Category Archives: Cystic Fibrosis

A Day in the Life of a Cystic Fibrosis Patient


Bob Aronson, the Author of most of these posts, does not  have CF.  He suffers from Chronic Obstructive Pulmonary Disease (COPD), a distant cousin of CF but related in the sense that he can identify with the struggle to breathe.  He is also a 2007 heart transplant recipient)

A few days ago I posted a blog on Cystic Fibrosis that has attracted a great deal of attention but my blog didn’t do justice to the heroic efforts these patients make every day just so that they can function.  Imagine if you can, getting up every day experiencing great difficulty breathing and then being required to exercise in order to clear your lungs…exercise that requires beating on your chest to loosen the mucus that restricts their ability to function.  Some people must physically beat on their own chests while others, if they can afford it, get a machine to do it for them, sometimes for up to an hour.

I found this account on the internet and rather than re-write it and lift excerpts I choose to publish the entire account unedited.    Your comments are welcome.

My Version of Normal

Lauren Beyenhof, Yahoo! Contributor Network

 http://tinyurl.com/946gllo

Diagnosed with cystic fibrosis at birth, I was not expected to celebrate my tenth birthday. Today, thanks to the hard work of many doctors and researchers, new drug therapies and the support of a wonderful family, I am living and breathing in my late twenties. A day in my life is somewhat routine with room for adjustments as needed.

The first thing I do every morning upon waking is cough. I cough because the thick, sticky mucus so characteristic of cystic fibrosis, has lodged itself in my airways during the night. Some mornings it only takes five minutes to clear my lungs of sputum. Cystic fibrosis patients like me have affectionately dubbed these secretions “lung oysters.”

My first round of coughing complete, I move on to a more dramatic approach to airway clearance. I stumble downstairs to the refrigerator where I keep one of my most effective medications. Pulmozyme, or DNase, is a mucolytic developed by Genetech. When inhaled as a mist via nebulizer, it works in my lungs to cut apart the bacteria that thrive there. Pulmozyme is just one of the inhaled medications I need to open up my airways to their fullest. A bronchodilator in the form of a metered dose inhaler (MDI) goes a long way to help with that. Later in the day, I may need that bronchodilator again if I feel my chest becoming tight.

The highlight of my morning routine is my high-frequency chest wall oscillation (HFCWO) vest. It looks like a life vest. On the front of the vest are two hoses that attach to an air compressor. As the vest fills with air and begins to vibrate, the controls on the compressor allow me to set the frequency, pressure and length of time for the vibration therapy. I wear the vest for a minimum of 20 minutes on days when I feel well and up to an hour when I’m struggling with chest congestion.

After the vest, it’s finally time for breakfast. I am a pancreatic insufficient CF patient, which means that my pancreas doesn’t secrete the enzymes I need to absorb the energy and nutrients from my food. To compensate for that I take enzyme supplements. My breakfast is usually on the order of 900 calories or more and typically includes some fruit, milk and some sort of complex carbohydrates like muffins or cereal.

Breakfast time is hardly complete without vitamin and mineral supplements to replace those I cannot absorb from food. Calcium, magnesium and a multivitamin of the fat-soluble vitamins A, B, D, E and K are swallowed together to save time. I learned long ago how to swallow multiple pills at a time.

Once I’ve taken my vitamins, I get ready for my day. My form of cystic fibrosis has progressed to the point that I have moderate loss of lung function. This keeps me from working a 40-hour week like I used to when I was younger. Three days a week, I go in to work where I enjoy a part-time schedule as an environmental scientist. My employer is very understanding of my needs for managing CF and has given me a reasonably flexible schedule. Some days I’m even able to work from home.

At lunch time I eat another calorie-laden meal, complete with enzymes. I try to eat almost the same thing every day, because I view food as fuel. Sandwiches (yes, plural on a single day), yogurt, pretzels, juice and a soda are my main fare. My lunch break is another chance for me to do some airway clearance and take my MDI to keep my airways open. I keep a small, hand-held nebulizer/compressor in my desk drawer along with a small stash of medications just in case.

