Monday, October 30, 2006

Behind the Scenes at the CFF

Gratitude is not only the greatest of virtues, but the parent of all others.

--Marcus Tullius Cicero

This morning I had the opportunity to meet with some of the staff and volunteers at the local chapter of the Cystic Fibrosis Foundation. The first word that comes to mind about the experience is "wow." The amount of energy and effort that goes on behind the scenes every day is astounding. The public events, the fund raisers, newsletters, the website--anything you may have seen from the CFF--is only a hair's breadth of what is really going on.

In the past I didn't know much about the CFF other than what I saw on their website. I figured that since I have cepacia, there was really nothing I could do to be involved. Back in August I started my first letter writing campaign to raise funds for the annual GREAT STRIDES Walk, which is the CFF's largest event. Today I learned so much, not just about the fund raising processes, but about where those funds go, which biotech companies are involved in finding a cure, etc.

For awhile I had been feeling as though being an adult with CF meant that I was out of the spotlight when I comes to what the CFF is doing. Today I had that attitude turned upside down, and I couldn't be happier. Granted, there will always be things that are more geared towards the younger CF population as far as treatments go, but that's because a younger person's body is less likely to have been ravaged by frequent lung infections. That's the reality. However, today I read about things that will improve quality of life for CF patients--things that may serve to take at least a fraction of the drudgery of our treatments away leaving more time for enjoying life.

The Cystic Fibrosis Foundation is adding tomorrows every day for everyone with this disease. I am truly grateful for the work they are doing, and I look forward to giving back in at least some small way for what they have worked so hard to give me.

If you would like to contribute to the Cystic Fibrosis Foundation, please click on this link. There are more ways to give than just financial resources! You can volunteer your time at a local chapter office, or donate items to be auctioned off at an upcoming fund-raising event. The possibilities aren't as limited as you may think! You can even sponsor me as I participate as an "Angel Walker" in the GREAT STRIDES walk.

Thursday, October 26, 2006

Coping Mechanisms of those with Chronic Illness

"Glass breaks so easily. No matter how careful you are."
(Part 4, Scene 7 Laura Wingfield)

--Tennessee Williams' "The Glass Menagerie"

Not until recently did I begin connecting with other people who have cystic fibrosis. Certainly I had known about others with CF, but because of cross-infection issues we weren't permitted to be around one another. Additionally, my parents had chosen--wisely I believe--not to engage in any sort of family counseling to "deal with" CF. Does this mean that we were in denial of the disease? In my opinion, no. For us, the best way to cope with CF was to learn how to cope with life in general, and that requires participating in life to the fullest extent possible.

A few months ago, after returning home after an unpleasant hospital stay, I began perusing the online cystic fibrosis support communities. At first I was thrilled to have discovered a resource that put so many people with similar experiences within easy reach. I quickly learned that just because we have the same experiences, our coping mechanisms are vastly different.

Some people choose the defeatist attitude. They don't embrace life because all they see is the big word "INCURABLE" in their minds when they think of cystic fibrosis.

Others lash out in anger. Their frustration of having CF and being limited by in it so many ways makes them unable to see the world beyond the end of their noses.

I have also observed that many CFers have nothing better to do all day than sit around in the message boards, clammoring for attention. They find a backwards sense of purpose and meaning in telling their sob stories to anyone who listens. I'm not talking about the people who truly are attempting to heal from a bad experience. I'm referring to those who outright refuse to put one foot in front of the other and make forward progress.

The few people I have have made friendly connections with thanks to the message boards and forums are nothing like the folks described above. There is a saying that "misery loves company" but since I do not consider myself miserable, I prefer to stay away from places that can drag me down emotionally. I only wish I had realized this about myself earlier. Because of my background of faith and the fact that I haven't been subjected to all the negativity of the "illness culture", I cope in different ways.

I cope by leaning on my family and friends. I cope through prayer. I cope by living normally and dealing with CF with as much routine as possible. I may be chronically ill--I know I'm certainly not invincible--but I choose to live in a world that isn't made of glass. I live in a world that's real, with its ups and downs, joys and disappointments. I cope by realizing that CF does not define me. My character defines me and that's what matters most.

Wednesday, October 25, 2006

Propping Up the Ones We Lean On

Even if you are strong, you still need people who would support you all the way.
--Corazon Aquino

I can't say enough how blessed I am to have the supportive husband that I do. I lean on him for so much more than things that are CF related. I know he leans on me in many ways as well. However, this is one way that I cannot provide support to him. I cannot, for all my best intentions, be what he needs when he is coping with the reality of being a "CF Spouse".

The social worker at the CF Adult Clinic that I attend has said that some day she would like to write a book about all the CF spouses she has encountered over the years. She says that there is something absolutely incredible about the kind of people she sees come through the clinic with their chronically ill spouses. It's a level of commitment that few people understand these days. Even outside of the CF community, the uncertainties and fears of what the future holds is overwhelming and can rip two people apart.Fortunately, this has not been our experience. My relationship with Brad has been solidified by each challenge we have faced together.

