conducted.

Ultimately I located them on random internet pages, which were subsequently taken down. After exploring the CF Services webpage (not sure I remember why.... I really despise that pharmacy), I came across the same studies I had referenced years ago:



I still have the pictures of the studies on my blog, but I just found it funny that this information has become a bit more "mainstream."

Happy eFlow-ing.

Sunday, December 9, 2012

C Dff

WARNING: this post contains extensive and graphic discussion of feces. Not for gratuitous purposes, but rather to help out other CFer's who want to prevent or who suffer from C Diff. You are warned. 

Maybe it's just my lack of exposure to the CF community, but I haven't heard many talk about struggles with C Diff (Clostridium difficile). 

So when I was diagnosed with this a week and a half ago, after my second hospital admission in 2 months (I was admitted the 2nd time for sinus surgery and we agreed more IV abx would set me up for a good chance of recovery), I searched the web feverishly for answers for how to treat this potentially deadly condition.

PWCF are no strangers to life-threatening conditions, this is true. But I've mostly been a pulmonary CF type gal for the 31 years I've been on the planet - no real weight problems, despite being PI. I take digestive enzymes, and as of 8 months ago I do have CFRD, but other than that, I don't give my digestive system much attention.

I've always taken probiotics off and on, mostly to either prevent a yeast infection while on IV abx or more recently to boost my immune system (thanks to smart Cyster Mandy). But that was really the extent to which I knew about these little heroes that live in our bodies.

So I'm sure you're absolutely shocked to hear that I went on a whirlwind of research upon being diagnosed with C Diff. I'll give you my research and actions in a few paragraphs - first I'll describe my symptoms. 

I was constipated without a BM for 72 hours - finally had a BM in the early morning of my 1st Friday in the hospital, and I ended up having 7 more that day that were pretty explosive diarrhea . I don't typically get diarrhea with IV abx, but sometimes loose stools. A quick C Diff culture showed I did in fact have the affliction. 

I felt slightly nauseated that Friday, and a bit distended, but nothing too horrendous other than frequent trips to the bathroom. Saturday, however, brought some of the worst pain I have felt since I passed gallstones. I was put on Simethicon and given a small dose or morphine to make me comfortable. I've never been on anything stronger than Tylenol + codeine, so I was pretty afraid of taking a strong pain killer. But the pain was too great and wasn't going away.

I was put on Oral Flagyl and Vanco (in addition to the other 3 IV abx I was on to treat the PA in my lungs) to treat the C Diff - very standard therapy. I was also given packets of lactobacillus to hopefully build up the good bacteria in my gut to combat the C Diff. The lactobacillus was refrigerated by the hospital, but I wasn't sure what the brand was or what the quality was.

Luckily the pain of Saturday never came back - thanks to lots of praying and I think the Flagyl and Vanco (and maybe even Simethicon) were doing their job. The diarrhea, however, did not go away until well over a week later. More on that later. 

As I was healing from my 1st sinus surgery and cranky lungs, I shook my head at myself for not proactively taking probiotics after my hospitalization discharge in October and prior to my admission in November. I knew in the back of my mind this was important, but with all the fevers, lethargy, blah blah blah I was dealing with I just didn't get it done. I asked my doc why he didn't put me on probiotics - he said the studies were mixed on whether probiotics really helped  prevent C Diff. There you go.

So back to the research - I first wanted to understand the exact mechanism of C Diff. Essentially, one can acquire C diff in two different ways - either getting it from someone else (not washing your hands....most commonly in hospital or long term care facilities) or through over use of abx. The abx CFer's normally use are "broad spectrum" - meaning the abx kill the PA we want dead, but the abx also kill some of the good bacteria in our intestines. 

As any of us who have had a yeast infection can attest, the balance between bacteria species, bacteria and fungus, etc etc is very fragile. And when the balance is disrupted, yeast infections and C Diff can result, to name a few afflictions. C Diff is particularly worrisome because the overgrowth of the bacteria releases toxins from the bacteria, causing inflammation of the colon or even damage to the intestines. Scary scary stuff.

