Breathing Deeply: My Life With Cystic Fibrosis

 

 

 

 

 

 

 

 

 

We thought if you got sick God was punishing you. Then we discovered germs and then we developed antibiotics to deal with germs. And then we discovered that it didn't make any difference if you were Hitler or Mother Teresa - penicillin works. It has nothing to do with your behavior.








CFers have to walk a fine line between being cautiously aware of the pathogens and allergens in the home, and outright germaphobia/paranoia. In addition to CF, I have asthma and allergies, which forces me to pay particular attention to eliminating clutter and other messes in the home. Whether or not the health of a CFer is affected by cleanliness depends on an individual's sensitivities to certain pathogens and allergens. I am allergic to things like animal dander, dust mites, pollen and mold spores, and fragrances (like candles, air fresheners, etc.) For that reason, the following items are a MUST for me:

1) protective pillow covers and mattress covers
2) Vacuum cleaner with 99.9% allergen reducing capability
3) 2 HEPA filters (one upstairs, one downstairs)

Pathogens (i.e. bacteria and viruses) in the home are inevitable despite our best efforts to diligently keep things clean. Some sources estimate that more than 65 percent of colds, 50 percent of all cases of diarrhea and 50 percent to 80 percent of food-borne illnesses are caught in the home.

Where shall we place blame for this? Suprisingly, not the bathroom, but on the kitchen! In fact, there could be up to 200 times more fecal bacteria on your kitchen cutting board than on your toilet seat. This is likely because people regularly disinfect their bathroom while kitchen items may be overlooked.

Kitchen Sponges and Rags

• The moist environment of sponges and rags is an ideal place for bacteria to flourish. Wiping your counters or dishes with a dirty sponge will only transfer the bacteria from one item to another.

• Replace kitchen sponges and rags often. Ideally, this should be about once a week.

• Allow them to dry out between uses. Most bacteria can only survive a few hours on dry surfaces.

Cutting Boards

• The cracks and crevices in your cutting board provide plenty of space for bacteria to grow.

• To clean the board, first hand-wash it using hot water and dish detergent to remove any food particles. You can then use a mixture of 1 teaspoon chlorine bleach in one quart of water to sanitize the board, leaving it to air dry.

• Alternatively, you can put the board in the dishwasher to sanitize it.

Kitchen Surfaces

• Kitchen countertops can still harbor germs even if they look clean. This is especially true if you've "cleaned" them with a dirty sponge or rag.

• The kitchen faucet and faucet handle is an often-overlooked source of potentially harmful bacteria.

Sink Drains

• The drains in both your kitchen sink and bathtub provide yet another moist environment that bacteria love.

• Use baking soda and an old toothbrush to get rid of stains, grit and grime around drains.

• Disinfect drains regularly as you would any other surface.

Other items in the home that are "hot spots" for bacteria are doorknobs, toothbrushes, phones and computer keyboards.

As tempting as it may be, a thorough cleaning of the home should not involve use of antibacterial products. Doing so will disrupt the balance of bacteria in your home, wiping out both good and bad varieties, which could pave the way for harmful bacteria. You are better off using antibacterial/disinfectant cleaners on an item-by-item basis rather than as an all-purpose cleaner. Also be aware of the ingredients in commercial cleaners as many contain harsh chemicals that can be harmful to your health. My personal recommendation is a product called "Simple Green". It's very safe (even safe enough to drink!) but tough enough to do outdoor cleaning.


For more information about keeping your home a healthy habitat for you and not for the bacteria, please visit some of the sites below.

Is Antibacterial Soap Better than Regular Soap?
Five Common Hygiene Mistakes
Why Hand Washing is So Important
Infection Control in Cystic Fibrosis
EPA Guide To Indoor Air Quality

Confound the nose! There's no end to it.

Cynicism is tough. A cynic's point of view is really pitiful. I derive pleasure out of a lot of things in life. As long as I'm fairly healthy, it's hard to stay dismal for very long.

If there's anything going, and I puts in for a bit of it, it's always the same story: "you're undeserving, so you can't have it." But my needs is as great as the most deserving widows that ever got money out of six different charities in one week for the death of the same 'usband. I don't need less than a deserving man, I need more!








Most news articles about cystic fibrosis follow the same basic outline. They begin with a story about a kid who "just wants to be like the other kids", then they go into minimal detail about the medications the child must take to be normal. After than they say how the future looks grim for said tot, unless of course, these new medications can be approved in time!