It saddens me to think of how easily the CF spouses are overlooked. Sure, they may not be the ones consuming dozens of pills each day, but that doesn't mean they don't have their own battles. There's nothing I would like to see more of in the CF community than a greater awareness of what these selfless souls dutifully and lovingly face day in and day out.

Recently I met Allie-- a young woman who is bearing the full weight of being a widowed CF spouse. I respect and admire her tremendously, even though I can't truly say I understand what she goes through. From the little bit I know of her, her marriage was beautiful and blessed, and the ache of not having her husband with her is, at times, nearly incapacitating. Still, she presses ahead, honoring her husband's memory and raising their daughter with the help and support of her husband's extended family.

Support. It's key to survival. I don't just mean the misery loves company type of support. I mean true, empathetic, sympathetic support, and encouragement. The ones we lean on for support need to be propped up as well, otherwise we all come tumbling down.

I encourage you to visit this online storefront which has been established as a way to raise awareness for cystic fibrosis and honor the memory of Ry Stentsland.

Friday, October 20, 2006

To Toot Your Own Horn, You Must Have Good Posture

I myself eat more cornbread than some small nations, and I'm an epitome of health, vigor, vim, charm, and good posture. I also control the weather

--Jeremy Jackson

According to an article published by R. Tattersall and M. J. Walshaw in the Journal of the Royal Society of Medicine (Posture and Cystic Fibrosis, 2003), people suffering from CF are likely to succumb to abnormal postures in an effort to relieve shortness of breath. In particular, CF patients tend to develop rounded shoulders, barrel chests, spine curvature and an overall stooped appearance. This may lead to additional health problems and back pain.

Slumping over as a means of reducing shortness of breath may feel better, but the hunching over isn't good. Like a lot of things these days, doing it "just 'cuz it feels good" isn't always the best thing when you look at the overall picture. The reason hunching over reduces shortness of breath is because then your blood--which is carrying much needed oxygen--can move more easily when it has less of the force of gravity to overcome.

For you science geeks out there, F=mg cos theta, where F=force, m=mass, g=acceleration due to gravity, and theta = angle of incline opposite the hypotenuse. This is why if you're leaning way over, it's easier to breathe than it would be if you're sitting up. It's the same reason why it's easier to push a heavy box up a ramp than to try to lift it to the same height straight up.

Okay, enough of the physics lesson. Eventually what happens as your body becomes more accostomed to the hunched over position, your physical ability to have enough force behind a cough for airway clearance is impaired. From there it's a vicious cycle as your lung capacity (FVC) and amount of inital expiratory force (FEV1) are adversely affected.

The moral of the story--your mother knows what she's talking about when she insists that you "quit slouching and sit up straight!"

My parents knew how important it was for me to do whatever it took to keep my lung function at its best. In the fifth grade I joined the school band. I wanted to play the xylophone, but my parents insisted that I play a big heavy brass instrument that provided good lung exercise and plenty of sustained breathing. They bought me a F. horn, and I loved learning to play it. I continued to play throughout high school. I eventually took up the trumpet as well. Even in college I participated in the band and orchestra.

I'm convinced that if it hadn't been for joining the band, I would not have done so well for the first 2 decades of my life. There's definitely something to be said for good posture and deep breathing. Together they can have a lasting, beneficial impact on overall lung health.

(Incidentally, the F. horn pictured above is a Conn 8D, which is the type of double horn I have--that's more than 12 feet of tubing to push air through! Mine's not quite as shiny as the one in the picture, but it sure does sound beautiful!)

Wednesday, October 18, 2006

Putting on the Mask

But let the world dream otherwise,
We wear the mask!

--Paul Lawrence Dunbar

Ever since my husband wrote music to accompany this poem, it has intrigued me. It was one of the first pieces of composition he shared with me when we were dating, and it has stayed in my mind ever since. He even included it as part of his composition recital in college. All I can say is that its timbre, mood and haunting vocalization are exceptional. My husband is brilliant and gifted.

But that's not why this poem came to mind today. Last night I had one of the better nights of sleep I've had in awhile. I credit my sister for providing a simple suggestion as to how I can sleep more peacefully.

Sleep mask-- $2.99
Foam ear plugs-- $4.29
Sleeping all night--PRICELESS

I admit, I initially turned up my nose at the idea of wearing a sleep mask. I thought only pampered, high maintenance, snooty broads wore those things with their satin night gowns and matching feathery slippers. Well, maybe I am just a little bit pampered, but I was at the point where I'd try just about any non-drug to get to sleep. I've been on Ambien in the past and that made me so weird it was frightening. I've also tried Unisom, Tylenol Simply Sleep, Advil PM and pretty much anything else with benadryl or alcohol in it that might make me conk out.