So, what I'm hoping to pass on to my fellow CFer's are a few foods for thought on treating and preventing C Diff. This is my research thus far, what has worked for me, and what else I have in my tool box in case things get worse.

  • Not all probiotics are made the same. Most notably, good bacteria can be destroyed in your probiotic quite quickly
    1. Ask the store where you are purchasing the probiotic if the item arrives to the store refrigerated. Often, probiotics need to be refrigerated, otherwise the bacteria die off. So if you're purchasing a probiotic from a refrigerator in the store, but the probiotic was transported by truck at room temp for 3 days just to get there, are you really getting your money's worth? Or more importantly, what your digestive tract needs? I was shocked that my favorite brand of probiotics didn't arrive at my store refrigerated. So I chose a different brand
    2. Read packaging to determine how the probiotics are created. Some yogurt products are infused with the good bacteria, then fermented. This can destroy the bacteria
    3. Location of the manufacturer is important as well. I chose a manufacturer just a few dozen miles from the store - I figure the refrigeration transport will have more integrity and the product is fresh

  • If things escalated, I was ready to visit a local doctor for a fecal transplant. Not the most glamorous, but scientifically and logically it makes sense. I have no desire to lose my colon over this C Diff infection http://fecalmicrobiotatransplantation.com/FMT/home.html. Things have not escalated and I haven't resorted to this method - but I just want to throw it out there in case anyone needs it/ wants to research it. C Diff is serious.

I realize this post is all over the place, but I want to get out as much info as possible. 2 days ago, Friday, I was released from the hospital, PICC line pulled, no more abx except for the Flagyl and Vanco. I immediately went to the store to pick out a probiotic. After looking at the criteria above, I chose http://www.biokplus.com/en-us   Strawberry probiotic (yes, it has a little added sugar Mandy, I know, I know....) with 50 billion live and active cultures for my struggling belly. Now I'm just telling you what I chose and what was best for me - it's important that you do your research for which probiotic is best for your situation.

Friday night I drank my 1st 1/2 jar of the probiotic (I didn't want to start off too aggressively... too many good bacteria can be disruptive as well) and I drank the other 1/2 Saturday morning. Saturday evening (last night) I have my first formed BM and much more appetite than I had had in over a week. This morning, Sunday, another formed BM. 

To be fair, I was taken off IV abx 36 hours before this solid BM. So it's possible that being off the IV abx helped the good bacteria start to grow. But I am convinced that the probiotic certainly helped the good bacteria and of course implanted more good bacteria for my digestive system to work and the C Diff to settle down a bit.

I will keep you guys posted, as I'm certainly not out of the woods yet. C Diff can re-occur after my Flagyl/Vanco cycle ends, or of course with my next round of abx. But I feel more confident with the right probiotic in hand I can try to PREVENT the infection in the future, and treat it if it comes back. 

Oh yes, and my sinuses and lungs are doing fine too. 

Hoping this info can help other CFer's out there who are need of help - C Diff is certainly a nightmare. FIGHT ON!

Saturday, April 14, 2012

5 weeks of Kalydeco

So I have now been on Kalydeco for 5 weeks. For those who don't know me, I do NOT have G551D nor do I have any sort of gating mutation.

That being said, as many of you know, there was a small subset of patients with DDF508 in the Phase III Kalydeco study that had a very large increase in FEV1 with the drug. Genes don't tell the whole story with this drug - as you see with many CF patients, their genes don't predict how healthy/unhealthy they are. Siblings, with the exact same CF mutations, have varying CF outcomes.

Diet, exercise, modifier genes all have an impact on how CFTR functions.

