We are here to extend our lives by THINKING DIFFERENT

Wednesday, March 25, 2009

You heard it HERE FIRST (5 days ago!!!)

Don't you love that you hear of news here FIRST before the CFF even posts? ;)

I posted this 5 days ago....


Shout out to my CFer's with Stop Mutations

Development of Novel Aminoglycoside (NB54) with Reduced Toxicity and Enhanced Suppression of Disease-Causing Premature Stop Mutations.


Saturday, March 21, 2009

REALLY exciting news

So these sites aren't recruiting quite yet, but they will be SOON for the drug that will help the DF508 gene, VX-809.

So check out the site to see if your clinic will be participating and keep on them to make sure you're right there when they begin trials. :)


Wednesday, March 18, 2009

Daily CF routine

I'm tweeting my daily CF routine to raise awareness of CF. Follow me on the button to your right :)

Thank you PacSun!


Tuesday, March 17, 2009

Superbug complicates treatment of infections in cystic fibrosis (Cepacia people, PLEASE READ)

A must read for cepacia patients....

Superbug complicates treatment of infections in cystic fibrosis

The unexpected behaviour of a family of "superbugs" called the Burkholderia cepacia complex (Bcc) could have implications for the treatment of cystic fibrosis (CF) patients. CF patients produce large quantities of sticky mucus in their lungs that is difficult to expel and is easily infected by bacteria. A recent treatment for CF, inhalation of a sugar called mannitol, works by attracting moisture into the lungs. This thins the mucus making it easier to disperse. However, recent research by Professor John Govan and colleagues at the University of Edinburgh, published in the journal Microbiology has shown that when Bcc bacteria are grown on mannitol they produce a sticky substance called exopolysaccharide (EPS) which could contribute to the very problem that the mannitol therapy was designed to solve.

Infections caused by slime-producing bacteria are particularly difficult to treat in CF patients. The bacterial slime combines with the debris of the body's own defence cells to form a biofilm which protects the bacteria against both natural defences and antibiotics. And Bcc is an especially virulent bug.

"Burkholderia make other superbugs look like wimps", said Professor Govan, "They not only have larger genomes (hence genetic potential) and are resistant to almost all antibiotics, they can even use antibiotics such as penicillin as a nutrient. One of the problems is that when they are grown under normal laboratory conditions they do not produce the exopolysaccharide slime so their potential for causing serious infection may have been underestimated. We grew them on onion tissue – they were first identified in the 1950's as the cause of onion rot – and found that then they produced copious amounts of slime. Onion tissue contains a lot of simple sugars, including mannitol".

Since Professor Govan's work was published, further potential complications in CF patients caused by the Burkholderia bacteria have been identified. Commenting on the work in the current issue of Microbiology, Dr David Reid, from the Menzies Research Institute, Hobart, Australia, and Dr Scott Bell from The Prince Charles Hospital, Brisbane, Australia, have suggested that the increased levels of sugar in the blood of CF patients with diabetes could contribute to Burkholderia infections.

"CF-related diabetes affects almost one-third of adults with CF", said Dr Reid, "But no comprehensive studies have been carried out to investigate the effect of diabetes on Burkholderia infection. We will need international collaboration to ensure there are sufficient patient numbers to make any survey statistically significant".

"CF patients known to have Burkholderia infections have been excluded from the clinical trials of mannitol therapy", he went on, "But the obvious concern is that, despite Professor Govan's findings, patients with Burkholderia infections will be prescribed mannitol – because mannitol works very well for CF patients with infections caused by a bug called Pseudomonas aeruginosa which consitute the vast majority of the CF population".

In a further twist to the tale, one of the most virulent strains of Burkholderia lacks the gene that causes the bacterium to produce slime – and so CF patients infected with this particular variety might be able to benefit from mannitol therapy. However less virulent Burkholderia strains can use the mannitol to produce slime and make the infections they cause much more severe.


Mechanisms of the noxious inflammatory cycle in cystic fibrosis

Mechanisms of the noxious inflammatory cycle in cystic fibrosis http://snipurl.com/e0obk

Multiple evidences indicate that inflammation is an event occurring prior to infection in patients with cystic fibrosis. The self-perpetuating inflammatory cycle may play a pathogenic part in this disease. The role of the NF-kappaB pathway in enhanced production of inflammatory mediators is well documented.