The problem with the medications was, I was at their mercy. I could not reasonably predict when the drug would take effect. If I took it at 8pm, sometimes I was sawing logs by 8:30. Other times I would take it at bedtime (approx. 10pm) only to have it kick in by 4a.m. which left me dragging well into the next day. Besides, I already take so many medications that I was loathe to add another one for something as dumb as sleep. I know sleep is important, but I figured there must have been a better way to get my much needed rest.

I'm hoping that the earplugs and sleep mask combination is the right solution for me. I'm so tired of being exhausted. I'll keep you posted. At the very least, it was nice to wake up this morning and not feel like I had only slept for 15 minutes. Even though I feel well (gaining weight, eating right, not coughing up anything funky) I have been tired for far too long now. Maybe this will be a much anticipated turning point.

Thursday, October 12, 2006

Knick Knack Paddy-Whack, Os-cal for my Bones

In order to achieve the best results to prevent osteoporosis and its complications, the prevention should ideally begin at the earliest possible age.

--Gro H. Brundtland

I grew up on a dairy, so naturally I'm a milk drinker--whole milk thank you very much. My husband and I even used champagne glasses filled with milk (instead of champagne) for the toasts at our wedding! Cute, no? Even with my fondness for snacking on cheeses, yogurts, ice cream and pretty much anything else milk related, my bone density isn't the greatest. I have never broken a major bone in my body, and I assumed my lactose laden diet was sufficient to protect me from any osteo-related problems. Afterall, milk does a body good, right?

Well, not my body. At least not, as much as it would if I didn't have cystic fibrosis. My body is just too lazy to do the work required to properly absorb the vitamins and nutrients I feed it. (And boy do I feed it!) As a result I, like many other CFers, and deficient in vitamins A, D, E and K. Vitamin D, which is a fat soluble vitamin, is directly responsible for how much calcium comprises the bones in our bodies. Without sufficient vitamin D and calcium to build strong bones, the risk of developing osteoporosis or osteopenia increases.

Many people have heard of osteoporosis, but osteopenia is not as commonly known. Osteopenia is when a person's bones are less dense than normal. Eventually it could lead to osteoporosis. Typically people who develop this condition are much older and are likely to be post-menopausal women. Bone density losses take place more frequently as people age. However, people with CF are likely to be diagnoses with osteopenia or osteoporosis at younger ages. I am one of them. I have osteopenia.

What do I do about it? Can I get rid of it?

My doctor has assured me that the diagnosis of osteopenia is not something that should keep me up at night. There are things I can do to make sure that I don't continue to lose bone density. These include:
  • eat a diet rich in calcium and vitamin D
  • limit my caffeine intake
  • limit my alcohol consumption
  • do weight-bearing exercise like running, walking, or hiking (basically anything that forces my body to work against gravity)
  • take care of my mental health issues so that they don't adversely affect my physical health
  • eat
  • eat
  • did I mention eat?
  • sit in the sun for about 15 minutes per day to give my body the opportunity to make its own vitamin D.
My body certainly requires a lot of maintenance, but since it's not just a "point A to point B vehicle" I'm more than happy to treat it with the attention and TLC it deserves so that I can continue living well with CF. It's not always a piece of cake (mmm...cake) to do all that's required, but that doesn't mean it's not worth it.

How Low Did I Go?

With blazing eyes you see my trembling hand.

--Led Zeppelin (The Wanton Song)

Although I'm only "pre-diabetic" and don't have cystic fibrosis related diabetes (CFRD), I'm in the habit of checking my blood sugar. A majority of the time I don't have any problems with it, but just to be safe, I check intermittently and especially when I feel a little out of whack. Last evening was one of those out of whack moments.

Throughout most of the day I'd felt hungrier than usual. I dismissed this as being a favorable sign that I was getting my typically ravenous appetite back, and remedied it with food. I suppose my food selections weren't quite adequate to keep up with my body's actual needs, because by the time I got home from work I was feeling a little woozy. I opted to lie down, thinking maybe I had just "overdone it" a bit during the day.

The wooziness (is that a word?) continued to the point where I began to feel like I was maybe having an anxiety attack or something. I couldn't concentrate, my heart was pounding, I was having a cold sweat...and my hands were trembling fiercely.

Aha! My hands tend to tremble a bit anyway from all the medications I am on, but this was noticeably different--at least a 7.0 on the Richter scale of manual quaking. The shakiness tipped me off that maybe I should check my blood sugar. I got out my trusty glucometer, inserted a test strip, pricked my finger and applied the blood sample and waited for 5 short seconds.

The readout on the screen confirmed why I was shaking so badly. My number was 22.

22?! Good heavens, that's a problem!

I hauled myself downstairs and retrieved the orange juice from its resting place in the refrigerator door. I didn't even bother to use a glass or close the fridge. I just stood there guzzling down the juice, praying that it would kick in quickly and the yucky feeling I was having would subside. Brad came home just at that moment and I told him what was going on. He ushered me to the couch and proceeded to bring me food for the next several minutes until I felt better. What a guy!

