Adults with selective IgA deficiency - Health-related quality of life (HRQL)

Adults with selective IgA deficiency

- Health-related quality of life (HRQL)-Risk factors for poor HRQL

ESID-INGID-IPOPI Meeting 2012

Ann Gardulf

HRQL in selective IgA deficiency

Dept of Medicine, University of Iceland, Dept of Immunology, Landspitali-University Hospital, Reykjavík, Iceland

and Dept of Laboratory Medicine, Section of Clinical

Immunology, Karolinska Institutet, Huddinge, Stockholm, Sweden

Guðmundur Jorgensen Ann Gardulf

Sigurdsson M. I., Sigurðardóttir S., Thorsteinsdottir I., Gudmundsson S., Hammarström L. and Ludviksson B.R.

HRQL in selective IgA deficiency

Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency

Definition: serum IgA <0.07 g/L

Estimated prevalence: 1/600

HRQL in selective IgA deficiency

Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency

Definition: serum IgA <0.07 g/L

Estimated prevalence: 1/600

Nordic countries 41,000 individualsUSA 511,000 individualsEU 835,000 individuals

HRQL in selective IgA deficiency

More infections, autoimmune disorders, allergic diseases

SIgAD32 individuals - 13 women, 19 men; mean age 48 years. Detected when screening blood donors and blood samples analysed at the University Hospital of Iceland. The blood from the 32 individuals was reanalysed to confirm inclusion criteria of S-IgA level <0.07g/L and no IgG subclass deficiency.Not under care, not informed

HRQL in selective IgA deficiency

SIgAD32 individuals - 13 women, 19 men; mean age 48 years. Detected when screening blood donors and blood samples analysed at the University Hospital of Iceland. The blood from the 32 individuals was reanalysed to confirm inclusion criteria of S-IgA level <0.07g/L and no IgG subclass deficiency.Not under care, not informed

Controls63 controls - 29 women, 34 men; mean age 50 years. A total of 96 age- and gender-matched controls were randomly selected from the Icelandic National Registry (1 SIgAD: 3 control individuals). 63 (66%) participated. All controls showed normal Ig levels.

HRQL in selective IgA deficiency

HRQL in selective IgA deficiency

No exclusion criteria for the individuals in the control group were set up

- thus, the controls might suffer from other chronic diseases/conditions and thereby better reflect the ordinary population.

HRQL in selective IgA deficiency

No exclusion criteria for the individuals in the control group were set up

- thus, the controls might suffer from other chronic diseases/conditions and thereby better reflect the ordinary population.

Unique study – first time in PID researchhistory that individuals with PID are compared to matched controls.

HRQL in selective IgA deficiency

Methods for HRQL and clinical status- Health and disease questionnaire (122 items) HRQLHRQL+CS+CS- SF-36 HRQL (36 items) HRQLHRQL- Infection-HRQL questionnaire (9 items) HRQLHRQL- Standardized interview by MD- Extensive physical examination- Lung function tests - Laboratory assessments- Skin prick tests

HRQL in selective IgA deficiency

Results clinical status – baseline (SIgAD vs controls)

We found that the SIgAD individuals significantly more often suffered from:

- upper- and lower respiratory tract infections

- autoimmune diseases

- allergies

- skin and nail-fungal infections

ESID Poster, 2012

HRQL in selective IgA deficiency

Remember the 30/70% slide?

ESID Poster, 2012

HRQL in selective IgA deficiency

Remember the 30/70% slide?Total disease burden when compared to controls

ESID Poster, 2012

HRQL in selective IgA deficiency

Remember the 30/70% slide?Total disease burden when compared to controls

ESID Poster, 2012

HRQL in selective IgA deficiency

Methods – 3 questionnaires for HRQL

SIgAD: Baseline 6 months 12 months

Controls: Baseline

HRQL in selective IgA deficiency

Results HRQL – baseline (SIgAD vs controls)

SF-36 results indicated poorer HRQL although not reaching statistical significances for the subscales (statistical power?)

Significantly increased fear of getting infected (p<0.01)

Correlation between fear of getting infection and physical health (p<0.01)

Significantly more SIgAD on long-term sick leave (p<0.05)

Baseline

Poor HRQL

12 months follow-up

6 months follow-up

Good HRQL Good HRQL

HRQL in selective IgA deficiency

Results – over time SIgAD

Baseline(dx)

Poor HRQL

12 months follow-up

6 months follow-up

Good HRQL Good HRQL

EducationTraining Home-therapy

HRQL in selective IgA deficiency

Results – over time SIgAD

Period of mixed feelings?

Risk factors for poor HRQLFactors Jörgensen

et al., ongoing

Age

Gender

Infections >4 organs

Number of infections/number of antibiotic treatments last 12 months

x

S-IgG level

Frequency of IVIG therapy

Type of diagnose

Home (instead of hospital)

Joint/muscle/skeletal x

No of any daily medication x

Allergic rhinoconjuctivitis x

Anxiety/insomnia x

Specific occurrence of stress last 2-3 months

Hospitalization last year

Unemployment

Smoking

Risk factors for poor HRQLFactors Jörgensen

et al., ongoing

Sigstad et al.,2005

Gardulf et al., 2008

Seeborg et al., ESID poster

Age x x (at dx)

Gender x

Infections >4 organs x

Number of infections/number of antibiotic treatments last 12 months

x x

S-IgG level x

Frequency of IVIG therapy x

Type of diagnose x

Home (instead of hospital) x

Joint/muscle/skeletal x x

No of any daily medication x

Allergic rhinoconjuctivitis x

Anxiety/insomnia x

Specific occurrence of stress last 2-3 months x

Hospitalization last year x

Unemployment x

Smoking x

HRQL in selective IgA deficiency

“What’s in it for me as a nurse?”

From a methodological point of view

The study highlights the importance to use matched controls in PID clinical research to better detect unexpected clinical manifestations, e.g. fungal infections for further research, and differences in HRQL.

Need to agree upon a basic “kit” of instruments/methods for international collaborations and comparisons betweencountries and patient groups.

HRQL in selective IgA deficiency

“What’s in it for me as a nurse?”

From a clinical point of view

SIgA is very common

Important to detect them as they have many health problems and are at risk for poor HRQL

Nursing interventions possible to prevent a worsening HRQL and/or to treat health problems

HRQL in selective IgA deficiency

“What’s in it for me as a nurse?”

From a clinical point of view

Aware of risk factors – more vulnerable subgroups?

Decreased HRQL for a period after being told about the PID- important for the nurse to know and to adapt educational/training based on this knowledge

HRQL in selective IgA deficiency

“What’s in it for me as a nurse?”

From a clinical point of view

Need for IgG therapy in some SIgAD individuals?

HRQL in selective IgA deficiency

Thank you !

Heimir & Pjakkur Màni (Moon)