The pathophysiologic mechanisms through which the intrinsic inflammatory response develops remain unclear. The unfolded mutated protein cystic fibrosis transmembrane conductance regulator (CFTRdeltaF508), accounting for this pathology, is retained in the endoplasmic reticulum (ER), induces a stress, and modifies calcium homeostasis.

Furthermore. CFTR is implicated in the transport of glutathione, the major antioxidant element in cells. CFTR mutations can alter redox homeostasis and induce an oxidative stress. The disturbance of the redox balance may evoke NF-kappaB activation and, in addition, promote apoptosis. In this review, we examine the hypotheses of the integrated pathogenic processes leading to the intrinsic inflammatory response in cystic fibrosis.

Glutathione - our good friend. Make sure to check out my NAC posts

Lung and heart-lung transplantation in children and adolescents


Monday, March 16, 2009

Immune cells play surprising role in cystic fibrosis lung damage, Stanford/Packard study shows


5 points to anyone who knows what the answer is to excess neutrophils....

You guessed it! NAC!

Yogurt over milk, please

So this article isn't necessarily CF specific, other than the fact that as CFer's age it's super important to avoid cancer so we can get a tx when the time comes (have to be cancer free for 5 years to get a tx at most centers).

I myself have replaced all the milk I drink with organic, plain (no sugar) yogurt mostly for the pro-biotic reasons (so many abx, so many yeast infections!!!). I mix my yogurt in my cereal and sprinkle a bit of honey to make it sweet (honey is one of the best sweetners).

But this article points out another reason why milk is really meant for children of breast-feeding age, not adults. I have a feeling organic milk is better than traditional, but there's still properties of organic milk that might not be so wonderful. Check it out....


Source of the study: National Cancer Institute.... the US Governments main cancer research arm

Friday, March 13, 2009

Full belly + exercise

Taiwanese sausage is an incredible dish. I've been eating it a little too much lately - I just can't get enough since I got back from my trip a few months back.

But man did this stuff just SIT in my stomach today. Yup, just sat there. So when it came to motivating for exercise this evening.... ya, wow. Didn't feel so good.

But I pushed myself anyway. So I'm 3/5 for cardio and 2/3 for weights for the week. And I might barf. No, I'm not joking. I do feel a little heavy in the yummy area and the water I'm drinking to re-hydrate isn't going down very easily.

Cheers! Here's to a great weekend guys :)

Thursday, March 12, 2009

Blood clot

Here are some fun pics I found on the interweb today.

The first pic is of normal red blood cells. The second is of red blood cells caught up in the web of a blood clot. The star looking thing is a white blood cell.

Preliminary evidence for cell membrane amelioration in children with cystic fibrosis by 5-MTHF and vitamin B12 supplementation


Wednesday, March 11, 2009

DVT is gone!!!!

Great news - my DVT is gone!!!!!!

I will still be on Lovenox for another 4 weeks, but at least I know that darn ticking time bomb in my arm is GONE!!!!!


Order your Stanford CF Education Day CD's

I learn SO MUCH for these DVD's ever year. I highly recommend ordering them....

VDs are now available of the presentations from this past weekend's Stanford Hospital/Lucile Packard Children's Hospital Cystic Fibrosis Education Day. You may or visit our website www.cfri.org for the DVD order form and return your order to our CFRI office. OR call us with your order at 650.404.9975.

Best Regards,
David Soohoo
Director of Programs & Operations
Cystic Fibrosis Research, Inc.
2672 Bayshore Parkway, Suite 520
Mountain View, CA 94043

34 Years of CF Research, Education and Support!
CFRI is a tax-exempt organization under 501 C(3) of the IRS.
Tax ID #51-0169988

Assessment of lung function from infancy to childhood in patients with cystic fibrosis.


