Lorraine Ozerovitch ( MSc, BSc, RGN ) Clinical Nurse Specialist

Lorraine Ozerovitch (MSc, BSc, RGN) Clinical Nurse Specialist

in Respiratory Infection and Immunology

• Background on Bronchiectasis and CVID

• Reported quality of life outcomes in Bronchiectasis and CVID

• Recent nursing research in patients with CVID-Bx compared to Id-Bx

Lorraine Ozerovitch 2012 - INGID

• Unwell in childhood (bronchitis)

• Period of good health

• Age 30 to 40 years: persistent coughs & colds

• Copious volume of purulent tenacious sputum

• Lethargy or decreased exercise tolerance

• Breathlessness/ Chest tightness/ Pleuritic pain


• HRCT with CXR and sinus XR

• Full PFT with reversibility

• Ciliary Studies (exhaled nasal & breath NO testing/ EM)

• Sputum cultures for AFB and M,C&S

• Bloods (FBC, U’s&E’s, LFT’s, CRP, IgG, IgA, IgM, IgE, SpAB, Asp Rast, Asp IgG,)


• Serum protein electrophorectic strip

• Skin prick testing

• Shuttle walking Test/ Borg Breathlessness Scale

• St George’s Respiratory Questionnaire

• Physiotherapy review

• ENT review, bronchoscopy, video-fluoroscopy, detailed immunology workup


• 33pts with confirmed Bx on HRCT

• 25pts completed the “CAT” (Jones et al 2009)

• CAT total scores correlated with worse bronchiectasis on HRCT scans: extent and severity of disease and airway wall thickness


CATCATQuestionsQuestions

Q1Q1CoughCough

Q2Q2PhlegmPhlegm

Q3Q3Chest tightnessChest tightness

Q4Q4BreathlessnessBreathlessness

Q5Q5ActivitiesActivities

Q6Q6ConfidenceConfidence

Q7Q7SleepSleep

Q8Q8EnergyEnergy

TotalTotalScoreScore

MeanMean (SD)(SD)

3.1 3.1 (0.95)(0.95)

3.13.1(0.91)(0.91)

2.22.2(1.30)(1.30)

2.4 2.4 (1.64)(1.64)

1.6 1.6 (1.82)(1.82)

1.4 1.4 (1.8)(1.8)

2.4 2.4 (1.71)(1.71)

2.2 2.2 (1.45)(1.45)

18.4 18.4 (9.72)(9.72)

• Chronic dilatation of peripheral airways, localised or widespread, with loss of ciliated epithelium

• Occurs from destruction of muscular and elastic components of the bronchial walls

• Stationary mucus acts as a breeding environment for bacteria to grow and which is the source of recurrent infections


• Common

– Pseudomonas aeruginosa– Haemophilus influenzae

• Less common

– Staphylococcus aureus– Streptococcus pneumoniae– Moraxella catarrhalis– Stenotrophamonas maltophilia– Klebsiella pneumoniae


• Cole (1986): The Vicious Cycle

• GOAL: Halt the bacterial process which in turn will impact on the inflammatory process

• The clinical course is variable


Microbial Infection(e.g.Haemophilus influenzae, Pseudomonas aeruginosa)

Impaired Lung Defences(e.g. Antibody Deficiency, Primary Ciliary Dyskinesia, Cystic Fibrosis)

Tissue Damage(To epithelial cells and the

structure of the airway wall leading to increased mucus production which

is poorly cleared)

Inflammation(Neutrophilic inflammation causes damage to the tissue through proteolytic enzymes

and oxidative stress)

A VICIOUS CYCLE OF INFECTION AND INFLAMMATION

• UK: 1:1000 hosp beds have a Bx pt (Sita-Lumsden and Wilson 2009)

• US /NZ: 3.7-4.2: 100, 000 higher in the elderly ≥ 75yrs (Weycher et al 2005; Twiss et al 2005)

• 1000 die a year, 3% increase yr on yr (Roberts and Hubbard 2010)

• BTS guideline (2010) may assist clinicians’ awareness in early detection and management


• Modern bronchiectasis is the end result of a number of different pathologies


Innate weakness in the lung’s defenses

(e.g. PCD) or deficiency in the

body’s ability to fight infection (e.g. CVID)

Born with normal host defenses then catches

a severe chest infection (e.g.

tuberculosis) or experience some other insult to the

airway (e.g. smoke inhalation)

Acquire an excessive immune response e.g.

allergic broncho-pulmonary

aspergillosis (ABPA)

Idiopathic – research suggest an upset in

the immune response causing an

exaggerated inflammatory response

Bronchiectasis


CausesCauses N (% of study popN (% of study popnn)) Age (SD)Age (SD) No: Males (% group)No: Males (% group)

Post InfectionPost Infection 52 (32)52 (32) 49 (16)49 (16) 17 (33)17 (33)

