Cystic Fibrosis

Cystic Fibrosis Overview

Cystic Fibrosis is an inherited disease that damages the lungs, as well as other organs such as the digestive system. This disease affects the cells that will produce sweat, mucus, and digestive juices. Most of the time, these fluids are slippery and thin, but in people will cystic fibrosis, these fluids will become sticky and thick. When the fluids are thin and slippery, they act as lubrication. On the other hand, if they are thick and sticky, then they will stop up tubes, passageways, and ducts.

Cystic Fibrosis

Cystic Fibrosis Symptoms

Newborns are screened for cystic fibrosis and can usually be diagnosed within their first month of life. If you do not believe you were tested as a child, then it is important to be checked immediately. It is important to know if you have cystic fibrosis so that you can treat it accordingly. Some symptoms that parents may notice include tasting salt when they kiss their child and various respiratory or digestive problems. Adults may notice they have cystic fibrosis because they may have infertility, pancreatitis, or recurring pneumonia.

Since cystic fibrosis is known to cause fluids to be thick and sticky, many of the symptoms are found in the respiratory system. You may notice symptoms such as wheezing, breathlessness, lung infections, cough with thick mucus, stuffy nose, or the inability to exercise.

Cystic fibrosis also attacks the digestive system and you may notice poor weight gain, intestinal blockage, and constipation. The severity of symptoms depends on the severity of the condition.

Cystic Fibrosis Causes

Cystic fibrosis is a defect in a gene which causes the protein that moves salt in and out of cells to not work properly. When this occurs, you will notice thick and sticky mucus in the digestive tract and in the respiratory system. Children will usually inherit this gene from both of their parents. If they only receive one of the genes from their parents, then they will be a carrier of the gene to their children.

Cystic Fibrosis Risk Factors

Since cystic fibrosis is hereditary, family history is a large factor on if you will also have cystic fibrosis. Cystic fibrosis is found in all cultures, but it is predominantly found in white people from Northern European ancestry. These are the main two risk factors of the disease. You should be able to tell if you possible have it based on your family and your ancestry.

Cystic Fibrosis Complications

There are so many complications found with cystic fibrosis. This disease is the leading cause of airway damage, which means it may be hard for you to move air in and out of the lungs and clear mucus from the airways too. When you cough, you will likely be coughing up blood since the airway walls will be so thin. Since this will likely result in thick mucus forming in the airways, you will also be at a high risk for chronic infections, such as bronchitis or pneumonia. This thick mucus will also aggravate the lining of the nose; you are also at a high risk for growths in the nose, which is also called polyps.  You may also experience chest pain, breathlessness, exacerbations, or overtime, respiratory failure.

The respiratory system is not the only part of the body that will be compromised. You will also notice that you have nutritional deficiencies, diabetes, intestinal obstruction, blocked bile ducts, thinning of bones, dehydration, or distal intestinal obstruction syndrome. Most men and women with cystic fibrosis have many reproductive system complications as well.

Cystic Fibrosis Prevention

As previously stated, newborns are typically tested for cystic fibrosis during their first month of life. In other cases, most parents may catch it as their child is growing up. If you or your partner has relatives that have cystic fibrosis, you may choose to go through with a blood test to see the likelihood of your child also have cystic fibrosis. If you are pregnant, you may decide to go through with a test to see if the baby has this disease.

Cystic Fibrosis Diagnosis

Newborns are routinely checked for cystic fibrosis, as early diagnosis means that they can also begin treatment sooner too. The first test that a newborn may go through includes a blood sample to check for the chemical that is the cause of cystic fibrosis. This test may not be perfectly accurate, as the chemical may be high in premature babies, or babies that have experienced a stressful delivery. Before the baby is born, the parents may decide to go through genetic testing. Once the baby is two weeks old or more, then they can do a sweat test. This occurs when the sweat is collected and if the sample is saltier than average, cystic fibrosis can be diagnosed.

In older children or adults, your physician may recommend testing if you have symptoms of cystic fibrosis. Just like a newborn, adults may go through genetic testing or sweat testing to confirm the diagnosis.

Cystic Fibrosis Treatment

There is no true cure for cystic fibrosis, but your physician will work to make you as comfortable and healthy as possible. The goals for treatment are to provide proper nutrition, prevent intestinal blockage, removing mucus from the lungs, and controlling infections in the lungs too. The best way to treat cystic fibrosis is to closely monitor symptoms and to properly diagnose as soon as possible.

Your physician may also recommend a few different medications. Antibiotics aid in preventing various lung infections. Anti-inflammatory medications may help decrease the swelling of the airways. Medications that aid in the thinning of mucus may be prescribed to help you cough up the mucus from your airways. Bronchodilators may also aid in relaxing the airways.

Pulmonary rehabilitation may help to improve your condition through education, nutritional counseling, breathing techniques, physical exercise, and continued counseling and support through your physician’s office.

There are other non-medication related treatments that may also be recommended for you based on severity. There are some surgical procedures that may help, which include: endoscopy and lavage, a feeding tube, lung transplant, oxygen therapy, or nasal polyp removal.