I'm happy to say that I recovered quickly and my blood sugar returned to a normal range without any problems. I'm not quite sure why it dipped as low as it did, but at least it came back up easily. The whole episode served to remind me that it's in my best interest to EAT EAT EAT to provide my crazy system with the fuel it needs to function as normally as possible.

Dangers of Hypoglycemia (from Texas A&M AgNews)

Wednesday, October 11, 2006

Breaking the Mold (or at least controlling it to some extent)

If they can make penicillin out of moldy bread, they can certainly make something out of you!

--Muhammad Ali

Indoor air quality is a topic of great importance to CFers. Here is California, people are becoming increasingly aware of the danger of certain types of molds and the impacts on respiratory health. Eventhough California is a relatively dry climate (not nearly as prone to humidity as the gulf and east coasts) we have the highest incident of reported mold problems in homes. Even rental agreements and real estate documents are dutifully highlighting the necessity for mold prevention.

In order to reproduce, mold generates what are called "spores." Spores are small enough to drift through the air in a home with ease, and some are even small enough to enter our airways.
The most effective way to control mold is to control moisture. Mold loves to grow in damp, dark areas, especially if there is a particularly warm temperature. As you can probably tell, the warm, moist environment of the human body provides and ideal setting for mold spores to reproduce and colonize.

The bad news is, mold is exceptionally resilient and is opportunistic, meaning it will lie in wait for just the right conditions to reproduce, even if that means waiting for a long time. Many of us have seen mold growing on a piece of fruit that has gone bad, or that leftover pizza that stayed under the bed in the college dorm for way too long. Mold doesn't only grow on foods--it can grow on just about any surface imaginable. Wood, paper, carpet, concrete...

Despite the fact that there is no way to remove all mold and mold spores from the home, there is good news. Plenty of things can be done to effectively control mold and avoid serious health effects such as allergic reactions. Also, dealing with potential moisture issues is beneficial to the soundness of your home.

Since I have ABPA and am allergic to molds in general, I make a point to combat mildew and mold in my home to the greatest extent practicable. We have laminate floors in the bedroom and our entire downstairs, since those are the areas where we spend the most time. By having less carpet, there is less likelihood that spores being tracked in from outside will lie unnoticed in the carpet threads. Also, having less surface area to vacuum ensures that we are not sucking up mold spores and making them airborne again through the vacuum cleaner's exhaust.

The bathroom can be a breeding ground for mold and mildew since there is a lot of moisture there. When showering, it is a good practice to run the exhaust fan. This helps keep the humidity from forming little pools of moisture in unseen places where mold might like to take hold. Also, once a week I use Tilex in our sinks and in the shower to kill off anything that may be growing in there. Our showers are fiberglass, which is a tremendous help when it comes to keeping mold and mildew away.

In the kitchen we make good use of our refrigerator's built-in humidity control function. This keeps mold from taking hold of the soft cheeses and other foods in there. Left overs are not put away or covered when they are at their hottest, since that could trap heat and moisture and promote mold growth. Instead, leftovers are allowed to cool to room temp before being stored in the fridge.

If you would like more information about molds and indoor air quality, ehe EPA has a very good resource listing things that can be done to control moisture and prevent mold growth.

EPA Mold Resources

Current mood: complacent
Current snack: pretzels and caffeine free cola
Health-o-meter: 96% of baseline
Emotional weather: patchy clouds

Read my published article on this topic here on Associated Content

Tuesday, October 10, 2006

Fungus Among Us

About three years ago I was having more than my share of sinus infections, and more asthma symptoms than usual. My doctor tried medication after medication, most of which were nasal sprays and antihistamines for my allergies. Nothing was working. One fall morning I blew my nose and was shocked to see that what came out of my nasal passage was something unlike I'd ever seen before--and for a person with CF who has seen every disgusting bit of mucus imaginable, that's saying something!

It was blue. And it was fuzzy.

The chances that a very tiny Muppet had crawled into my sinuses and had just exited into my tissue were highly improbable. I took the mysterious blue booger with me to my doctor appointment that afternoon.

"Ah yes, that is mold," she told me. "Aspergillus."

As if I hadn't already been a walking science project for the better part of two decades, now I had to deal with the fact that a very persistent mold/fungus had taken up residence in my body. Not only that, but since I am allergic to mold spores, pollen and basically anything else that likes to grow in God's great outdoors, my body was reacting to the aspergillus in a manner consistent with a condition referred to as ABPA.

What is ABPA?

ABPA stands for "allergic broncho pulmonary aspergilliosis." It means that in addition to culturing aspergillus (a fungus), the body reacts to it with an allergic response. Some people can culture aspergillus but don't have ABPA. The body responds to the allergen by increasing the amount of IgE (immunoglobulin E) in the blood. To bring the IgE back down and control the fungal infection, doctors prescribe antifungal medications like Vfend or Sporanox in combination with corticosteroids (such as prednisone) to reduce inflammation in the lungs and/or sinuses.

