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
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
- 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
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.”
- http://tinyurl.com/9swbmsn (CF Living)
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 firstname.lastname@example.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 email@example.com 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.
Posted on September 15, 2012, in Cystic Fibrosis and tagged assistance, brreathing, CF, chest, Chronic, cure, Cystic Fibrosis, dehydration, digestive, Disease, enzymes, gene therapy, genes, lungs, Medicine, pancreas, pounding, research, resources. Cystic Fibrosis Foundation., salt, sweat, transplant, treatment, vest. Bookmark the permalink. 3 Comments.