15. Immunoglobulin Patient Experience: 2024 IgNS Patient 360 Survey Analysis

Background: Immunoglobulin (Ig) therapy is a critical treatment for patients with serious, chronic disorders. However, despite the life-saving potential of timely diagnosis and treatment, patients often face unacceptably long delays in receiving appropriate care. This delay can be attributed to several interconnected factors:

Lack of Awareness and Practical Screening Tools: Many non-specialist practitioners lack awareness of the signs and symptoms of disorders requiring Ig therapy. The absence of readily available and practical screening tools further hinders early diagnosis. Fragmented Healthcare System: The complexity of navigating a fragmented healthcare system often leads to prolonged diagnostic journeys and delays in accessing specialized care.

The rarity of Disorders: The rare nature of these disorders contributes to a lack of familiarity among healthcare providers, potentially leading to misdiagnosis or delayed recognition. These challenges underscore a critical gap in practice standards. Systematic education and training of clinicians are urgently needed to bridge this gap and ensure timely diagnosis and access to Ig therapy. The data highlights the importance of patient-centric research, like that conducted by the Immunoglobulin National Society (IgNS), in identifying the need for highly specialized practice and promoting the adoption of evidence-based standards like the IgNS Standard of Practice.

Purpose: This initiative aims to improve patient outcomes by addressing the critical gaps in the timely diagnosis and treatment of patients with serious, chronic disorders requiring immunoglobulin (Ig) therapy.

Methods: This exploratory, confidential and deidentified survey of patients receiving Ig was distributed to Patient 360 subscribers via the Jotform HIPAA-compliant survey tool between May and July 2024. An interim analysis of 308 respondents was conducted.

Results: 167 respondents (54%) had primary immunodeficiency (PI), either with or without an autoimmune comorbidity, 49 (16%) had a neuromuscular disorder, 42 (14%) had a rheumatological disorder and 24 reported other conditions for which they are receiving Ig therapy. PI patients’ delay in diagnosis was 17.74 years, compared to 4.72 years for neuromuscular disorders, 2.3 years rheumatological disorders, and 5.31 for other conditions. When assessing the use of emergency departments, urgent care, and primary care prior to diagnosis, all were associated with increased delays in diagnosis.

127 SCIG /fSCIG patients rated their satisfaction with their self-administration training: 35% reported being very satisfied with their training, 43% satisfied, 12% neither satisfied nor dissatisfied, 8% dissatisfied, and 2% very dissatisfied. In examining the relationship between satisfaction with SCIG/fSCIG self-administration training and infusion related issues, there was no statistical significance between these parameters for issues such as getting the needle to stay in throughout the Ig treatment, leaking at the infusion site, the infusion taking too long or the infusion rate slowing down. 91% of IVIG respondents received premedication, which had no statistical significance in reduction of IVIG side effect severity or frequency.

Discussion: This analysis demonstrated that patients with PI continue to experience the longest delays in diagnosis, followed by neuromuscular and rheumatologic diagnoses. Primary care-treated patients experienced increased delays in diagnosis, suggesting urgent educational needs in these settings. The continued high incidence of SCIG/fSCIG issues signals to gaps in administration technique and lack of continued assessment of independent patients. Surprisingly, highly-mitigatable infusion issues such as leaking, pain and needle coming out had no significant relationship with patients’ satisfaction with their training. This finding suggests that even with optimal patient education and training which normally occurs over just 3-4 initial nursing visits, patients must be assessed for these specific issues on a regular basis. Additionally, premedication was not associated with a reduction in IVIG ADRs, underscoring the importance of mitigating ADRs by appropriate administration.

Conclusions: As one of the most complex therapeutics, Ig is used to treat patients with serious, chronic disorders. Timely diagnosis and access to treatment can be life-saving, and yet, patients continue to experience unacceptably long delays. Lack of awareness and absence of practical screening tools for the non-specialist practitioners, a fractionated healthcare system, and the rare nature of these disorders are all contributing factors. Systematic education and training of clinicians is needed to continue to solve for gaps in practice standards. Our data emphasize the significance IgNS’ patient research that reflects the needs for highly specialized practice and the adoption of the IgNS Standard of Practice.