Cystic Fibrosis

cystic-fibrosis

Cystic Fibrosis (CF) is an inherited, chronic disorder that affects the lungs and digestive system, as well as other organs in the body. It causes mucus throughout the body to be thick and sticky, which clogs up glands and ducts throughout the body, creating a variety of issues that can become serious if left untreated. But what causes CF? And what are the best treatment options available?

Causes of Cystic Fibrosis

Cystic fibrosis is an inherited disorder caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It only occurs when a child receives copies of the mutated gene from both parents. Someone with only one copy of the mutated gene will not have cystic fibrosis, but they can be a carrier of the disorder.

Symptoms of Cystic Fibrosis

While cystic fibrosis can produce complications across the entire body in various ways, the most common symptoms include:

  • Chronic sinus infections
  • Bronchiectasis (damaged airways)
  • Nasal polyps
  • Increased respiratory symptoms, such as cough and shortness of breath
  • Respiratory failure
  • Fertility problems in men and women
  • Diabetes
  • Liver disease
  • Growth deficiencies due to nutritional complications
  • Intestinal obstruction
  • Osteoporosis
  • Dehydration and electrolyte imbalance

Diagnosis of Cystic Fibrosis

Diagnosis of cystic fibrosis is generally done in a series of tests and can be performed on newborns, older children, and adults. Diagnosing cystic fibrosis often includes sweat tests, genetic screenings, and other means of sampling chemicals in the body. Once the diagnosis is confirmed, the healthcare provider will determine a treatment plan based on how the condition is affecting the particular patient.

Treatment Options for Cystic Fibrosis

While there is no cure for cystic fibrosis, many of the symptoms are treatable. Today, patients who suffer from CF are living longer, more fulfilling lives than at any other point in history.

Medications

Medication options may vary on the severity of your condition and family history. The most common medications for CF include:

  • Antibiotics to treat recurrent infections, especially in the respiratory system
  • Anti-inflammatory and steroidal medications to open up and relax the airways
  • Mucus thinners to combat the thick, sticky mucus produced by CF
  • Genetic medications that modulate the CFTR protein

Therapies

In addition to medications, there are also several therapy options to improve bodily function of someone with CF. These include:

  • Airway clearance techniques or chest physical therapy (CPT) to reduce mucus obstruction
  • Pulmonary rehabilitation to increase lung performance over time
  • Oxygen therapy and ventilation to relieve the effort of breathing and to prevent the occurence of high blood pressure in the lungs

Surgical Treatments

In some cases more invasive measures might be required to keep CF in check. These methods commonly include:

  • Lung transplant
  • Liver transplant
  • Intestinal surgery
  • Nasal surgery

If You’re Interested in Genetic Screening or Treatment Plans for Cystic Fibrosis, Nurse Practitioners of Florida Can Help

At Nurse Practitioners of Florida, we have a dedicated team of certified nurse practitioners who have an unwavering commitment to providing you with care and compassion. When you call any of our locations, you will be greeted by a live person who’s ready to offer acute medical care as well as preventive measures — including pneumonia vaccine. And, above everything else, you will be treated like family.

If you need assistance, call us at (727) 290-6116 or fill out our online contact form.

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