Abstract
Primary immunodeficiency disease (PIDD) with hypogammaglobulinemia is characterized by recurrent and severe bacterial infections and IgG replacement is the standard of care in many of these patients. Health-related quality of life (HRQOL) is becoming increasingly recognized as a factor that affects patient well-being and treatment preferences. In an effort to better understand what factors affect HRQOL in patients with PIDD, we reviewed the published literature that used standardized instruments for the measurement of HRQOL. We investigated HRQOL in PIDD patients compared with normal controls and patients with other chronic diseases; we also investigated the impact of treatment administration on patient satisfaction. The most commonly encountered health-related quality of life instruments were the child heath questionnaire parental form 50, short form 36, PedsQL 4.0, Lansky’s play performance scale, and Life Quality Index. Patients with PIDD scored significantly lower on many of the instruments compared with normal controls. Also, while it appears that many patients appreciate home-based and subcutaneous IgG replacement therapy, patient satisfaction ultimately involves various clinical factors and individual patient preferences. By further analyzing what factors impact HRQOL, therapy adjustments can be made to maximize patient well-being and minimize disease impact on daily functioning.
Highlights
Primary immunodeficiency disease (PIDD) is a group of genetic disorders characterized by increased susceptibility to infection
Another study comparing children with PIDD with children with other chronic diseases found that they generally experience similar health-related quality of life (HRQOL) to patients with juvenile inflammatory arthritis (JIA) but that they score lower than the JIA group with respect to perception of general health and limitations on parental time and family activities [20]
A recent study of 50 PIDD patients shifting from intravenous immunoglobulin (IVIG) to subcutaneous immunoglobulin (SCIG) found that these patients subsequently had a significant improvement in their total mean life quality index (LQI) at 6 months that was sustained over time [52]
Summary
Primary immunodeficiency disease (PIDD) is a group of genetic disorders characterized by increased susceptibility to infection. A six-year longitudinal study of patients with common variable immunodeficiency (CVID), a common form of PIDD, found that they scored lower on the General Health scale in comparison to other patients with chronic diseases, and that they had a worse perception of their general personal health and limitations due to their physical health [19]. Another study comparing children with PIDD with children with other chronic diseases found that they generally experience similar HRQOL to patients with juvenile inflammatory arthritis (JIA) but that they score lower than the JIA group with respect to perception of general health and limitations on parental time and family activities [20]. Comprised of 15 items with assessments made on a 7-point Likert scale ranging from extremely good (= 7) to extremely bad (= 1)
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
