Smart Pseudomonas

Adapting To Clogged Airways Makes Common Pathogen Resist Powerful Antibiotics

People with cystic fibrosis frequently have lung infections that defy treatment. Cystic fibrosis is an inherited disease that clogs airways with thick mucous. While the life expectancy for children with cystic fibrosis has increased over the past few decades, many lives are still shortened in young adulthood by the ravages of lung infections.

These chronic infections are often caused by common, environmental microbes that mutate in ways that let them live and thrive in viscous lung secretions. The same adaptations also make the pathogens less likely to be killed off by powerful antibiotics, according to a recent study led by Dr. Lucas “Luke” Hoffman, University of Washington assistant professor of pediatrics.

Surprisingly, he added, the pathogens don’t need any previous exposure to the antibiotics to resist their effects. The results were published in the latest edition of PLoS Pathogen.

The researchers looked at Pseudomonas aeruginosa, a microbe that can infect a cystic fibrosis patient early in life and then undergo various changes as it establishes a chronic lung infection. Pseudomonas aeruginosa with specific alterations tend to give patients a poor outcome. Some of those alterations diminish the chances of eradicating the infection with antibiotics.

It’s believed that these adaptive alterations in Pseudomonas, all of which are caused by genetic changes, could be selected for by the environment inside a patient’s airways, the researchers noted. Characteristics that facilitate microbial survival begin to emerge.

The specific airway conditions that select for these genetic changes, Hoffman said, remain unclear. “But,” he added, “we have some clues from what is known about airway mucus.”

From the point of view of Pseudomonas, the physical properties of cystic fibrosis mucus, Hoffman said, “make it a great place for the stuff people routinely breathe in to set up shop.” Cystic fibrosis secretions contain a lot of nitrates and amino acids, which Pseudomonas can use to grow.

Inside mucus plugs oxygen levels are low. Some Pseudomonas strains can live in this oxygen-poor, nutrient-rich environment. Hoffman and his team found that a mutation that occurs commonly in Pseudomonas from cystic fibrosis patients allows the pathogen to grow better in the nutrient environment in cystic fibrosis secretions. This particular mutation inactivates a gene named lasR. Pseudomonas with this mutation apparently undergo a metabolic shift: consuming less oxygen while utilizing nitrate more efficiently. lasR mutant bacteria also can handle oxidative stress resulting from an imbalance of damaging substances called free radicals forming faster than they can be detoxified.

One source of oxidative stress encountered by Pseudomonas is the antibiotic treatment that is frequently given to people who have cystic fibrosis. Antibiotics like ciprofloxacin and tobramycin kill bacteria partly by inducing the overproduction of free radicals and causing oxidative stress. Hoffman and his team found that, because these mutant microbes are resistant to oxidative stress, they were relatively resistant to these antibiotics when grown in conditions that were like cystic fibrosis mucus.

“We learned that simply by adapting to the conditions inside the airways of cystic fibrosis patients, mutated Pseudomonas can withstand the effects of ciprofloxacin and tobramycin,” Hoffman said. They did not need any previous exposure to these antibiotics to reduce their susceptibility.

Hoffman and his team suspect that Pseudomonas is not the only microbe that can do this. Some of the characteristics conferred by the mutation in Pseudomonas are also exhibited in other microbes found in chronic lung infections, such as tuberculosis or the fungal pathogen, Cryptococcus neoformans, Hoffman noted. Metabolic shifts may be a way many microbes get the upper hand over their hosts — and over antibiotics.

This report, Hoffman said, may point to new ideas for treating chronic lung infections. Luckily, colonies of Pseudomonas with the lasR mutation are relatively easy to identify in hospital laboratories by their distinctive iridescent sheen. Because lasR mutant Pseudomonas has been associated with worse outcomes in cystic fibrosis patients, indentifying Pseudomonas with the lasR mutation may be of prognostic value and may indicate the need for treatment with specific antibiotics like monobactams, tetracyclines, or polymyxin, whose mode of action differs from ciprofloxacin and tobramycin. Other treatment methods may be targeted at preventing adaptive changes, such as the lasR mutation, in Pseudomonas, the researchers said.

