Caregivers’ role in managing hereditary angioedema and perceptions of treatment-related burden

2021 ◽  
Vol 42 (3) ◽  
pp. S11-S16 ◽  
Author(s):  
Timothy J. Craig ◽  
Aleena Banerji ◽  
Marc A. Riedl ◽  
Jessica M. Best ◽  
Jinky Rosselli ◽  
...  
Keyword(s):  
Hereditary Angioedema ◽  
Genetic Disease ◽  
Online Survey ◽  
Scientific Literature ◽  
Upper Airway ◽  
The United States ◽  
Care Recipient ◽  
Psychosocial Impacts ◽  
Life Threatening ◽  
Familial Caregivers

Hereditary angioedema (HAE) is a rare genetic disease that results in recurrent, debilitating, and potentially life-threatening swelling episodes in the extremities, genitals, gastrointestinal tract, and upper airway. Patients can experience significant burdens related to their disease. Informal or familial caregivers often support patients with HAE and likely share in the disease-related burdens, although there are limited HAE caregiver‐focused reports in the scientific literature. In the United States, we conducted an online survey of adults caring for an individual with HAE to better understand their experiences with the disease and identify psychosocial impacts of providing care for a patient with HAE. Thirty caregivers provided responses to the survey. Most caregivers were family members of the care recipient and many had HAE themselves. Caregivers reported participating in a number of medical-related tasks and experiencing some burdens as a result of caring for a person with HAE.

scholarly journals Blastomycosis of the Vocal folds with Life-Threatening upper Airway Obstruction: A Case Report

2002 ◽  
Vol 81 (12) ◽  
pp. 852-855 ◽  
Author(s):  
Celso T. Ebeo ◽  
Kenneth Olive ◽  
Ryland P. Byrd ◽  
Girish Mirle ◽  
Thomas M. Roy ◽  
...  
Keyword(s):  
Lower Respiratory Tract ◽  
Pulmonary Infection ◽  
Upper Airway ◽  
Vocal Folds ◽  
The United States ◽  
Inappropriate Therapy ◽  
Airway Compromise ◽  
Inflammatory Phase ◽  
Life Threatening ◽  
Laryngeal Involvement

Blastomycosis is a chronic fungal disease that primarily affects the lower respiratory tract. The acute inflammatory phase of the primary pulmonary infection is characterized by a lymphohematogenous spread to extrapulmonary sites, especially the skin. The presence of disseminated infection with Blastomyces dermatitidis in the larynx is unusual. In areas of the United States where this fungus is endemic, failure to consider laryngeal involvement might lead to inappropriate therapy and thus worsening inflammation and airway compromise.

Hereditary Angioedema: Management of Laryngeal Attacks

2011 ◽  
Vol 25 (6) ◽  
pp. 379-382 ◽  
Author(s):  
Sandra C. Christiansen ◽  
Bruce L. Zuraw
Keyword(s):  
Hereditary Angioedema ◽  
Treatment Options ◽  
The United States ◽  
Fresh Frozen Plasma ◽  
C1 Inhibitor ◽  
Acute Therapy ◽  
Fresh Frozen ◽  
Life Threatening ◽  
Frozen Plasma ◽  
Future Management

Background Hereditary angioedema (HAE) patients suffering from laryngeal attacks in the United States faced severely limited treatment options until 2008. These potentially life-threatening episodes occur in over one-half of the patients affected by HAE during their lifetimes. Acute therapy had been relegated to supportive care, intubation, and consideration of fresh frozen plasma (FFP)–-the latter with the potential for actually accelerating the speed and severity of the swelling. Methods In this article we will review the recently approved and emerging HAE treatments that have evolved from the recognition that bradykinin generation is the fundamental abnormality leading to attacks of angioedema. Results Acute therapy for laryngeal attacks will be discussed including purified plasma–derived C1 inhibitor (C1INH), recombinant C1INH, an inhibitor of plasma kallikrein (ecallantide), and a B2 receptor antagonist (icatibant). Prophylactic care has also been transformed from a reliance on attenuated androgens with their attendant side effects to C1INH replacement. Conclusion The arrival of these novel therapies promises to transform the future management of HAE.

scholarly journals Ecallantide for the Treatment of Hereditary Angiodema in Adults

2011 ◽  
Vol 5 ◽  
pp. CMC.S4434 ◽  
Author(s):  
Michael Lunn ◽  
Erin Banta
Keyword(s):  
Upper Airway ◽  
The United States ◽  
Reversible Inhibitor ◽  
Plasma Kallikrein ◽  
Autosomal Dominant Disorder ◽  
Clinical Disorder ◽  
Life Threatening ◽  
The Complement System ◽  
Submucosal Edema ◽  
Esterase Inhibitor