Severe Bone Demineralisation is Associated with Higher Mortality in Cystic Fibrosis


EPIX Pharma earns addnl. $500K from Collaboration with Cystic Fibrosis Foundation Therapeutics


Clinical Trials

I wanted to update you all on the newly listed clinical trials, as well as trials outside the US as I'm noticing I'm getting more and more visitors from around the globe. (*waves*)

  • Multidose Safety and Tolerability Study of (Arikace™) for Inhalation In Cystic Fibrosis Patients in Alabama http://snipurl.com/dkr4e
  • Assessment of Quadriceps Muscle Electrostimulation Used in Patients Suffering From Cystic Fibrosis (STIMUCO) in France http://snipurl.com/dkr8y
  • Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of QAU145 in Patients With Cystic Fibrosis in Alabama http://snipurl.com/dkrl3
  • Aztreonam Lysine for Inhalation in Patients With Cystic Fibrosis, Mild Lung Disease, and P. Aeruginosa (AIR-CF4) all over the US and Canada and Australia (yes, they're still recruting) http://snipurl.com/dkrrm
  • Do Musculoskeletal Techniques Improve Forced Expiratory Volume in One Second in Adults With Cystic Fibrosis? in London http://snipurl.com/dkrug

More tomorrow when I finish my midterm :)

Done with the midterm. Here's some more:

Tuesday, March 10, 2009

Busy Day + Exercise

I snuck in some exercise today (cardio.... 2/5). Tuesdays aren't usually my exercising days because I'm so darn busy, but I wanted to sneek it in in homes to increase my chances of reaching 5/5 for cardio. It's HARD. But I can do it.

Tomorrow I get re-scanned for my DVT (can you believe it's been 6 weeks already???). Hopefully we have shown some progress. As I've told many of you, swelling and tenderness went down 10 hours after my very 1st Lovenox dose 6 weeks ago and I haven't seen any symptoms since.

The Lovenox hasn't been too hard to remember - I just do what I do with all my other meds - set an iCal appointment on my computer and have it pop up 2x a day to remember to take the shot. I've had quite a bit of bruising from the shots on both sides but it doesn't really hurt and goes away quickly.

I'll let you know the verdict tomorrow.

Azithromycin increases survival & reduces lung inflammation in CF mice



Running off to bed because I have class early tomorrow, but I get my cardio and weights in today. (1/5) and (1/3).

I'm watching dancing with the stars and I feel dumber for it. Denise Richards looks exactly like barbie. *dies*

Hope you guys are all doing well!

Sunday, March 8, 2009

Roller skating as cardio

I went roller skating yesterday, which was so exhausting I'm counting it as cardio. I wish I wasn't so private because I'd show you how ridiculously i was dressed up..... ahhhh so funny. So that was 4/5 for cardio for the week.

Oh and I just did my eliptical and weights right now so that's 5/5 and 3/3. Yey!!!!

Hop everyone is doing great with their exercise goals. I'd love any updates if you have them. I follow some of you on your blogs so I get them that way.

Here's to a great week!

Friday, March 6, 2009

Secret Weapon

Some of my biggest CF issues throughout my life have been inflammation - mostly from allergies but the past few years it's also due to residual inflammation from colds. Oh yes and having a company retreat in Vegas where I was dealing with cig smoke EVERYWHERE and perfume pumped in to cover the cig smell. Ugh.

I have a long, tortuous history with coughing so hard I barf from bronchospasm due to inflammation.

For years (like 1989-2004) I tried various techniques like hot tea, eating food, breathing through my nose and out through my mouth. The highlight was always lettuce coming out of my nose from coughing so hard, throwing up, and now catching it in my mouth.

I was in the ER in 2004 due to bronchospasm (and not being able to catch my breath) after cleaning my house (I hired a house cleaner from there on out) and the doc gave me what I now call my secret weapon: benzonatate (or tessalon pearls, their brand name).

Basically, it numbs the stretch sensors in the lungs. Yes, I know our docs don't like us to take cough suppressants but sometimes you need HELP to cut off the cycle of bronchospasm. Benzonatate works in 15-20 minutes (although my experience is more like 2 minutes) and lasts for 3 hours. Drowsiness can be a side effect but I've never experienced it. Oh yes, and it's been on the market since 1954 (it's an Rx - FDA approved!)

My CF doc is OK with Rx-ing me it now because he knows I use it responsibly and only in those horrible situations. I do enough mucus clearance with the Vest and exercise that of course I'm not surpressing mucus clearance ....

Not sure why more CF docs (and even mine) don't talk about this. But it's a true God-send.....

