Gina Lacuesta

Canadian hereditary angioedema guideline

Hereditary angioedema (HAE) is a disease which is associated with random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased Health Related Quality of Life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada is neither optimal nor uniform across the country. It lags behind other countries where there are more organized models for HAE management, and where additional therapeutic options are licensed and available for use. The objective of this guideline is to provide graded recommendations for the management of patients in Canada with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. It is anticipated that by providing this guideline to caregivers, policy makers, patients and their advocates, that there will be an improved understanding of the current recommendations regarding management of HAE and the factors that need to be considered when choosing therapies and treatment plans for individual patients. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency physicians, gastroenterologists, dentists and otolaryngologists, who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.

Insights and advances in chronic urticaria: a Canadian perspective

In the past few years there have been significant advances which have changed the face of chronic urticaria. In this review, we aim to update physicians about clinically relevant advances in the classification, diagnosis and management of chronic urticaria that have occurred in recent years. These include clarification of the terminology used to describe and classify urticaria. We also detail the development and validation of instruments to assess urticaria and understand the impairment on quality-of-life and the morbidity caused by this disease. Additionally, the approach to management of chronic urticaria now focuses on evidence-based use of non-impairing, non-sedating H1-antihistamines given initially in standard doses and if this is not effective, in up to 4-fold doses. For urticaria refractory to H1-antihistamines, omalizumab treatment has emerged as an effective, safe option.

Severe allergic reaction to diethyltoluamide (DEET) containing insect repellent

Case presentation A 53 year-old female bridge inspector presented with allergic reaction to diethyltoluamide (DEET) containing insect repellent. She had prior use without difficulty. In 2013, she used the insect repellent and with only a small amount making contact with her forehead, she had immediate pruritus and erythema on her forehead, persisting for an hour. The following week, she used a different insect repellent and sprayed her face and body. Within minutes, she became diffusely pruritic with generalized urticaria and angioedema of her eyes. She called 911 and was given intramuscular diphenhydramine. Her symptoms gradually eased and she was subsequently well. Her regular medications include venlafaxine and ketorolac. She has no history of atopy. Since the reaction, she has been avoiding all forms of insect repellent, including riding in separate vehicles as her co-workers who use insect repellent. She carries an epinephrine device at all times. Skin testing was performed using two DEET-containing insect repellents: Lloyd’s Bug Spray (23.75% DEET) and OFF Family Care Bug Spray (5% DEET). She had positive skin prick test to both insect repellents, more prominent with the higher containing DEET repellent. There was also a significant reaction on the skin adjacent to the test site where the repellent had not made contact. She developed significant pruritus and was treated with oral anti-histamine. She had appropriate controls. A control subject tested in the office was negative to both insect repellents. Conclusion The patient had a severe allergic reaction to insect repellent, and exhibits sensitization based on skin testing. This represents a unique case of severe cutaneous reaction to insect repellent and such patients may be at risk of anaphylaxis with future exposure.

Canadian Physician Survey on the Medical Management of Hereditary Angioedema.

BACKGROUND Hereditary angioedema (HAE) is a rare disease that has significant morbidity and may be potentially fatal because of airway obstruction. OBJECTIVE To determine practice patterns in physicians treating HAE. METHODS A survey was designed to determine HAE practice patterns among Canadian physicians. These physicians were identified by sending the survey to members of 3 physician organizations (Canadian Hereditary Angioedema Network, Canadian Society of Clinical Immunology and Allergy, and Canadian Hematology Society). RESULTS Thirty-six physicians responded to the survey. Thirty-four physicians were included in the analysis. Most referrals to HAE-treating physicians were from family and emergency department physicians. The most common sites of swelling reported by patients to physicians were facial, peripheral, and abdominal. A mean of 53.9% of patients with type 1 and 2 HAE and 53.4% of patients with HAE with normal C1 esterase inhibitor were undergoing long-term prophylaxis. A mean of 41.9%, 19.4%, and 93.5% of respondents had some patients taking danazol, tranexamic acid, and C1-esterase inhibitor, respectively. Most physicians believed that severity and frequency of attacks were the most important determinants in deciding when to use prophylaxis. A mean of 88.2% of physicians used C1-esterase inhibitor to treat acute attacks and 79.4% used icatibant. All respondents were aware of HAE guidelines. CONCLUSION Physicians are using guidelines to support their practice and using agents suggested by guidelines with confidence. C1 inhibitor is being used widely for prophylaxis and treatment of acute attacks along with icatibant. However, certain special patient populations may require additional focus in future guidelines.

Raw wheat allergy: a case report

Results A 28 year-old female with a history of persistent allergic rhinitis and Arterial Tortuosity Syndrome had two episodes of anaphylaxis following a meal. In each instance she had ingested food cooked in batter. Skin prick tests to all foods ingested during the meals, including commercial wheat extract, were negative. Skin prick testing to wheat flour, white flour, the uncooked batter in question, as well as several commercial pancake mixes, were strongly positive. Our patient did not wish to undergo a double-blinded placebo controlled food challenge due to her underlying medical condition.