A IgE number of 100 or greater is considered "high" for adults. Mine has run in the thousands when I'm experiencing an accute exacerbation from the aspergillus that has colonized me. Normally, the first thing the doctors would try is prednisone. For me though prednisone is worse than the infection itself since it brings about so many horrible side effects, and I avoid it to the greatest extend possible. These days I do the antifungal meds all the time, and do what I can to make sure my home environment is free from the conditions that would encourage mold growth (i.e. moisture, dampness, live plants, etc.)

High IgE levels are presumed to be associated with a decrease in lung function. The significance and danger of a high IgE number is that if IgE numbers stay high for a prolonged period of time, the degree of severity of lung damage can potentially increase. However, this is largely just a hypothesis on my part based on things I've read. IgE and its connection with CF and lung function is something that isn't fully understood at this point in time but several studies are ongoing.

Antifungal Therapies for ABPA

Current mood: detached
Current snack: strawberry yogurt
Health-o-meter: 95%
Emotional weather: patchy clouds

Monday, October 09, 2006

A Typical Day (if there is such a thing)

A new day...I knew I'd make it through.

--Celine Dion

It's a few minutes after 6 a.m. and I'm doing the first of my nebulized medications for the day. I've been slightly slack on starting them earlier enough lately, opting for additional sleep in the mornings instead of just getting my rear in gear. My medication of choice these days for a bronchodilator is Xopenex--it doesn't make me nearly as jittery as the albuterol does, and since I do a lot of note taking at work lately, I want to make sure I can read my own handwriting later on!

If you've arrived at this page because you are looking for information about what a "day in the life" of someone with cystic fibrosis looks like--perhaps even specificially an adult with CF--then allow me to share a little corner of my world with you. My healthcare maintenance schedule is fairly rigorous even when I'm not fighting an infection, so you may find yourself experiencing a bit of information overload. Nonetheless, I hope you are able to develop a greater understanding of what people like me (CFers as we call ourselvs) go through in a typical day.

Take a deep breath! Here we go...

Starting the morning

I try to get in at least 8 hours of sleep each night. Last night I was fortunate to have gotten in nearly 10, which means today promises to to be a good day. The first thing I do after waking is take a Prilosec OTC pill. This helps protect my stomach in case my other medications aggravate it or in case my digestive problems cause some acid reflux. After the Prilosec I begin my inhaled medications. This helps open up my airways and make it easy for me to cough out the thick mucus that would otherwise obstruct my breathing and invite nasty lung infections. Xopenex, like albuterol is a bronchodilator--it opens up the airways.

Now it's 6:15 and the Xopenex is finished, so it's time to move on to the hypertonic saline treatment. I mix two concentrations of saline(10% and 3%) and put 4 mL of the resulting 7% solution in the nebulizer cup. I actually like this part of the routine the best. I'm a scientist by profession, so playing with syringes and turning my bathroom into something of a mini-lab is fun. It helps break up the monotony in the morning.

I'm starting to cough more now, which is good. The hypertonic saline (HS) is stimulating my lungs to produce moisture, which makes it easier for the mucus to slip and slide around. When I cough, I bring it up and out of my lungs and, I hope, bringing me out of harm's way. I have a pretty speedy nebulizer/compressor system, so the HS only takes about 10 minutes, depending on how much I have to pause to cough. Today it's looking like a 15 minute event.

As the HS winds down, I'm getting ready to inhale two powder-form medications: Advair and Spiriva. They are both long-acting medications that should help keep my airways from closing up between now and my evening treatments. Advair is something that is also used by asthmatics, which is why it has some benefit for me as a CFer too. Spiriva was originally intended for patients with chronic obstructive pulmonary disease (COPD) and although that's not the same as CF, it's similar enough to warrant the use of the med.

It's 6:30 and I'm ready to head downstairs to make breakfast for the two of us.


Even my selection of food preparation has to be done with CF in mind. I need close to 5,000 calories in a day for the level of energy I exert and in order to gain some weight. A high calorie, high protein diet is what I follow. Unfortunately, part of having CF means that my body does not absorb much of what I eat--a lot of it goes sliding right through, especially if it's got a lot of fat content. To combat this I take enzyme supplements to substitute for the enzymes my pancrease refuses to excrete because it is blocked by mucus. (And you thought CF just meant I had lousy lungs...ha!)

Today I plan to make cinnamon rolls and wash it down with a small glass of orange juice and a tall glass of whole milk mixed with Carnation instant breakfast. This is a whopping calorie meal and slightly higher in fat than what I usually do, but I'm feeling lucky today. Just to make sure I don't overdo it, I'll take 6 enzymes with the meal instead of 4 like I do with something less fatty. Additionally, I will take vitamin supplements--a multivitamin, vitamin E and vitamin A&D softgels,--to replace what is lost to malabsorption--and Os-Cal since I have osteopenia (a precursor of osteoporosis.)

