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

1. Adults with selective IgA deficiency - Health-related quality of life (HRQL) • Risk factors for poor HRQL • ESID-INGID-IPOPI Meeting 2012 Ann Gardulf

2. HRQL in selective IgA deficiencyDept of Medicine, University of Iceland, Dept of Immunology, Landspitali-University Hospital, Reykjavík, Iceland andDept of Laboratory Medicine, Section of Clinical Immunology, Karolinska Institutet, Huddinge, Stockholm, SwedenGuðmundur Jorgensen Ann GardulfSigurdsson M. I., Sigurðardóttir S., Thorsteinsdottir I., Gudmundsson S., Hammarström L. and Ludviksson B.R.

3. HRQL in selective IgA deficiencySelective IgA deficiency (SIgAD) is the most common primary immunodeficiencyDefinition: serum IgA <0.07 g/LEstimated prevalence: 1/600

4. HRQL in selective IgA deficiencySelective IgA deficiency (SIgAD) is the most common primary immunodeficiencyDefinition: serum IgA <0.07 g/LEstimated prevalence: 1/600 Nordic countries 41,000 individuals USA 511,000 individuals EU 835,000 individuals

5. HRQL in selective IgA deficiencyMore infections, autoimmune disorders, allergic diseases

6. HRQL in selective IgA deficiency SIgAD • 32 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

7. HRQL in selective IgA deficiency Controls 63 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 Iglevels. SIgAD • 32 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

8. HRQL in selective IgA deficiencyNo 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.

9. HRQL in selective IgA deficiencyNo 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.

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

11. HRQL in selective IgA deficiencyResults 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

12. HRQL in selective IgA deficiencyRemember the 30/70% slide? ESID Poster, 2012

13. HRQL in selective IgA deficiencyRemember the 30/70% slide?Total disease burden when compared to controls ESID Poster, 2012

14. HRQL in selective IgA deficiencyRemember the 30/70% slide?Total disease burden when compared to controls ESID Poster, 2012

15. HRQL in selective IgA deficiencyMethods – 3 questionnaires for HRQLSIgAD: Baseline 6 months 12 monthsControls: Baseline

16. HRQL in selective IgA deficiencyResults 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)

17. HRQL in selective IgA deficiencyResults – over time SIgAD Good HRQL Good HRQL Baseline 12 monthsfollow-up Poor HRQL 6 monthsfollow-up

18. HRQL in selective IgA deficiencyResults – over time SIgAD Period of mixed feelings? Good HRQL Good HRQL Education Training Home-therapy Baseline (dx) 12 monthsfollow-up Poor HRQL 6 monthsfollow-up

19. Risk factors for poor HRQL

20. Risk factors for poor HRQL

21. HRQL in selective IgA deficiency“What’s in it for me as a nurse?”From a methodological point of viewThe 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.

22. HRQL in selective IgA deficiency“What’s in it for me as a nurse?”From a clinical point of viewSIgA is very commonImportant to detect them as they have many health problems and are at risk for poor HRQLNursing interventions possible to prevent a worsening HRQL and/or to treat health problems

23. HRQL in selective IgA deficiency“What’s in it for me as a nurse?”From a clinical point of viewAware 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

24. HRQL in selective IgA deficiency“What’s in it for me as a nurse?”From a clinical point of viewNeed for IgG therapy in some SIgAD individuals?

25. HRQL in selective IgA deficiencyThank you !Heimir & PjakkurMàni (Moon)