A Media Advocacy Toolkit for the Allergist-Immunologist.

For clinicians involved in improving healthcare for patients with allergic and immunologic conditions, advocacy on a broader level through public outreach is key to advancing value-based care. In this article, we provide a toolkit of strategies and resources that can be used to raise public awareness of important issues through various mediums, including podcasts and social media, newspapers, testimonies, presentations, and interviews. A simple approach to effective media interactions is described using the acronym "RATIO", which stands for Research, Audience, Targeted topic, Interview rephrasing, and Optimism. The acronym also reminds the person who is presenting information that only a fraction of what is discussed will be recalled, and an even smaller proportion will be implemented. Key points should be made early. Examples of key talking points are provided for selected topics, including food allergy, anaphylaxis, asthma, rhinitis, and broader healthcare advocacy.

The Impact of Prior Authorization on Clinical Practice and Patient Care Outcomes: A Work Group Report of the AAAAI Prior Authorization Task Force.

The Prior Authorization Task Force of the American Academy of Allergy, Asthma & Immunology (AAAAI), a presidential initiative of David Khan, MD, FAAAI, was established to develop an AAAAI position statement outlining ways to improve health care for our patients, to support legislation that advocates for prior authorization (PA) reform and identify the impact PA has on its membership using a questionnaire survey. This article describes the results of this survey. An electronic anonymous survey questionnaire was developed to assess the impact and burden of PA on AAAAI members and their staff and patients. Surveys were sent to randomly selected members and fellows of the AAAAI in the United States. Descriptive statistics were used to analyze the results by the Information Services team of the AAAAI and the authors of this work group report. The questionnaire responses from allergy immunology specialists demographically reflected the AAAAI membership and indicate that PAs can significantly affect patient care delivery and increase administrative burden to clinical practices, leading to serious adverse events in some circumstances. Differential responses regarding PAs for various medication classes likely reflect the physician's patient population, which can shift prescribing patterns. Prior authorization is a serious health care problem that is wasting financial resources and needlessly placing patients in danger when they are unable to access medications or medical services required for clinical management. The results of this questionnaire study support the recommendations made in the recent AAAAI position statement on PA.

Vaccine-acquired rotavirus in infants with severe combined immunodeficiency.

Live pentavalent human-bovine reassortant rotavirus vaccine is recommended in the United States for routine immunization of infants. We describe three infants, two with failure to thrive, who had dehydration and diarrhea within 1 month after their first or second rotavirus immunization and subsequently received a diagnosis of severe combined immunodeficiency. Rotavirus was detected, by means of reverse-transcriptase-polymerase-chain-reaction (RT-PCR) assay, in stool specimens obtained from all three infants, and gene-sequence analysis revealed the presence of vaccine rotavirus. These infections raise concerns regarding the safety of rotavirus vaccine in severely immunocompromised patients.

Proteinases as molecular adjuvants in allergic airway disease.

BACKGROUND: Asthma and related respiratory tract allergic diseases are among the most common chronic diseases of adults and children. Despite their importance, disease course cannot be predicted and treatment remains non-specific and potentially hazardous, with no means for cure. Improved clinical management of asthma will require an improved understanding of the fundamental factors that initiate allergic inflammation, especially T helper type 2 (T(H)2) cell induction. SCOPE OF REVIEW: In this review, we explore the Proteinase Hypothesis of allergic airway disease, considering specifically how organismal proteinases contribute to the expression of allergic disease and potentially important proteinase signaling pathways. MAJOR CONCLUSIONS: Proteinases from diverse sources (bacteria, fungi, plants) may cause occupational asthma by acting as immune adjuvant factors that specifically elicit T(H)2 cell-dependent allergic inflammation. However, more conventional allergic airway diseases (asthma, allergic sinusitis) are more likely to arise from contained fungal or viral infections of the airway in which proteinases are produced and serve as major virulence factors. Proteinases may elicit allergic disease by disrupting numerous cellular proteins, potentially including Toll like receptor (TLR) 4, but critical proteinase-activated signaling pathways remain largely unknown. GENERAL SIGNIFICANCE: Clarification of how proteinases cause allergic disease, specifically confirming an infectious basis for airway proteinase exposure, will likely radically advance how asthma and related respiratory tract disorders are diagnosed and treated. This article is part of a Special Issue entitled Biochemistry of Asthma.

Outcomes of patients with severe combined immunodeficiency treated with hematopoietic stem cell transplantation with and without preconditioning.

