RESPIRATORY ASPECT OF CYSTIC FIBROSIS

ULVAN OZAD

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

RESPIRATORY SYMPTOMS 1,2

RECURRENT CHEST INFECTIONS

PSEUDOMONAS AERUGINOSA

STAPHLOCOCCUS AUREUS

MUCUS BUILD UP-CLOGGING

DECREASED MUCOCILIARY CLEARENCE

INFLAMMATION

COUGH

PHLEGM

EXERCISE INTOLERANCE

CLUBBING

HEART FAILURE

NASAL POLYPS

SINUSITIS

DIAGNOSIS

ANTENATAL TESTING: CHORIONIC VILLOUS SAMPLING

NEWBORN TESTING: HEEL PRICK TEST

SWEAT TESTING

COMMON RESPIRATORY INVESTIGATIONS:

SPIROMETRY

CHEST X-RAY

MANAGEMENT AIM

NEARLY NORMAL LUNG FUNCTION

PREVENT INFECTION

AGE

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

ACTIVE CYCLE OF BREATHING TECHNIQUES

POSTURAL DRAINAGE

CHEST PHYSICAL THERAPY

DEVICES: MECHANICAL PERCUSSOR, THERAPY VEST/MASK, FLUTTER, RC CORNET, ACAPELLA

PHYSIOTHERAPY6

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

ANTIBIOTICS

ORAL-MILD INFECTIONS

INHALED-PSEUDOMONAS (CHRONIC COLONISATION)

INTRAVENOUS-SEVERE INFECTIONS

TOBRAMYCIN INHALATION POWDER (2013)

PSEUDOMONAS TREATMENT, IMPROVEMENT IN FEV1

MEDICATIONS6, 14

EVIDENCE 8, 9, 10, 12, 13, 15

INHALED ANTIBIOTICS

ANTISTAPHYLOCOCCAL ANTIBIOTICS

OTHER ANTIBIOTICS

MACROLIDES

LEUKOTRIENE MODIFIERS

CROMOLYN

INHALED ANTICHOLINERGICS

CHRONIC N-ACETYLCYSTEINE

DORNASE A.INHALED STEROIDS

ORAL STEROIDSNSAIDS

INHALED MEDICATION ORDER

BRONCHODILATOR

HYPERTONIC SALINE

DORNASE ALFA

AIRWAY CLEARENCE THERAPY

AEROLISED ANTIBIOTICS

EXACERBATIONS7

INCREASE:

AIRWAY CLEARENCE

BRONCHODILATOR

PHYSIOTHERAPY

PULMOZYME

ANTIBIOTICS

CYSTIC FIBROSis

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.

REFERENCES

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