View the full article here: http://www.bmedreport.com/archives/10015

First Time Finding the Baby in a Cake

As some of you know a couple of weeks ago a super sweet cyster out in the community decided to send me a King Cake so I could be a part of their Mardi Gras celebration. You can find the post about it here. Well, video has finally surfaced of us finding the famous baby in that cake. Let me just tell you, a bit creepy!!!

Thankful for New Drugs!!!

This Thankful Thursday is a pretty easy one! I'm so thankful for the new drug just approved by our FDA :) I've heard nothing but great things about this drug from fellow cysters and fibros and I really can't wait to try it out myself. Make sure to call your clinic ASAP to see if this is a drug that can help you and one that you can get on sooner rather than later.

The drug by the way is Cayston, also known as AZLI. Think of it as another TOBI type of drug that can be done during your off month of TOBI or can be done instead of TOBI all together. It has to be taken three times a day, but the great part is, the treatment takes 2 to 3 minutes!!!

Bring it on!

Gilead Sciences, Inc. today announced that the U.S. Food and Drug Administration (FDA) has granted marketing approval for Cayston(R) (aztreonam for inhalation solution) as a treatment to improve respiratory symptoms in cystic fibrosis (CF) patients with Pseudomonas aeruginosa (P. aeruginosa). Cayston's safety and efficacy have not been established in pediatric patients below the age of 7, patients with forced expiratory volume in one second (FEV1) of less than 25 percent or greater than 75 percent predicted, or patients colonized with Burkholderia cepacia.

Cayston is administered at a dose of 75 mg three times daily over a 28-day period and is delivered via the Altera(R) Nebulizer System, a portable, drug-specific delivery device using the eFlow(R) Technology Platform, developed by PARI Pharma GmbH. PARI Pharma also contributed to the development of Cayston's drug formulation for delivery with the Altera Nebulizer System. Cayston will be available in the United States by the end of next week through certain specialty pharmacies.

"All of us at Gilead extend our thanks to the investigators and to the people with cystic fibrosis who took part in the Cayston clinical trials," said Norbert Bischofberger, PhD, Gilead's Executive Vice President, Research and Development and Chief Scientific Officer. "We look forward to making Cayston available to the cystic fibrosis community as soon as possible."

CF is a chronic, debilitating genetic condition that affects the respiratory and digestive systems of approximately 70,000 people worldwide, including 30,000 people in the United States. Chronic respiratory tract infection with P. aeruginosa contributes to the decline in pulmonary function, which is often associated with morbidity and mortality among CF patients.

"Since its founding in the 1950s, the Cystic Fibrosis Foundation has worked to advance the care and treatment of cystic fibrosis and we are pleased with the progress to date," said Robert J. Beall, PhD, President and Chief Executive Officer, Cystic Fibrosis Foundation. "However, a significant need for new treatments remains for people with cystic fibrosis, particularly for those with chronic pseudomonal infection. As the first new inhaled antibiotic approved for use in cystic fibrosis in more than a decade, Cayston therefore represents an important therapeutic option in the care of patients with cystic fibrosis."

Cayston received conditional marketing authorizations in the European Union and Canada in September 2009 and was approved in Australia in January 2010. Applications for marketing approval of Cayston are currently pending in Switzerland and Turkey.

Reimbursement and Access to Care

Gilead also announced today the establishment of a program designed to minimize barriers to access for Cayston for uninsured, privately insured and government-insured people with cystic fibrosis.

Additionally, Gilead is launching the Cayston(R) Access Program, a call center developed with Cystic Fibrosis Foundation Pharmacy, LLC, a wholly owned subsidiary of the Cystic Fibrosis Foundation. The program will assist people with cystic fibrosis and members of their care team with insurance verification, referral to participating specialty pharmacies, claims support and co-pay assistance. For information about the Cayston Access Program, call 1-877-7CAYSTON (877-722-9786) or visit www.cayston.com.