Thursday, March 5, 2009

Stem cells offer new hope to CF patients


That tickle...

That post-cost tickle nearly sidelined me from working out today. But I snuck in a 1/2 hour of cardio when I felt the tickle monster wouldn't totally dominate me. That's 3/5 for cardio and 2/3 for weights.

In addition to my cardio, I did plee's (however you spell it) to mix things up. Oooooooh my inner thighs will feel it tomorrow!

Too many dates

So I did my cardio today (2/5) and weights (2/3). I'm still battling the remants of this cold (you know how that goes, right? The virus is gone but the inflammation in the lungs certainly is not... and neither are the accompanying bronchospams ugh!!!).

But I managed a decent work out despite eating an inappropriate amount of dates today. I have no idea why I ate so many... they taste SO GOOD. so sweet. man.

Ya and I did that two days ago too. I don't think it's necessarily too too bad for me other than the fact that it's taking the place of other healthy food I normally eat and I think diversity in food is probably a good thing. Anyway.

TOO MANY DATES :) But not too much exercise. Hope everyone is well!

Tuesday, March 3, 2009

Singulair in CF 3

Paediatr Drugs. 2005;7(6):353-63.Links

Leukotriene receptor antagonists in children with cystic fibrosis lung disease : anti-inflammatory and clinical effects.

Department of Pediatrics, University of Bonn, Bonn, Germany. s.schmitt.grohe@uni-bonn.de

Cystic fibrosis (CF) lung disease is characterized by chronic endobronchial infection resulting in progressive pulmonary destruction; this is a major cause of mortality and morbidity. Neutrophils are the primary effector cells responsible for the progressive deterioration of lung function. Peptido-leukotriene B4 antagonists, new anti-inflammatory agents that block the neutrophil-dominated inflammation, could have had the potential for long-term use.

A trial on the pharmacokinetics of amelubant administered orally as a single dose of up to 75 mg in pediatric patients with CF and 300 mg in adults, and as a repeated dose of 75 mg and 150 mg, respectively, once daily for 15 days provided evidence that amelubant metabolism in adult and pediatric patients with CF is similar to that in healthy adults. In another study using the same dosage regimen, amelubant appeared to be safe and well tolerated.

Safety measures included physical examination, vital signs, spirometry, oximetry, ECG, and clinical laboratory testing. However, a randomized, double-blind, placebo-controlled, multinational, phase II trial (Boehringer Ingelheim 543.45) was conducted to investigate the clinical efficacy of 24 weeks of treatment with amelubant in patients with CF with mild-to-moderate lung disease. Two doses of amelubant (75 and 150 mg) were tested in adult patients (> or = 18 years) and one dose of amelubant (75mg) was tested in pediatric (6-17 years) patients. The trial was terminated early due to a statistically significant increase in the risk of pulmonary-related, serious adverse events in adults receiving amelubant.

Cysteinyl leukotrienes, eosinophilic inflammation, and viral infections also contribute to progressive pulmonary destruction in CF. Cysteinyl leukotrienes are potential targets for cysteinyl leukotriene receptor antagonist use. A study on the pharmacokinetics of montelukast in children with CF provided evidence that the dose of montelukast and the administration interval does not need to be modified if the goal is to mimic the serum concentrations used to treat asthma.

In a randomized, double-blind, crossover, placebo-controlled study, 16 children with mild CF (median age 9.5 years; vital capacity [VC] >70%) were treated with montelukast (5 to < or ="14">14 years; 10 mg) or placebo as a once-daily tablet for 21 days. There was a significant (p < or = 0.02) reduction in serum eosinophil cationic protein levels and eosinophils (p < or = 0.027) with montelukast. However, neither lung function tests (VC, forced expiratory volume in 1 second [FEV1], maximum expiratory flow at 25% of forced VC), nor clinical symptom scores changed significantly.

In another study, 26 patients aged 6-18 years with moderate CF (VC between 40% and 69% predicted) received montelukast or placebo for 8 weeks in a 20-week, randomized, double-blind, crossover, placebo-controlled trial. After treatment with montelukast there was a significant improvement in FEV1, peak expiratory flow, and forced expiratory flow between 25% and 75%, and a significant decrease in cough and wheezing scale scores (p <>

Another Singulair Study in CF

Ann Allergy Asthma Immunol. 2005 Oct;95(4):372-80.Links

Effects of montelukast treatment on clinical and inflammatory variables in patients with cystic fibrosis.