The lyrics may be different, but the tune remains the same. Even Forbes magazine, which rarely resorts to what I call the "tear-jerker tactic," has brought me to a new level of cynicism by publishing Mary Ellen Egan's article "Breathing Easier."

I'm willing to wager that Ms. Egan had minimal knowledge of cystic fibrosis before she wrote the article. I have read other examples of her writing and they too are very formulaic. Although I'm not particularly impressed with her writing style--even the article's title makes me cringe as I search desperately for an adverb accompanied by a modifier--I am pleased that she has attempted to garner some publicity for cystic fibrosis.

Unfortunately, not all of her facts about the disease are correct. She states that the average age of CF sufferers is 37. In point of fact, the median survival rate is approximately 37 years old. This means that half of CF patients will expire before this age, and the other half after. Based on the number of patients in the CF registry, one can reasonably assume that there are literally thousands of adult patients out there. Thousands.

We're the ones who are in more urgent need for medical breakthroughs. In my opinion, something better than finding medications to halt the progressive decline in lung function would be developing those that could reverse it completely.


"Breathing Easier"
Mary Ellen Egan


Now researchers hope new compounds may halt the deadly progression of cystic fibrosis. Inspire's drug, denufosol tetrasodium, emerged in the early 1990s from research by Dr. Richard Boucher at the University of North Carolina at Chapel Hill.

Patients with CF produce a defective form of a protein called CFTR (cystic fibrosis transmembrane conductance regulator) that screws up the inflow/outflow process. Boucher long suspected that epithelial cells have an alternative chloride transport channel; stimulate the right receptor, and he might jump-start the backup channel.

In 1991, after 15 years of searching, Boucher and his UNC lab coats discovered a nucleotide called uridine triphosphate (UTP) that activates this alternative chloride passageway. UTP turns on P2Y2 receptors--protein molecules on the surfaces of mucosal cells. These receptors prompt the cells to secrete salt (which draws water onto the airway surface) and prompt the hairlike cilia on cell membranes to beat faster to sweep mucus out of the airways and into the trachea, where it is disposed of by swallowing or coughing.

UNC licensed the breakthrough to Inspire in 1995. Inspire raised $9 million to develop a compound that would mimic UTP, tapping venture capitalists at Burr, Egan Deleage & Co. (now Alta Communications), Domain Associates and Medical Science Partners. The firm's scientists spent five years developing a synthetic version of UTP, now called denufosol, which can be inhaled through a nebulizer. Compound in hand, the company went public in August 2000; since then its shares have dropped 73%, in part because of higher R&D costs.

Vertex's compound, VX-770, would go a step further than Inspire's drug. It tries to restore function not only to the lungs but also to other organs affected by CF. VX-770 resulted from a five-year collaboration between Vertex and the Cystic Fibrosis Foundation. Vertex landed $13.3 million from the group to work on the compound and received a total of $40 million in foundation grants (although the organization doesn't own an equity stake in Vertex).

Instead of switching on a backup chloride channel like Inspire's drug, VX-770 works directly on the faulty CFTR channel. It targets parts of the CFTR protein involved in the opening and closing of the CFTR channel, propping open the protein to afford a more normal flow of chloride.

In young patients the pancreas and digestive tract also may benefit from the drug, says Vertex Chief Medical Officer John Alam, because the CFTR function is suspected to play a role there as well.

"I think it's a terrific idea," says UNC's Boucher, although he allows that VX-770 is only in early-stage trials. "There's hope now that you could take a pill and fix CF in the lungs, pancreas and other organs," he says. Patients may benefit from both the Vertex compound and Inspire's denufosol, he adds. "In college kids, who typically have significant amounts of lung damage, denufosol can keep the healthy portions of the lung healthy."

Cast you off into exile!


--Beastie Boys







A recent discussion thread in the cysticfibrosis.com forums has made me aware of how many misperceptions are out there about cepacia. In the CF community, this exceptionally resistent bacteria is a bone of contention for patients and healthcare professionals alike. Strict adherence to cross-infection avoidance protocols must be a priority. The gravity of the potential probelms for CF patients cannot be denied. However, many patients and their caregivers seem to have almost an irrational fear of being exposed to cepacia.

Because of the different genomovars (i.e. species) of cepacia, it is impossible to predict how an individual's lung function and overall health will be affected once cepacia has been cultured. For some people, decline is rapid and devastating. Others (like myself) may experience some degree of loss of lung function and become more susceptible to other infections.