After work I come home, take a quick cat-nap on the couch and prepare dinner. Dinner time is where I really shine! I love to cook and my husband loves it when I have a meal ready to go when he walks through the door. I prepare two plates every night. One for me, which has a double helping of almost everything and one for him, a lighter version of the evening’s food selection. When it comes to food at our house, grocery shopping can be difficult since I need high-calorie foods, and my husband, who watches his weight in a manner completely different from mine, likes low-fat or non-fat items.

The dinner dishes having been cleared, my husband and I relax and enjoy the evening together. Usually we watch a bit of television or watch a Netflix movie together. By the time 8:30 p.m. rolls around it’s time for me to once again begin a series of airway clearance and nebulizer medications. I put on the vibrating vest and set it to a pressure that allows me to move relative freely. During this time I lift hand weights and do a lot of stretching to build muscles. Since muscle weighs more than fat, it is important that I have enough of both to give my body the energy it needs not only to sustain me throughout the day’s activities, but to help fight off any flare-ups of lung infections.

Half an hour later, my treatments are complete and I spend a half hour playing with my pet rabbit. Initially we were concerned that an indoor animal would be a problem for my allergies. Our worries turned out to be unfounded. The rabbit only sheds a few times a year, and as long as I keep his cage and litter box neat, there’s no odor. Having a pet is a great source of therapy for me, especially when I’m home sick.

I try to get to bed at the same time every night. Cystic fibrosis patients are encouraged to get at least 10 hours of sleep. I usually manage to get between 8 and 9 hours. After such a full day of taking care of myself, doing my share around the house and making sure my husband is happy, a good night’s sleep is not only much needed, it’s much welcomed.

Aside from all the medications and airway clearance treatments, a day in my life as a cystic fibrosis patient really isn’t that different from anyone else’s day. I just have to be more careful about how I budget my time and my energy so that I can be as normal as possible and enjoy the highest quality of life this world has to offer.

Suggested resources

Cystic Fibrosis is a terrible disease and while progress is being made in treating it, lifespans for its victims are still relatively short. If you’d like to help fund research or offer assistance in other ways contact the Cystic Fibrosis Foundation http://www.cff.org/GetInvolved/ManyWaysToGive/MakeADonation/

Bob Aronson of Bob’s Newheart is a 2007 heart transplant recipient, the founder of Facebook’s nearly 2,500 member Organ Transplant Initiative and the author of most of these donation/transplantation blogs.

You may comment in the space provided or email your thoughts to me at bob@baronson.org. And – please spread the word about the immediate need for more organ donors. There is nothing you can do that is of greater importance. If you convince one person to be an organ and tissue donor you may save or positively affect over 60 lives. Some of those lives may be people you know and love.

Please view our video “Thank You From the Bottom of my Donor’s heart” on http://www.organti.org This video was produced to promote organ donation so it is free and no permission is needed for its use.

If you want to spread the word personally about organ donation, we have another PowerPoint slide show for your use free and without permission. Just go to http://www.organti.org and click on “Life Pass It On” on the left side of the screen and then just follow the directions. This is NOT a stand-alone show; it needs a presenter but is professionally produced and factually sound. If you decide to use the show I will send you a free copy of my e-book, “How to Get a Standing “O” that will help you with presentation skills. Just write to bob@baronson.org and usually you will get a copy the same day.

Also…there is more information on this blog site about other donation/transplantation issues. Additionally we would love to have you join our Facebook group, Organ Transplant Initiative The more members we get the greater our clout with decision makers.

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Cystic Fibrosis — The Victims are Heroes


CF is Cystic Fibrosis. Most of us know nothing about it even though we are familiar with the term. CF is a devastating disease causing constant discomfort and requiring intense and frequent treatment.

A CF patient must start every day with an extended and sometimes agonizing period of therapy. Often that therapy has to be repeated several times during the day. I’ve known several CF patients and to me they are special because of the heroic efforts they must put forward every day just to be able to approach normal functioning. Most diseases are difficult to manage but CF patients need to get physical in order to function. They are amazing people.

