Cystic Fibrosis


What is cystic fibrosis?

Cystic fibrosis is an inherited disease that causes a buildup of very thick mucus which can cause damage to the lungs, digestive system and other organs in the body. Due to a genetic mutation cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. In a person without cystic fibrosis, mucus is typically thin and slippery. In patients with cystic fibrosis a genetic mutation causes the production of abnormal mucus that is thick and sticky. This abnormal mucus doesn’t lubricate tissues as it would normally, but rather it plugs up or blocks tubes, ducts and passageways in the body. This abnormal mucus usually affects the passageways of the lungs and pancreas, thus leading to respiratory infections and problems with digestion. Furthermore, mucus buildup can lead to permanent lung damage, malnutrition and weight loss.

Cystic fibrosis was previously considered a fatal childhood disease. With improvements in screening and treatment, the prognosis of cystic fibrosis has become a lot brighter.

At CompreCare, we provide a variety of specialty therapies for Cystic Fibrosis. Please feel free to contact us at 1-888-644-8326 or email us at info@comprecarehealth.com to learn how we can help you.

What are the causes of cystic fibrosis?

Cystic fibrosis is caused by a mutation in a gene known as the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This gene controls certain particles of chloride, an important element in our bodies. The flow of chloride particles helps control movement of water in your tissues and the production of normal, thin and slippery mucus. As a result of the CFTR gene mutation, chloride doesn’t flow freely and allow for normal mucus production. Therefore, this mutation ultimately leads to unusually thick and sticky mucus that clogs various passageways in the body. Environmental factors can also worsen this condition as well.

What are the signs and symptoms of cystic fibrosis?

Presently, most children are diagnosed with cystic fibrosis before symptoms occur as part of routine screening at birth. However, it is still important to be aware of signs and symptoms for this condition. Symptoms can worsen and improve with time and some children may not even experience symptoms until later in life. Most symptoms are due to respiratory and digestive complications that occur. A key sign may also be a particularly salty taste to the skin.

Respiratory sign and symptoms:
  • Breathlessness and wheezing
  • Persistent cough with thick mucus production
  • Stuffy nose
  • Recurring lung infections such as pneumonia
  • Limited ability to exercise
Digestive signs and symptoms:
  • Poor growth and reduced weight gain
  • Severe constipation
  • Intestinal blockages
  • Foul-smelling, greasy stools
How is cystic fibrosis diagnosed?

Screening of newborns for cystic fibrosis is now routinely performed in the United States. Therefore, cystic fibrosis can be diagnosed within the first month of life. Parents that have family members with cystic fibrosis can also be genetically tested in order to predict the risk of having a child with cystic fibrosis. Newborn screening is not diagnostic and cannot confirm a cystic fibrosis diagnosis. However, mutations in the CFTR gene may indicate following-up testing is needed. There are several diagnostic tests for cystic fibrosis but the standard test usually used is called a “sweat chloride test”. This test indicates a high salt level in sweat to be a sign of the disease.

How is cystic fibrosis treated?

Cystic fibrosis has many treatments that may help to reduce thick mucus and prevent serious complications of the disease. There are treatments for lung problems and digestive or nutritional problems that are routinely used in order to prevent further damage.

Lung treatments:
  • Inhaled medicines that help reduce airway inflammation such as inhaled corticosteroids.
  • Inhaled bronchodilator medications that help open up the airways.
  • Medications that help thin the mucus such as highly concentrated salt solutions (hypertonic saline)
  • Antibiotics that can prevent and treat lung and sinus infections such as inhaled tobramycin.
  • Flu and pneumococcal vaccines may be needed yearly to prevent these infections.
  • Oxygen therapy may be required if lung disease gets worse.
Digestive/ nutritional treatments:
  • A special diet that is high in protein and calories may be required.
  • Enzymes that mimic those secreted from the pancreas (pancreatic enzymes) may be prescribed and may be taken with every meal.
  • Vitamin supplements may also bee needed including vitamins A, D, E and K.
Other treatments:
  • Newer medicines such as Ivacaftor (Kalydeco) and Ivacaftor/Lumacaftor (Orkambi) may be prescribed for particular forms of cystic fibrosis. These medications work to improve function of the defective gene (CFTR) that causes cystic fibrosis. As a result, these medications help to reduce production of thick, abnormal mucus.
References:

https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700
https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/
https://ghr.nlm.nih.gov/condition/cystic-fibrosis
https://www.guideline.gov/summaries/summary/50221/clinical-practice-guidelines-from-the-cystic-fibrosis-foundation-for-preschoolers-with-cystic-fibrosis

How to place an order?

CompreCare can accept electronic prescriptions as well as original prescriptions through the mail. Faxed prescriptions must come from your physician’s office only. CompreCare will verify all new controlled substance prescriptions with your physician’s office for validity.

We look forward to servicing all of your prescription medication needs in a fast and friendly way! If you have any questions, please do not hesitate to call us at 1-888-644-8326.

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