Hereditary angioedema (HAE) is a clinical disorder characterized by a deficiency of C1 esterase inhibitor (C1-INH). HAE has traditionally been divided into two subtypes. Unique among the inherited deficiencies of the complement system, HAE Types I and II are inherited as an autosomal dominant disorder. The generation of an HAE attack is caused by the depletion and/or consumption of C1-inhibitor manifested as subcutaneous or submucosal edema of the upper airway, face, extremities, or gastrointestinal tract mediated by bradykinin. Attacks can be severe and potentially life-threatening, particularly with laryngeal involvement and treatment of acute attacks in the United States has been severely limited. In December 2009 the FDA approved ecallantide for the treatment of acute HAE attacks. Ecallantide is a small recombinant protein acting as a potent, specific and reversible inhibitor of plasma kallikrein which binds to plasma kallikrein blocking its binding site, directly inhibiting the conversion of high molecular weight kininogen to bradykinin. Administered subcutaneously, ecallantide was demonstrated in two clinical trials, EDEMA3 and EDEMA4, to decrease the length and severity of acute HAE attacks. Although there is a small risk for anaphylaxis, which limits home administration, ecallantide is a novel, safe, effective and alternative treatment for acute HAE attacks.

scholarly journals Hereditary angioedema: the challenges of cross-border family investigation and treatment

2020 ◽  
Vol 13 (4) ◽  
pp. e231906
Author(s):  
Anna Trier Heiberg Brix ◽  
Trine Mehlbye Svensson ◽  
Malin Sandberg ◽  
Anette Bygum
Keyword(s):  
Case Report ◽  
Family History ◽  
Hereditary Angioedema ◽  
Genetic Disorder ◽  
Upper Airway ◽  
Index Patient ◽  
Cross Border ◽  
The Family ◽  
Life Threatening ◽  
Family Investigation

Hereditary angioedema (HAE) is a rare genetic disorder characterised by recurrent swellings involving subcutaneous and submucosal tissue that can be potentially life threatening in cases involving the upper airway. In this case report, we present a Syrian refugee family with HAE who have lived in Denmark since 2014. The index patient is an 8-year-old girl diagnosed with HAE after being hospitalised in Denmark with an angioedema attack. Her younger sister and father were diagnosed later, following investigation of the family. Exploring the family history, deaths due to suffocation were described in previous generations and other family members based in Sweden, Germany, Turkey, Saudi Arabia, USA and Syria could also potentially be affected. This highlights the need for a cross-border effort to diagnose and treat this inherited disorder.

Assessment on hereditary angioedema burden of illness in Brazil: A patient perspective

2019 ◽  
Vol 40 (3) ◽  
pp. 193-197 ◽  
Author(s):  
Anderson Abdon Barbosa ◽  
Raquel de Oliveira Martins ◽  
Renata Martins ◽  
Anete Sevciovic Grumach
Keyword(s):  
Hereditary Angioedema ◽  
Burden Of Illness ◽  
Family Members ◽  
The United States ◽  
Consent Form ◽  
South American ◽  
Similar Data ◽  
Autosomal Dominant Disorder ◽  
Life Threatening ◽  
Subcutaneous Tissues

Background: Hereditary angioedema (HAE) is an autosomal dominant disorder that affects ∼1 in 50,000 individuals. The disorder is characterized by spontaneous and gradual episodes of edema in subcutaneous tissues or mucosal membrane that may endanger the patient's life. Previous studies that concern HAE treatment and burden of illness in the United States and Europe suggest an improvement in the control of HAE. However, no similar data are available in South American patients. The purpose of the present study was to evaluate the burden of disease and compliance to therapy of patients with HAE in Brazil. Methods: We developed a structured questionnaire to assess the variables that influence the diagnosis and treatment of Brazilian patients with HAE. The questionnaire was evaluated by allergists with expertise in HAE and was reformatted before applying it. The consent form was included before survey access; approval from the ethic committee of Faculdade de Medicina do ABC (Santo André, São Paulo, Brazil) was ensured. The Brazilian association of patients with HAE distributed the survey by e-mail to associated patients all over the country, and responses were accepted within a period of 3 months after signing a consent form. Results: Ninety patients (67 women, 23 men; average age, 36.5 years) responded; the main findings were the following: 53 of 90 (64%) had no need of visiting emergency departments in the past 6 months; 71 of 90 (79%) reported life-threatening fear due to their diagnosis; 73 of 90 (83%) had other family members affected, but 54 of 73 of these affected relatives did not look for specific HAE treatment; and 26 of 90 (29%) reported death due to asphyxia or throat swelling in family members. Conclusion: Patients with HAE report understanding how severe their diagnosis represent, but they did not ponder how important their commitment to treatment may decrease the constant fear brought by the disease in its possible swelling crisis. Family data supported this conclusion.