Most of the anxiety about cepacia stems from the basic reality that cepacia is highly transmissible from patient to patient(1). However, not every species has the same transmissiblity characteristics.

The CFF patient registry tracks approximately 23,000 patients with cystic fibrosis who receive care at CF Centers throughout the country. Overall, only 3-5% of CF patients culture cepacia in any form. This translates to anywhere from 690 to 1150 patients who culture cepacia. In the United States there are 115 care centers. Just from rouhg estimation, this could mean that only 10 patients at each care center have cepacia. Keep in mind that not all of them are present each time the clinic holds appointments, and not all of them continually culture cepacia.

I am not intending to downplay the seriousness of cepacia, nor am I expressing the idea that precautions should be ignored. There are epidemic strains of cepacia. According to an academic paper published in November, 2003 on the study of cepacia in the UK (2)

"Minimising the risk of transmission of this organism from one individual to another requires a multifactorial approach. The polymerase chain reaction [PCR] has allowed for earlier detection of this organism in the clinical progression of the infection, which may allow for earlier and more aggressive intervention with appropriate antibiotics. In addition, such molecular diagnostic approaches may help by giving direction to segregation policies, thus minimising transmission in both the in-patient and the out-patient setting. Most importantly perhaps is vigilance on the part of the CF patient to adhere to infection control practices. Although tedious and socially difficult to maintain properly, these practices do work. " (Emphasis mine)

The more common pathogens of concern in CF clinics are those such as Pseudomonas aerginosa, and various staph infections. I am confident in my doctors' ability to execute proper infection control measures that protect me and others with CF. It's the rest of the world I worry about--I find myself far more likely to catch a nasty bug from the world beyond my health care center's walls. Public places like movie theatres, swimming pools, shopping malls, etc. pose a greater risk to my overall health.


--
(1) source: Prevention of Cross Infection in cystic fibrosis. Claus Moser, MD, PhD & Neils Hoiby, MD
(2) source: Burkholderia cepacia and cystic fibrosis. John E. Moor & J. Stuart Elborn. November, 2003. Northern Ireland Public Health Library.

Steve Irwin Killed By Stingray

I have no fear of losing my life - if I have to save a koala or a crocodile or a kangaroo or a snake, mate, I will save it.

Television personality and environmentalist Steve Irwin has died after being stung by a stingray while filming off north Queensland.

Known worldwide as the Crocodile Hunter, the 44-year-old was famous for his enthusiasm for wildlife and his catchcry "Crikey!''.

The Queensland Police Service issued a statement saying Mr Irwin collapsed after being stung at Batt Reef, Low Isles off Port Douglas about 11am. He had been filming a documentary.

"Steve was hit by a stingray in the chest,'' said local diving operator Steve Edmondson, whose Poseidon boats were out on the Great Barrier Reef when the accident occured.

"He probably died from a cardiac arrest from the injury,'' he said.

Police said after he stingray attack, Irwin's crew called for medical treatment at 11am and the Queensland Rescue Helicopter responded with a doctor and paramedic on board.

Puncture wound

Mr Irwin had a puncture wound to the left side of his chest and was pronounced dead at the scene.

Police said Mr Irwin's family has been advised and Irwin's body was being flown to Cairns.

It is believed his American-born wife Terri was trekking on Cradle Mountain in Tasmania when the incident happened.

Police in Tasmania say she has been informed of her husband's death.

The Irwins have two children, an eight-year-old a daughter, Bindi Sue, and a three-year-old son, Robert Clarence, usually known as Bob.

When asked if he had ever heard of anyone dying from a stingray barb Matthew Hurley, general manager of Quicksilver Group, whose company has taken tours to Low Isles for 26 years, said: "No, definitely not."

"We've never heard of or been involved with anything like that."

Ross Coleman, acting director at at University of Sydney Institute of Marine Science, told smh.com.au it was "quite rare" for someone to die from contact with a stingray and he couldn't recall hearing of another incident.

Stingrays were "dangerous if provoked", he said.

"As a recreational diving instructor you hear of people getting injured by standing on them ... but they rarely die."

I think the information explosion has created noise to the extent that people get trigger-happy, which can be detrimental.







Of of my ambitions is to provide the CF community at large with viable, accurate information. Whenever possible I consult print-media in the form of scientific journals and news articles. Like many others I also make use of online resources since those are readily available thanks to powerful search engines. However, not every source on the web is a credible one.