About Cayston

Cayston (aztreonam for inhalation solution) 75 mg is an inhaled antibiotic for patients with cystic fibrosis who have P. aeruginosa. Aztreonam has potent in vitro activity against gram-negative aerobic pathogens including P. aeruginosa. Cayston contains aztreonam formulated with lysine, a proprietary formulation of aztreonam developed specifically for inhalation. Aztreonam formulated with arginine has previously been approved by FDA for intravenous administration.

Cayston is administered three times a day for a 28-day course, followed by 28 days off of Cayston therapy. Cayston is administered by inhalation and should only be used with an Altera Nebulizer System. Patients should use a bronchodilator before administration of Cayston.

Article from Business Wire. February 22, 2010. To read the full article please click here.

Top Ten Toys I Never Grew Out Of

(**Written by Mandi)

Sunday night Ronnie and I went to listen to live music with friends of ours to celebrate one of our good friend's birthdays. As a little birthday gift, Ronnie and I bought her a Giant Googly Ball (see the picture to the left) that lights up when you push a button inside. It was a huge hit. Even people that weren't there with us took their turn playing with it. That got us talking on the car ride home about classic toys we still loved as adults. Here's our list in no particular order:

10. Etch a Sketch - You all know this one well. Two knobs on the lower left and right hand sides under the screen. The toy that takes massive amounts of concentration to make good images on even at an old age.

9. Pin Art - What other toy can you spent hours with making your hand and face into metal art? I used to love seeing that I could create, and would find myself fascinated with just the mere feel of it.

8. Slinky - Who walks the stair without a care. It shoots so high in the sky. Bounce up and down just like a clown. Everyone knows its Slinky. The best present yet to give or get. The kids will all want to try. The hit of the day when you're ready to play. Everyone knows it's Slinky. It's Slinky, It’s Slinky for fun it's the best of the toys It's Slinky, It’s Slinky the favorite of girls and boys.

7. Yo Yo - Now, I never was good enough to compete in any competitions, and never attended any conventions. But I did enjoy them and mastered the art of walking the dog and around the world.

6. Lite Bright - Ah there's nothing like making images out of little neon light bulbs. There's no wonder this is a toy that's been around since the 60s!

5. Slap Bracelet - It's metal covered in fabric, it's a bracelet, it's dangerous and removed from the market, it's CLASSIC!

4. Magnadoodle - I think my love for this toy stems from a fascination that it actually works!

3. View Master - Like your own personal slide show, no electricity needed.

2. Rubiks Cube - I've never once been able to actually make all the sides the same color, so I've never actually been GOOD at this toy. But I can waste hours playing with it.

1. Magic 8 ball - Will I marry him? Will my hair look good blonde? Will I get an A on my math test? The answer to all life's questions can be found in the infinite wisdom of the Magic 8 ball.

What are your favorite toys that you just never grew out of. We all have them!

Pre-marriage Counseling by Rondi

Drug Improved Survival in Mice With Cystic Fibrosis

It may only be mice, but it's a start....

In the search for new treatments for cystic fibrosis, U.S. researchers have identified a defective signaling pathway that contributes to the severity of the inherited lung disease.

Cystic fibrosis causes thick, sticky mucus to build up in the lungs and digestive tract, and is one of the most common potentially lethal genetic diseases in children and young adults.

In the new study, the researchers found that correcting the defective signaling pathway for a protein called peroxisome proliferator-activated receptor-y (PPAR-y) reduced cystic fibrosis symptoms in mice.

"Cystic fibrosis results from a genetic mutation in a channel, or membrane pore, that facilitates the transport of chloride and bicarbonate electrolytes from inside the cell to the spaces outside the cell," lead investigator Dr. Gregory Harmon, of the University of California, San Diego School of Medicine, said in a news release from the school.