BACKGROUND: The effect of pretransplantation conditioning on the long-term outcomes of patients receiving hematopoietic stem cell transplantation for severe combined immunodeficiency (SCID) has not been completely determined. OBJECTIVE: We sought to assess the outcomes of 23 mostly conditioned patients with SCID and compare their outcomes with those of 25 previously reported nonconditioned patients with SCID who underwent transplantation. METHODS: In the present study we reviewed the medical records of these 23 consecutive, mostly conditioned patients with SCID who underwent transplantation between 1998 and 2007. RESULTS: Eighteen patients (median age at transplantation, 10 months; range, 0.8-108 months) received haploidentical mismatched related donor, matched unrelated donor, or mismatched unrelated donor transplants, 17 of whom received pretransplantation conditioning (with 1 not conditioned); 13 (72%) patients engrafted with donor cells and survive at a median of 3.8 years (range, 1.8-9.8 year); 5 (38%) of 13 patients require intravenous immunoglobulin; and 6 of 6 age-eligible children attend school. Of 5 recipients (median age at transplantation, 7 months; range, 2-23 months) of matched related donor transplants, all 5 engrafted and survive at a median of 7.5 years (range, 1.5-9.5 year), 1 recipient requires intravenous immunoglobulin, and 3 of 3 age-eligible children attend school. Gene mutations were known in 16 cases: mutation in the common gamma chain of the IL-2 receptor (IL2RG) in 7 patients, mutation in the alpha chain of the IL-7 receptor (IL7RA) in 4 patients, mutation in the recombinase-activating gene (RAG1) in 2 patients, adenosine deaminase deficiency (ADA) in 2 patients, and adenylate kinase 2 (AK2) in 1 patient. Early outcomes and quality of life of the previous nonconditioned versus the present conditioned cohorts were not statistically different, but longer-term follow-up is necessary for confirmation. CONCLUSIONS: Hematopoietic stem cell transplantation in patients with SCID results in engraftment, long-term survival, and a good quality of life for the majority of patients with or without pretransplantation conditioning.

Work Group Report: COVID-19: Unmasking Telemedicine.

Telemedicine adoption has rapidly accelerated since the onset of the COVID-19 pandemic. Telemedicine provides increased access to medical care and helps to mitigate risk by conserving personal protective equipment and providing for social/physical distancing to continue to treat patients with a variety of allergic and immunologic conditions. During this time, many allergy and immunology clinicians have needed to adopt telemedicine expeditiously in their practices while studying the complex and variable issues surrounding its regulation and reimbursement. Some concerns have been temporarily alleviated since March 2020 to aid with patient care in the setting of COVID-19. Other changes are ongoing at the time of this publication. Members of the Telemedicine Work Group in the American Academy of Allergy, Asthma & Immunology (AAAAI) completed a telemedicine literature review of online and Pub Med resources through May 9, 2020, to detail Pre-COVID-19 telemedicine knowledge and outline up-to-date telemedicine material. This work group report was developed to provide guidance to allergy/immunology clinicians as they navigate the swiftly evolving telemedicine landscape.

Hemophagocytic lymphohistiocytosis in a patient with x-linked lymphoproliferative disease.

X-linked lymphoproliferative disease (XLP) is a primary immunodeficiency affecting approximately 1 to 3 per million live male births. Patients are generally healthy until facing a viral infection such as Epstein-Barr Virus and then may develop fulminant infectious mononucleosis and die. XLP patients are also at increased risk of hemophagocytic lymphohistiocytosis (HLH), which may be triggered by assorted viruses. Here we report a novel case of HLH in a patient with XLP. Significant to his presentation is a paradoxical increase in natural killer (NK) cell activity. We hypothesize that this indicates that Parvovirus B19 activates NK cells via a signaling lymphocytic activation molecule-associated protein (SAP)-independent mechanism. Our case demonstrates an important etiology to consider in the differential diagnosis of XLP patients with nonfocal findings and febrile illnesses.

Long-term outcomes of nonconditioned patients with severe combined immunodeficiency transplanted with HLA-identical or haploidentical bone marrow depleted of T cells with anti-CD6 mAb.

