Cystic Fibrosis

Thanks to earlier diagnosis and better treatment, today’s life expectancy for people with CF is late thirties and beyond. And INTEGRIS Health will be with you every step of the way.

Living longer – and better – than ever before.

A Remarkable Prognosis

Cystic fibrosis (CF) is a genetically inherited, life-threatening disease that affects many organs, and there is no known cure. It's always life-changing to learn your child has a disease, but there is good news with cystic fibrosis.

Thanks to earlier diagnosis and better treatment, in the past few decades remarkable improvements have been made in the prognosis for cystic fibrosis. Life expectancy used to be in the early teens or younger. Today, it's late thirties and beyond. And INTEGRIS Health will be with you every step of the way, with the best physicians and specialists, the latest technology and the emotional support you need.

Get Tested

If someone in your family has CF, you might be carrying the CF gene without being affected by the disease – or being aware you're carrying it. Even if you're not affected by it, you could pass CF on to a child. So it's important to do a simple blood sample or cheek test to see if you're a carrier.

Understanding Cystic Fibrosis

The symptoms of CF differ for each person. Infants born with CF usually show symptoms by age two. Some children, though, may not show symptoms until later in life. The following signs are suspicious of CF, and infants having these signs may be tested for CF:

  • Diarrhea that does not go away
  • Foul-smelling stools
  • Greasy stools
  • Frequent episodes of wheezing
  • Frequent episodes of pneumonia or other lung infections
  • Persistent cough
  • Skin tastes like salt
  • Poor growth despite a good appetite

Additional CF Symptoms & Problems

  • Abnormal heart rhythms
  • Low blood pressure
  • Shock
  • Sinusitis
  • Nasal polyps
  • Coughing up blood
  • Abdominal pain
  • Excess gas in the intestines
  • Rectal prolapse
  • Liver disease
  • Diabetes
  • Gallstones
  • Thick mucus in the lungs and intestines, causing:
    • Malnutrition
    • Poor growth
    • Frequent respiratory infections
    • Breathing difficulties
    • Chronic lung disease
  • Clubbing of fingers and toes. A condition marked by extremely thickened fingertips and toes due to decreased oxygen in the blood.
  • Pneumothorax, collapse of the lung often due to intense coughing
  • Cor pulmonale, enlargement of the right side of the heart due to increased pressure in the lungs.
  • Pancreatitis. Inflammation of the pancreas that causes severe abdominal pain.
  • Congenital bilateral absence of the vas deferens (CBAVD) in males.

Most cases of cystic fibrosis are now identified with newborn screening. In addition to a complete medical history and physical examination, diagnostic procedures for cystic fibrosis include a sweat test to measure the amount of sodium chloride (salt) present. Higher than normal amounts of sodium and chloride suggest cystic fibrosis. Other diagnostic procedures include:

  • Chemical Tests
  • Chest X-Rays
  • Lung Function Tests
  • Sputum Cultures
  • Stool Evaluations

Currently, there is no cure for CF. A cure would call for gene therapy at an early age and this has not been developed yet, although research is being done in this direction. Goals of treatment are to ease severity of your symptoms and slow the progress of the disease. Specific treatment for cystic fibrosis will be determined by your doctor based on your age, overall health, and medical history, extent of the disease, expectations for the course of the disease, your tolerance for specific medications, procedures, or therapies and your opinion or preference.

Management of problems that cause lung obstruction, which may involve:

  • Physical therapy
  • Exercise to loosen mucus, stimulate coughing, and improve overall physical condition
  • Medications to reduce mucus and help breathing
  • Antibiotics to treat infections
  • Anti-inflammatory medications

Management of digestive problems, which may involve:

  • Appropriate diet
  • Pancreatic enzymes to aid digestion
  • Vitamin supplements
  • Treatments for intestinal obstructions

Newer therapies include lung transplantation for patients with end-stage lung disease. The type of transplant done is usually a heart-lung transplant, or a double lung transplant. Not everyone is a candidate for a lung transplant. Discuss this with your doctor.

Our pulmonary rehabilitation programs at INTEGRIS help you by creating individualized plans and education, so you can do more things you enjoy. Support services include:

  • Stress management, relaxation exercises and emotional support
  • Medication management
  • Exercises for physical conditioning programs
  • Assistance with obtaining respiratory equipment and portable oxygen
  • Lung medication
  • Infection control
  • Oxygen and equipment

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