Speech-Language Pathologists' Perceptions of Their Preparation and Confidence for Providing Dysphagia Services

2020 ◽  
Vol 5 (6) ◽  
pp. 1666-1682
Author(s):  
Lena G. Caesar ◽  
Merertu Kitila
Keyword(s):  
Service Delivery ◽  
Graduate School ◽  
Online Survey ◽  
The United States ◽  
Academic Preparation ◽  
Speech Language Pathologists ◽  
Confidence Levels ◽  
Graduate School Preparation ◽  
Graduate Preparation ◽  
Significant Relationships

Purpose The purpose of this study was to investigate the perceptions of speech-language pathologists (SLPs) regarding their academic preparation and current confidence levels for providing dysphagia services, and the relationship between their perceptions of graduate school preparation and their current levels of confidence. Method This study utilized an online survey to gather information from 374 American Speech-Language-Hearing Association–certified SLPs who currently provide dysphagia services in the United States. Surveys were primarily distributed through American Speech-Language-Hearing Association Special Interest Group forums and Facebook groups. The anonymous survey gathered information regarding SLPs' perceptions of academic preparation and current confidence levels for providing dysphagia services in 11 knowledge and skill areas. Results Findings indicated that more than half of respondents did not feel prepared following their graduate academic training in five of the 11 knowledge and skill areas related to dysphagia service delivery. However, about half of respondents indicated they were currently confident about their ability to provide services in eight of the 11 knowledge and skill areas. Findings also indicated that their current confidence levels to provide dysphagia services were significantly higher than their perceptions of preparation immediately following graduate school. However, no significant relationships were found between respondents' self-reported current confidence levels and their perceptions of the adequacy of their academic preparation. Conclusions Despite SLPs' low perceptions of the adequacy of their graduate preparation for providing dysphagia services in specific knowledge and skill areas immediately following graduation, they reported high confidence levels with respect to their actual service delivery. Implications of these findings are discussed.

School-Based Speech-Language Pathologists' Perceived Self-Efficacy in Conducting Multidimensional Treatment With Children Who Stutter

2020 ◽  
Vol 51 (4) ◽  
pp. 1172-1186
Author(s):  
Carolina Beita-Ell ◽  
Michael P. Boyle
Keyword(s):  
Self Efficacy ◽  
Clinical Training ◽  
Online Survey ◽  
The United States ◽  
Comfort Level ◽  
Social Emotional ◽  
Speech Language Pathologists ◽  
Fluency Disorders ◽  
Cognitive Components ◽  
School Based

Purpose The purposes of this study were to examine the self-efficacy of school-based speech-language pathologists (SLPs) in conducting multidimensional treatment with children who stutter (CWS) and to identify correlates of self-efficacy in treating speech-related, social, emotional, and cognitive domains of stuttering. Method Three hundred twenty randomly selected school-based SLPs across the United States responded to an online survey that contained self-efficacy scales related to speech, social, emotional, and cognitive components of stuttering. These ratings were analyzed in relation to participants' beliefs about stuttering treatment and their comfort level in treating CWS, perceived success in therapy, and empathy levels, in addition to their academic and clinical training in fluency disorders as well as demographic information. Results Overall, SLPs reported moderate levels of self-efficacy on each self-efficacy scale and on a measure of total self-efficacy. Significant positive associations were observed between SLPs' self-efficacy perceptions and their comfort level in treating CWS, self-reported success in treatment, beliefs about the importance of multidimensional treatment, and self-reported empathy. There were some discrepancies between what SLPs believed was important to address in stuttering therapy and how they measured success in therapy. Conclusions Among school-based SLPs, self-efficacy for treating school-age CWS with a multidimensional approach appears stronger than previously reported; however, more progress in training and experience is needed for SLPs to feel highly self-efficacious in these areas. Continuing to improve clinician self-efficacy for stuttering treatment through improved academic training and increased clinical experiences should remain a high priority in order to enhance outcomes for CWS. Supplemental Material https://doi.org/10.23641/asha.12978194

Suicide-Bereaved Individuals’ Attitudes Toward Therapists

Crisis ◽  
2015 ◽  
Vol 36 (2) ◽  
pp. 135-141 ◽  
Author(s):  
Erin F. Ward-Ciesielski ◽  
Madeline D. Wielgus ◽  
Connor B. Jones
Keyword(s):  
Mental Health Treatment ◽  
Online Survey ◽  
The United States ◽  
Limited Attention ◽  
Time Of Death ◽  
Loved One ◽  
Important Group ◽  
Positive Attitudes ◽  
Suicide Bereaved ◽  
Research Domain