CF is one of those diseases in which a Lung Transplant is sometimes necessary and quite helpful but the procedure does not cure the disease. We’ll discuss that option more later.

I don’t have CF but I do have Asthma and Chronic Obstructive Pulmonary Disease (COPD) so I can at least relate to the part of CF that causes difficult breathing.  The Clinical description of CF sounds bad enough but until you’ve experienced what it’s like to struggle for air it Is difficult if not impossible to understand.  One CF patient said, “It feels like you’re breathing through a small straw all the time.”

Difficult breathing alone is a terrible affliction but CF is much more than difficult breathing, it affects almost the entire body.   This definition from Medicine Net seems to sum up the disease in graphic, therefore understandable terms (http://www.medicinenet.com/cystic_fibrosis/article.htm).

“Cystic fibrosis mostly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs. Mucus is a substance made by the lining of some body tissues. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. However, if you have cystic fibrosis, your mucus becomes thick and sticky.

The mucus builds up in your lungs and blocks your airways—the tubes that carry air in and out of your lungs. The buildup of mucus makes it easy for bacteria to grow. This leads to repeated, serious lung infections. Over time, these infections can severely damage your lungs.

The thick, sticky mucus also can block tubes, or ducts, in your pancreas. As a result, the digestive enzymes that your pancreas makes can’t reach your small intestine.

These enzymes help break down the food that you eat. Without them, your intestines can’t fully absorb fats and proteins. This can cause vitamin deficiency and malnutrition because nutrients leave your body unused. It also can cause bulky stools, intestinal gas, a swollen belly from severe constipation, and pain or discomfort.

Cystic fibrosis also causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. This can upset the balance of minerals in your blood and cause a number of health problems. Examples include dehydration (a condition in which your body doesn’t have enough fluids), increased heart rate, tiredness, weakness, decreased blood pressure, heat stroke, and, rarely, death.” The Clinical description of CF sounds bad enough but until you’ve experienced what it’s like to struggle for air it Is difficult if not impossible to understand.

The Cystic Fibrosis Foundation (www.cff.org/home/) Says this about the disease.

“Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide.   An additional ten million more—or about one in every 31 Americans—are carriers of the defective CF gene, but do not have the disease. CF is most common in Caucasians, but it can affect all races”.

In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.”

The sad fact of life for the approximately 30,000 Americans who suffer from cystic fibrosis (CF) is that they must get their chests pounded at least twice a day.

Chest pounding, also known as chest percussion, loosens the thick mucus that forms in the lungs of CF patients, allowing them to cough or sneeze up mucus and consequently breathe more easily. Chest pounding is a primary therapy for treating the disease.

To achieve chest percussion, CF patients today have two main choices: they can have a respiratory therapist perform the chest-pounding or they can purchase a CF “vest.” The vest, once the patient puts it on, uses air waves to shake the whole upper body, helping to loosen mucus in the lungs.

In this video a young woman not only demonstrates the vest but has some fun with it.  http://www.youtube.com/watch?v=NEBM7ediRic&feature=related

Symptoms of Cystic Fibrosis

From the Mayo Clinic

http://www.mayoclinic.com/health/cystic-fibrosis/DS00287/DSECTION=symptoms

Respiratory signs and symptoms
The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause:

  • A persistent cough that produces thick spit (sputum) and mucus
  • Wheezing
  • Breathlessness
  • A decreased ability to exercise
  • Repeated lung infections
  • Inflamed nasal passages or a stuffy nose

Digestive signs and symptoms
The thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these digestive enzymes, your intestines can’t fully absorb the nutrients in the food you eat. The result is often:

  • Foul-smelling, greasy stools
  • Poor weight gain and growth
  • Intestinal blockage, particularly in newborns (meconium ileus)
  • Severe constipation

Frequent straining while passing stool can cause part of the rectum — the end of the large intestine — to protrude outside the anus (rectal prolapse). When this occurs in children, it may be a sign of cystic fibrosis. Parents should consult a physician knowledgeable about cystic fibrosis. Rectal prolapse in children may require surgery.