BACKGROUND: Between 1981 and 1995, 20 children with severe combined immunodeficiency (SCID; median age at transplant, 6.5 [range, 0.5-145] mo, 12 with serious infection) were treated with haploidentical T cell-depleted (anti-CD6 antibody) bone marrow (median number of 5.7 [0.8-18.8] x 10(8) nucleated cells/kg) from mismatched related donors (MMRDs), and 5 children with SCID (median age at transplant, 1.8 [0.5-5.0] mo, 1 with serious infection) were given unmanipulated bone marrow from matched related donors (MRDs). No conditioning or graft-versus-host disease (GvHD) prophylaxis was used. OBJECTIVE: To assess the outcomes of patients with SCID who received bone marrow from MMRDs or MRDs. METHODS: We reviewed the medical records of these 25 consecutive patients with SCID (4 with Omenn syndrome). RESULTS: Of the 20 patients who received bone marrow from MMRDs, 12 engrafted, 10 survived at a median age of 15.2 [10.0-19.1] years, 4 had chronic GvHD (lung, intestine, skin), 5 required intravenous immunoglobulin, and 8 attended school or college. Two of 5 patients who died had chronic GvHD, and 2 developed lymphoproliferative disease. Of the 5 patients who received bone marrow from MRDs, 5 engrafted, 5 survived at a median age of 23.3 [18.5-26] years, 1 had chronic GvHD (lung, skin), 2 required intravenous immunoglobulin, and 4 attended school or college. CONCLUSIONS: Treatment of critically ill patients with SCID with anti-CD6 antibody T cell-depleted MMRD marrow resulted in an overall 50% long-term survival of patients (83% survival of those engrafted). The principal barriers to long-term survival were delay in diagnosis, life-threatening infection, failure to engraft, and chronic GvHD. Educational goals were achieved in most of the survivors.

Reducing Environmental Allergic Triggers: Policy Issues.

The implementation of policies to reduce environmental allergic triggers can be an important adjunct to optimal patient care for allergic rhinitis and allergic asthma. Policies at the local level in schools and other public as well as private buildings can make an impact on disease morbidity. Occupational exposures for allergens have not yet been met with the same rigorous policy standards applied for exposures to toxicants by Occupational Safety and Health Administration. Further benefit may be obtained through policies by local, county, state, and national governments, and possibly through international cooperative agreements. The reduction of allergenic exposures can and should be affected by policies with strong scientific, evidence-based derivation. However, a judicious application of the precautionary principle may be needed in circumstances where the health effect of inaction could lead to more serious threats to vulnerable populations with allergic disease. This commentary covers the scientific basis, current implementation, knowledge gaps, and pro/con views on policy issues in reducing environmental allergic triggers.

Allergens in school settings: results of environmental assessments in 3 city school systems.

Environmental allergens are major triggers for pediatric asthma. While children's greatest exposure to indoor allergens is in the home, other public places where children spend a large amount of time, such as school and day care centers, may also be sources of significant allergen encounters. The purpose of this article is to describe schoolroom allergen levels from 3 different geographic sites obtained from dust samples collected in the fall and in spring. Environmental dust samples were collected from elementary schools in Birmingham (AL), Detroit (MI), and Houston (TX), from 4 room locations, including the cafeteria, library, upper grades, and lower grades. Samples were assayed for dust mite (Dermatophagoides pteronyssinus and Dermatophagoides farinae), cat (Felis domesticus), and cockroach (Blatella germanica 2) allergen levels. Allergen levels varied by geographic location and type of schoolroom. Schoolroom settings differed by the type of flooring (hard and carpet), room characteristics and use (food service, library shelves with books, and general classroom with multiple types of materials [individual desks and different types of furniture]), and the average age of the schoolroom dwellers (younger vs. older children). Dust mite, cat, and cockroach allergens were present in all schoolrooms and all sites at varying levels by season and by type of room. Schools may be important sources of direct allergen exposure and reservoirs that could potentially contribute to allergic sensitization and disease exacerbation in children. Further studies are needed to carefully examine the environmental allergen load in schools and its effect on children.

Partners in school asthma management: evaluation of a self-management program for children with asthma.

The "Partners in School Asthma Management" program for inner-city elementary school children comprises (1) case finding; (2) linkage of school nurses, parents, and clinicians; (3) a computer-based tailored educational program; and (4) school environmental assessment and intervention. Case finding identified 1730 children in 60 elementary schools with probable asthma; 835 (96% Hispanic or African American) joined the study. Baseline, posttest, and follow-up measures of asthma knowledge, self-efficacy, and self-management behavior were obtained from the children, and data on symptoms, emergency department visits, and hospitalizations were obtained from their parents. The schools provided data on grades and absences. Each school had a baseline and follow-up environmental assessment. The children in the intervention group showed greater increases in knowledge, self-efficacy, and some aspects of self-management. No differences between groups were found in health status variables, school performance, attendance, or levels of environmental allergens in schools. In 15 schools, an enhanced intervention allowed children and their parents to meet with a project physician, develop an asthma action plan, and receive a 1-month supply of medication; the project physician then followed up with the child's community physician. Children participating in this enhanced intervention had better school performance and fewer absences than the comparison group. Overall, the program was effective in improving children's asthma self-management but not in improving their health status. While the case-finding, computer-based self-management training program and linkage system were successfully implemented, the program failed in creating needed changes in the medical (action plans by community physicians) and physical environments (reduced school allergen levels) of the children.

