Symptoms+and+Treatments

__Symptoms of Cystic Fibrosis __
There are many possible symptoms patients with cystic fibrosis may experience that can vary greatly from person to person. Most symptoms tend to occur a some time after birth. Symptoms typically categorize into the following body systems: respiratory, digestive, and reproductive.


 * Respiratory System Symptoms **

One of the most common symptoms in cystic fibrosis patients is the thick, sticky mucus buildup in the airways of the lungs. This buildup makes it very difficult for a patient to breath which results in frequent coughing up mucus (sometimes with blood) and discomfort. With such a high amount of mucus in the airways, it makes it very easy for bacteria to grow and cause infection. Infections can further block the airways and cause further complications. Bacterial infections in patients with cystic fibrosis tend to be caused by unusual bacteria that typically do not response to standard antibiotics that would be prescribed by a doctor. This is due to such a unique environment in the airways of a great amount of mucus for these bacteria to grow.

People with cystic fibrosis will usually experience frequent bouts of sinusitis, bronchitis, and pneumonia. Since these illnesses tend to be reoccurring, long term lung damage can result. Cystic fibrosis is a disease that progressively gets worse, resulting with more threatening illnesses such as pneumothorax and bronchiectasis. Both of these rare diseases result from damage to the airways in the lungs. Pneumothorax can be treated, but bronchiectasis cannot. These diseases cause so much strain and damage to the respiratory system that they usually end up being fatal. Pneumothorax can result in the collapsing of the lung if left untreated and bronchiectasis results in respiratory failure, both fatal conditions.


 * Digestive System Symptoms **

The mucus resulting from cystic fibrosis can also affect ducts in the pancreas. This results with the blockage of essential enzymes from reaching the intestines to digest food material. With a lack of enzymes, the intestines cannot function normally resulting in the inability to absorb fats and proteins. This can result in chronic or acute diarrhea or greasy stools. Intestinal blockage may also occur, predominantly in newborns. This causes gas buildup and constipation within the intestines, which leads to stomach pain.

A hallmark symptom usually seen in children is poor weight gain and growth. This is due to the lack of fats and proteins being absorbed by the intestines, resulting with not enough nutrients gained from their food for their body.

As cystic fibrosis progressively gets worse, other problems may occur. These include; pancreatitis, rectal prolapse, liver disease, diabetes, or gallstones.


 * Reproductive System Symptoms **

Woman with cystic fibrosis will usually have a hard time getting pregnant due to the excess mucus buildup blocking the cervix. Most men who have cystic fibrosis have congenital bilateral absence of the vas deferens. This is a conditions in which the tubes that carry sperm from the testes to the penis are blocked by mucus and do not develop correctly.


 * Other Symptoms **

Due to the excess secretion of chloride ions from the CFTR chloride channel, a common symptom seen in cystic fibrosis patients is very salty sweat. This causes a great amount of salt leaving the body resulting in dehydration, fatigue, increased heart rate, decreased blood pressure, and heat stroke.

Clubbing is also a symptom seen in older patients with cystic fibrosis. This is the rounding and widening of the finger tips and toes, which is a result of the lungs not moving enough oxygen into the bloodstream.

__Statistics of Cystic Fibrosis __
<span style="font-family: Times New Roman,Times,serif;">Cystic Fibrosis is most common among the white population of United States. The disease is seen to occur in 1 in 2,500 to 3,500 white newborns. In other ethnicity groups, cystic fibrosis is not as common with the disease being present in 1 in 17,000 African Americans and 1 in 31,000 Asian Americans. About 30,000 people in the United States and 70,000 worldwide are living with cystic fibrosis in which almost half are the age of 18 or older. Approximately, 1,000 new cases of cystic fibrosis are disposed each year.

