1. Work and Cystic Fibrosis Dr Jane Dewar Respiratory Physician Director Adult CF Service East Midlands

2. Overview:  CF: A refresher!  Tales from the City!  CF and employment Rates and types Rates and types Employment Choice Employment Choice Predicting disability Predicting disability Vocational rehabilitation Vocational rehabilitation Facilitating employment Facilitating employment  Questions!

3. What is Cystic Fibrosis?  Commonest inherited disorder in UK  Affects 9000 people UK  Faulty salt handling  Thick, sticky secretions

4. What is Cystic Fibrosis?  Multi-organ disease  Mainly affects lung, pancreas and liver.  Most patients die of respiratory failure.  Multi-disciplinary team approach.  Average survival 40 yrs

5. CF Genetics

6. CFTR Mutations

7. CF Pathophysiology  Failure to exchange Na and CL  High intracellular Na levels  High extracellular CL levels  Dehydrated cell surface  Thick mucus layer

8. Diagnosis:  Positive sweat test x2- Cl> 60mmol  CFTR Mutational analysis- 2 mutations  Abnormal nasal PD  One or more Phenotypic features

9. Bronchiectasis Pancreatic Insufficiency Failure to thrive, short stature Diabetes-CFRDM Malabsorption DIOS Liver Cirrhosis Gallstones Joint disease, vasculitis Male Infertility CAVD Osteoporosis

10. CF Lung Disease  Cystic Bronchiectasis  Pneumothoraces- 19% in adult males  ABPA- 49% SPT, 27% SERUM PPT.  Aspergilloma

11. CF Lung Disease  Major Haemoptysis- 7%  ? Bronchial Hyperreactivity  Kyphoscoliosis  Environmental Mycobacterium  Respiratory Failure

12. Significant complications  20% liver involvement, 5% clinical liver disease  30% CFRDM  Pancreatic insufficiency 85% from neonates  DIOS 7-15%

13. CF Microbiology

14. Infection Control

15. Medical Treatment  Antibiotics  Anti-inflammatory  Chest Physio  Exercise  Nutrition Spotting decline Attention to other complications Timely transplant referral End-of-life issues

16. Treatment Summary: Typical moderately severe patient will be on:  Creon, ADEKS  Supplemental feeding  Nebulised colistin  Nebulised DNAse  Azithromycin  +/- Insulin  On average 2-3 hrs therapy per day

17. What are the unique challenges facing CF Patients?

19. Pseudomonas auriginosa

25. What are the challenges in caring for patients? Adolescence Concordance Complex complications Psychosocial impact Denial! Transplant referral End-of-life issues Skilled teamworking

26. CF Outcome

27. CF Survival

28. Case 1:  34 yr female, primary teacher  Worked part-time  Very dedicated to job, poor concordance  Poor health, low BMI, CFRD, many Ivs  Perceived poor support in workplace  Difficult career to be absent from…  Retired on health grounds  Now much health better, but not fulfilled…..

29. Case 2:  29 yr female, great FEV1, great BMI.  Never worked  Lives with parents  “My CF is my job. I spend all my energy on that, on keeping well. And look at me, I’m fantastic!! I would just wear myself out working, and why should I when I have so much less life to live than other people?”

30. Case 3:  32 yr female, SALT  Worked full-time until 1 yr pre-transplant  Part-time until 3mnths pre-transplant  Strong work ethic  Huge support at work  Many miles covered each day…..  Planning to return as soon as OK!

31. Case 4:  26yr male, chef.  Shift working  Long hours  Coughing and cooking???  Incompatible with multiple admissions  Succession of lost positions  Low morale and self-esteem

32. Rate of Employment  70% France unit- Laborde-Casterot et al, 2012  55% California unit- Gillen et al, 1995  48% North Carolina unit -Burker et al, 2004  72% Australia unit- Hogg et al, 2007  56% Canada unit- Frangolias et al, 2003

33. Rate of Employment:  Approx. 50% in each of 6 studies currently in work- Saldana et al, 2012  51% Full time, 34% part time- Frangolias et al, 2003  7/10 post-transplant re-employed (Laborde-casterot et al, 2012)

34. Types of employment  Very few adolescents have career guidance (Demars et al 2011)  No difficulty setting vocational goals, regardless of disease severity (Isralsky et al, 1979)  Higher educational attainment (Saldana et al,2012)

35. Types of employment:  Majority in professional employment (Gillen et al, Goldberg et al, Saldana et al)  10-16% Health profession (Saldana et al,2012)

37. Working with CF:  “For individuals with disabilities and chronic health care conditions, work goes beyond the financial benefit, and is thus therapeutic by enhancing identity, increasing social contact, and decreasing isolation.” Johnson et al, 2004

38. Working with CF:  ? Deterioration in health status- Havermans et al, 2009  Associated with better HrQOL scores and lower depression scores-Hogg et al, Burker et al  33% disclosed CF at interview  62% disclosed to colleagues

39. Working with CF:  >50% perceived limitations in their job due to CF  67% felt CF prevented them having a career  37% felt CF caused lower income  70% declared one sick leave in 12 mnths study  54% of cases led to service disruption Laborde-Casterot et al, 2012

40. Employment Choices: Discouraged:  Health care work  Working with children  Exposure to dusts/fumes  Outdoor work  Working with animals  Hairdressing Laborde-Casterot et al, 2012

41. Employment choices: Nottingham Experience:  Blue-collar workers find sick leave etc much more difficult to negotiate than white-collar  Working for large firms advantageous  Health care workers well supported but problems with MRSA etc  Patients often delay/avoid treatment due to work pressures

42. Employment choices: Nottingham Experience:  Shift working bad news  Part-time working a good option  Little support for adapting to disability and loss of vocational identity.  Many patients with severe CF successfully work.  Many patients have ‘high flying’ careers

43. Predicting work disability: FEV 1 :  Moderate/severe disease not a predictor of employment (Frangolias et al, 2003, Hogg et al, 2007)  No significant difference FEV 1 between working/not working groups (Burker et al, 2004)  In particular low FEV 1 poor discrimator (Frangolias et al, 2003)  FEV 1 not good predictor of work status

44. Predicting work disability:  Laborde-Casterot et al, 2012: FEV 1 and educational level best predictors of work status.  Hogg et al, 2007: Age, hospitalisations, disease mastery  Gillen et al, 1995: age, female, being single  Burker et al, 2004: Elevated depression scores, lower educational level

45. To work or not to work…….  Financial benefits versus benefits system  ? Worse health  Juggling treatment  Improved quality of life  Self-esteem and identity  Career adjustment to disability and ill health

46. Vocational rehabilitation  Goldberg et al, 1979: Less realistic in considering limitations and planning careers; Higher commitment, work values, and occupational info.; Strong work ethic.  Laborde-Casterot 2012: Workplace adjustments in 22%, usually decrease in hrs

47. What is needed to improve the work experience?  Better transition  Career advice in adolescence  Role models  Realistic aspirations  Vocational rehabilitation

48. What is needed to improve work experience?  Vocational assessments  Transferable skills analysis  Retraining  More public understanding of CF.  A more sensible and flexible benefits system…

49. Summary:  Survival is now 50 yrs plus  Most patients with CF work  Work poses considerable challenges  Great scope for input from occupational health physicians…!!!

50. Key References:  Burker et al, Paed Pulm 38 (2004) 413- 418  Saldana et al, Work 42 (2012) 185-193  Laborde-Casterot et al, JCF 11 (2012) 137-143