Work and Cystic FibrosisWork and Cystic Fibrosis

Dr Jane DewarDr Jane Dewar

Respiratory PhysicianRespiratory Physician

Director Adult CF Service Director Adult CF Service

East MidlandsEast Midlands

Overview:Overview:

CF: A refresher!CF: A refresher! Tales from the City!Tales from the City! CF and employmentCF and employment

Rates and typesRates and types Employment ChoiceEmployment Choice Predicting disabilityPredicting disability Vocational rehabilitationVocational rehabilitation Facilitating employmentFacilitating employment

Questions!Questions!

What is Cystic Fibrosis?What is Cystic Fibrosis?

Commonest inherited disorder in UKCommonest inherited disorder in UK Affects 9000 people UKAffects 9000 people UK Faulty salt handlingFaulty salt handling Thick, sticky secretionsThick, sticky secretions

What is Cystic Fibrosis?What is Cystic Fibrosis?

Multi-organ diseaseMulti-organ disease Mainly affects lung, pancreas and Mainly affects lung, pancreas and

liver.liver. Most patients die of respiratory failure.Most patients die of respiratory failure. Multi-disciplinary team approach.Multi-disciplinary team approach. Average survival 40 yrsAverage survival 40 yrs

CF GeneticsCF Genetics

CFTR MutationsCFTR Mutations

CF PathophysiologyCF Pathophysiology

Failure to exchange Failure to exchange Na and CLNa and CL

High intracellular Na High intracellular Na levelslevels

High extracellular CL High extracellular CL levelslevels

Dehydrated cell Dehydrated cell surfacesurface

Thick mucus layerThick mucus layer

Diagnosis:Diagnosis:

Positive sweat test Positive sweat test x2- Cl> 60mmolx2- Cl> 60mmol

CFTR Mutational CFTR Mutational analysis- 2 mutationsanalysis- 2 mutations

Abnormal nasal PDAbnormal nasal PD One or more One or more

Phenotypic featuresPhenotypic features

•Bronchiectasis

•Pancreatic Insufficiency

•Failure to thrive, short stature

•Diabetes-CFRDM

•Malabsorption

•DIOS

•Liver Cirrhosis

•Gallstones

•Joint disease, vasculitis

•Male Infertility CAVD

•Osteoporosis

CF Lung DiseaseCF Lung Disease

Cystic BronchiectasisCystic Bronchiectasis Pneumothoraces- Pneumothoraces-

19% in adult males19% in adult males ABPA- 49% SPT, ABPA- 49% SPT,

27% SERUM PPT.27% SERUM PPT. AspergillomaAspergilloma

CF Lung DiseaseCF Lung Disease

Major Haemoptysis-Major Haemoptysis-7%7%

? Bronchial ? Bronchial HyperreactivityHyperreactivity

KyphoscoliosisKyphoscoliosis Environmental Environmental

MycobacteriumMycobacterium Respiratory FailureRespiratory Failure

Significant complicationsSignificant complications

20% liver 20% liver involvement, 5% involvement, 5% clinical liver diseaseclinical liver disease

30% CFRDM30% CFRDM Pancreatic Pancreatic

insufficiency 85% insufficiency 85% from neonatesfrom neonates

DIOS 7-15% DIOS 7-15%

CF MicrobiologyCF Microbiology

Infection ControlInfection Control

Medical TreatmentMedical Treatment

AntibioticsAntibiotics Anti-inflammatoryAnti-inflammatory Chest PhysioChest Physio ExerciseExercise NutritionNutrition

• Spotting decline• Attention to

other complications

• Timely transplant referral

• End-of-life issues

Treatment Summary:Treatment Summary:

Typical moderately severe patient will be on:Typical moderately severe patient will be on: Creon, ADEKSCreon, ADEKS Supplemental feedingSupplemental feeding Nebulised colistinNebulised colistin Nebulised DNAseNebulised DNAse AzithromycinAzithromycin +/- Insulin+/- Insulin On average 2-3 hrs therapy per dayOn average 2-3 hrs therapy per day

What are the unique challenges What are the unique challenges facing CF Patients?facing CF Patients?

Pseudomonas auriginosaPseudomonas auriginosa

What are the challenges in What are the challenges in caring for patients?caring for patients?