__<span style="font-family: 'Times New Roman',Times,serif;">Treatment and Therapy Options __
<span style="font-family: Times New Roman,Times,serif;">There are different treatment and therapy options available for patients with cystic fibrosis that may help their overall health to allow them to live a longer life. Treatment plans tend to be unique, tailored to the needs of each person. There are a few different options patients may chose to consider:
 * **<span style="font-family: Times New Roman,Times,serif;">Airway Clearance **<span style="font-family: Times New Roman,Times,serif;">-used to loosen thick, sticky mucus buildup from the airways by coughing. This technique works to decrease lung infections and to help improve lung functions.
 * **<span style="font-family: Times New Roman,Times,serif;">Inhaled Medication **<span style="font-family: 'Times New Roman',Times,serif;">-medication can be given in a form of an aerosol solution that can reach deep into the airways. Mucolytics are common inhaled treatments used to help keep mucus in the lungs thin to allow patients to more easily cough it up. Mucoltyics come in two types: dornas alfa, commonly known as Pulmozyme®, and hypertonic saline.
 * **<span style="font-family: Times New Roman,Times,serif;">Nutrition **<span style="font-family: Times New Roman,Times,serif;">-like any other disease or illness, nutrition plays a huge role in overall health. It is recommended for patients to eat a high-calorie diet along with vitamins, minerals, and pancreatic enzymes.
 * **<span style="font-family: Times New Roman,Times,serif;">Fitness **<span style="font-family: Times New Roman,Times,serif;">-regulator fitness activity holds many benefits for patients with cystic fibrosis, such as; improved lung function and strengthening bones.
 * **<span style="font-family: Times New Roman,Times,serif;">CFTR Modulators **<span style="font-family: 'Times New Roman',Times,serif;">-these modulator therapies work to correct the function of the defective CFTR protein. Ivacaftor is an approved CFTR modulator drug that is given to patients with specific rare mutations in the CFTR gene. A combination drug of ivacaftor and lumacaftor, Orkambi®, has been approved for patients 12 and older with the more common mutation, F508del. Even more effective modulators are in development for other mutations in the CFTR gene.
 * **<span style="font-family: Times New Roman,Times,serif;">Lung Transplantation **<span style="font-family: Times New Roman,Times,serif;">-patients have the option of receiving a lung transplantation to help improve and lengthen a person's life. This option usually isn't always the easiest due to extensive evaluation processes and a long time waiting for a donor, ("Treatments and Therapies").

<span style="font-family: Times New Roman,Times,serif;"> Since cystic fibrosis patients experience many reoccurring respiratory bacterial infections, three main types of antibiotics can be prescribed:
 * <span style="font-family: 'Times New Roman',Times,serif; line-height: 1.5;">**Oral** **<span style="font-family: Times New Roman,Times,serif;">Antibiotics **<span style="font-family: 'Times New Roman',Times,serif;">-Piperacillin​/​Tazobactam, Azithromycin (Zithromax), and Ciprofloxacin (Cipro).
 * **<span style="font-family: Times New Roman,Times,serif;">Inhaled Antibiotics **<span style="font-family: 'Times New Roman',Times,serif;">-TOBI®, Bethkis®, Cayston®, and TOBI® Podhaler™.
 * **<span style="font-family: Times New Roman,Times,serif;">Intravenous (IV) Antibiotics **<span style="font-family: 'Times New Roman',Times,serif;">-Gentamicin, Azithromycin (Zithromax), Meropenem, Amikacin, Aztreonam (Azactam), Ciprofloxacin (Cipro), Tobramycin, Ceftazidime (Fortaz), (MayoClinic Staff).

<span style="font-family: 'Times New Roman',Times,serif;">Many of the bacterial infections patients experience are somewhat unique to people with cystic fibrosis due to the thick mucus environment of the airways.

<span style="font-family: 'Times New Roman',Times,serif;">Next: **<span style="font-family: 'Times New Roman',Times,serif;"> CFTR Gene **


 * <span style="font-family: 'Times New Roman',Times,serif;">Comparative Genomics **
 * <span style="font-family: 'Times New Roman',Times,serif;">Results **
 * <span style="font-family: 'Times New Roman',Times,serif;">Resources **
 * <span style="font-family: 'Times New Roman',Times,serif;">Background **

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