So I wanted to try Kalydeco. Keep in mind, as I started the drug, I was diagnosed with CFRD. Meaning I have had out of control blood sugars that are artificially suppressing my lung function - most likely over the past 1.5 - 2 years I think (I have been having false negative OGTT results).

Upon starting Kalydeco, my FEV1 was approximately 2.3L, which is around 81% (waaaaaaay down from my normal of mid 90s, I know. It's been a rough year for me).

2 weeks in to taking Kalydeco, using my home FEV1 monitor, my FEV1 went up to 2.56. This was prior to taking any diabetes medication or doing any other changes in my diet, exercise or medication routine.

That's an 11% increase in FEV1. And I felt it.

I could laugh without coughing my brains out for the first time in years.

(EDITED TO ADD:I did PFTs on Monday, April 9 at my clinic and then came home to compare to my home meter - the FEV1's were 0.03 apart. So although I didn't have the opportunity to take a look at my FEV1 on the same machine a few weeks ago because I didn't have a clinic appointment, I do believe my home machine correlates pretty closely to my clinic machine.)

Unfortunately I got bad allergies from really really crazy winds that were blowing around that turned me in to an inflammation nightmare. That, coupled with experimenting with my blood sugar log (I wanted to see what would happen with my sugars when I ate a "treat" aka sugary stuff, vs normal stuff vs. healthy stuff) I think really set me back. I blew about 2.32L last Tuesday (at about 4.5 weeks).

Due to diabetes, they did my first urinalysis and it came back that I was super dehydrated (they were checking for me spilling protein in my urine - big sign of uncontrolled sugars) - and of course DUH! Dehydration wasn't helping my lungs either. This was prior to starting insulin as well - no changes in medications until 4/11 where a basal insulin was added.

So bottom line is, I don't have G551D and I had no expectation of having miraculous, mind-blowing FEV1 results with Kalydeco. But I do know that one's genes aren't the only story with CFTR function, so I was bound and determined to get my hands on this drug off label.

So we shall see where my lung journey will go - I am anticipating that my lungs will feel a bit better with some  insulin on board. But any change in FEV1 from here on out can be attributed to better CFRD control, not necessarily Kalydeco. So I a grateful I had a handful of weeks to change nothing about my care other than Kalydeco to see the true changes.

I will continue to keep you all posted.

But there is no doubt in my mind that Kalydeco had some impact......

........and I'm so grateful that my country doesn't have nationalized healthcare or socialized medicine so I could get a hold of the drug off label and my government couldn't restrict me. I'm willing to bet getting a hold of Kaly in Australia, England and Canada for non-G551D patients will be nearly impossible. I already know of more than a dozen here in the States, and I'm sure there are more.

Yes, it's my blog, and I can make a political statement if I want.

"A government big enough to give you everything you want, is also big enough to take it away." 
-Thomas Jefferson, 3rd President of the United States and principle author of the Declaration of Independence 

Regular periods - another benefit of Green Smoothies

Another lovely benefit I have experienced from drinking Green Smoothies (thanks to Mandy http://saltyspark.blogspot.com/ ) is regular periods.

I have long suffered from irregular, loooooong, heavy periods. Oral, estrogen-based BC pills would help with this, but since I have throw 2 PICC line clots, these meds are now out of the question for me.

Since drinking green smoothies regularly, I have been able to time my periods like clock work - January, February, March and now April 2012 have all been perfectly 28 days apart. Amazing!

Periods are still a bit loooong and heavy, but at least I'm spending less $ on replacing soiled undies! :)


Monday, March 19, 2012

The effect of N-acetylcysteine on chloride efflux from airway epithelial cells

Cell Biol Int. 2010 Jan 27;34(3):245-52.

The effect of N-acetylcysteine on chloride efflux from airway epithelial cells.