While breakfast was in the oven, I made our lunches for the day. I am very much a creature of habit, so I don't vary too much in what I eat for my noon meal. Although I enjoy food, I have come to think of it merely as fuel for my body. I have learned what foods work well to give me the energy and calories I need, and can be digested relatively easily with the enzyme supplements.

After breakfast I take one more medication--sporanox. This is a liquid and although I've gotten used to its taste, I still hate it. I do it after breakfast because this way it doesn't upset my stomach, and also, because I know that I'll soon be able to brush my teeth to get the sporanox taste out of my mouth! Blech!

Off to Work!

I work full-time as an environmental scientist. Most of my job is indoors working at a desk. While I'm at work I continually strive to meet my caloric need by snacking on pretzels or other small foods. I find that pretzels are particularly easy for me to digest with a minimal number of enzymes. They are non-fat, and since they are made up of complex carbohydrates I know that they are a beneficial resource for my body. Another good snack for me is nuts, but since they are higher in fat, that doesn't always work out too well, so I have to be careful. Most of the day I sip water so I stay hydrated. Lately I've also been drinking cranberry juice. It's good for my kidneys and it has more calories than the same amount of cola. It's rare that I find something healthy in a small package that offers decent competition for the junk food!

Between 9 and 5

While I'm at work, CF doesn't really factor in too much unless I'm working on an outdoor project. Since this is not the case right now, the main thing that I do that is CF related is to wiple down my desk and other surfaces in my office with alcohol wipes. I do this every morning to make sure that my "CF cooties" aren't sticking around and making me sick. I wipe down the desk once a day, but the phone gets a going-over two times, since that comes in contact with my face. Maybe I'm a bit of a germa-phobe, but I like to feel like I have at least SOME degree of control.

Things to Consider

The effects of CF can be widlly variable, and no matter how well I try to take care of myself, eat right, etc., sometimes I will fall prey to a lung infection anyway. That's just the reality. Even when I'm feeling "well", I cannot forget that I have CF. I have to pace myself so that I don't deplete my energy. There are times (quite often, in fact) when I would love to do many activities, take on multiple projects at work, and try to be Super-wife at home, but the physical reality is that I cannot do those things.

I have to pick and choose. If I want to make sure I have the energy to drive out to spend time with my prayer group friends, then I have to plan ahead. If a project at work will require an early morning meeting, I have to be sure to go to be early enough the night before. If my husband wants to take me on a date, we need to decide in advance if it would be better to go out to an early movie then come home for dinner, or eat first and then see a movie. For me, having CF means planning as much as possible, given my limitations, but still being flexible enough to accomodate those limitations as the need arises. That is the biggest mental challenge of having CF.

After work

I arrived home about 15 minutes before my husband did, which gave me a chance to have a quick snack (I don't like to tempt him with my high calorie goodies!) while I sat down to unwind from my day at work. It seems like I'm always eating, which I suppose is true. Like I already said, food is fuel to me, so I'm not much good without it. If I get hungry I get crabby, and that's no fun for anyone.

Dinner preparations again involve careful consideration for nutritional value. Tonight we had marinated tri-tip steaks, baked potatoes, and California mixed vegetables. My potato, however, had about 2 tablespoons each of butter, cheddar cheese, and sour cream on it. I washed down my meal with 2 cups of whole milk. And let's not forget the enzymes...dinner is typically my largest meal of the day, and the one with the most fat content. In order to process the evening meal, I took 7 enzymes to handle it all. In addition to the enzymes, I took another multivitamin and another Os-Cal tablet.

After Dinner

As my body digests my dinner, I plan to use the energy I have left to get some housework done. We did all of our heavy-duty chores on Saturday, so tonight's "to-do" list really only involves a bit of laundry. I like doing laundry. It makes me feel productive, and it's relatively low-impact, so I don't get too worn out doing it. It's also methodical enough that it helps calm me down at the end of the long day and put me in a state of calm that is suitable for sleeping. To aid in that process, I'll also be taking 75mg of a medication called Anafranil. It helps keep my brain from running continuously all night. It's an anti-anxiety med, so it's not exactly unique to CF, but it's not uncommon for people with CF to take anti-anxiety medications or anti-depressants.

Even something as simple as doing the laundry must involve consideration of CF. We have to be sure to turn on the vent so that fiber particles don't blow around everywhere when the dryer is running. We only use detergents and dryer sheets that are "free-style" (aka no perfumes or dyes) so that my allergies and asthma aren't aggravated.

After taking care of some laundry, or perhaps even while I fold some of it, I will do a repeat performance of all the same nebulizer treatments and inhalers that I did this morning. All of it should take about an hour, and then it's off to bed, but not without one last snack to tide me over until morning. After the snack, taken with the appropriate number of enzymes, I'll take the last of my evening meds. Singulair will help keep my asthma in check throughout the night. Zithromax is a preventive antibiotic taken every other day to ward off lung infections caused by Pseudomonas. Last but not least, it's time for sporanox. I'll brush my teeth, take a quick soak in the tub, and then it's off to bed.