Background: Suicide-bereaved individuals represent an important group impacted by suicide. Understanding their experiences following the suicide of a loved one is an important research domain, despite receiving limited attention. Although suicide-bereaved individuals may benefit from mental health treatment, their attitudes toward therapy and therapists are poorly understood. Aims: The present study aimed to understand the extent to which bereaved individuals’ attitudes toward therapy and therapists are impacted by whether their loved one was in therapy at the time of death. Method: Suicide-bereaved individuals (N = 243) from the United States were recruited to complete an online survey about their experience with and attitudes toward therapy and therapists following the suicide of a loved one. Results: Bereaved individuals whose loved one was in therapy at the time of death (N = 48, 19.8%) reported more negative and less positive attitudes toward the treating therapist than those whose loved one was not in therapy at the time of death (N = 81, 33.3%) or whose loved one was never in therapy/the deceased’s therapy status was unknown (N = 114, 46.9%). Conclusion: The deceased’s involvement with a therapist appears to be an important factor impacting the experience of bereaved individuals and should be considered when attempting to engage these individuals in postvention.

Alteraciones sistémicas y metabólicas producidas por lesión medular

2019 ◽  
Vol 1 (1) ◽  
pp. 59-75
Author(s):  
Gabriel Guízar Sahagún
Keyword(s):  
Spinal Cord ◽  
Daily Living ◽  
Autonomic Function ◽  
Clinical Presentation ◽  
Scientific Literature ◽  
International Standards ◽  
Therapeutic Approaches ◽  
Cord Injury ◽  
Life Threatening ◽  
The Impact

Besides the well-known loss of motor and sensory capabilities, people with spinal cord injury (SCI) experience a broad range of systemic and metabolic abnormalities including, among others, dysfunction of cardiovascular, respiratory, gastrointestinal, urinary, and endocrine systems. These alterations are a significant challenge for patients with SCI because such disorders severely interfere with their daily living and can be potentially life-threatening. Most of these disorders are associated with impairment of regulation of the autonomic nervous system, arising from disruption of connections between higher brain centers and the spinal cord caudal to the injured zone. Thus, the higher and more complete the lesion, the greater the autonomic dysfunction and the severity of complications.This article summarizes the medical scientific literature on key systemic and metabolic alterations derived of SCI. It provides information primarily focused on the pathophysiology and clinical presentation of these disorders, as well as some guides to prevent and alleviate such complications. Due to the impact of these alterations, this topic must be a priority and diffuse to those involved with the care of people with SCI, including the patient himself/herself. We consider that any collaborative effort should be supported, like the development of international standards, to evaluate autonomic function after SCI, as well as the development of novel therapeutic approaches.

Learning Electrocardiogram Interpretation: Insights from Residents and a Proposed Solution in an Observational Study (Preprint)

2020 ◽  
Author(s):  
Mohammad Alakchar ◽  
Abdisamad M. Ibrahim ◽  
Mohsin Salih ◽  
Mukul Bhattarai ◽  
Nitin Tandan ◽  
...  
Keyword(s):  
Internal Medicine ◽  
Correlation Coefficient ◽  
Online Survey ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Resident Attitudes ◽  
Internal Medicine Residents ◽  
Life Threatening ◽  
Degree Of Participation ◽  
Electrocardiogram Interpretation

BACKGROUND Interpretation of electrocardiograms (EKG) is an essential tool for every physician. Despite this, the diagnosis of life-threatening pathology on EKG remains suboptimal in trainees. The purpose of this study is to study resident attitudes and behaviours towards EKGs, and describe an innovative way to teach EKGs. OBJECTIVE Study attitudes and behaviours towards EKGs. Describe an innovative way to teach EKGs. METHODS Design: An observational cross-sectional study through an anonymous online survey of resident attitudes and comfort with EKG interpretation. This was followed by creation of a WhatsApp group for discussion and interpretation of EKGs with peers. At the end of the day, the official EKG interpretation was posted. Setting: Internal medicine residency at Southern Illinois University. Participants: Internal medicine residents Interventions: Creation of WhatsApp group to aid with EKG interpretation Measurements: A 17 item questionnaire, followed by detection of degree of participation in a WhatsApp group. RESULTS Forty-one out of 63 residents (65%) completed the survey. 85% of respondents thought that an interactive way to teach EKGs is the best method of teaching, and 73% did not feel confident interpreting EKGs. 30% often rely on automated EKG interpretation. Further analysis indicated that PGY-1 residents reported ordering fewer EKGs (correlation coefficient -0.399, p = 0.012) and were uncomfortable diagnosing QT prolongation on an EKG (correlation coefficient -0.310, p = 0.049). Residents in the third or greater year of training ordered more EKGs (correlation coefficient 0.379, p = 0.015), less frequently relied on the computer for EKG diagnosis (correlation coefficient 0.399, p = 0.010), and were comfortable diagnosing an acute myocardial infarction and atrial arrhythmias. CONCLUSIONS In conclusion, most IM trainees do not feel comfortable interpreting EKG, however, this does improve with PGY year. WhatsApp is a possible platform for teaching EKGs.

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