Department of Medical Cell Biology, Uppsala University, Box 571, SE-75123 Uppsala, Sweden. georgia.varelogianni@orebroll.se


Defective chloride transport in epithelial cells increases mucus viscosity and leads to recurrent infections with high oxidative stress in patients with CF (cystic fibrosis). NAC (N-acetylcysteine) is a well known mucolytic and antioxidant drug, and an indirect precursor of glutathione. Since GSNO (S-nitrosoglutathione) previously has been shown to be able to promote Cl- efflux from CF airway epithelial cells, it was investigated whether NAC also could stimulate Cl- efflux from CF and non-CF epithelial cells and through which mechanisms. CFBE (CF bronchial epithelial cells) and normal bronchial epithelial cells (16HBE) were treated with 1 mM, 5 mM, 10 mM or 15 mM NAC for 4 h at 37 degrees C. The effect of NAC on Cl- transport was measured by Cl- efflux measurements and by X-ray microanalysis. Cl- efflux from CFBE cells was stimulated by NAC in a dose-dependent manner, with 10 mM NAC causing a significant increase in Cl- efflux with nearly 80% in CFBE cells. The intracellular Cl- concentration in CFBE cells was significantly decreased up to 60% after 4 h treatment with 10 mM NAC. Moreover immunocytochemistry and Western blot experiments revealed expression of CFTR channel on CFBE cells after treatment with 10 mM NAC. The stimulation of Cl- efflux by NAC in CF airway epithelial cells may improve hydration of the mucus and thereby be beneficial for CF patients.

Sunday, March 11, 2012

Day 2 - Kalydeco

Well, suffice it to say that I feel a bit like I'm flashing back to Fall 2006, about 5 1/2 years ago, when I first started taking NAC. (http://noexcusesnoexcuses.blogspot.com/2008/08/nac.html)

It's true, we look for little signs of medication working or for side effects particularly right when we start something new. I took my first dose of Kalydeco right before bed on 3/9 Friday. Here is what I observed:

  • Prior to starting Kalydeco, I had been a bit non-compliant with my green smoothies and felt quite a bit more tight and non-moving mucus-y in my right middle lobe. I would cough but nothing would come up - but I could tell it was there. My FEV1 on 3/5 at clinic was 80%
  • When I woke up yesterday (3/10) AM, the first thing I noticed was how clear my sinuses felt. I could feel air moving in my sinuses where I feel like I've never felt air move before. Like up above my eyebrows - it was almost distracting because it was so different and weird
  • I coughed up pretty watery, much thinner than what I had the past few day's mucus. My right middle lobe felt much clearer
  • I have kind of avoided exercising the past few weeks because it was so exhausting to cough so much - but exercising yesterday was much easier and I could breathe deeper in my right middle lobe. I coughed while exercising but it was mostly a dry cough
  • Today I don't notice as much air flowing through my sinuses, but it's possible that I'm used to it or it was just a fluke yesterday. Not sure
  • I for sure have a plug stuck in my right middle lobe and it's a bit harder for me to breathe in my right lobe today. I am going to try to go for a run later today to cough it up (that usually does the trick) - it will be cool to see how my endurance is
  • I had another feminine side effect that normally I would post here because I'm comfortable posting anything - but I realize that all my readers might not be comfortable reading all I feel to share. Ha. So if you're curious, drop me an email or a PM and I'll fill you in :)

So all in all, some changes that I am cautiously optimistic about. It may be complete placebo effect, but that's fine with me - I feel good, whatever the cause. 

I'll continue to keep you guys posted

Saturday, March 10, 2012

1st Kalydeco dose

I was very fortunate and blessed to get my 1st Kalydeco dose in the mail today.

Luckily my doc was willing to give it a try, even though I don't have G551D (I have DF508 and Di507). And my insurance covered Kaly for a really reasonable co-pay.

I'm really not expecting it to do much, but I figured it most likely won't hurt me so I should just give it a shot.

There are so many things that can influence how genes are expressed, such as modifier genes, exercise (Stanford states that exercising actually increases CFTR function) and the foods we eat. So I think our CF genes don't tell the whole story - another reason why just trying Kaly is a good idea in my mind.

