Treating Cystic Fibrosis - a DAILY task

There is NO cure for Cystic Fibrosis, only ways to minimize the symptoms.

Treating those with Cystic Fibrosis is best done by a multidisciplinary team of specialists. The team consists of a combination of, or all of the following:

• Pulmonary Physician
• Nurse Clinician
• Nutritionist
• Pharmocologist
• Social Worker
• Respiratory Therapist
• Gastroenterologist
• Genetic Counselors (for those wishing to have children)
• Physical Therapist

The Foundation for cystic fibrosis has developed guidelines by which all accredited cf centers must follow. One recommendation is that each patient been seen by their cf team at least once every 90 days, and more if needed. The main purpose is to monitor the patients overall lung health by spirometry tests - a decline can signal a flare-up of infection

The goals of multidisciplinary treatment are maintenance of adequate nutritional status, prevention or aggressive treatment of pulmonary and other complications, encouragement of physical activity, and provision of adequate psychosocial support. With appropriate support, most patients can make an age-appropriate adjustment at home and school. Despite myriad problems, the occupational and marital successes of patients are impressive.

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The two major components in treating those with cystic fibrosis include treating the respiratory system and treating the digestive system.

Managing Respiratory - Airway Clearance --->Open, Thin, Clear

Daily breathing treatments are necessary in attempt to keep the cycle of lung damage from progressing too quickly.

Step 1 - The first step in the breathing treatment routine is the Nebulized Medicines - liquid solutions which are turned into a mist that can be more readily absorbed into the airways. This step can take 10 - 45 minutes and include one or all of the following:

• Bronchodilators - Medications such as albuterol which help open and keep open, the airways in the lungs which allow clearing mucus easier a task.
• Mucus Thinning Drugs - DNA, the basic material in your white blood cells, is released when these cells attack bacteria in your airways, making the mucus in the airways thicker.
• Antibiotics - Antibiotics are crucial to treating CF lung infections The benefits of nebulized antibiotics are that they go directly to the site of a lung infection. They can minimize the number of flare-ups requiring hospitalizations.

Step 2 - The second step in the breathing treatment routine is Chest Percussion Therapy (CPT). CPT is performed for a minimal of 30 minutes each treatment.

• Bronchial Airway Clearance - Since the medications only aren't powerful enough to complete the job, the person with cf needs a way to physically remove thick mucus from their lungs. Daily CPT, also known as percussion and postural drainage, helps to loosen lung secretions and stimulates coughing. In times past, the lungs were "percussed" by manual clapping with cupped hands. Today the patient can perform CPT without assistance with the investion of The Vest.

Standard reccomended therapy requires these two steps performed twice daily, although it's done more frequently when the person has an active lung infection, or in progressed lungs. Regular exercise also helps to loosen and move secretions.

Additionally, some patients benefit from a maintenance dose of anti-inflammatories such as ibuprofen, which help to reduce inflammation or swelling of the body tissues. People with CF have chronic inflammation in their lungs, and it's now known that this is one cause of lung damage.

Management of Digestion

The digestive problems in Cystic Fibrosis are typically less serious and more easily managed than those in the lungs.

To enhance digestion, supplemental pancreatic enzymes are taken before every meal and snack, and extra enzymes to help digest high-fat foods. Those with severe digestive impairment may require supplemental feedings either with a feeding tube or by vein. Patients who fail to take these enzymes with each meal can cause life threatening intestinal blockages.

People with CF must eat a well-balanced, high-calorie, high-protein diet. Their reduced absorption of nutrients often means that as children, they must consume up to 150% of the daily recommended allowances to meet their growth needs. Multivitamins and supplements of the fat-soluble vitamins (A, D, E, and K) are also important.

As you can see, CF is relentless. Management can slow, without guarantees, the progression of lung damage, but it requires dedicated and aggressive efforts, both by the patient, their family, and their health care team.