What is Cystic Fibrosis?
Cystic fibrosis is an inherited disease affecting the glands that
produce mucus, tears, sweat, saliva and digestive juices in the
body. Normally, these secretions are thin and slippery, but in CF,
a defective gene causes the secretions to become thick and sticky
leading to obstruction of the lungs and preventing the pancreas
from secreting the enzymes necessary for the digestion of fats.
Respiratory failure is the most dangerous consequence of CF.
What is the cause? Cystic fibrosis is a genetic disease. The genes cause
abnormal mucus in the respiratory and gastrointestinal tracts and
sweat glands. This causes lung obstruction and infection, poor
digestion and food absorption. The greatest risk factor for CF is
a family history of the disease. If both parents come from
families with CF, the chances are one in four that each of their
children will also have CF. Cystic fibrosis occurs mainly in
whites of Northern European ancestry. It is relatively rare in
black Americans and Asian Americans. Hence, if one has a family
history of cystic fibrosis, it is advisable to seek genetic
counselling before starting a family. People who carry the CF gene
are healthy and have no symptoms of the disease. The environment,
diet, exercise, or other lifestyle behaviours do not cause CF. It
is not contagious and cannot be passed from one person to another
except through inheritance.
What are the symptoms?
The specific signs and symptoms of CF vary depending on the
severity of the disease. In some newborns the first sign may be a
blockage of the intestines due to thick, sticky stools (meconium)
Later symptoms:
* poor weight gain despite good appetite
* bad-smelling, large, fatty stools
* a barrel-chested appearance
* chronic cough
* frequent and severe respiratory infections with sticky sputum
* salty sweat
* enlarged liver and spleen
* blockage in the bowel
* delayed growth
* frequent chest and sinus infections with recurring pneumonia or
bronchitis
* enlargement or rounding (clubbing) of the fingertips and toes
* cirrhosis of the liver due to inflammation or obstruction of the
bile ducts
How is the diagnosis made?
The standard diagnostic test for CF is a sweat test, which
measures the amount of sodium and chloride in a person's sweat. A
consistently high level of salt indicates CF. In some cases a
genetic analysis of a blood sample is performed to confirm the
diagnosis of CF. Tests may also help determine the extent and
severity of CF.
What are the complications?
Pneumonia
Chronic bronchitis
Bronchiectasis
Fluid and electrolyte imbalance
Malnutrition
Nasal polyps
Rectal prolapse
What is the treatment?
Cystic fibrosis is currently considered incurable and is often
fatal in childhood. However, careful long-term care by parents and
professionals can help the patients lead reasonably comfortable
lives. Children with milder forms can live till adulthood,
especially if the disorder is detected early.
Researchers have identified the gene that causes cystic fibrosis.
New strategies are aimed at developments to prevent and treat this
disease. Management of the disease varies widely from person to
person and generally focuses on respiratory and digestive
problems. It usually involves a combination of medications and
home treatments (including respiratory and nutritional therapies),
as well as hospital care for some complications. Newer treatments
are allowing some of those with CF to live well into their 40s,
50s, and later years. However, there is no cure for the disease.
MEDICATIONS
* Mucus-thinning drugs: these drugs help in making mucus thinner
and easier to cough up
* Bronchodilators: use of medications such as albuterol may help
keep the airways in the lungs open by helping to clear thick
bronchial secretions
* Bronchial airway drainage: people with CF need a way to
physically remove thick mucus from their lungs. This is often done
by manually clapping on the front and back of the chest. In some
cases an electric chest clapper, known as a mechanical precursor,
is used. Some people may use an inflatable vest with a machine
attached that vibrates at high frequency to help cough up
secretions
* Oral enzymes and better nutrition: CF can make the patients
malnourished because the pancreatic enzymes needed for digestion
do not reach the small intestine. As a result, the patient may
need 50 percent to 100 percent more calories than normally
required.
* Supplemental high-calorie nutrition, special vitamins and
enteric-coated oral pancreatic enzymes can enable the patients to
maintain and even gain weight
* Antibiotics may also be required in case of lung infections
DIET
* The patient should eat a low-fat diet with adequate proteins
* Vitamin and mineral supplements may be necessary
* Intake of liquids should be encouraged, which help in thinning
mucus
* Consultation with a dietician is advisable for specific
instructions
Many treatments exist for the symptoms and complications of CF.
The main goal is to treat infections, keep the lungs free of
sputum, improve airflow and maintain adequate calories and
nutrition.
Lung transplants not
harmful for children with cystic fibrosis
Inherited Disease
Cystic Fibrosis - treatment of Cystic Fibrosis,
Cystic Fibrosis types, Disease medicines, Cystic Fibrosis symptoms, Cystic
Fibrosis and Disease symptoms, Cystic Fibrosis symptoms Disease and
diagnosis, Symptoms and Solutions, Signs and Symptoms, type of Cystic
Fibrosis, cause common, common Cystic Fibrosis, Cystic Fibrosis List, causes
list, Infectious Cystic Fibrosis, Causes, Diseases , Types, Prevention,
Treatment and Facts, Cystic Fibrosis information, Cystic Fibrosis:
Definition, Cystic Fibrosis names, medical Cystic Fibrosis, medical Cystic
Fibrosis and disorders, cell Cystic Fibrosis, Cystic Fibrosis Worldwide,
Cystic Fibrosis Research, Cystic Fibrosis Control, Cystic Fibrosis Center,
Digestive Cystic Fibrosis Week, Information about Cystic Fibrosis, causes of
different Cystic Fibrosis, Cystic Fibrosis Articles, Cystic Fibrosis and
conditions, Health and Cystic Fibrosis, Cystic Fibrosis Patients, Cystic
Fibrosis and Sciences, causes of alzheimer's Cystic Fibrosis, Cystic
Fibrosis causes, alternative medicine heart Cystic Fibrosis, body ailments,
Cystic Fibrosis medicines, medical antiques, type of blood Cystic Fibrosis