I look forward to keeping you all posted on my progress or lack thereof over the next month(s).

Update so far is that I took my 1st Kaly at around 9pm with my dear friend and parents watching me over video chat.

Bottom's up!

According to Vertex: "In recombinant cells VX-770 increased CFTR channel open probability (P(o)) in both the F508del processing mutation and the G551D gating mutation."

From Annals of Human Genetics, 2003, by Rowntree et al,:
"Measurements of Cl- conductase of intestine and respiratory tissues of DF508 homozygote CF patients suggest, in vivo, that at least some DF508 CFTR can reach the plasma membrane"

Saturday, January 21, 2012

Aquagenic palmar wrinkling

Again, I think we as a CF community are so fortunate to have Mandy and all of her amazing research around treatments for CF. I have learned so much from her and this post is another testament to her dedication to the lives of all CFers.

As many of you know, I have been drinking green smoothies in an attempt to get more greens in my diet - I truly eat like a crazy teenager, which I know is not good for my health. I really love the smoothies and they taste wonderful.

A benefit I didn't realize I could expect from these wonderful smoothies is my aquagenic palmar wrinkling has disappeared. It took about 4 weeks of drinking the smoothies everyday, but it worked!

Now what exactly the health consequences are of this, we aren't sure. But we do know that those with CF and those who are CFTR carriers wrinkle much faster when submerged in water.

I'll continue to keep you all posted on any other exciting changes!


In July 2011, I was so bummed to be admitted to the hospital with very, very few antibiotic options to treat my PA. Basically I had colistin and sorta kinda beta-lactams (intermediate resistance). Not too fun.

Well, thanks to the amazing wisdom of Ms. Mandy - who is always giving me great ideas), I have been taking Chilated Magnesium every day since October.

And in clinic on Jan 12, for the first time in 3 years, my clinic took me off isolation because I no longer have multi-drug resistant PA. Meaning all 3 of my PA strains are susceptible to nearly all classes of antibiotics (one strain is resistant to one class).

Talk about amazing. Incredible. Truly, a God-send.

Here are some studies supporting this outcome (thank you to Mandy's blog for these references):

Proteomic analysis of Pseudomonas aeruginosa grown under magnesium limitation.

Department of Pediatrics, Division of Infectious Diseases, University of Washington, Seattle, Washington 98195, USA. tguina@u.washington.edu 

In this study, large-scale qualitative and quantitative proteomic technology was applied to the analysis of the opportunistic bacterial pathogen Pseudomonas aeruginosa grown under magnesium limitation, an environmental condition previously shown to induce expression of various virulence factors. For quantitative analysis, whole cell and membrane proteins were differentially labeled with isotope-coded affinity tag (ICAT) reagents and ICAT reagent-labeled peptides were separated by two-dimensional chromatography prior to analysis by electrospray ionization-tandem mass spectrometry (ESI-MS/MS) in an ion trap mass spectrometer (ITMS). To increase the number of protein identifications, gas-phase fractionation (GPF) in the m/z dimension was employed for analysis of ICAT peptides derived from whole cell extracts. The experiments confirmed expression of 1331 P. aeruginosa proteins of which 145 were differentially expressed upon limitation of magnesium. A number of conserved Gram-negative magnesium stress-response proteins involved in bacterial virulence were among the most abundant proteins induced in low magnesium. Comparative ICAT analysis of membrane versus whole cell protein indicated that growth of P. aeruginosa in low magnesium resulted in altered subcellular compartmentalization of large enzyme complexes such as ribosomes. This result was confirmed by 2-D PAGE analysis of P. aeruginosa outer membrane proteins. This study shows that large-scale quantitative proteomic technology can be successfully applied to the analysis of whole bacteria and to the discovery of functionally relevant biologic phenotypes. 

PMID: 12837596 [PubMed - indexed for MEDLINE]