Cystic fibrosis

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CYSTIC FIBROSIS
Cystic fibrosis is an inherited life-threatening disorder that damages the

lungs and digestive system.
Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. It causes these fluids to become thick and sticky. They then plug up tubes, ducts and passageways.

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Cystic fibrosis affects the cells that produce sweat, mucus, and digestive

enzymes. Normally, these secreted fluids are thin and smooth like olive oil. They lubricate various organs and tissues, preventing them from getting too dry or infected.
In people with cystic fibrosis, however, a faulty gene causes the fluids to become thick and sticky. Instead of acting as a lubricant, the fluids clog the ducts, tubes, and passageways in the body.

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Symtoms :-
Symptoms vary and can include cough, repeated lung infections, inability

to gain weight and fatty stools.

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Symptoms may appear at infancy, but for other children, symptoms may

not begin until after puberty or even later in life. As time passes, the symptoms associated with the disease may get better or worse.
One of the first signs of cystic fibrosis is a strong salty taste to the skin. Parents of children with cystic fibrosis have mentioned tasting this saltiness when kissing their children.
Other symptoms of cystic fibrosis result from complications that affect: the lungs the pancreas the liver other glandular organs

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Respiratory problems The thick, sticky mucus associated with cystic fibrosis often blocks

the passageways that carry air into and out of the lungs. This can cause the following symptoms- wheezing a persistent cough that produces thick mucus or phlegm shortness of breath, especially when exercising recurrent lung infections a stuffy nose stuffy sinuses

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Digestive problems The abnormal mucus can also plug up the channels that

carry the enzymes produced by the pancreas to the small intestine. Without these digestive enzymes, the intestine can’t absorb the necessary nutrients from food. This can result in - greasy, foul-smelling stools constipation nausea a swollen abdomen loss of appetite poor weight gain in children delayed growth in children

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Cystic Fibrosis Causes :-
Cystic fibrosis is caused by a change, or

mutation, in a gene called CFTR (cystic fibrosis transmembrane conductance regulator). This gene controls the flow of salt and fluids in and out of your cells. If the CFTR gene doesn’t work the way it should, a sticky mucus builds up in your body.
About 10 million Americans are CF carriers. Every time two CF carriers have a baby, there’s a 25% (1 in 4) chance that their baby will be born with CF.

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Cystic fibrosis occurs as a result of a defect in what’s

called the “cystic fibrosis transmembrane conductance regulator” gene, or CFTR gene. This gene controls the movement of water and salt in and out of your body’s cells. A sudden mutation, or change, in the CFTR gene causes your mucus to become thicker and stickier than it’s supposed to be. This abnormal mucus builds up in various organs throughout the body, including the: intestines pancreas liver lungs

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It also increases the amount of salt in your sweat. Many different

defects can affect the CFTR gene. The type of defect is associated with the severity of cystic fibrosis. The damaged gene is passed on to the child from their parents. In order to have cystic fibrosis, a child must inherit one copy of the gene from each parent. If they only inherit one copy of the gene, they won’t develop the disease. However, they’ll be a carrier of the defective gene, which means that they may pass the gene on to their own children.

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Cystic Fibrosis Diagnosis :-
Early diagnosis means early treatment and better health

later in life. Tests newborns for cystic fibrosis using one or more of these three tests: Blood test :- This test checks the levels of immunoreactive trypsinogen (IRT). People with CF have higher levels of it in their blood. DNA test :- This looks for mutations to the CFTR gene. Sweat test :- It measures the salt in your sweat. Higher than normal results suggest CF.

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Approximately 1,000 people are diagnosed with cystic fibrosis every year in

the United States. Although people with the condition require daily care, they can still lead a relatively normal life and work or attend school. Screening tests and treatment methods have improved in recent years, so many people with cystic fibrosis can now live into their 40s and 50s.