If my medications do what they are supposed to and I don't cough much during the night, I'll be able to get in a good 8 or 9 hours before I start the whole process over again.

Thanks for sharing my day with me. I hope you've learned a lot. Feel free to ask any questions or leave your comments by clicking on the "comments" link below!

Friday, October 06, 2006

If You're HEPA and You Know It...

I got allergies.

--Betty Hill

Let me start by defining HEPA Filter. It stands for High Efficiency Particulate air filter. From what I can tell, the term "true HEPA" is used to refer to where the filter is placed in the unit in conjunction with the motor. This is more of a consideration for HEPA vaccuums (which have exhaust) as opposed to the stationary room filters. When something says it is a true HEPA, it is basically saying that its efficiency in removing certain contaminants from the air is 99.97% effective at removing particles that are .3 microns and larger in diameter.

What exactly is a micron? A micron is one millionth of a meter, but that probably isn't very helpful to you, so I'll put it another way. A grain of salt is approximately 60 microns in size. To give you an even better idea of how small this is, here is a list of airborne pathogens and their sizes. The items listed in blue are generally visible to the naked eye. Those in red are very difficult to see as individual particles. The smaller the diameter, the easier it is for the pathogen to get into our small airways and cause irritation.

  • Pollen 10-60 microns
  • Mold spores 1-60 microns
  • Lint 10-60 microns
  • Dust 1-60 microns
  • Bacteria .25-20 microns
  • Smog .05-20 microns
  • Tobacco Smoke .05-5 microns
  • Viruses .01-.1 microns

As you can probably infer by looking at the list above, HEPA filters--which can remove particles .3 microns in diameter and larger-- are most effective at removing almost all of the things in that list. With the exception of viruses, airborne pathogens of the greatest concern to CFers are easily filtered from the air with a HEPA filter.

What about ionizing filters?

Ionic air cleaners work by breaking up the contaminant and pushing it around the room in a different form. They split the offending molecule into separate positive and negative charges (ions). Ionizing units are great for removing odor, and many people like them because all you have to do is wipe of the metal plates inside the unit as opposed to changing out filters. However, when the airborne pathogen is merely ionized, the separated atoms, particularly oxygen atoms, have the potential to combine with one another and form what is called "ground level ozone."

Ground level ozone varies slightly from the stratospheric ozone that makes up the ozone layer surrounding the earth. Stratospheric ozone is "good" ozone; ground level ozone is "bad" ozone. "Bad" ozone (i.e. ground level ozone) has been shown to impair lung function. Naturally, anything that reduces lung function is something a CFer should avoid. The technology is coming along to make ionizing air purifiers better, but as a CFer I would urge other CFers to steer clear of them.

What about units (like the one I have) with an optional ionizing function?

My general though is that, yes, this is okay. If you need to deal with a strong contaminant that may have a particularly problematic odor, I recommend using the ionizing function while you are not in the same room where it's being used. Or, if you'd rather not even take a chance with the ionizer, spray some Neutra-air or Febreeze into the room and let the air filter run full blast for awhile.

Many people are attracted to air purifying units (like the Ionic Breeze or the Living Air Purifier) that do not have filters that need to be replaced. Although the idea of less maintenance and less expense is nice, I strongly recommend biting the bullet and getting a unit with filters that can be replaced. Yes, it's an added expense, but I think it's a worthwhile one. My reasoning on that is based on my understanding of mold spores. If you have a unit that has been sucking up mold spores but you never physically remove them from your home, you are only perpetuating the problem. Mold spores can lie dormant for long periods of time and can flare up again when just the right conditions are in place. If you are sensitive to molds (perhaps if you have ABPA) this can be a problem. I'd much rather buy replacement air filters than spend large portions of my paycheck on expensive anti-fungal medications like Vfend and itriconozole.

People with allergies and CF should look into HEPA filter systems. My personal and professional choice for an air filter is the Bionaire HEPA Tower. It is very efficient; the price is great; it's far and away the quietest air filter I've found for its size. My favorite features are that it oscillates (which means it is able to do an even better job of moving the air) and it has a remote control.

SEARS has two different sizes of the Bionaire HEPA tower. They both have an independent ionization function, which, for the most part I leave turned off. The only time I had the ionizer switched on was when we first moved into our new house and the air conditioner wasn't installed yet. Since our windows were open a lot of the time for ventilation, I ran the filter with the ionizer on (while I was out of the room) to break down the emissions from passing cars (we live on a busy street.

I am exceptionally pleased with the Bionaire product. I have researched air filters and purifiers for a number of years now, and this one has the technology and aesthetics that I deem acceptable. I hope that has been helpful. If anyone else has other questions about how to effectively keep their home as allergen free as possible, please let me know and I'd be happy to help.

