Hi there. My name is Genevieve, and I was born on the 23rd of July, 1975. I was diagnosed with Cystic Fibrosis when I was 3 and a half years old. I am the youngest of 5 in my family, none of whom have CF, but 6 years ago, my eldest sister also had a child with CF.
My life, despite its up and downs that come with the disease, has been absolutely fabulous. I have been most fortunate in being given some wonderful experiences and memories that have happened because of CF.
As more and more CF children become CF adults, new issues are arising and new bridges are being crossed. Things like having children, marriage, independent living, "cross infection", family planning and financial concerns are all becoming more prominent.
And sure enough, I am one of these adults - yet my primary concern is (and has been for the last 10 years) wanting to explore new territories in alternative medicine, other than relying solely on western medicine and my doctor's opinion, to treat my disease. So far, I am succeeding. This is one of my main motivations for wanting to do this website.
My other motivation is to inspire you. To give you some hope, some faith. I am a CF adult getting out there and doing all the things I love. CF is riddled with negativity and sadness and rightly so, as we all experience it. It is a devastating disease, and for a majority of CFers, a lifelong battle. But there is also a question of not letting CF get you down, and also not conforming to what is expected - particularly with the "expected", "eventual", deterioration. Beliefs are powerful, and I have found it difficult at times to stand my own ground and not be brought down by the expected eventualities of CF. More and more I become more determined to live a long life and I encourage CF sufferers, parents of CF children and adults and anyone else who is somehow affected by CF, to not give up and believe in the power of your own healing ability and strength.
WHAT IS CYSTIC FIBROSIS?
Cystic Fibrosis (CF) is an inherited disease which causes glands in the body to function abnormally. The glands that are affected are the exocrine glands.
These glands normally make thin, slippery secretions - these include sweat, mucus, tears, saliva and digestive juices. These secretions are carried through small tubes to the external surface of the body, or into hollow organs like the airways. The exocrine glands and their secretions play a major role maintaining the normal functions of the body.
In CF, the exocrine glands produce mucus that is thick and sticky. This sticky mucus may block up the ducts and other passageways. Trapped mucus occurs mostly in the lungs and intestines and can interfere with important body functions, such as breathing. If not removed, this thick mucus and plugs can lead to repeated lung infections and lung damage. Lung infections are really dangerous to people with CF, because- repeated infections can damage the small cilia (hair like structures) on cells in the lungs that force mucus to the throat and the airways, making them even less able to get mucus, particles and germs from the lungs. AND
- infections usually cause even more mucus to be produced. This in turn can cause more mucus blockages and make an infection even harder to get rid of.
From all this, a nasty cycle can develop. CFs are also very prone to two particular types of lung infections. Staphylococcus aureus and Pseudomonas aeruginosa love love love CF lungs. I have had both of these present in my sputum. I have also had a mould present in my sputum sample called Aspergillus Fumigatus, but after blood tests, my doctors decided I was not actually having any reaction to this particular bug. Some CFs, have been known to though.
To help with removing mucus, CFs have to have daily physio. There are a variety of ways to do this, the aim being to get up as much mucus as possible and unclog the airways. Exercise is also encouraged, to help loosen mucus and cough it up, to build up strength and stamina and increase the general level of cardiovascular fitness.
Aerosols (Bronchodilators, Mucolytics, decongestants) and Antibiotics (oral, intravenous and aerosolized) are also heavily used in the treatment of Cystic Fibrosis.
Also, for the majority of CF sufferers, digestive problems occur. Thick mucus prevents the flow of digestive enzymes from the pancreas into the intestines where the enzymes usually help to digest the food. People with CF may therefore have difficulty in gaining weight and the undigested food results in large bulky bowel actions. So that they can digest food, people with CF take digestive enzymes before food. Some popular brands of enzymes taken these days are Pancrease, Cotazym and Creon Forte
Another problem is that the sweat glands are also affected in CF. More salt and potassium is lost during sweating, so dehydration can result. Salt tablets taken once a day, combat this salt loss, or just increased amounts of salt in food. Increased dosages would be in hot weather, when exercising strenuously, or when sufffering from a fever.
POSSIBLE COMPLICATIONS of CYSTIC FIBROSIS
Lung Damage, Diabetes, Osteoporosis, Liver Damage (Cirrhosis), Kidney Failure, Sinusitis, Rectal Prolapse ( can occur in children as a result of digestive complications - happened to me when I was young), Pneumonia, Nasal Polyps, Meconium Ileus, Meconium Ileus Equivalent, Hyperglycemia, Clubbing (rounded, enlarged tips of the fingers and toes. Usually indicates a chronic deficiency of oxygen in the bloodstream), Bronchitis, Dehydration, Tiredness, Thrush, Candida, Lethargy, Stunted Growth, Digestion problems, poor appetite and being underweight just to name a few things!What Are the Symptoms of CF?
- Persistent cough, particularly with physical effort
- some difficulty in breathing or wheezing with effort
- tiredness, lethargy or lower tolerance to exercise
- frequent visits to the loo
- salt loss in hot weather - this can produce cramps, weakness or dehydration
- poor appetite (not one of my problems!!!)
Me and CF - my story
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