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Other diagnostic tests that may be performed include:
1:- Immunoreactive trypsinogen (IRT)

test
2:- Sweat chloride test
3:- Sputum test
4:- Chest X-ray
5:- CT scan 6:- Pulmonary function tests (PFTs)

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Treatment :-
Although there’s no cure for cystic fibrosis, there are various

treatments available that may help relieve symptoms and reduce the risk of complications.
Medication :- Antibiotics may be prescribed to get rid of a lung infection and to prevent another
infection.. In more severe cases, injections or infusions of antibiotics can be given intravenously (through a vein).
Mucus-thinning medications make the mucus thinner and less sticky. They also help you to cough up the mucus so it leaves the lungs. This significantly improves lung function Surgical procedures:-
Bowel surgery :- This is an emergency surgery that involves the removal of a section of the bowel. It may be performed to relieve a blockage in the bowels.

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Feeding tube :- Cystic fibrosis may interfere with digestion and prevent

the absorption of nutrients from food. A feeding tube to supply nutrition can be passed through the nose or surgically inserted directly into the stomach.
Double-lung transplant :- When medical management alone can no longer maintain lung health and physical function, this procedure can improve the length and quality of life for a person with cystic fibrosis.

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Transplant benefits :-
Most transplant recipients report improved strength and energy, and

freedom from symptoms such as coughing and shortness of breath While having a double-lung transplant can’t cure cystic fibrosis, as the defective gene remains in the body, the donor lungs don’t contain the mutated gene
Home care :-
Cystic fibrosis can prevent the intestines from absorbing necessary nutrients from food If you have cystic fibrosis, you might need more calories per day than people who don’t the disease. You may also need to take pancreatic enzyme capsules with every meal.
Drink plenty of fluids, because they can help thin the mucus in the lungs. Exercise regularly to help loosen mucus in the airways. Walking, biking, and swimming are great options. Avoid smoke, pollen, and mold whenever possible. These irritants can make symptoms worse. Get influenza and pneumonia vaccinations regularly.

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The long-term outlook for people with cystic fibrosis :-
The outlook for

people with cystic fibrosis has improved dramatically in recent years, largely due to advances in treatment. Today, many people with the disease live into their 40s and 50s, and even longer in some cases. However, there’s no cure for cystic fibrosis, so lung function will steadily decline over time. The resulting damage to the lungs can cause severe breathing problems and other complications.
How can cystic fibrosis be prevented?
Cystic fibrosis can’t be prevented. However, genetic testing should be performed for couples who have cystic fibrosis or who have relatives with the disease. Genetic testing can determine a child’s risk for cystic fibrosis by testing samples of blood or saliva from each parent. Tests can also be performed on you if you’re pregnant and concerned about your baby’s risk.

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OVERVIEW OF DISEASE

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Questions :-
1:- What is cystic fibrosis ?
2:- What is the symroms

of cystic fibrosis ?
3:- How is cystic Fibrosis diagnosed ?
4:- What is the treatment of cystic fibrosis

Cystic fibrosis

Содержание

CYSTIC FIBROSIS

CYSTIC FIBROSISCystic fibrosis is an inherited life-threatening disorder that damages the

Cystic fibrosis affects the cells that produce sweat, mucus, and digestive

Symtoms :-Symptoms vary and can include cough, repeated lung infections, inability

Symptoms may appear at infancy, but for other children, symptoms may

Respiratory problems The thick, sticky mucus associated with cystic fibrosis often blocks

Digestive problems The abnormal mucus can also plug up the channels that

Cystic Fibrosis Causes :-Cystic fibrosis is caused by a change, or

Cystic fibrosis occurs as a result of a defect in what’s

It also increases the amount of salt in your sweat. Many different

Cystic Fibrosis Diagnosis :-Early diagnosis means early treatment and better health

Approximately 1,000 people are diagnosed with cystic fibrosis every year in

Other diagnostic tests that may be performed include:1:- Immunoreactive trypsinogen (IRT)

Treatment :-Although there’s no cure for cystic fibrosis, there are various