More than 30,000 people in the U.S. live with cystic fibrosis about 1,000 new cases are been diagnosed by the Doctors each year.
According to a 62 years ago record many children with Cystic Fibrosis died even before reaching elementary school age. However, advances in treatment mean that people with CF often live into their 30s, 40s, and beyond.
Although people with the condition require daily care, they can still lead a relatively normal life and work or attend school.
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What is cystic fibrosis?
Cystic fibrosis is a serious genetic condition that causes severe damage to the respiratory and digestive systems, which means you get if from your parents at birth. Cystic fibrosis (CF) affects the way your body makes mucus, this is a substance that helps your organs and systems work, Mucus should be thin and slippery like olive oil as they help your system to lubricate. but when you have Cystic Fibrosis, it becomes thick and glue-like. This blocks tubes and ducts throughout your body and it can also result in fatal complications such as liver disease and diabetes.
Organs that cystic fibrosis affect.
Cystic fibrosis affects the cells that produce sweat, mucus, and digestive enzymes. The most commonly affected organs include the:
Cystic Fibrosis Complications
The lungs aren’t the only part of your body CF damages. Cystic fibrosis also affects the following organs:
Pancreas: The thick mucus caused by CF blocks ducts in your pancreas. This stops proteins that break down your food, called digestive enzymes, from reaching your intestine. As a result, your body has a hard time getting the nutrients it needs. Over time, this can also lead to diabetes.
Liver: If the tubes that remove bile get clogged, your liver gets inflamed. This can lead to severe scarring called cirrhosis.
Small intestine: Because it can be hard to break down high-acid foods that come from your stomach, the lining of the small intestine can wear away.
Large intestine: The thick fluid in your stomach can make your poop large and harder to pass. This can lead to blockages. In some cases, your intestine may also start to fold in on itself like an accordion, a condition called intussusception.
Bladder: Chronic or long-lasting coughing weakens your bladder muscles. Almost 65% of women with CF have stress incontinence. This means that you leak a little pee when you cough, sneeze, laugh, or lift something. Though it’s more common in women, men can have it, too.
Kidneys: Some people with CF get kidney stones. These small, hard globs of minerals can cause nausea, vomiting, and pain. If you don’t treat them, you could get a kidney infection.
Reproductive organs: Excess mucus affects fertility in men and women. Most men with CF have problems with the tubes that move sperm, called the vasa deferentia. Women with CF have very thick cervical mucus, which can make it harder for a sperm to fertilize an egg.
Other parts of the body. CF can also lead to muscle weakness and thinning bones, or osteoporosis. Because it upsets the balance of minerals in your blood, it can also cause low blood pressure, fatigue, a fast heart rate, and a general feeling of weakness.
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Cystic Fibrosis Symptoms
The symptoms of cystic fibrosis can vary depending on the person and the severity of the condition. But the most common symptoms of CF are:
- Trouble with bowel movements –
- Wheezing or trouble breathing
- Frequent lung infections
- Infertility, especially in men
- Trouble growing or gaining weight
- a persistent cough that produces thick mucus or phlegm
- shortness of breath, especially when exercising
- recurrent lung infections
- a stuffy nose
- stuffy sinuses
- poor weight gain in spite of excessive appetite
- greasy, bulky and foul smell stools
- nasal polyps, or small, fleshy growths found in the nose.
- swollen abdomen
What Causes Cystic Fibrosis?
Cystic fibrosis occurs as a result of a defect, change, or mutation, in a gene called CFTR (cystic fibrosis transmembrane conductance regulator). This gene controls the movement of water and salt in and out of your body’s cells. But For most people CF is an inherited condition. they inherited the defective gene from both of their parents.
If you inherit only one copy, you won’t have the condition or its symptoms, but you will be a carrier of the disease which means you do not have CF . But this also means you risk the chance of passing it on to your children.
- Every time two CF carriers have a baby, there’s a 25% (1 in 4) chance that their baby will be born with CF,
- 50 % (1 in 2) chance the child will be a carrier but will not have CF
- 25 % ( 1 in 4) chance the child will not be a carrier and will not have CF
- According to reports About 10 million Americans are CF carriers and they very much unaware.
Diagnosis of cystic fibrosis.
All newborns in the U.S. are screened for CF by testing a small blood sample or samples. This can indicate that a baby might have a health condition and require further investigation.
Because Early diagnosis means early treatment and better health later in life. Every state in the U.S. tests newborns for cystic fibrosis using one or more of these tests:
Sweat Chloride Test
The sweat chloride test is the most commonly used test for diagnosing cystic fibrosis. It checks for increased levels of salt in the sweat. The test is performed by using a chemical that makes the skin sweat when triggered by a weak electric current. Sweat is collected on a pad or paper and then analyzed. A diagnosis of cystic fibrosis is made if the sweat is saltier than normal.
This test checks the levels of immunoreactive trypsinogen (IRT). People with CF have higher levels of it in their blood.
- A chest X-ray is useful in revealing swelling in the lungs due to blockages in the respiratory passageways.
- DNA test. This method seems to be the most important of them all as it looks for mutations to the CFTR gene.
- Most people who were not tested at birth do not know they are diagnosed with CF until they become adults, so it’s best adviced to carry out test for your kids at birth.
Treatment for cystic fibrosis
Currently There is no cure for Cystic Fibrosis. The Treatment available can only manage the symptoms of the disease, however, and improve quality of life. Symptoms can vary and so also treatment plans will be individualized.
Medications: Your doctor may give you drugs to open your airways, thin mucus, prevent infections, and help your body get nutrients from food to help relieve symptoms and reduce the risk of complications. These include:
Antibiotics: Antibiotics may be prescribed to get rid of a lung infection and to prevent another infection from occurring in the future. They’re usually given as liquids, tablets, or capsules. In more severe cases, injections or infusions of antibiotics can be given intravenously, or through a vein.
Combination therapy: The new medication elexacaftor/ivacaftor/tezacaftor (Trikafta) combines three CFTR modulators to target the CFTR protein and make it work for efficiently.
Nonsteroidal anti-inflammatory drugs (NSAIDs): Drugs such as ibuprofen and indomethacin, may help reduce any pain and fever associated with cystic fibrosis.
Chest Physical therapy: this Method helps loosen the thick mucus in the lungs, making it easier to cough it up. It’s typically performed one to four times per day. As This involves tapping or clapping on your chest or back to clear mucus from your lungs. Someone else does this for you.
Oscillating devices: You breathe into a special device that oscillates, or vibrates, your airways. This loosens mucus and makes it easier to cough up. You can wear an oscillating chest vest instead.
Home Care / Physical therapy for CF: This includes breathing exercises designed to push air between layers of mucus and your chest wall. They make it easier to cough up the gunk and ease blocked airways. Some common exercises include:
Autogenic drainage: To do this, you breathe out hard, or huff. This moves mucus from your smaller airways to the central airways and makes it easier to get out.
Avoid smoke, pollen, and mold whenever possible. These irritants can make symptoms worse.
Get influenza and pneumonia vaccinations regularly.
Active cycle of breathin:. This controls your breath and relaxes the upper chest and shoulders, which can help clear mucus and prevent airway blockages. You breathe in deeply, hold it, and then huff for different lengths of time.
Can Cystic Fibrosis Be Prevented?
To be frank Cystic fibrosis can’t be prevented. But it’s best advised 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.
In conclusion To Cystic Fibrosis
The median predicted age of survival for people with CF is currently in the early 40s. Life expectancy is strongly affected by the severity of the disease, age of diagnosis, and the type of CF gene mutation.
With routine therapies and healthful lifestyles, most people with CF can lead active lives.