scholarly journals Primary Immune Deficiency: Patients’ Preferences for Replacement Immunoglobulin Therapy

2021 ◽  
Author(s):  
Juan Marcos Gonzalez ◽  
Mark Ballow ◽  
Angelyn Fairchild ◽  
M. Chris Runken
Keyword(s):  
Immune Deficiency ◽  
Patient Preferences ◽  
Immunoglobulin Therapy ◽  
Patient Characteristics ◽  
Treatment Preferences ◽  
Primary Immune Deficiency ◽  
Duration Of Treatment ◽  
Life Saving ◽  
Improving Patient Care ◽  
Time Since Diagnosis

Abstract Purpose: Immunoglobulin (Ig) replacement therapy is an important life-saving treatment modality for patients with primary antibody immune deficiency disorders (PAD). IVIG and SCIg are suitable alternatives to treat patients with PAD but vary in key ways. Existing evidence on patient preferences for Ig treatments given the complexities associated with IVIG and SCIg treatment is limited and fails to account for variations in preferences across patients. For this reason, we sought to evaluate PAD patient preferences for features of IVIG and SCIg across different patient characteristics.Methods: 119 PAD patients completed a discrete-choice experiment (DCE) survey. The DCE asked respondents to make choices between carefully constructed treatment alternatives described in terms of generic treatment features. Choices from the DCE were analyzed to determine the relative influence of attribute changes on treatment preferences. We used subgroup analysis to evaluate systematic variations in preferences by patients’ age, gender, time since diagnosis, and treatment experience. Results: Patients were primarily concerned about the duration of treatment side effects, but preferences were heterogeneous. This was particularly true around administration features. Time since diagnosis was associated with an increase in patients’ concerns with the number of needles required per infusion. Also, patients appear to prefer the kind of therapy they are currently using which could be the result of properly aligned patient preferences or evidence of patient adaptive behavior. Conclusions: Heterogeneity in preferences for Ig replacement treatments suggests that a formal shared decision making process could have an important role in improving patient care.

The Use of Immunoglobulin Therapy for Patients With Primary Immune Deficiency: An Evidence-Based Practice Guideline

2010 ◽  
Vol 24 ◽  
pp. S28-S50 ◽  
Author(s):  
Nadine Shehata ◽  
Valerie Palda ◽  
Tom Bowen ◽  
Elie Haddad ◽  
Thomas B. Issekutz ◽  
...  
Keyword(s):  
Immune Deficiency ◽  
Practice Guideline ◽  
Evidence Based Practice ◽  
Immunoglobulin Therapy ◽  
Primary Immune Deficiency ◽  
Evidence Based

Implanted ports in adults with primary immunodeficiency

2018 ◽  
Vol 19 (4) ◽  
pp. 375-377
Author(s):  
James C Barton ◽  
Jackson Clayborn Barton ◽  
Luigi F Bertoli
Keyword(s):  
Immune Deficiency ◽  
Immunoglobulin G ◽  
Venous Access ◽  
Primary Immune Deficiency ◽  
Subcutaneous Immunoglobulin ◽  
Duration Of Treatment ◽  
Immunoglobulin G Subclasses ◽  
Catheter Fracture ◽  
Immunoglobulin G Subclass ◽  
Common Variable

Background: We sought to learn more about the utility and safety of implanted ports for monthly immunoglobulin G infusions in adults with primary immune deficiency. Methods: We reviewed charts of adults who were referred to a single practice during the interval 2006–2016 for evaluation and management of frequent or severe upper and lower respiratory tract and other infections, subnormal total immunoglobulin G or immunoglobulin G subclasses, and suboptimal responses to polyvalent pneumococcal polysaccharide vaccinations; were diagnosed to have primary immune deficiency; and were advised to undergo immunoglobulin G therapy. Results: Of 606 patients, 20 (19 women, 1 man; 16 immunoglobulin G subclass deficiency, 4 common variable immunodeficiency; 3.3%) needed implanted ports because they had inadequate upper extremity superficial venous access. Median age at diagnosis was 48 years (range: 32–65 years). In total, 17 of the 20 patients preferred monthly in-office intravenous immunoglobulin G treatment to weekly at-home subcutaneous immunoglobulin G. The other three patients could not be treated with subcutaneous immunoglobulin G (unfavorable self-treatment experiences and insurance limitations). Median duration of treatment via implanted ports was 73 months (range: 10–153 months). In the man, the first implanted port was replaced after 26 months due to catheter fracture of unknown cause. His second port has been used for 112 months. We observed no other port-related failure, infections, thrombosis, or other adverse events. Conclusion: The utility and safety of implanted ports in adults with primary immune deficiency for whom subcutaneous immunoglobulin G therapy is not desired or feasible are probably similar to those of ports in patients without primary immune deficiency.

scholarly journals Medical and Nonmedical Information during Multidisciplinary Team Meetings in Cancer Care

2021 ◽  
Vol 28 (1) ◽  
pp. 1008-1016
Author(s):  
Jessica Wihl ◽  
Linn Rosell ◽  
Tobias Carlsson ◽  
Sara Kinhult ◽  
Gert Lindell ◽  
...  
Keyword(s):  
Decision Making ◽  
Multidisciplinary Team ◽  
Cancer Care ◽  
Family Relations ◽  
Patient Characteristics ◽  
Psychological Status ◽  
Treatment Preferences ◽  
Case Discussions ◽  
Case Presentations ◽  
Data Elements

