How To Know If You Have Cystic Fibrosis – The lungs, digestive system and other organs of the body are severely damaged by the genetic condition known as cystic fibrosis (CF).
Cystic fibrosis affects the cells that make mucus, sweat and digestive secretions. These generated fluids are often thin and slippery. The secretions are thick and sticky in CF patients, but due to a defective gene.
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Especially in the pancreas and lungs, secretions clog the tubes, ducts and ducts instead of lubricating them.
What Is Cystic Fibrosis (cf)
Cystic fibrosis (CF) is an inherited disease that you get from your parents at birth. It affects the way your body produces mucus, a fluid that helps your organs and systems function.
When you have cystic fibrosis, your mucus is thick and glue-like, rather than the normal thin, smooth consistency. As a result, the channels and pipes of your body become clogged.
It can also lead to serious lung damage such as fibrosis and cysts (fluid-filled sacs) (scarring). CF got its name that way.
Depending on how severe the condition is, there are different assumptions and symptoms of cystic fibrosis. Symptoms can improve or worsen over time, indeed in the same person.
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Some people may not start showing symptoms until they are teenagers or adults. Older cases are usually diagnosed with mild disease and are more likely to experience atypical symptoms similar to intermittent pneumonia, infertility, and pancreatitis attacks.
This gene regulates the movement of fluid and salt in and out of your cells. Sticky mucus builds up in your body if the CFTR gene is not working properly.
You must inherit the gene with the mutation from both parents to develop CF. Ninety percent of affected individuals have the F508del mutation in at least one copy.
If you have only one copy, you will not show any signs of the disease, but you will still be a carrier. Therefore, there is an opportunity to pass it on to your child.
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Early diagnosis means early treatment and better health later in life. Every state in the United States 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 this in their blood.
Medicines – Your doctor may give you medicines to open your airways, thin out mucus, prevent infections, and help your body absorb nutrients from food. These include:
Antibiotics – These can prevent or treat lung infections and help the lungs work better. You can get them as tablets, in an inhaler, or by injection.
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Mucus Thinners – These will help flush mucus from your airways. You will get them from an inhaler.
CFTR Modulators – These help CFTR work properly. They can make your lungs work better and help you gain weight.
Combination therapy – The new drug elexacaftor/ivacaftor/tezacaftor (Trikafta) combines three CFTR modulators to target the CFTR protein and make it work effectively.
Chest therapy or percussion – This involves tapping or slapping the chest or back to clear mucus from the lungs. Someone else will do it for you.
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Oscillating device – You breathe into a unique machine that vibrates or oscillates your airways. As a result, the mucus dissolves, which makes it easier to cough. An oscillating chest vest is an alternative.
Physical therapy for cystic fibrosis – This involves breathing techniques that push air through thick layers of mucus and against the chest wall. They make it easier to clear blocked airways and make it easier to cough up debris. General exercises consist of:
Autogenous Drainage – To achieve this, exhale forcefully or huff. This makes it easier for mucus to move from your smaller airways to your central airways and makes it easier to clear.
Active Breathing Cycle – This controls your breathing and relaxes your upper chest and shoulders, which can help clear mucus and prevent airway blockage. You take a deep breath, hold it, and then exhale for different amounts of time.
About Cystic Fibrosis
Your lungs are not the only part of your body affected by cystic fibrosis. Cystic fibrosis also affects the following organs:
Pancreas – Your pancreatic ducts are blocked by the thick mucus caused by CF. This prevents digestive enzymes, which are proteins that help break down food, from reaching your intestines. Therefore, your body struggles to get the nutrients it needs. As a result, diabetes can develop over time.
Liver – Your liver becomes inflamed if the ducts used to drain bile become blocked. This can lead to cirrhosis, a severe scarring condition.
Small intestine – The lining of the small intestine can deteriorate as highly acidic foods from your stomach can be a challenge to digest.
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Colon – Thick fluid in your stomach can make your stool large and harder to pass. This can lead to blockage. In some cases, your intestines may also begin to fold like an accordion, a condition called intussusception.
Bladder – A chronic or prolonged cough weakens the muscles of the bladder. Almost 65% of women with CF have stress incontinence. This means that you leak a little urine when you cough, sneeze, laugh or lift something. Although it is more common in women, men can have it too.
Kidneys – Some people with CF get kidney stones. These small, hard mineral balls can cause nausea, vomiting and pain. If you don’t treat it, you can get a kidney infection.
Reproductive organs – Both male and female fertility is affected by excess mucus. Most CF sufferers have problems with the vasa deferentia, which transports sperm. Because of their extremely thick cervical mucus, women with CF may have trouble getting sperm to fertilize eggs.
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Other parts of the body – In addition, osteoporosis and muscle weakness are side effects of CF. Low blood pressure, fatigue, rapid heart rate, and a general feeling of weakness can also result from it because it disrupts the balance of minerals in your blood.
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Cystic fibrosis (CF) is a genetic disorder that causes a buildup of thick, sticky mucus in organs including the lungs and pancreas. In a healthy person, the mucus that lines the body’s organs and cavities, such as the lungs and nose, is slippery and watery. In people with cystic fibrosis, thick mucus clogs the airways, making breathing difficult. Obstruction of the ducts in the pancreas causes problems in the digestion of food. Babies and children with CF may not be able to absorb enough nutrients from food. Other organs that CF affects are the liver, sinuses, intestines, and genitals. CF is a chronic (long-lasting) and progressive (gets worse over time) condition.
CF is a disease that is passed on by genes called CFTR genes. People with CF inherit two faulty genes, one from each parent. The parents do not have to have CF. Many families have no family history of CF. In this case, the person with the defective gene is called a carrier. About 1 in 31 people in the United States are carriers who do not have symptoms of CF.
Among white children in the United States, the incidence rate of CF is one in every 3,500 newborns. CF affects about one in 17,000 black newborns and one in 31,000 Asian newborns.
In most cases, CF is diagnosed in childhood. Doctors diagnose CF through a careful evaluation and the use of various tests. These include:
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• Newborn screening — A healthcare provider takes a few drops of blood from a heel prick, usually while the newborn is in the hospital. The provider places the drop of blood on a special paper known as Guthrie paper. Laboratory tests can diagnose conditions including CF. All states in the United States require testing of newborns at birth as well as several weeks later.
• Sweat test — This is the most conclusive test for CF. Sweat tests measure the amount of chloride in the body’s sweat, which is elevated in people with CF. People of all ages can take a sweat test. In the test, a health care provider applies a chemical called pilocaprin to the skin. He or she then applies a small amount of electrical stimulation to encourage the sweat glands to produce sweat. The provider then collects the sweat in a plastic spiral or on a piece of filter paper or gauze. The test is painless and does not use a needle.
• Genetic tests — These are done using a blood sample that is tested for the faulty genes that cause CF.
• Chest X-ray — A chest X-ray is used to
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