Medical Treatment of Cystic Fibrosis

Cystic Fibrosis treatment aims at helping patients to maintain their health so they can live a long, quality life. Each treatment plan will is individualized and tailored to the needs of every Cystic Fibrosis patient. The main structure of treatment encompasses airway clearance techniques, nutritional therapies, oral\inhaled\nebulized medications, and a fitness plan.

Airway clearance Techniques

Airway clearance techniques (ATC’s) helps to loosen the viscous, sticky mucus so it can be cleared from the patient’s lungs by coughing or deep breathing. The process of clearing the airways helps to decrease the risk of developing a lung infection and improves overall lung function.

ACT’s can be coupled with other treatments such as inhaled bronchodilators, mucus thinning medications, and antibiotics. Bronchodilators help to widen the airways by relaxing the lining in the airway walls; they should be inhaled before starting ACT’s. Mucus thinners help thin and mobilize the mucus out of the airways so it is easier for the patient to expel the mucus by coughing; they should be taken by nebulizer during ACT”s. Antibiotics help to terminate any infectious pathogens; they should be inhaled after ACT’s are finished because the lungs will be the most clear of mucus at that point allowing the antibiotics to travel deeper into the lungs.

Airway Clearance Techniques include:

Coughing

Reflex that helps clear mucus due to high-speed airflow. Sometimes even after long periods of coughing, mucus will not be loosened, this will cause shortness of breath.  

Huffing
Technique performed by taking a breath in a forcefully exhaling afterwards. You progressively start at softer huffing and work up to a more deep and forceful huff. Huffing is not as forceful as coughing, but can work better for longer periods of time without causing shortness of breath or fatigue.

Active Cycle of Breathing (ACBT)
This cycle of breathing asks the patient to take normal, gentle breathing in the lower while relaxing the upper chest and shoulders. Then patient then begins deep breathing exercises- holding the breath for 3 seconds to allow more air into the lungs and loosen the mucus. This may also be done with chest clapping or vibrating, following by more normal breaths. The cycle finishes with a force expiration technique where huffs vary in length of duration.

Autogenic Drainage (Self Drainage) This drainage uses various airflow levels to move mucus. It uses very high airflows in different parts of the lung. The patient inhales to different levels and then adjusts how they breath out to maximize airflow and expel mucus. Breathing out may best be done by huffing in a certain pattern tailored to the patient’s ability to breath.

High Frequency Chest-Wall Oscillation (The Vest)
A specialized vest is attached to a machine that vibrates the vest at high frequency. A small amount of pressure is applied to the chest through the vibration of the chest. The best vibrates and moves mucus into bigger airways so the patient can expel them by coughing. The patient can turn the machine whenever needed so they may huff or cough.

Nutritional therapies

Good nutrition is an important part of maintaining health with Cystic Fibrosis. Maintaining a balanced, high-calorie diet will help supply your body with nutrients it needs to stay healthy and fight off infection. 

Oral\inhaled\nebulized medications

These medications go directly into to source of inflammation and mucus build-up in the lungs. This makes then highly effective. Two mucus thinning medications are Dornase Alfa (Pulmozyme) and Hypertonic Saline solution.

Dornase Alfta

A mucus thinning medication that helps a patient in making it easier to expel mucus by coughing or huffing. Getting this mucus out of the lungs helps to reduce the risk of lung infections or slow down damage from chronic lung infections. It’s mechanism of action is “cutting” into the mucus like scissors. It cuts up the DNA strands outside of the cell that makes Cystic Fibrosis mucus thick and sticky.

Adverse effects are usually mild and are temporary. They include changes in or loss of voice, discomfort in the throat, red watery eyes, rash, dizziness, fever or runny nose.

Hypertonic Saline Solution

Solution that is composed of water and high amounts of sodium; this makes the water sterile and prevents germs from getting into it. Because patients with Cystic Fibrosis have very dry airways, the hypertonic saline solution mist acts as a mucus thinner by helping clear the thick mucus from the lungs and reducing the risk of lung infections.

Adverse effects include increasing coughing, sore throat and chest tightness (saline can sometimes irritate the airways.  

Fitness plans

Maintaining regular physical activity provide several benefits for patients with Cystic Fibrosis. These benefits include improved lung function, increased bone strength, more energy, management of diabetes and heart disease and better overall health. Just adding 21 minutes of physical activity (walking, running, lifting, biking) can help improve your health. Participating in physical activity can also be planning in a pattern during the weak- an example would be Monday, Wednesday, Friday are running days. Tuesday and Thursday are lifting and core exercising days. During physical activity, the patient is allowed to perform in as much as they feel comfortable with.

http://www.dailymail.co.uk/health/article-3085695/New-treatment-brings-hope-cystic-fibrosis.html

https://www.cff.org/Living-with-CF/Treatments-and-Therapies/

https://libbyasbestos.org/education/maintenance/airway.cfm

 http://kidshealth.org/teen/food_fitness/nutrition/cf_diet.html