"Loss of the cystic fibrosis pore channel results in inflammation and mucus accumulation. It also results in dehydration of the cell surfaces that make up the lining spaces inside the lungs and other affected organs, such as the intestinal tract," he explained.

Working with cells from mice and human cell lines from cystic fibrosis patients, Harmon and his colleagues determined that multiple genes affected by PPAR-y were reduced in cystic fibrosis.

The researchers then treated mice with cystic fibrosis with the drug rosiglitazone (a drug that binds and activates PPAR-y) and found that gene expression was largely normalized and survival improved. Among the other findings:

• Drug treatment also corrected part of the inflammatory process associated with cystic fibrosis.
• Deleting PPAR-y in the intestine of mice worsened cystic fibrosis.
• Activating PPAR-y can increase bicarbonate production in intestinal tissue by increasing the activity of bicarbonate-producing enzymes called carbonic anhydrases.

"For the first time, we are able to use a drug that activates bicarbonate transport without affecting chloride transport, and see improvement in the disease," Harmon said.

The findings, published in the Feb. 14 issue of Nature Medicine, may lead to new treatments for cystic fibrosis.

Find original article at http://www.palmbeachpost.com/health/drug-improved-survival-in-mice-with-cystic-fibrosis-253691.html

Rondi and J-Dizzle Sing Wild Horses

For this sound off Saturday - you get the hear the WONDERFUL sounds of our voices.

Sorry Jezzy doesn't chime in more form the backseat. Enjoy!

New Drug Class Offers Hope Against "Superbugs"

This is very new discovery, but exciting to hear nonetheless!! For full article click here.

LONDON, Feb 18 (Reuters) - Swiss scientists have found a new class of antibiotics, offering drug developers a fresh weapon in the fight against multi-drug resistant bacteria or "superbugs".

Researchers from a privately held Swiss biotech company Polyphor and the University of Zurich said the potential medicines are effective against a type of bacteria known as "gram-negative", and offer hope for new treatments for serious and often life-threatening infections.

The antibiotics work by deactivating a protein vital for the formation of the bacteria's outer cell membrane.

Polyphor's chief financial officer said the firm was in talks with pharmaceutical firms about possible licensing deals on the most advanced drug candidate, called POL7080, which selectively kills the dangerous pseudomonas aeruginosa, a common bacteria that can cause lung infections.

"There is a big need for new antibiotics that can overcome rising resistance," Michael Altorfer told Reuters. "And if you look back in history, finding a new class of antibiotics is an event that probably happens about every 20 years."

Until recently, antibiotics have been viewed by drugmakers as a low-growth area but the emergence of superbugs has rekindled interest in the field.

A study published in the journal Science found that POL7080 was able to target and deactivate an essential protein of the pseudomonas bacteria, killing the bug.

A report in December found that gram-negative bacteria account for around 63 percent of infections in hospital intensive care units. Experts commenting on that study said they feared resistance among gram-negative bugs was rising while the number of medicines to treat them was shrinking. [ID:nN01516996]

Drug-resistant bacteria kill about 25,000 people a year in Europe and about 19,000 in the United States.

Altorfer said Polyphor is planning to start Phase I clinical trials in healthy volunteers in the second quarter of this year and had begun out-licensing negotiations with potential pharma partners. He declined to name any of the firms in talks.

Pseudomonas aeruginosa is a particular problem in hospital acquired infections and in patients with cystic fibrosis, whose lungs and digestive systems become clogged with a thick, sticky mucous.

Altorfer said he was keen not to raise hope, but the drug could potentially be made in an inhalable form to help cystic fibrosis patients, of which there are around 70,000 worldwide.

"But there are other indications that could come first, such as hospital acquired infections," he said.

Full article can be found at http://www.reuters.com/article/idUSLDE61G0OX20100218

Let's Help This Cyster Out!!!

I'd like to introduce you guys to a fellow cyster, Chelsey Werchan. She is 23 and currently working on her Master's degree in Health Psychology.

