Cystic Fibrosis
Published on February 24th, 2021. Information will be updated.
Cystic fibrosis (CF) is a chronic disease that produces thick mucus that can clog the lungs, pancreas, and other organs. Although the thick mucus clogs up other organs, the primary organs the mucus clogs are the lungs and pancreas which inhibits the function of the respiratory and digestive systems respectively. When the lungs get clogged with the mucus, then it would be very difficult to breathe resulting in infections, inflammation, lung damage and respiratory failure. When the mucus clogs the pancreas, then it cannot release digestive enzymes that break down food and absorb essential nutrients. Furthermore, the thick mucus can affect the liver by blocking the bile duct resulting in liver disease.
Causes
This thick mucus comes from the parents, therefore the cause of CF is genetics. When parents have CF, they would each inherit one defective gene (making it two genes) to their offspring. This defective gene produces an undeveloped protein which creates the thick mucus in the body. Later on, this mucus allows germs to multiply in the body causing other diseases and/or infections to occur.
Risk Factors
Due to the leading cause being inheritance, more than 30,000 children and adults have CF in the United States, and 70,000 individuals have CF worldwide. Although not mentioned in the article, male individuals are more likely to have CF.
Signs and Symptoms
There are multiple symptoms for cystic fibrosis that range from being mild to severe. The symptoms also differ from person to person. However, individuals that were diagnosed shared some symptoms that were the same. One of the most common symptoms is having a very salty-tasting skin and that’s because of the thick mucus in the body which contains sodium and other solutes. Another very common symptom is coughing persistently, but not a dry cough, a cough that contains phlegm. Individuals would have lung infections, pneumonia, or bronchitis as the thick mucus primarily affects the lungs. Additionally, because of the thick mucus shortness of breath (SOB) occurs. Other common symptoms include lack of weight gain, difficulty of bowel movements, nasal polyps, chronics sinus infections, enlargement of fingertips and/or toes, rectal prolapse, and male infertility. Depending on whether the patient is female or male, males tend to have severe symptoms than females.
Diagnosis
Having the symptoms in mind, physicians would diagnose a patient with cystic fibrosis either by a sweat chloride test, carrier test, or newborn-screening. Nowadays, newborns have been screened to identify the mucus in the body. The majority of the parents who were affected with CF have offspring also affected with CF by the age of two. The sweat chloride and carrier tests are done at adulthood or late adolescents if they weren’t diagnosed during infancy.
Treatment
Despite the symptoms the individuals with CF have to face, they are living healthy lives by taking a regular treatment routine to manage their symptoms. Patients would add nutrition in their diet (a fulfilling diet) and make a fitness plan. An alternative to these lifestyle changes is to take medications to clear up the thick mucus from the airways and pancreas to promote breathing and enzyme functions in the digestive system respectively. Additionally, there are antibiotics to treat cystic fibrosis lung infections which combine other therapies that prevent the cystic fibrosis transmembrane conductance regulator (CFTR) protein to produce, and eventually die out.
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Resources
Diagnosed with cystic fibrosis. (n.d.). Retrieved March 20, 2021, from https://www.cff.org/What-is-CF/Diagnosed-With-Cystic-Fibrosis/