• Adolescence• Concordance• Complex complications• Psychosocial impact• Denial!• Transplant referral• End-of-life issues• Skilled teamworking

CF OutcomeCF Outcome

CF SurvivalCF Survival

Case 1:Case 1:

34 yr female, primary teacher34 yr female, primary teacher Worked part-timeWorked part-time Very dedicated to job, poor concordanceVery dedicated to job, poor concordance Poor health, low BMI, CFRD, many IvsPoor health, low BMI, CFRD, many Ivs Perceived poor support in workplacePerceived poor support in workplace Difficult career to be absent from…Difficult career to be absent from… Retired on health groundsRetired on health grounds Now much health better, but not fulfilled…..Now much health better, but not fulfilled…..

Case 2:Case 2:

29 yr female, great FEV1, great BMI.29 yr female, great FEV1, great BMI. Never workedNever worked Lives with parentsLives with parents ““My CF is my job. I spend all my energy on that, My CF is my job. I spend all my energy on that,

on keeping well. And look at me, I’m fantastic!! I on keeping well. And look at me, I’m fantastic!! I would just wear myself out working, and why would just wear myself out working, and why should I when I have so much less life to live should I when I have so much less life to live than other people?”than other people?”

Case 3:Case 3:

32 yr female, SALT32 yr female, SALT Worked full-time until 1 yr pre-transplantWorked full-time until 1 yr pre-transplant Part-time until 3mnths pre-transplantPart-time until 3mnths pre-transplant Strong work ethicStrong work ethic Huge support at workHuge support at work Many miles covered each day…..Many miles covered each day….. Planning to return as soon as OK!Planning to return as soon as OK!

Case 4:Case 4:

26yr male, chef.26yr male, chef. Shift workingShift working Long hoursLong hours Coughing and cooking???Coughing and cooking??? Incompatible with multiple admissionsIncompatible with multiple admissions Succession of lost positionsSuccession of lost positions Low morale and self-esteemLow morale and self-esteem

Rate of EmploymentRate of Employment

70% France unit-70% France unit-Laborde-Casterot et al, 2012Laborde-Casterot et al, 2012

55% California unit-55% California unit-Gillen et al, 1995Gillen et al, 1995

48% North Carolina unit48% North Carolina unit-Burker et al, 2004-Burker et al, 2004

72% Australia unit-72% Australia unit-Hogg et al, 2007Hogg et al, 2007

56% Canada unit-56% Canada unit-Frangolias et al, 2003Frangolias et al, 2003

Rate of Employment:Rate of Employment:

Approx. 50% in each of 6 studies Approx. 50% in each of 6 studies currently in work-currently in work-Saldana et al, 2012Saldana et al, 2012

51% Full time, 34% part time-51% Full time, 34% part time- Frangolias Frangolias et al, 2003et al, 2003

7/10 post-transplant re-employed 7/10 post-transplant re-employed (Laborde-casterot et al, 2012)(Laborde-casterot et al, 2012)

Types of employmentTypes of employment

Very few adolescents Very few adolescents have career guidance have career guidance (Demars et al 2011)(Demars et al 2011)

No difficulty setting No difficulty setting vocational goals, vocational goals, regardless of disease regardless of disease severity severity (Isralsky et al, (Isralsky et al, 1979)1979)

Higher educational Higher educational attainment attainment (Saldana et (Saldana et al,2012)al,2012)

Types of employment:Types of employment:

Majority in professional Majority in professional employment employment (Gillen et al, (Gillen et al, Goldberg et al, Saldana Goldberg et al, Saldana et al)et al)

10-16% Health profession 10-16% Health profession (Saldana et al,2012)(Saldana et al,2012)

Working with CF:Working with CF:

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

Working with CF:Working with CF:

? Deterioration in health status-? Deterioration in health status-Havermans et al, 2009Havermans et al, 2009

Associated with better HrQOL Associated with better HrQOL scores and lower depression scores and lower depression scores-scores-Hogg et al, Burker et alHogg et al, Burker et al

33% disclosed CF at interview33% disclosed CF at interview 62% disclosed to colleagues62% disclosed to colleagues

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Working with CF:Working with CF:

>50% perceived limitations in >50% perceived limitations in their job due to CFtheir job due to CF

67% felt CF prevented them 67% felt CF prevented them having a careerhaving a career

37% felt CF caused lower 37% felt CF caused lower incomeincome

70% declared one sick leave in 70% declared one sick leave in 12 mnths study12 mnths study

54% of cases led to service 54% of cases led to service disruptiondisruption

Laborde-Casterot et al, 2012Laborde-Casterot et al, 2012

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Employment Choices:Employment Choices:

Discouraged:Discouraged:Health care workHealth care workWorking with childrenWorking with childrenExposure to dusts/fumesExposure to dusts/fumesOutdoor workOutdoor workWorking with animalsWorking with animalsHairdressingHairdressing

Laborde-Casterot et al, Laborde-Casterot et al, 20122012

Employment choices:Employment choices:

Nottingham Experience:Nottingham Experience:Blue-collar workers find sick leave etc Blue-collar workers find sick leave etc much more difficult to negotiate than much more difficult to negotiate than white-collarwhite-collarWorking for large firms advantageousWorking for large firms advantageousHealth care workers well supported Health care workers well supported but problems with MRSA etcbut problems with MRSA etcPatients often delay/avoid treatment Patients often delay/avoid treatment due to work pressuresdue to work pressures

Employment choices:Employment choices:

Nottingham Experience:Nottingham Experience:Shift working bad newsShift working bad newsPart-time working a good Part-time working a good optionoptionLittle support for adapting to Little support for adapting to disability and loss of vocational disability and loss of vocational identity.identity.Many patients with severe CF Many patients with severe CF successfully work.successfully work.Many patients have ‘high flying’ Many patients have ‘high flying’ careerscareers

Predicting work disability:Predicting work disability:

FEVFEV11::

Moderate/severe disease not a Moderate/severe disease not a predictor of employment predictor of employment ((Frangolias et Frangolias et al, 2003, Hogg et al, 2007)al, 2003, Hogg et al, 2007)No significant difference FEVNo significant difference FEV11 between between

working/not working groups working/not working groups (Burker et (Burker et al, 2004)al, 2004)In particular low FEVIn particular low FEV11 poor discrimator poor discrimator

((Frangolias et al, 2003)Frangolias et al, 2003)FEVFEV11 not good predictor of work status not good predictor of work status

Predicting work disability:Predicting work disability:

Laborde-Casterot et al, 2012: Laborde-Casterot et al, 2012: FEVFEV1 1 and educational level best and educational level best

predictors of work status.predictors of work status. Hogg et al, 2007: Hogg et al, 2007: Age, Age,

hospitalisations, disease masteryhospitalisations, disease mastery Gillen et al, 1995: Gillen et al, 1995: age, female, age, female,

being singlebeing single Burker et al, 2004: Burker et al, 2004: Elevated Elevated

depression scores, lower depression scores, lower educational leveleducational level

To work or not to work…….To work or not to work…….

Financial benefits Financial benefits versus benefits systemversus benefits system

? Worse health? Worse health Juggling treatmentJuggling treatment Improved quality of lifeImproved quality of life Self-esteem and identitySelf-esteem and identity Career adjustment to Career adjustment to

disability and ill healthdisability and ill health

Vocational rehabilitationVocational rehabilitation

Goldberg et al, 1979: Goldberg et al, 1979: Less realistic in Less realistic in considering limitations considering limitations and planning careers; and planning careers; Higher commitment, work Higher commitment, work values, and occupational values, and occupational info.; Strong work ethic.info.; Strong work ethic.

Laborde-Casterot 2012: Laborde-Casterot 2012: Workplace adjustments in Workplace adjustments in 22%, usually decrease in 22%, usually decrease in hrshrs

What is needed to improve the What is needed to improve the work experience?work experience?

Better transitionBetter transition Career advice in Career advice in

adolescenceadolescence Role modelsRole models Realistic Realistic

aspirationsaspirations Vocational Vocational

rehabilitationrehabilitation

What is needed to improve work What is needed to improve work experience?experience?

Vocational assessmentsVocational assessments Transferable skills Transferable skills

analysisanalysis RetrainingRetraining More public More public

understanding of CF.understanding of CF. A more sensible and A more sensible and

flexible benefits system…flexible benefits system…

Summary:Summary:

Survival is now 50 yrs plusSurvival is now 50 yrs plus Most patients with CF workMost patients with CF work Work poses considerable Work poses considerable

challengeschallenges Great scope for input from Great scope for input from

occupational health occupational health physicians…!!!physicians…!!!

Key References:Key References:

Burker et al, Paed Pulm 38 (2004) 413-Burker et al, Paed Pulm 38 (2004) 413-418418

Saldana et al, Work 42 (2012) 185-193Saldana et al, Work 42 (2012) 185-193 Laborde-Casterot et al, JCF 11 (2012) Laborde-Casterot et al, JCF 11 (2012)

137-143137-143