Cystic Fibrosis And Treatment John Plante RRT Melissa Damas RRT-NPS, AE-C Children’s Hospitals and Clinics of MN

What is CF? A Chronic, progressive and life limiting autosomal recessive genetic disease characterized by chronic respiratory disease, pancreatic insufficiency, elevation of sweat electrolytes and male infertility.

CFTR CFTR stands for Cystic Fibrosis Transmembrane Conductance Regulator. The CFTR protein functions as a channel for the movement of chloride ions in and out of cells, which is important for the salt and water balance on epithelial surfaces, such as in the lungs or pancreas

What is CF? Cystic fibrosis occurs when there is a mutation in the CFTR gene.

What is CF? this results in: airway surface liquid depletion 9/15/2018 What is CF? this results in: airway surface liquid depletion Abnormal mucus that becomes more purulent and tenacious Ciliary collapse and decreased mucociliary transport The consequence of this is a vicious circle of phlegm retention, infection, and inflammation that ultimately results in decreased lung function.

What is CF?

Who gets CF? Both parents need to be carriers of the gene mutation 50% chance of being a carrier 25% chance of having disease 25% chance of healthy child

Sweat Chloride Testing Diagnosis Sweat Chloride Testing The analysis of sweat for increased electrolyte concentration is used to confirm the diagnosis of cystic fibrosis. The sweat test occurs in three phases: sweat stimulation, sweat collection and sweat chloride analysis. 0-29 mEq/L Negative 30-59 mEq/L Borderline >60 mEq/L Positive

Manifestations Common Presentations Chronic cough Recurrent pulmonary infiltrates Failure to thrive

Body Systems Affected Gastrointestinal Pancreas: Absence of CFTR limits function of chloride-bicarbonate exchanger to secrete bicarbonate, leading to plugged pancreatic ducts and enzyme retention. Malabsorption of nutrients from food Need to take supplemental enzymes before all meals Meconium illeus at birth

BMI and FEV1

Body Systems Affected Pulmonary Mucus adhere to airway surface, leading to decreased mucus clearing Predisposition to Staph and Pseudomonoas infections. Pseudomonas aeruginosa Burkholderia Cepatia Klebsiella MRSA, MSSA Zanthomonas Aspergillis

Body Systems Chronic Sinusitis Nasal obstruction Rhinorrhea Nasal Polyps-often requiring surgery

Major Treatment Goals Promote airway clearance Control lung infections Provide adequate nutrition

Airway Clearance Modalities Chest Physiotherapy ACBT (Active Cycle of Breathing Technique), AD (Autogenic drainage) HFCWO Intrapulmonary Percussive Ventilation (IPV) AKA: MetaNeb or Percussionaire Positive Expiratory Pressure Blowing bubbles Vibratory PEP-Aerobika, Acapella, Flutter

See Education Handout for diagram of each position Chest Physiotherapy birth to 2 years age 2 and older 9 positions 1-3 minutes per position See Education Handout for diagram of each position

HFCWO The Vest

Mechanism of Action Flow Rate During Oscillation Cycle The Vest® System produces cough-like shear forces

What is needed to move mucous? Airflow behind the mucous!!!! Airway Clearance What is needed to move mucous? Airflow behind the mucous!!!!

Oscillatory PEP Therapy Examples: Flutter Aerobika Acapella

Oscillatory PEP Goals: Vibrate the airways (which loosens mucus from the airway walls) Intermittently increase Endobronchial pressure (which helps maintain the patency of the airways during exhalation so that mucus does not become trapped as it moves up the airways) Accelerate expiratory airflow (which facilitates the upward movement of mucus through the airways so that it can be more easily cleared)

Increasing Airflow and Collateral Ventilation Pathways Air movement between adjacent lung segments Pores of Kohn Interalveolar Canals of Lambert Broncho-alveolar Canals of Martin Interbronchial

Aerobika

Aerobika Slowly inhale beyond a normal breath, but do not fill the lungs completely. Hold breath for 2-3 seconds. Place the mouthpiece in mouth. Use nose clips if necessary. Exhale through Aerobika at a reasonably fast, but not to forceful speed. Exhale beyond a normal breath, but do not empty lungs completely. exhalation should be 3 to 4 seconds while the device vibrates. Select resistance setting so that each exhalation last 3-4 times longer than inhalation. The I:E flow ration should be 1:3 or 1:4 After 5-10 breaths, perform 2-3 forceful cough or huff/cough maneuvers Repeat steps 1 through 7 as prescribed.

Aerobika Always used with a filter. Filter is changed daily. Select resistance setting so that each exhalation last 3-4 times longer than inhalation. The I:E flow ration should be 1:3 or 1:4

9/15/2018 ACAPELLA

ACAPELLA Slowly inhale beyond a normal breath, but do not fill the lungs completely. Hold breath for 2-3 seconds. Place the mouthpiece in mouth. Use nose clips if necessary. Exhale through ACAPELLA at a reasonably fast, but not to forceful speed. Exhale beyond a normal breath, but do not empty lungs completely. Exhalation should be 3 to 4 seconds while the device vibrates. If exhalation cannot be maintained for this length of time, increase resistance by turning the dial clockwise. be After 5-10 breaths, perform 2-3 forceful cough or huff/cough manuevers Repeat steps 1 through 7 as prescrid

Flutter Valve During exhalation, the steel ball rolls and bounces up and down and creates an opening and closing cycle that repeats itself many times throughout exhalation. Generating PEP between 10-25 CmH2O and oscillation frequencies between 6-20 Hz.

HUFF-COUGH Huffing is similar to coughing. Instead of a forceful cough effect, air is let out steadily while whispering the word “huff,” or as if trying to fog up a mirror. This “huff” forces the air and mucus from the small airways in the lung, making the cough more effective.

How do I Huff-Cough? A huff can be done after taking a large, medium, or small breath. Sit with your back straight and your mouth slightly open. Hold a tissue about two to three inches away from your mouth. Take in a breath and hold for one to two seconds. Breathe out steadily, exhaling as much air as you can with your mouth open. Breathe out forcefully enough to move the tissue. Use stomach muscles to help exhale. Do two or three huff breaths followed by a forceful cough. NOTE: If a “wheeze” sound is heard, you are breathing out too quickly. Breathe out more slowly

Nebulizer Treatments Neb treatment order: 1.     Bronchodilator(Albuterol) neb treatment 2.     HTS neb treatment 3.     Pulmozyme neb treatment 4.     Antibiotic neb (TOBI or Cayston)

7% HTS Therapy

Infection Control All patients with CF are to be placed in contact precautions upon admission CF patients are to wear a surgical mask when outside of the hospital room CF patients may participate in activities outside of the hospital room only when no other person with CF is present Avoid direct contact with another CF patient in the hospital unless they live in the same household Patients with CF may not visit another patient with CF Dedicate patient airway devices to single patient use

Infection Control Hand held nebulizers:   Rinse with sterile water and air dry away from a sink after each use and discard within 24 hours All staff should wear gown and gloves for all patient contact