Her thesis for her Master's is examining airway clearance adherence and quality of life in adults with CF. She says, "I'm so excited for this opportunity to advance knowledge in the field of psychology and medicine to help the medical system better communicate and work with our population!".

Here's where we come in. She's conducting a study to help her for her thesis and she needs participants to answer some questions. I did the survey myself and it took me 10 minutes. The only qualification that I know of is that you have to be an adult CFer. She will collect no personal information in which you can be identified and an email isn't required. It's painless, quick and simple...and I actually found the questions very interesting. Promise.

Here's the link to the survey and I highly encourage you guys to take 10 minutes out of your day to help out Chelsey and support her great efforts:

http://www.surveymonkey.com/s/CFadherencestudy

Is Loving Salt a CF Thing??

I'm probably the only one, but I found the following article fascinating for two reasons: I'm from Arizona and I love salt.

Now, I do have a little background to give you on my love AND hate relationship with salt however. While growing up I added salt to anything and everything...and I mean everything. I was one of those, salt first, eat second, salt again and then decide if I should put more salt or not. I distinctly remember adding salt to my red jello on Thanksgiving Day 1991 and I LOVED it. I'm telling you guys, I was a walking and talking salt-adding machine.

Like many of you, I also became a salt lick with any sort of physical activity. I used to always make jokes with the guys and lean over there fries and ask them if they needed any more salt with that. My hats were always surrounded with a salt rim and I was constantly becoming blind by the salt crystals that would drip into my eyes.

My relationship with salt took a turn for the worse however in 2003 when my kidneys were having a rough go of it in the hospital. I was actually flushed out with fluids and then asked to leave by my docs cause they couldn't in good faith give me anymore antibiotics. My kidneys were being damaged and they didn't want to harm them any further. I asked them about some simple steps I could take to help my kidney function and they came up with two suggestions: Drink more water and stop adding table salt to my food. To make a long story short, I've added table salt to my food ONE time since 2003. It was to some canned corn last year that I just wanted to see what it tasted like again. I've had a few issues here and there with my kidneys, but they've never been as bad as they were in 2003. Was it the strict "no table salt diet"? Who really knows right?

Ok, so I totally got sidetracked there, but I'm still wondering; Do cysters and fibros have a special affinity for salt? Is it just a coincidence that I've met many who crave salt just as our cells crave the balance of salt and water (super layman's terms there)? I don't know, you tell me.

Here's the article though:

Arizona is taking steps to improve the health of its residents. The state joined a national initiative to cut 20 percent of sodium from diets in the next five years.

"Most people are eating twice as much salt as they should and about 80 percent of it comes from pre-processed foods, like canned soups and frozen dinners," said Will Humble, Interim Director of the Arizona Department of Health Services.

"The fact is the sodium line in the nutrition facts label is just as important as the fat line, but it's often ignored. The overall goal is to get food processors to reduce the amount of salt in their products. Until that happens, everyone has to pay a lot more attention to the sodium information in the nutritional facts."

A recent study in the New England Journal of Medicine found that cutting dietary salt by three grams per day could reduce coronary heart disease and stroke.

Humble says if we cut 30 percent of the salt out of our diet, it would have the same benefit as half the smokers in the state quitting.

"If we were able to knock back 30 percent, the amount of salt that folks eat here in Arizona by 30 percent, it would be the public health equivalent of getting half of Arizona smokers to quit cold turkey today," said Humble.

Too much salt causes things like high blood pressure which leads to higher medical costs.

"Every year in Arizona, we spend between 200 and 400 million dollars that we don't need to spend on controlling blood pressure and the consequences that come from eating too much salt," said Humble.

Humble says a lot of salt comes from processed foods which makes cutting back on salt harder than you think.

"Initially, folks think, ‘Well, I don't use much salt from the salt shaker so I'm okay.' Well, you're not okay because 80 percent of the salt that you're getting is coming from processed foods," said Humble.

One way to reduce sodium intake is to watch what you buy at the grocery store and the places you eat. Fresh fruits, vegetables and meats are naturally low in sodium, while the amount in processed foods varies greatly. Nutrition labels help make healthy choices about sodium in bread, sausage, chips, etc.