Background: Multidisciplinary team (MDT) meetings provide treatment recommendations based on available information and collective decision-making in teams with complementary professions, disciplines and skills. We aimed to map ancillary medical and nonmedical patient information during case presentations and case discussions in MDT meetings in cancer care. Methods: Through a nonparticipant, observational approach, we mapped verbal information on medical, nonmedical and patient-related characteristics and classified these based on content. Data were collected from 336 case discussions in three MDTs for neuro-oncology, sarcoma and hepato-biliary cancer. Results: Information on physical status was presented in 48.2% of the case discussions, psychological status in 8.9% and comorbidity in 48.5% of the cases. Nonmedical factors, such as family relations, occupation, country of origin and abode were referred to in 3.6–7.7% of the cases, and patient preferences were reported in 4.2%. Conclusions: Provision of information on comorbidities in half of the cases and on patient characteristics and treatment preferences in <10% of case discussions suggest a need to define data elements and develop reporting standards to support robust MDT decision-making.

scholarly journals Real-World Evidence of Caplacizumab Use in the Management of Acute TTP

Blood ◽  
2020 ◽  
Author(s):  
Tina Dutt ◽  
Rebecca J Shaw ◽  
Matthew James Stubbs ◽  
Jun Yong ◽  
Benjamin Bailiff ◽  
...  
Keyword(s):  
Platelet Count ◽  
Real World ◽  
Patient Characteristics ◽  
Patient Access Scheme ◽  
Fda Approval ◽  
Immune Mediated ◽  
Real World Evidence ◽  
Life Saving ◽  
The Uk ◽  
Von Willebrand

The cornerstone of life-saving therapy in immune mediated thrombotic thrombocytopenic purpura (iTTP) has been plasma exchange (PEX) combined with immunomodulatory strategies. Caplacizumab, a novel anti-von Willebrand factor nanobody, trialled in two multicentre, randomised-placebo-controlled trials leading to EU and FDA approval, has been available in the UK through a patient-access scheme. Data was collected retrospectively from 2018-2020 for 85 patients receiving caplacizumab, including 4 children, from 22 UK hospitals. Patient characteristics and outcomes in the real-world clinical setting were compared with caplacizumab trial endpoints and historical outcomes in the pre-caplacizumab era. 84/85 patients received steroid and rituximab alongside PEX; 26% required intubation. Median time to platelet count normalisation (3 days), duration of PEX (7 days) and hospital stay (12 days) was comparable with RCT data. Median duration of PEX and time from PEX initiation to platelet count normalisation was favourable compared with historical outcomes (p&lt;0.05). TTP recurrence occurred in 5/85 patients; all with persistent ADAMTS13 activity &lt;5iu/dL. Of 31 adverse events in 26 patients, 17/31 (55%) were bleeding episodes and 5/31 (16%) were thrombotic events (two unrelated to caplacizumab); mortality was 6% (5/85), with no deaths attributed to caplacizumab. In 4/5 deaths caplacizumab was introduced &gt;48 hours after PEX initiation (3-21 days). This real-world evidence represents the first and largest series of TTP patients receiving caplacizumab outside clinical trials, including paediatric patients. Representative of true clinical practice, the findings provide valuable information for clinicians treating TTP globally.

Efficacy of a new chromatography-based IVIG (10%-formulation) on validated sinopulmonary infections in primary immune deficiency

2002 ◽  
Vol 109 (1) ◽  
pp. S190-S190
Author(s):  
Harold Schroeder ◽  
Melvin Berger ◽  
Anita T Gewurz ◽  
Phillip Korenblat ◽  
Chain Roifman ◽  
...  
Keyword(s):  
Immune Deficiency ◽  
Primary Immune Deficiency

Adult Primary Immune Deficiency: What Are We Missing?

2012 ◽  
Vol 125 (8) ◽  
pp. 779-786 ◽  
Author(s):  
Bharat T. Srinivasa ◽  
Reza Alizadehfar ◽  
Martin Desrosiers ◽  
Joseph Shuster ◽  
Nitika Pant Pai ◽  
...  
Keyword(s):  
Immune Deficiency ◽  
Primary Immune Deficiency

scholarly journals Recognizing Primary Immune Deficiency in Clinical Practice

2006 ◽  
Vol 13 (3) ◽  
pp. 329-332 ◽  
Author(s):  
Hale Yarmohammadi ◽  
Lissette Estrella ◽  
John Doucette ◽  
Charlotte Cunningham-Rundles
Keyword(s):  
Immune Deficiency ◽  
Clinical Practice ◽  
Organ Dysfunction ◽  
Primary Immunodeficiency ◽  
Scoring System ◽  
Clinical Information ◽  
Chronic Illnesses ◽  
Primary Immune Deficiency ◽  
Recurrent Infections

ABSTRACT Primary immunodeficiency results in recurrent infections, organ dysfunction, and autoimmunity. We studied 237 patients referred for suspicion of immunodeficiency, using a scoring system based on clinical information. The 113 patients with immunodeficiency had higher scores and more episodes of chronic illnesses and were more likely to have neutropenia, lymphopenia, or splenomegaly.

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