Currently, there is no cure for cystic fibrosis. However, specialized medical care, aggressive drug treatments and therapies, along with proper CF nutrition, can lengthen and improve the quality of life for those with CF.

Each day most people with CF:

  • Take pancreatic enzyme supplement capsules with every meal and      most snacks (even babies who are breastfeeding may need to take enzymes).
  • Take multi-vitamins.
  • Do some form of airway clearance at least once and sometimes up to four      or more times a day.
  • Take aerosolized      medicines—liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer.

Because CF is a complex disease that affects so many parts of the body, proper care requires specialized knowledge. The best place to receive that care is at one of the more than 110 nationwide CF Foundation-accredited care centers

Lung transplants

While lung transplants are an option for CF patients, the procedure will not cure the disease, because the defective gene that causes it is in all of the cells in the body, not just in the lungs. At this time, scientists are not able to “fix” genes permanently but they are working on it. . While a transplant does give a person with CF a new set of lungs, the rest of the cells in the body still have CF and may already be damaged by the disease. Further, organ rejection is always possible and drugs that help prevent organ rejection can cause other health problems.

Cost and available help

As is the case with most chronic diseases treating CF can become very expensive but there are programs that exist to help patients with these challenges.  Many people with CF use Cystic Fibrosis Services, Inc., a specialty pharmacy that is a subsidiary of the Cystic Fibrosis Foundation. It provides access to CF drugs, offers patient assistance programs and works to help resolve complex insurance issues. CF Services is a participating provider with more than 5,000 insurance plans and nearly 40 state and federally funded programs. Visit www.cfservicespharmacy.com or call (800) 541-4959.

In 2008, the CF Foundation launched the Cystic Fibrosis Patient Assistance Program (CFPAF) as a nonprofit subsidiary. The CFPAF helps people with CF (who qualify) who need FDA-approved medication or paired drug-delivery devices for the nebulized treatment of CF-related pulmonary disease, or an FDA-approved medication for the treatment of pancreatic insufficiency related to CF. Case managers at the CFPAF help people with CF with ways to reduce out-of-pocket costs for CF drugs. All funds distributed by the CFPAF are provided by grants from drug manufacturers. Visit http://www.cfpaf.org or call (888) 315-4154.

CF drug companies often offer a range of patient assistance programs — from giving out samples of new CF products, to providing free nutritional supplements, to accepting voucher payments for CF drugs. Find out more information in the Foundation’s archived Web cast entitled, ” Patient Advocacy: Issues and Answers for CF.”

Suggested resources

Cystic Fibrosis is a terrible disease and while progress is being made in treating it, lifespans for its victims are still relatively short.   If you’d like to help fund research or offer assistance in other ways contact the Cystic Fibrosis Foundation http://www.cff.org/GetInvolved/ManyWaysToGive/MakeADonation/

Bob Aronson of Bob’s Newheart is a 2007 heart transplant recipient, the founder of Facebook’s nearly 2,500 member Organ Transplant Initiative and the author of most of these donation/transplantation blogs.

You may comment in the space provided or email your thoughts to me at bob@baronson.org. And – please spread the word about the immediate need for more organ donors. There is nothing you can do that is of greater importance. If you convince one person to be an organ and tissue donor you may save or positively affect over 60 lives. Some of those lives may be people you know and love.

Please view our video “Thank You From the Bottom of my Donor’s heart” on http://www.organti.org This video was produced to promote organ donation so it is free and no permission is needed for its use.

If you want to spread the word personally about organ donation, we have another PowerPoint slide show for your use free and without permission. Just go to http://www.organti.org and click on “Life Pass It On” on the left side of the screen and then just follow the directions. This is NOT a stand-alone show; it needs a presenter but is professionally produced and factually sound. If you decide to use the show I will send you a free copy of my e-book, “How to Get a Standing “O” that will help you with presentation skills. Just write to bob@baronson.org and usually you will get a copy the same day.

Also…there is more information on this blog site about other donation/transplantation issues. Additionally we would love to have you join our Facebook group, Organ Transplant Initiative The more members we get the greater our clout with decision makers.

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