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Symptoms related to the respiratory system include:
Normally, mucus is thin and can be easily cleared by the airways. With CF, mucus becomes thickened, sticky and gradually blocks the airways. Eventually, cysts may develop due to this blockage. This can result in lung infections. These are quite common in children with CF, because bacteria that are normally cleared by the airways remain in the thickened mucus. Many of these infections are chronic and commonly caused by either the pseudomonas aeruginosa or staphylococcus (staph) bacteria.
About 10 to 20 percent of children with CF have nasal polyps — small protrusions of tissue from the lining of the nose — that need to be surgically removed.
Children with CF also have a high rate of sinus infections.
Symptoms related to the digestive system include:
The gene that has the CF mutation — the CFTR gene — controls the flow of water and certain salts in and out of the body's cells. The mutation means that mucus throughout your child’s body becomes thickened. The pancreas secretes substances that help digestion. In around 90 percent of children with CF, these substances become thick, and cause ducts (passageways between organs) to become blocked, resulting in fewer enzymes.
With fewer digestive enzymes, a child will have difficulty absorbing the nutrients the body needs, especially:
In addition to affecting the respiratory and digestive systems, cystic fibrosis can also lead to diabetes. This is because problems with the pancreas may become so severe that some of its cells are destroyed, leading to glucose intolerance and insulin-dependent diabetes.
About 35 percent of CF patients develop this type of diabetes in their 20s, and more than 40 percent develop the disease after age 30.
A small number of patients may also develop liver disease. Signs of liver disease include:
Cystic fibrosis is a genetic disease. Two things are true of all genetic diseases: their root cause is located on the cellular level and they are inherited. Because of this, we can look at what causes cystic fibrosis in two different ways — how it occurs on the cellular level and how a child comes to inherit the disease.
As the name implies, a “genetic disease” is caused by something in the body’s genes. Genes are found on chromosomes — structures in the cells of our bodies that usually come in pairs.
One pair of chromosomes includes a gene called the cystic fibrosis transmembrane conductance regulator (CFTR) gene, and mutations or errors in this gene are what cause CF. This gene is large and complex, and more than 1000 different CF-related mutations have been identified.
If a baby is born with CF, it means two CF genes were inherited, one from the mother and one from the father.
A person with only one CF gene is healthy and said to be a "carrier" of the disease. If both parents are carriers of the CF gene (they each have one copy of the defective gene, but do not have the disease themselves), their child has:
This type of inheritance is called "autosomal recessive." Autosomal means that the gene is on one of the pairs of chromosomes that do not determine gender, so that the disease equally affects males and females. Recessive means that two copies of the gene, one inherited from each parent, are necessary to have the condition.
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”