IdiopathicIdiopathic 43 (26)43 (26) 51 (14)51 (14) 15 (35)15 (35)

PCDPCD 17 (10)17 (10) 36 (13)36 (13) 5 (29)5 (29)

ABPAABPA 13 (8)13 (8) 54 (13)54 (13) 6 (46)6 (46)

Immune deficiencyImmune deficiency 11 (7)11 (7) 47 (18)47 (18) 1 (9)1 (9)

Ulcerative ColitisUlcerative Colitis 5 (3)5 (3) 48 (20)48 (20) 2 (40)2 (40)

YoungYoung’’s Syndromes Syndrome 5 (3)5 (3) 56 (5)56 (5) 3 (60)3 (60)

Pan BronchiolitisPan Bronchiolitis 4 (2)4 (2) 46 (21)46 (21) 3 (75)3 (75)

Yellow Nail SyndromeYellow Nail Syndrome 4 (2)4 (2) 55 (14)55 (14) 2 (50)2 (50)

Mycobacterium infectionsMycobacterium infections 4 (2)4 (2) 62 (20)62 (20) 0 (0)0 (0)

Rheumatoid ArthritisRheumatoid Arthritis 3 (2)3 (2) 65( 4)65( 4) 1 (33)1 (33)

AspirationAspiration 2( 1)2( 1) 67 (13)67 (13) 1(50)1(50)

Cystic FibrosisCystic Fibrosis 2 (1)2 (1) 41 (13)41 (13) 2 (100)2 (100)

TotalTotal 165165 49 (16)49 (16) 58 (35)58 (35)

ABPA = allergic brochopulmonary aspergillosis; PCD = primary ciliary dyskinesiaABPA = allergic brochopulmonary aspergillosis; PCD = primary ciliary dyskinesia

Shoemark et al (2007)Shoemark et al (2007)

• CVID is a heterogeneous group of conditions characterised by: Antibody deficiency, Autoimmune disorders and Granulomatous disease

• Commonest cause of primary antibody deficiency (PID)

• ESID criteria of CVID is “marked decrease in IgG and a reduction of a least one isotypes; IgM or IgA”

• Average time between onset of symptoms and diagnosis is 7 years in the UK


• Prevalence 1 in 25, 000 individuals (Parks et al 2008)

• ESID database identifies 20.7% with PID has CVID (Gathmann et al 2009)

• Mean age of CVID diagnosis is early 30’s

• RBH bx study identified 2% had CVID, 4% had other immune deficiencies (Ozerovitch et al 2006)


• Symptoms of cough and phlegm did not impact on patients’ activity or confidence levels (Ozerovitch et al 2010)

• CRP and Total WCC are systemic markers of inflammation that correlate with quality of life (Wilson et al 1998)

• Dyspnoea, FEV1 and sputum production are the strongest factors of HRQL in stable bronchiectasis patients (Martinez-Garcia et al 2005)

• Improved quality of life scores on follow-up compared to time of referral (Ozerovitch et al 2004)


• ↑HRQoL in patients with PID on IVIG – based on self-reported measures of physical functioning (Hedderick et al 1986)

• Patients reported on QoL, function and self-rated health status with IgG therapy (Gardulf et al 1993)

• Studies remark of medical and clinical measures of success measures (Gardulf et al 2006)

• Positive outcome in days off sick 6.1 compared to 23.3 (Eades-Perner et al 2007)


• To assess QoL and functional ability in adult stable patients with Bx due to CVID, compared with historical controls with idiopathic bronchiectasis (Ozerovitch et al 2004)

(Note: stable patients – no acute infective event requiring additional antibiotics in the preceding month)


• Bx confirmed on HRCT

• CVID confirmed by ESID criteria CVID confirmed by ESID criteria

• Severity of Bx noted by presence Severity of Bx noted by presence of of Pseudomonas aeruginosa (Pa) Pseudomonas aeruginosa (Pa)

• Analysis: Student t-testsAnalysis: Student t-tests


• Spirometry

• SGRQ (Wilson et al 1997; Jones 2002)

• Exercise Capacity – SWT (Singh et al 1992)

• The Borg Breathlessness Scale (Borg 1982)

• Sputum Results (Wells et al 1993; Davies et al 2006; Loebinger et al 2009)


0 Nothing at all

0.5 Very, very slight (just noticeable)

1 Very slight

2 Slight

3 Moderate

4 Somewhat severe

5 Severe

6

7 Very severe

8

9 Very, very severe(almost maximal)

10 Maximal


Biomedical and social characteristics Biomedical and social characteristics of study participantsof study participants CVID-Bx n(%)CVID-Bx n(%) Id-Bx n(%)Id-Bx n(%)

CVID associated bronchiectasis patients within the immunology database 34 0

Study participants 22 (65) 36Subjects excluded 12 (35) 0Male participants (%) 9 (41) 12(33)Female participants (%) 13 (59) 24 (67)

Mean age(SD)(age range)

45yrs(22.8)

(17-67)

54yrs(11.30)(32-75)

n of participants positive to Pa in the preceding 6 months 1 (5) 13 (36)

Mean FEV1 % pred (SD) 64 (26) 71 (28)Participants on IgG replacement therapy (%) 20 (91) 0

• CVID-Bx patients had better scores for all SGRQ components and better SWT distance, than Idiopathic Bx (these were clinically relevant although not statistically significant).


