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

2. 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.

3. 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

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

5. 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.

6. What is CF?

7. 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

8. 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

9. Manifestations
Common Presentations Chronic cough Recurrent pulmonary infiltrates Failure to thrive

10. 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

11. BMI and FEV1

12. 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

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

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

15. 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

16. 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

17. HFCWO
The Vest

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

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

20. Oscillatory PEP Therapy
Examples:
Flutter
Aerobika
Acapella

21. 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)

22. 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

23. Aerobika

24. 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.

25. 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

26. 9/15/2018
ACAPELLA

27. 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

28. 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 CmH2O and oscillation frequencies between 6-20 Hz.

29. 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.

30. 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

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

32. 7% HTS Therapy

33. 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

34. 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