Full article at http://ktar.com/?nid=6&sid=1264096

A Young Girl's Cry for Help

I thought I would re-post this question and response because it applies to so many of us...

Young Girl's Post:
Mods, feel free to move this, as I am a teenager, but I'm seeking advice that adults can help with. I have a feeling I've posted things like this before. In Jan, I was in the hospital, got a gtube..lungs went from 50% to 100%. Now, I am not sure..but I believe they are back at 50%. My weight has also dropped a few pounds because the machine was messing up. I don't know what to do with myself. I go into this cycle..hospital is great, but I don't do many of my meds at home. I can't keep doing this, I want to live a long life, and this isn't helping. I feel like crap, when I wake up I DREAD coughing because I know that I will be bringing a lot of stuff up..and many times I end up throwing up because of it. I have zero energy. I get out of breathe going up the stairs... At PE Thursday we I couldn't even run a full lap..I ran 1/4th, after that I felt horrible. What is wrong with me? Why does my mind not want to do these meds? I get so frustrated with myself..but I have no willpower.

My response:
I can't stress enough the importance to doing your treatments faithfully at your age. Believe me, we've all been there. I haven't met ONE CFer that is dying to do his or her treatments. I totally agree with previous posters that the future of CF is looking brighter by the day and although there may never be a cure, they WILL come up with a med that takes away many of the affects. The key is to keep your lungs healthy enough so when that day comes, you're lungs are somewhat normal. They've told me that I have a lot of scarring in different areas of my lung. That lung is gone and it's never coming back. Now my focus is to not let any more areas get scarred.

I agree with coming up with a routine. Maybe every morning you sit on your vest and do the nebs while doing something on the computer? Homework? Facebook? Myspace? Personal blog? Do you watch TV? Every time your show comes on, make it a habit to do your treatments while watching it.

I figure that anytime I sit down, I can be doing my treatments. Believe me, it wasn't this was when I was your age, but if I could turn back the clock and do it over again I would. I'm paying the price now, for my neglect in the past.

I really hope this helps and you can message me if you'd like to talk further.

Ronnie

Readers: What would your response have been?

The Weekend of Love: Engagement Photos, Bridal Shower and Dinner Date

With Sunday being Valentine's day being the day of love, we decided to make this the weekend of love. Well, maybe it wasn't a conscious decision, but somehow all of our weekend activities were love-based.

On Saturday Ronnie and I had our engagement photo session with a great guy and great friend of ours, David. You'll have to check out his work because he's spectacular. He is so great at snapping reality, not posed pictures. And that's EXACTLY what we needed because Ronnie and I aren't exactly the most photogenic couple, so David's ability to capture us, just being us, was crucial. So anywhoooo, we all headed to downtown Phoenix a few hours before sunset, and began posing all over town. We had a blast. With Ronnie and David cracking jokes, I think we were able to capture a handful of genuine smiles. After a long photo session, that even included a wardrobe change (sounds legit huh?) we headed to a GREAT bbq joint in the city that I have never been to that the boys raved about and had some bbq pork sandwiches, french fries and beans. It was a FUN shoot and a FUN time watching the photo master, David, work his magic. As soon as we have the photos, we'll be sure to post some for your viewing pleasure (and possibly a few good laughs at our "picture smiles").

THEN Sunday morning was my brother's fiancee bridal shower (Her name is Chrissy if you missed any blogs about my bro and his new fiancee, getting hitched on March 28th). It was a great opportunity to meet the other ladies in her life and her family's lives that I didn't already know. We did the standard lunch, gifts, and a few fun games. It was a great way to spend Valentine's day morning, basking in the glory of my brother and his bride-to-be's love.