SGRQ ComponentsSGRQ Components

Mean scores (SD):Mean scores (SD):

CVID-Bx (n=22)CVID-Bx (n=22)

(Age range 17-67yrs)(Age range 17-67yrs)

Mean Scores (SD):Mean Scores (SD):

Idiopathic Bx (n=36)Idiopathic Bx (n=36)

(Age range 32-75yrs)(Age range 32-75yrs) p:p:

Symptoms 58.3 (23.7) 65.8 (22.3) 0.23

Activity 37.0 (27.0) 45.3 (25.0) 0.23

Impact 27.8 (22.2) 34.4 (18.1) 0.22

Total Score 35.8 (23.0) 43.0 (18.7) 0.19

SWT (m) 513 (213.0) 432 (157.7) 0.10

*There was only a statically significant difference between the exercise tolerance scores (t-test p<0.03).


Total QofL

Mean Score

Symptom QofL

Mean ScoreActivity QofL

Mean ScoreImpact QofL

Mean ScoreSWT metres

Mean Score

SCIG(SD)

28.9 53.2 27.5 21.9 614*

-18.5 -17.2 -21.4 -19 -180.5

IVIG(SD)

45.5 66.8 48.6 36.8 402*

-26.3 -29.0 -31.3 -28.8 -218.6


PathogenPathogen CVID-BxCVID-Bx

(n=22)(n=22)CVID-Bx CVID-Bx

(baseline)(baseline)Id-BxId-Bx

(N=36)(N=36)Id-BxId-Bx

(baseline)(baseline)

Pseudomonasaeruginosa

1 1 14 9

Steptococcus pneumoniae 0 4 2 0

Haemophilus influenzae 2 8 1 5

StaphlococcusAureus

0 0 1 0

Stenotrophomonas maltophilia 0 0 1 1

Moroxella Catarrhalis 0 1 0 0

Multi Pathogens (Hi & Strep/Staph)

0 2 0 3

No growth 1 3 5 16

No sputum (well) 18 3 12 2

• SWT –59% walked 4-600m; total range 50-940m– Id-Bx 42% walked 4-600m; total range 0-890m

• Borg scores: 59% no breathlessness pre-exertion; 64% scored between 2 to 3 (slight to mod) post exertion– Id-Bx 33% no breathlessness pre-exertion; 39% scored

between 2 to 3 post exertion (Borg score≥4=39%)

• Spirometry: (FEV1 64% pred) correlated negatively with Activity component only– Id-Bx no relationship


• Patients with CVID-Bx have clinically better health Patients with CVID-Bx have clinically better health status and functional ability than demographically status and functional ability than demographically similar Id-Bxsimilar Id-Bx

• SCIG therapy was found to be associated with better SCIG therapy was found to be associated with better exercise tolerance and health status scores: howeverexercise tolerance and health status scores: however

• Small no of patients studied in each groupSmall no of patients studied in each group• ? Interaction of other confounding factors such as age ? Interaction of other confounding factors such as age

or presence/absence co-morbiditiesor presence/absence co-morbidities

• Little data on the utility of the Borg breathlessness scores in this specialist area.


• Baseline values obtained at diagnosis or referral

• Comparison group rather than historical controls

• Research study used disease specific QoL tool - ?fitted to existed published work


• This study provides the first report on the impact of CVID-Bx on quality of life and physical functioning using a disease specific respiratory tool

• CVID-Bx QoL scores were generally better CVID-Bx QoL scores were generally better than Id-Bx possibly due as a result of specific than Id-Bx possibly due as a result of specific therapy (IgG replacement) in the majority of therapy (IgG replacement) in the majority of these patientsthese patients


• Patients • Dr Peter Kelleher• Dr Rob Wilson

• Samantha Prigmore• Winston Banya• Dr Jill Riley


http://uk.wrs.yahoo.com/_ylt=A0PDodnrkrlO_0AAjudWBQx.;_ylu=X3oDMTBpcGszamw0BHNlYwNmcC1pbWcEc2xrA2ltZw--/SIG=12g63fi4g/EXP=1320813419/**http:/www.arvato-services-healthcare.com/en/references.html

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Lorraine Ozerovitch ( MSc, BSc, RGN ) Clinical Nurse Specialist

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