CYSTIC FIBROSIS, RESPIRATORY ASPECTS

1. RESPIRATORY ASPECT OF
CYSTIC FIBROSIS
ULVAN OZAD

2. 65 ROSES

CYSTIC FIBROSIS

MUCOVISCIDOSIS

1:2500 BABIES

AUTOSOMAL RECESSIVE GENETIC DISORDER

CFTR GENE MUTATION (CYSTIC FIBROSIS TRANSMEMBRANE REGULATOR)

ABNORMAL CHLORIDE AND SODIUM TRANSPORT IN EPITHELIUM-THICK MUCUS

3. RESPIRATORY SYMPTOMS

MUCUS BUILD UP-CLOGGING

DECREASED MUCOCILIARY
CLEARENCE

INFLAMMATION

COUGH

PHLEGM

EXERCISE INTOLERANCE

CLUBBING

HEART FAILURE

NASAL POLYPS

SINUSITIS
1,2
RECURRENT CHEST INFECTIONS
PSEUDOMONAS AERUGINOSA
STAPHLOCOCCUS AUREUS

4. DIAGNOSIS

ANTENATAL TESTING: CHORIONIC VILLOUS SAMPLING

NEWBORN TESTING: HEEL PRICK TEST

SWEAT TESTING
COMMON RESPIRATORY INVESTIGATIONS:

SPIROMETRY

CHEST X-RAY

5. MANAGEMENT AIM
 NEARLY
NORMAL LUNG FUNCTION
 PREVENT
INFECTION
AGE

6. RESPIRATORY MANAGEMENT 2, 3, 4, 5,11

BRONCHODILATORS- DILATE AIRWAYS

CORTICOSTEROIDS- REDUCE INFLAMMATION, LONG TERM SIDE EFFECTS ON GROWTH

NSAIDS- FEV1>60%, SLOW DOWN LOSS OF LUNG FUNCTION

ANTIBIOTICS- CONTROL PERSISTENT INFECTIONS

MUCOLYTICS / PULMOZYME (DNASE/DORNASE ALPHA)- BREAK DOWN MUCUS

VACCINATION AND FLU JAB

PHYSIOTHERAPY

EXERCISE

OXYGEN-ADVANCED LUNG DISEASE

LUNG TRANSPLANT

7. PHYSIOTHERAPY6

ACTIVE CYCLE OF BREATHING TECHNIQUES

POSTURAL DRAINAGE

CHEST PHYSICAL THERAPY
DEVICES: MECHANICAL PERCUSSOR, THERAPY VEST/MASK, FLUTTER, RC CORNET, ACAPELLA

8. MEDICATIONS- EBM 8, 9, 10, 12, 13, 15
HYPERTONIC SALINE (2006)

INCREASE AIRWAY SURFACE HYDRATION

ELKINS ET AL DEMONSTRATED IMPROVEMENT

PULMONARY THERAPIES COMMITTEE- LONG TERM USE AFTER AGE OF 6
INHALED MANNITOL (2012)

IMPROVEMENT IN LUNG FUNCTION
IVACAFTOR/KALAYDECO (2012)

CFTR POTENTIATOR

RAMSEY ET AL-LUNG FUNCTION IMPROVEMENT AND EXACERBATION DECREASE

9. MEDICATIONS6, 14
ANTIBIOTICS

ORAL-MILD INFECTIONS

INHALED-PSEUDOMONAS (CHRONIC COLONISATION)

INTRAVENOUS-SEVERE INFECTIONS
TOBRAMYCIN INHALATION POWDER (2013)
PSEUDOMONAS TREATMENT, IMPROVEMENT IN FEV1

10. EVIDENCE 8, 9, 10, 12, 13, 15
INHALED ANTIBIOTICS
ANTISTAPHYLOCOCCAL ANTIBIOTICS
OTHER ANTIBIOTICS
MACROLIDES
DORNASE A.
INHALED STEROIDS
ORAL STEROIDS
NSAIDS
LEUKOTRIENE MODIFIERS
CROMOLYN
INHALED ANTICHOLINERGICS
CHRONIC N-ACETYLCYSTEINE