Computer-based decision support for pediatric asthma management: description and feasibility of the Stop Asthma Clinical System.

Clinical guidelines can assist in the management of asthma. Decision support systems (DSSs) can enhance adherence to clinical guidelines but tend not to provide clinicians with cues for behavioral change strategies to promote patient self-management. The Stop Asthma Clinical System (SACS) is a DSS designed for this purpose. To assess feasibility, seven clinicians used SACS to guide well visits with 26 predominantly persistent pediatric asthma patients. Data were collected via survey and in-depth semi-structured interviews. SACS improved assessment of asthma severity and control, classification of and intervention in medicine and environmental trigger management problems, and development of an action plan (all p < 0.05). Clinician-patient communication was enhanced. The primary challenge was that SACS increased clinic visit time. SACS can enhance clinician behavior to improve patient asthma self-management, but more studies are indicated to mitigate temporal constraints and evaluate impact on clinician and patient communication and behavior as well as clinical outcomes.

Indoor Environmental Control Practices and Asthma Management.

Indoor environmental exposures, particularly allergens and pollutants, are major contributors to asthma morbidity in children; environmental control practices aimed at reducing these exposures are an integral component of asthma management. Some individually tailored environmental control practices that have been shown to reduce asthma symptoms and exacerbations are similar in efficacy and cost to controller medications. As a part of developing tailored strategies regarding environmental control measures, an environmental history can be obtained to evaluate the key indoor environmental exposures that are known to trigger asthma symptoms and exacerbations, including both indoor pollutants and allergens. An environmental history includes questions regarding the presence of pets or pests or evidence of pests in the home, as well as knowledge regarding whether the climatic characteristics in the community favor dust mites. In addition, the history focuses on sources of indoor air pollution, including the presence of smokers who live in the home or care for children and the use of gas stoves and appliances in the home. Serum allergen-specific immunoglobulin E antibody tests can be performed or the patient can be referred for allergy skin testing to identify indoor allergens that are most likely to be clinically relevant. Environmental control strategies are tailored to each potentially relevant indoor exposure and are based on knowledge of the sources and underlying characteristics of the exposure. Strategies include source removal, source control, and mitigation strategies, such as high-efficiency particulate air purifiers and allergen-proof mattress and pillow encasements, as well as education, which can be delivered by primary care pediatricians, allergists, pediatric pulmonologists, other health care workers, or community health workers trained in asthma environmental control and asthma education.

Environmental allergens and irritants in schools: a focus on asthma.

As part of the Partners in School Asthma Management Program, environmental data were collected from 385 rooms in 60 elementary schools in southeast Texas, using an Environmental Observation Checklist and a Q-TRAK Indoor Air Quality Monitor. Dust samples for allergen analysis were collected from floors, carpets, and area rugs in 80 classrooms in a subset of 20 schools. CO2 levels > 1,000 ppm were found in 86% of rooms; 69% had indoor humidity above recommended levels. Der p I dust mite allergen levels > 2,000 ng/g were present in 20% of rooms, but only 2.5% of rooms had Der f I mite allergen levels exceeding recommended tolerances. Detectable levels of cockroach allergen (Bla g II) were found in all schools (median 5.5 ng/g), with 10% of rooms over the recommended threshold. Almost two-thirds of classrooms had mold spore counts > 10,000 col/g (median, 14,400 col/g; range, 2,000-52,000 col/g).

A 5-week-old HIV-1-exposed girl with failure to thrive and diffuse nodular pulmonary infiltrates.

A 5-week-old female infant with vertical HIV-1 exposure, progressive cough, and failure to thrive was given a diagnosis of bilateral diffuse nodular lung lesions. The child was without fever, leukocytosis, anemia, peripheral adenopathy, or hepatosplenomegaly, and the results of repeated blood tests for HIV-1 DNA were negative. A needle biopsy of the lungs revealed granulomatous inflammation and giant cells, with fungal organisms suggestive of Aspergillus species. A nitroblue tetrazolium dye test performed on the patient's blood specimen demonstrated absence of dye reduction, suggesting a diagnosis of chronic granulomatous disease. Further analysis revealed that the child had a deficiency of the p47(phox) component of the nicotinamide adenine dinucleotide phosphate oxidase system. Thus this child with vertical HIV-1 exposure and diffuse pulmonary nodules actually had an autosomal recessive form of chronic granulomatous disease. This case study clearly demonstrates that children with suspected HIV-1 infection might also need evaluation for primary immunodeficiency and that the clinical immunology laboratory is a powerful adjunct in coming to a correct diagnosis.