A recent search for the key words "cystic fibrosis" using Blogger's search tool pointed me toward the following website: e-cysticfibrosis.com You will note that I am not providing a hyperlink to this particular site. The site's disclaimer says that it does not guarantee the accuracy of the information contained therein. The website contains links to information such as "cystic fibrosis", "cystic fibrosis symptoms", "causes of cystic fibrosis" and "cystic fibrosis treatments". Each of these pages regurgitates the same summary descriptions about CF, and those descriptions, although not wholly without merit, are sorely lacking. I was able to find contact information about the site, but no credentials that would lead me to believe that it is a credible source.

Sadly, sites that this are everywhere. In my opinion, such sites breed ignorance. The Cystic Fibrosis Foundation recognizes the problem and has put out a consumer fact sheet outlining some basic principles that should be taken to heart when combing the internet looking for medical information. Here are the main points to consider according to the fact sheet:

• Be a cyberskeptic
• Consider the source
• Focus on the quality of the information
• Look for evidence
• Check the date
• Beware of bias
• Protect your privacy
  

Source : "Guide to 'Healthy' Web Surfing (c) 2005 CFF

What is the extent of my credibility with this site? I am a patient with CF, therefore I have a vested interest in obtaining accurate information about medical topics. I am also a member of the scientific community. I have a degree in environmental science and have been involved as a researcher in different types of air quality projects. This combination of education, professional and personal experience makes me uniquely qualified to address certain topics. As a scientist I am bound to uphold the principles of academic integrity. Finally, and here's my disclaimer, this site is mostly a personal one. My opinion, the information I link at this site, and any comments provided by others are in no way meant to substitute the advice of your own team of medical professionals.

"Recurrent wheeze and breathlessness are common in people with cystic fibrosis, and bronchodilators are commonly prescribed," according to lead author Clare Halfhide, M.D., of the Royal Liverpool Children's Hospital in England. "Despite their wide-scale and often long-term use, there is limited objective evidence about their efficacy in cystic fibrosis."

Source: Center for Advancement in Health. Article "Little Evidence Behind Bronchodilator Therapy For Cystic Fibrosis"


The findings of that study were published in October, 2005. It is the most current information I can find on the topic. Dozens of health-related news media ran the article, so I'm confident that it's not just a hack study published by some entity with a hidden agenda.

I often do CPT without a bronchodilator unless my asthma is acting up or I'm fighting infection. I also am asked why I do Xopenex. Usually RTs in the hospital ask me that because they are more familiar with albuterol. I choose Xopenex over albuterol because of the extreme jittery/anxious feelings I exhibit with albuterol that don't occur with Xopenex. For me, that's a quality of life decision, not necessarily a scientific one.

I do use bronchodilators both as a "rescue med" and as part of my preventive maintenance even if I don't do them at the same time as CPT. Bronchodilators are most effective when they are done first in the lineup of "usuals." Below is the proper order of med-neb treatments, as reported by another CF patient's clinic's recommendation:

1. Bronchodilator (albuterol or xopenex)

2. Mucolytics (pulmozyme THEN hypertonic saline)

3. Inhaled antibiotic (TOBI or colistin)

4. Long acting bronchodilator (serevent or foradil)

5. Inhaled steroid (flovent, advair, or pulmicort)

Here's why the order of operations is so important when managing CF.

The bronchodilator helps open up the airways. The more open the airways, the better the chances of inhaling the other medications deeply enough into the lungs where they can be most effective. Mucolytics are "mucus cutting" medications. They help break up the secretions so that they can be coughed up more easily. In CF patients, the mucus and the lungs are typically dry, which is why hypertonic saline is so useful. Hypertonic saline works by adding moisture to the lining of the lungs, resulting in a slippery surface conducive coughing out sputum. Following sputum clearance of the airways, the lungs are further able to take in the nebulized mist of the antibiotics. At this point there is a greater likelihood of the antibiotics taking hold in both small and large airways. The treatment concludes with inhaled steroids which help keep the airways open for an extended period of time (i.e. 8-12 hours when it is time for another treatment.)

The length of time to complete all these steps varies somewhat depending on the type of nebulizer/compressor system being used. For me this process takes approximately half an hour when I am not in a TOBI month and not producing much sputum. When I am not feeling well, am more congested or taking TOBI, it can take me as long as 1-1/2 hours to complete the process.

Although the article I cited at the beginning of this post says that bronchodilators are shown to be ineffective treatment, bronchodilators used in tandem with the other treatments CF patients need are effective indeed. My opinion is that they are an important piece of the overall treatment. While they may be ineffective on their own, they should not be discounted altogether.