11. INHALED MEDICATION ORDER

BRONCHODILATOR

HYPERTONIC SALINE

DORNASE ALFA

AIRWAY CLEARENCE THERAPY

AEROLISED ANTIBIOTICS

12. EXACERBATIONS
7
INCREASE:

AIRWAY CLEARENCE

BRONCHODILATOR

PHYSIOTHERAPY

PULMOZYME

ANTIBIOTICS

13. CYSTIC FIBROSis

14. REFERENCES

1: Cystic Fibrosis, Disease Library, Online Resource: http://www.lpch.org/diseaseHealthInfo/HealthLibrary/respire/cfrespir.html, Last Accessed: 21/01/2014 18:54.

2: Cystic Fibrosis, NHS UK, Online Resource: http://www.nhs.uk/Conditions/cystic-fibrosis/Pages/Treatment.aspx , Last Accessed: 21/01/2014 18:22.

3: Pai VB, Nahata MC (2001) Efficacy and safety of aerosolized tobramycin in cystic fibrosis. Pediatr. Pulmonol. 32 (4): 314–27.

4: Westerman EM, Le Brun PP, Touw DJ, Frijlink HW, Heijerman HG (2004) Effect of nebulized colistin sulphate and colistin sulphomethate on lung function in patients with cystic fibrosis: a pilot study. J. Cyst. Fibros. 3 (1): 23–8.

5: McCoy KS, Quittner AL, Oermann CM, Gibson RL, Retsch-Bogart GZ, Montgomery AB (2008) Inhaled aztreonam lysine for chronic airway Pseudomonas aeruginosa in cystic fibrosis. Am J Respir Crit Care Med. 1;178(9):921-8.

6:Cystic Fibrosis, National Health Library, Online resource: http://www.nhlbi.nih.gov/health/health-topics/topics/cf/treatment.html Last Accessed: 22/01/2014 19:56.

7: Cystic Fibrosis, Medscape, Online Resource:http://emedicine.medscape.com/article/1001602-treatment, Last Accessed:22/01/2014 20:15.

8: Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC (2006) Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med.354(3):241-50.

9: Elkins MR, Robinson M, Rose BR, et al. (2006) A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med. 354(3):229-40.

10: Flume PA, O'Sullivan BP, Robinson KA, et al. (2007) Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 176(10):957-69.

11:Cheng K, Ashby D, Smyth RL (2011) Oral steroids for long-term use in cystic fibrosis. Cochrane Database Syst Rev. CD000407.

12: Yu H, Burton B, Huang CJ, Worley J, Cao D, Johnson JP Jr, et al. (2012) Ivacaftor potentiation of multiple CFTR channels with gating mutations. J Cyst Fibros.

13: Aitken ML, Bellon G, De Boeck K, Flume PA, Fox HG, Geller DE, et al. (2012) Long-term inhaled dry powder mannitol in cystic fibrosis: an international randomized study. Am J Respir Crit Care Med. 85(6):645-52.

14: FDA (2013) TOBI Podhaler treat a type of bacterial lung infection in cystic fibrosis patients. FDA News Release.

15: Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC (2006) Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med. 354(3):241-50.

Rose Image: Seattle Childrens,Online Resource: http://www.seattlechildrens.org/kids-health/page.aspx?kid=22901, Last Accessed: 21/01/2014 18:22.

CF Image: CF Charity, Online Resource: http://www.ukskydive.com/charity/cysticfibrosistrustcharity.htm, Last Accessed: 21/01/2014 19:24.

Lung Image: Living Lungs, Online Resource: http://johnhendrix.blogspot.co.uk/2011/06/living-lungs.html, Last Accessed: 22/01/2014 22:34.

Superman Image: CF Awaremess, Online Resource, http://kalmek182.deviantart.com/art/Cystic-Fibrosis-awarness-58783997, Last Accessed: 22/01/2014 19:22.

15. THANK YOU