Tuesday, October 03, 2006

Chlorine and Potential Concerns to CF

Chlorine occurs naturally as a component of salt. It is a diatomic molecule and is a toxic, non-metallic flammable gas. Because of its high degree of effectiveness in killing harmful microorganisms (e.g. bacteria), chlorine is a widely used chemical for the treatment of public water supplies. The purpose of this report is to present a brief description of how chlorine is used as a disinfectant for water supply. Using information gathered from reputable websites, medical journals and regulatory documentation, it will also describe how chlorine reacts with other compounds to form trihalomethanes and why this is of concern to persons with impaired pulmonary function due to asthma or cystic fibrosis.

The Disinfection Process: Beneficial and Necessary

Clean, safe water is vital to the overall health of every individual. Before water can be fit for human consumption or use, it must undergo a series of physical and chemical processes. The practices of water filtration and treatment vary only slightly depending on whether the end use of the water needs to comply with Federal regulatory requirements of the Safe Drinking Water Act or the Clean Water Act.

The general process for water treatment takes place as follows:

Raw water from surface water (i.e. local rivers or reservoirs) pass through a large screen. This removes the largest debris such as leaves, rocks, trash and sediment from the water. The water then flows toward a treatment tank where it undergoes rapid mixing. This phase is part of what is called "pre-treatment." During pretreatment strong disinfectant chemicals are added to the water. These chemicals are chlorine, ammonia, lime, and aluminum sulfate.

When the chlorine reacts with ammonia, it forms chloramines. These powerful new compounds are able to kill harmful bacteria and viruses. Because chloramines are highly stable and persistent, they are able to continue protecting the water long after it has left the treatment facility. This ensures that the water flowing out of our tap at home is safe to drink and free from bacteria.

The purpose of adding lime is to adjust the pH of the water. pH is a measure of how acidic something is. Water should be of neutral pH. Addition of aluminum sulfate into the disinfected water promotes even distribution of the chemicals that have been added. It also acts as a coagulant, meaning that it causes any other particles that remain in the water to stick together forming a "floc." Because flocs are made up of several particles, they become heavier than the water molecules and sink to the bottom of the mixing tank. Once the flocs have collected, they are removed. Flocs are harmless bits of sediment and can be recycled back into the environment sort of like applying mulch to a garden.

The water then undergoes another round of filtration and another series of treatment with chloramines and lime. Some cities are required to add fluoride to their drinking water supply, and that takes place during this phase of treatment. Another chemical, called orthophosphate is added to protect the pipes that the water will travel through. It works by preventing corrosion which could adversely affect the pH of the water or cause small amounts of metals to be present.

Clean, safe drinking water is then pumped from the treatment facility to consumers.

Chlorine's effects on lung health

Throughout the last decade, as asthma has undergone increased scrutiny by epidemiological researchers, several resources have come to light regarding the hazards associated with chlorine exposure. Chloramines are known to cause an increase in the frequency and severity of bronchospasms/bronchoconstriction associated with asthma and other pulmonary diseases, such as cystic fibrosis. Public drinking water supplies and the disinfection process it undergoes, do not present nearly the same level of concern as do sources like public swimming pools. It is the author's recommendation that areas where chlorine bleach or pool chlorine are used in noticeable amounts (i.e. detected by smell) are to be reasonably avoided by persons with compromised lung function.

Additional resources

EPA Chlorine Hazard Study

Chlorine in Pools May Cause Breathing Trouble
The Connection Between Chlorine and Asthma
European Investigators Identify Potential Cause of Asthma in Swimmers (Doctor's Guide)
Office of Environmental Health (OEHA) Toxic Air Contaminants
Aqualyse (R) Pool Chlorine Linked to Asthma Epidemic

Sunday, October 01, 2006

If Less is More, Just Imagine How Much More is More!

The best cure for the body is a quiet mind.

--Napoleon Bonaparte

Meropenem is a medication that I have been on twice now as part of my IV tune-up regimen. By itself I don't think it would be completely sufficient for knocking out the types of infections I get (PA, B. cepacia and the like) but its synergistic effects when combined with medications like Tobra or Colistin make it pretty powerful.

Meropenem, commonly referred to as Merrem, is the first carbapenem to be approved for use in the United States and others are sure to follow. A recently published article about a study carried out by Johnson & Johnson Pharmaceuticals highlights the findings of yet another, more powerful carbapenem (Doripenem) that may soon be available. If Merrem is good, is this new one even better? As is usually the case for us CFers awaiting new and better treatments, one can only hope.

SAN FRANCISCO, Sept. 29 /PRNewswire/ -- Johnson & Johnson Pharmaceutical Research and Development, L.L.C. today announced that doripenem, an investigational carbapenem(1) antibiotic in Phase III trials, achieved a high clinical cure rate of 83 percent in patients with intra-abdominal infections. Doripenem also was active against a wide range of Gram-positive(2) and Gram-negative(3) bacteria that cause serious hospital infections -- including Pseudomonas.

Read the original article as it appears here

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