Then it was home to my own Valentine for the remainder of the day. Since my mom is in town, we decided to postpone the romance to next weekend, even though she INSISTED that we go out. We have many many days to be romantic, and only 2 more until she heads back home. BUT we had a great afternoon and evening together. Ronnie and I found a great recipe online for a yummy chicken casserole and headed to the grocery store (one of our favorite activities together) to get the raw materials for our masterpiece. We came home from the store and did some prep work for the meal (I love cooking with my man in the kitchen...he always pauses to crack some jokes, give me sweet little kisses or give me love pats :) !!) After dinner prep, came some nice cheese and crackers while watching Fool's Gold (anyone seen that movie? It's CUTE). It was then onto the main course and a new show, Undercover Boss (we HIGHLY suggest it). And that was just about it. We spent the remainder of the night snuggling up on the couch channel surfing and just enjoying time together.

All in all it was a wonderful weekend of love. As I told Ronnie earlier today, it's the only Valentine's day we'll spend as an engaged couple, and this weekend was a perfect way to celebrate. What did you all do to celebrate love or rebel against it? Any single people out there have an anti-valentine's day party? Have any Valentine's day parties or dates? Let me hear it so I can snag some romantic ideas for next weekend when we have our V-day bash numero dos.

The First (and only) Time I Got Engaged!!!!

**I thought this video would be appropriate for Valentines Day :)**

What began as a nice leisurely hike up a mountain, ended with a proposal on Thursday! I couldn't have picked a more perfect setting: a mountain top, city views and the sunset. I was also able to capture it all on film, so enjoy!

If you watch until the very end you get to see the ring!

Do you procreate if you have bad genes??

I found this article and the subsequent comments very interesting. To view the comments and full article, click here.

Do you procreate if you have a bad gene? Discuss

January 22, 2010 at 11:27 am by Tracy Ormsbee

I’m reading a gripping and scary novel called “Still Alice,” by Lisa Genova, about a Harvard psychology professor who develops early onset Alzheimer’s. The novel is written from Alice’s perspective so you are in her head as she slowly loses her grip on reality and what’s going on around her.

Bear with me, this does relate to parenting: After she learns she has the disease, she is told that it is genetic and a test exists to screen her three grown children. One of her grown daughters is undergoing fertility treatment to try to have a baby. Alice thinks about what she would have done had she known she carried the gene before she got pregnant.

Would she have conceived these children or taken precautions to prevent them? Would she have been willing to risk the random roll of meiosis? Her amber eyes, John’s aquiline nose, and her presenilin-1. Of course, now, she couldn’t imagine her life without them. But before she had children, before the experience of that primal and previously inconceivable kind of love that came with them, would she have decided it would be better for everyone not to?

This is interesting because most of us carry a gene for disease in our family history. My mother has diabetes and I knew the gene could be passed on. I decided to have children. Part of my decision was based on the fact that my mother took good care of herself and lived a normal life without complications (she continues to do great). I also developed diabetes at 28 after having gestational diabetes with my first pregnancy. My oldest daughter does not have diabetes; my youngest was diagnosed when she was 4. Don’t know how I could possibly have regrets. She lives a normal life on the insulin pump and is perfectly healthy. I would make the decision again today.

In the book “Still Alice,” her daughter decides to have children. Since she is doing in vitro fertilization, they will screen the embryos and only implant those without the gene for Alzheimer’s.

To view the full article and comments, please go to http://blog.timesunion.com/parenting/6222/do-you-procreate-if-you-have-a-bad-gene-discuss/

Confessions of a Chronic Cougher

I received a great question by a mom over in the CysticLife community that I wanted to take the time and answer over here on RSR. Here is her background story with (bolded) questions:

levi has been sick for about a week and now he is to the point where he is coughing while laying down, running around and basically on and off all day. its a wet junky cough and i swear i have been using my stethoscope for the past 3 1/2 years and i never can tell what the heck im hearing if its a wheeze if its not? so anyways the last three times he has been in over the last few months with colds he has not cultured anything not normal so the doc has been going back and forth as to whether or not to admit him so i really dont want to push taking him in because id hate to see him go in at this age. last time he was a champ but he was a little over 2 and honestly would get poked and then forget about it but now he is older and brighter so he knows whats coming he still does really well and isnt afraid of the doctors or anything like that but we want to do what is best for him. he usually gets a cold and starts with a runny nose then he starts to get a wet cough and then he starts the wheeze so most of the time he starts an oral antibiotic and then prednisone. he already does albuterol 3 times a day and his vest 3 times a day for 30 minutes. we started that after his last hospital stay because they did it 3 times a day when he was in so we thought is three better than 2 and they said yes so why the heck wouldnt we continue that if it is better so right now he is doing his vest 3 times a day for 30 minutes and he doesnt mind it at all he actually likes it because that is the only time we let him watch tv that way he looks forward to it and an hr and a half is enough tv in my option for a 3 year old. so anyways he is already doing that all the time so is there anything else you think we could be doing to help him. its hard to because he totally wants to play and run around but then he just coughs more. i usually let him play until he decides its too much or if hes coughing a ton we just do something else like draw or something more calm. anyways i just totally rambled so we are at that place of not knowing what is best. any suggestions or insight to how you feel when you are going through something like this. its hard because he can only tell us so much of how he is feeling. we know his body pretty well but only externally from what we see we see and hear but we cant tell how he is feeling. basically we do all that we can he never misses a treatment well i shouldnt say never (well maybe 2 or three when we go to disneyland or the beach or something fun but basically we are all over his health and do all that we can to keep him healthy. so any advice would be awesome! sorry for rambling. k the boy is off the vest gotta run. we will be in touch. have a great night!

First of all, I can't stress enough that it sounds like you're doing a wonderful job in raising your boy! It sounds like you've found a nice balance between his life and his cystic life. A couple things I would say to you however...

One, coughing does not always mean sick. I'm a chronic cougher. There, I said it. No seriously though, I cough all of the time. I've coughed since I was his age and I continue to cough now. I coughed when my lung function was over 100 percent and I cough now with it in the 60's. Point is this, it's important to look for other signs that your child is giving in order to determine an exacerbation or not. You can check mine off the list every time I get sick. I'm more tired but I sleep less. I have to force myself to eat. I wake up in the middle of the night coughing; all night long. I usually wake up more tired than when I went to bed. I have a REALLY tough time motivating myself to exercise. My stools float more. They're stinkier. I'm generally nauseated. And I can't get my mucus to ever be clear at any point of the day. Whew!! I know, quite a list. But, it's important to note, that that is MY list. As a parent, you want to make mental notes about some things that ALWAYS happen when they are really getting CF sick. For me, coughing isn't one of them. I'm always doing it.

It sounds like you have your son on a very strict treatment regiment. Here's a couple of things I would try to switch it up. Have you ever thought about hand pounds? For me (apart from running), nothing works better at getting the mucus up and out. Ultimately, that's what is important. Getting the gunk out of there. If you can manage the mucus, you can manage Cystic Fibrosis. Is there something that ALWAYS gets your son coughing? It sounds like it may be just running around and playing. I would suggest having him do that more. There's nothing magical about the vest, I hate to break it to you. I mean, it does a great job for most people in doing it's job, but it's not the only horse in the race. We as a community have to figure out what works best for us at clearing out our lungs and forcing us to take deep breaths. You, as his mother, have that responsibility for your young son.

Also, to get back to what you hear when you listen to Levi. You may never hear him wheeze. In my 30 years, the doctors have told me that they have heard me wheeze less than 5 times. In fact, 9 out of 10 times, even while in the hospital, they say I sound "pretty good". Maybe it's because of all that coughing I'm doing :)

Last piece of advice. Use your instincts as a mother. There's no guide out there written specifically on how to manage your son's CF. We're all different. Sure, we can take bits from some and pieces from others, but like I say to people all of the time, "You're CF has NOTHING to do with anybody else's CF". The proverbial different strokes for different folks. Don't ever make decisions based on being the mother of a child with CF; make decisions based on being the mother of a child.