What Is Cystic Fibrosis?

Cystic Fibrosis (CF) is an inherited disease, affected about 1 in 2500 white babies (much less common in other races).

A healthy person has a constant flow of mucus over the surfaces of the air passages in the lungs. This removes dust particles and bacteria. In someone with CF, the mucus is too sticky and doesn't function right. Instead, it becomes a perfect environment for bacteria to grow in.

CF tends to cause heavy and sometimes constant coughing, associated with the excess mucus production. Sufferers also tend to have too much saliva, bouts of wheezing, and they get short of breath doing many ordinary activities.

Babies with CF are often diagnosed soon aftter birth, but for many babies the condition only becomes apparent as they have trouble putting on weight (due to their bowels clogging up with mucus). Constipation is a common life-long problem, and severe (life-threatening) abdominal blockage may occur. The life expectancy for sufferers is low, but most live into young adulthood.

What Causes CF?
CF is an inherited disease, which means that it's passed down from parent to child. Someone who has CF was born with it. Maybe you've heard someone say, "It's in your genes." They don't mean your blue jean pants. Genes make up the blueprint each of your cells follows to make you a unique individual.
 
Genes determine your eye and hair color and also are responsible for certain health problems. People with CF got the disease because their moms and dads each had a gene for CF. You need two CF genes (one from mom and one from dad) to have cystic fibrosis. Most people don't know that they carry the gene for CF until they have a child who has the disease because carriers of the CF gene do not have the disease themselves.
 
How Is CF Diagnosed?
Although someone with cystic fibrosis is born with it, it isn't always obvious at birth. It may take a while for symptoms to develop. Doctors may suspect that a baby has CF if he or she coughs a lot and gets a lot of lung infections. The baby also might have unusual large, bulky bowel movements or may not gain weight as expected.
 
To know for sure, the doctors can do a simple test that doesn't hurt. Kids with CF have more salt in their sweat than other kids, so by simply collecting a sample of sweat and testing to see how much salt is in it the doctors can determine if a person has CF. Other tests may be done, including one that looks for the gene that causes CF.
 

A multi system disorder, cystic fibrosis is characterized by a disturbance of exocrine gland function, resulting in raised levels of sodium chloride in the sweat and thick mucous secretions from the secretary glands. It is the most common life threatening genetic disease in the white – skinned people and is less common in those with a black skin. More than one child in a family may be affected as the disease is transmitted as a recessive gene carried by each parent.

The production of the abnormal mucus results in chronic lung infections, pancreatic insufficiency and intestinal obstruction in the newborn (meconium ileus). Unlike children suffering from celiac disease, children with cystic fibrosis are friendly and good tempered. The appetite remains good in the absence of general infection but in spite of this there is failure to gain weight, growth is shunted, the abdomen is distended. The stools are pale and bulky. Repeated chest infections are often a presenting symptom.

When the disease is well - established at birth, intestinal obstruction results due to the failure to pass meconium. This is because the enzyme which would normally liquefy the meconium is absent. Urgent treatment either by surgery or treatment by gastrografin enema under X-ray control is required.

Cystic fibrosis or CF, also known as Mucoviscidosis, is a type of genetic disease which has an effect on the whole body, being the cause of gradual disability over a period of time and eventually death at an early stage. It was earlier called as cystic fibrosis of the pancreas, now it is called just as cystic fibrosis.

The symptom most seen in people suffering from this condition is finding it difficult to breathe. This takes place due to recurrent infections of the lungs, which get treatment but not necessarily cured by means of antibiotics and other types of drugs.

Several other symptoms like sinus infections, slow growth, loose motions etc are effects of cystic fibrosis. The odds of any child getting cystic fibrosis are one in every 2500, and it is one of the most widely occurring hereditary terminal conditions.