Department of Pediatrics and Allergy, M Curie Hospital, Zgierz, Poland. interna@wss.izerz.pl

BACKGROUND: In cystic fibrosis (CF), the inflammatory process contributes to progressive lung tissue damage. Cysteinyl leukotrienes have been found in the sputum of patients with CF at high concentrations sufficient to cause potent biological effects. OBJECTIVE: To evaluate the effect of anti-inflammatory treatment with montelukast sodium in patients with CF.

METHODS: Twenty-six patients aged 6 to 18 years were recruited to this 20-week, randomized, double-blind, placebo-controlled, crossover trial. Patients received montelukast or placebo for 8 weeks in addition to their regular CF treatment. Before and after treatment, findings from spirometry, whole-body plethysmography, and the clinical wheezing and cough scales were evaluated. At the same time, serum and sputum samples were obtained for the measurement of eosinophil cationic protein, interleukin 10 (IL-10), IL-8, and myeloperoxidase levels.

RESULTS: Twenty-three patients completed the study. Compared with placebo use, montelukast treatment significantly improved forced expiratory volume in I second, peak expiratory flow, and forced expiratory flow between 25% and 75% and significantly decreased cough and wheezing scale scores (P < .001 for all). There were no significant changes in vital capacity, thoracic gas volume, airway resistance, and residual volume after treatment. Compared with placebo use, montelukast treatment decreased serum and sputum levels of eosinophil cationic protein and IL-8, decreased sputum levels of myeloperoxidase, and increased serum and sputum levels of IL-10 (P < .001 for all).

CONCLUSIONS: Montelukast may have measurable anti-inflammatory properties in patients with CF.

Singulair in CF

Ann Allergy Asthma Immunol. 2002 Dec;89(6):599-605.Links
Comment in:
Respir Med. 2007 Mar;101(3):684.

Anti-inflammatory effects of montelukast in mild cystic fibrosis.

Children's Hospital Medical Center, University of Bonn, Bonn, Germany. s.schmitt.grohe@uni-bonn.de

BACKGROUND: Immune-mediated inflammation contributes to progressive pulmonary damage in cystic fibrosis (CF). Sputum cysteinyl leukotriene levels, eosinophil cationic protein (ECP), and interleukin-8 (IL-8) are significantly related to disease severity.

OBJECTIVE: The aim of this study was to evaluate the anti-inflammatory and clinical effects of the cysteinyl leukotriene receptor antagonist montelukast in children with CF.

METHODS: A double-blind, randomized, crossover design was used. Patients received montelukast (6 to < or =" 14"> 14 years, 10 mg) or placebo as a once-daily tablet for 21 days and then, after a washout period of at least 4 weeks, crossed over to receive the alternative treatment. Blood and native nasal fluid were taken on days 1 and 21 of each treatment block, and WBC count, ECP, and IL-8 were analyzed using a chemiluminescent immunometric assay.

RESULTS: Sixteen CF patients (10 boys, 6 girls; age, 5 to 18 years, median 9.5 years) completed the trial. There was a significant (P < or = 0.02) reduction of serum ECP (median reduction: montelukast 7.7 microg/L vs placebo 0.15 microg/L) and eosinophils (P < or = 0.027; median reduction: montelukast 85/microL vs placebo 0/microL). There was no significant change in nasal ECP, IL-8, or serum IL-8 after a 21-day course of montelukast. Clinical symptom scores did not change significantly.

CONCLUSIONS: Montelukast reduces eosinophilic inflammation in CF patients. Multicenter trials providing more patients to create more data to prove the hypothesis that montelukast is an effective tool to cut down disease severity in CF patients are needed.

Monday, March 2, 2009

Exercise + Cold virus

It took lots of talking myself into it, but I exercised with this cold. I'm always glad I do it after the fact - I get up lots of junk and I swear the hard breathing forces my constricted air open!

I didn't exercise like I was supposed to this weekend because I was sleeping the whole dang time.

I'm glad you guys liked my CF Story post.... I hope some of the rest of you will do it too!

Exercise on.......