Cystic fibrosis is an inherited disease characterized by the accumulation of thick, sticky mucus that can damage many organs in the body. The most common signs and symptoms of this disorder include progressive damage to the respiratory system and chronic digestive problems.. more.

What is cystic fibrosis disease?

Mucus is a slippery substance that lubricates and protects the mucous lining of the respiratory tract, digestive system, reproductive system, and other organs and tissues. In people with cystic fibrosis, the body produces mucus that is abnormally thick and sticky. Cough becomes chronic, wheezing and inflammation....Over time, the accumulation of mucus and infection leads to permanent lung damage, including the formation of scar tissue and cysts in the lungs.. Most people with cystic fibrosis also have digestive problems.. Some babies with meconium ileus develop a thick intestinal obstruction after birth.. Sticky mucus in the pancreas... delivers and impairs its ability to produce insulin and digestive enzymes..

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Symptoms of cystic fibrosis

CF usually affects the lungs and causes respiratory symptoms, such as:

• whistle
• shortness of breath
• Persistent cough, which may produce blood or mucus
• Other respiratory problems

Also, in people with CF, the mucus that obstructs lung function creates favorable living conditions for pathogens... As a result, a person's risk of developing lung infections such as bronchitis and pneumonia increases. CF symptoms can vary from person to person depending on which organ is affected..

Some other possible symptoms and complications are:

• Frequent sinus infections
• Digestive problems such as: abdominal pain, constipation, diarrhea
• greasy and smelly feces
• Nasal polyps, which are small, fleshy lumps inside the nose
• salty and sweaty skin
• Night sweat
• Fever
• Jaundice
• Joint and muscle pain
• Low body weight
• Restricted growth in children
• late puberty
• Pancreatic obstruction, which can lead to malnutrition and growth restriction, is also associated with an increased risk of diabetes and osteoporosis.

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The cause of cystic fibrosis

The CFTR gene contains the codes to make a protein that controls the flow of salt and water across cell membranes.... In a person with CF, the gene does not work normally, leading to a decrease in the level of salt and water outside the cell.... The result is abnormally thick and sticky mucus.. Biologics must carry the incorrect gene.... According to the National Organization for Rare Disorders, if two carriers have children, there are:

• There is a 25% chance of the child getting CF in each pregnancy
• 50% probability in each pregnancy that the child is a carrier but does not have it
• 25% chance that the child is not a carrier and does not have CF
• More than 10 million trusted individuals in the United States may be CF carriers, and many do not know it.

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Risk factors for cystic fibrosis disease

• Genetic factors: People are more likely to develop CF if one or both of their biological parents are carriers of the disease.
• Race and ethnicity: CF usually affects people with certain genes from different parts of the world.

Diagnosis of cystic fibrosis

Some symptoms of CF may appear shortly after birth, but in milder cases symptoms may not appear until adulthood.

• Screening involves taking a small blood sample from the heel area and sending it to a laboratory for analysis. People can be screened to see if they carry the genetic mutation for CF....The pediatrician takes a sample of blood, saliva, or cheek cells for DNA testing....People may get this screening before or during pregnancy....Prenatal screening involves taking a sample of the genetic fluid from the fetus....The standard CF genetic test checks for 23 common gene mutations...However, more than 1,700 mutations in There is a CFTR gene.

In order to communicate easily and quickly, you can communicate with a pediatrician through the possibility of online consultation.

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Treatment of cystic fibrosis

Treatments can help manage symptoms and improve quality of life... The best combination of approaches depends on the individual's symptoms... Here are some treatment options:

Cleaning the respiratory tract

To ease breathing and minimize the risk of infection, it is important to loosen and clear mucus from the lungs... Airway clearing techniques can help.

Some techniques for cleaning the airways include:

• Percussion: Percussion on the chest can help loosen the mucus.
• Vibration: This involves exhaling while the therapist uses their hands to make light, repetitive movements on the chest. These vibrations help loosen the mucus.
• Breathing techniques: some breathing exercises can help loosen and clear mucus.

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Drug therapy

Some medications can help reduce the symptoms of CF and otherwise manage the disease.

Some of the most effective ones are:

• Bronchodilators: These drugs help relax the muscles around the lungs, giving the airways more room to expand.. Some also help to clear the mucus from the lungs.
• Hypertonic salt: sterilized salt water can help clear mucus and improve lung function in people over 6 years old.
• Ibuprofen: High doses may help slow the decline in lung function.
• Antibiotics: People with CF may take antibiotics orally or IV to treat bacterial lung infections...Pediatric pulmonologists do not usually recommend inhaled antibiotics to treat exacerbations of CF due to bacterial infections...If you have any questions about this, you can consult with a pediatric pulmonologist online at GCORP LLC Get medical advice.

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Nutrition therapy for digestive symptoms

CF can affect digestive function and nutrient absorption, so getting professional nutritional guidance is key. A nutritionist may recommend:

• Eat a high-fat diet: Eating a high-fat diet and taking fat-soluble vitamin supplements may help fight nutrient malabsorption, according to 2021 research.
• Increase your calorie intake: This can help prevent unwanted weight loss.. Studies show that adult women may need 2,500 to 3,000 calories per day, and adult men may need 3,000 to 3,700 calories per day.
• Increased salt intake: People with CF may need to eat more salt to combat sweating because they exercise or live in hot climates.

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Prevention of cystic fibrosis

Based on research, researchers have concluded that there is no surefire way to prevent CF.. If people are planning to have children and are CF carriers, they may want to discuss this with a genetic counselor and consider their options.

Why do children get cystic fibrosis?

Cystic fibrosis was considered a fatal disease in childhood... With improved treatments and better ways to manage the disease, many people with cystic fibrosis are now living well into adulthood... Adults with cystic fibrosis experience health problems that affect the respiratory, digestive, and reproductive systems. Men with cystic fibrosis are unable to have children unless they undergo fertility treatment. Women with cystic fibrosis may experience complications during pregnancy.

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Summary

CF is a genetic disorder associated with mutations in the CFTR gene. This causes the abnormal proteins to become thick and sticky. The disease mainly affects the lungs, although it can also affect the digestive system and other parts of the body.