The beginning of school or a major holiday is often the time when parents discover that they not only have a child with allergies, but that the allergy is serious enough to cause death. This then is their first contact with anaphylaxis. Derived from the Greek and meaning ‘without protection’, anaphylaxis implies that the body’s natural defence systems are lowered. This can be fatal. Anaphylaxis has been known to occur in infants as young as 2 weeks and in the elderly at age 79.1
Symptoms of anaphylaxis are shown in Figure 1.
Epinephrine should be used immediately to treat anaphylaxis. Antihistamines and glucocorticoids are not frontline treatments.1 Individuals with anaphylaxis must be taught to use an epinephrine autoinjector and to seek emergency medical assistance immediately.
The risk factors for anaphylaxis include age, current physiologic state, concomitant diseases, current medications and co-factors.
For children, food allergy is the most common trigger for anaphylaxis while themost common source of allergens includes insect stings, exercise, latex and medication. Other common food triggers are peanuts, tree nuts, milk, eggs, wheat, corn, beef, pork, fish, shell fish, chocolate, soy and food additives. Gelatin and sesame can be added to the list. However an individual may have a severe life-threatening allergy to any food.
Consider the effect on the family of a child with such a potentially life-threatening condition. Caution has to be carefully balanced with the need for a normal daily life. The constant fear of an anaphylactic reaction is likely to cause parents to become strict, cautious and fearful. Sicherer2 and colleagues surveyed 253 parents of children (range 5 - 18 years, mean 10.8 years) with food allergies. 59% of the children were male. 68% were allergic to one or two foods with 32% allergic to more than two foods. They noted a significant reduction in general health perception, high emotional impact on the parent and an increased limitation on family activities. Concomitant diseases included
- 13% with atopic dermatitis
- 33% with asthma
- 33% with asthma and atopic dermatitis
At risk for anaphylaxis are individuals
- with severe, uncontrolled asthma
- allergic to insect (hymenoptera) venom
- with systemic mastocytosis
- on Xolair
- undergoing immunotherapy – whether subcutaneous or sublingual
- who take NSAIDs
Health care workers, individuals with spina bifida or genitourinary abnormalities are also at high risk for anaphylaxis from latex exposure. There is also the risk of occupational exposure to latex which can result in anaphylaxis.3
When Jerschow and colleagues4 analyzed death certificates from the US National Mortality Database, they found the following causes of death due to anaphylaxis:
- 58.8 % from medication
- 15.2% from venom
- 6.7% from food
- 19.3% from other unspecified causes.
Where medications were identified, of the 2,458 deaths from anaphylaxis between 1999 and 2010, nearly 50% were due to antibiotics, followed by radio contrast media for diagnostic imaging; and chemotherapeutics for cancer treatment.
Cofactors of Anaphylaxis
- exercise (after eating certain foods or in the presence of certain foods)
- emotional stress
- acute infection including upper respiratory tract infections
- premenstrual status6
In adolescents, cofactors may include lack of adherence to asthma medication regimen, denial of symptoms, exercise, fasting, and delay in seeking help.
Anaphylaxis is a risk factor in the elderly, particularly those
- allergic to hymenoptera venom
- with pre-existing cardiovascular disease, or
- with mast cell disorder, or
- taking either beta-adrenergic blockers or ACE inhibitors
A retrospective review7 of 601 cases of anaphylaxis found that 62% were female (more females than males are anaphylactic); 22% were due to food; 11% due to medication and 5% to exercise. It should be noted that in most cases the cause of anaphylaxis cannot be determined, and that over time and with increasing age, anaphylactic episodes reduce in severity and frequency.
There are currently a variety of epinephrine autoinjectors available. These include the EpiPen, Adrenaclick and Auvi-Q (Allerject in Canada). Instructions for their administration can be found at the respective websites.
Parents and Anaphylaxis
Patients and parents of children with severe allergies are reluctant for many reasons to use the epinephrine autoinjector. And herein lies the great difficulty with anaphylaxis: this reluctance results in increased morbidity and risk of death. Some parents are hindered by the thought of injecting their child; others by the fear of hurting the child; others think it unnecessary and many do not understand the severity of the reaction that can lead to death.
Pouessel et al.8 contacted 152 families of children with food allergies that included 111 children with a mean age of 6.5 years. Using questionnaires, they noted that 76% of parents were taught with a training device and 49% were either taught or given written instructions. However, when asked to list symptoms that required the used of the epinephrine autoinjector, only 48% were able to list more than one response; 54% of the children had an anapylaxis “plan” and only 60% of the children had a complete emergency kit that included epinephrine, inhaled beta-agonist, corticosteroid and anti-histamine. Even the children with asthma had often forgotten their beta agonist at school. Pouessel concluded that the use of an epinephrine autoinjector was “inadequately demonstrated” and that more was required in the form ofinformation, educational programs, and follow-up.
100 mothers were taught how to use EpiPens and Anapens. In a follow-up, researchers found that 15% of them could not use the devices correctly despite a one-on-one demonstration; 4% could not fire the Anapen; and an alarming 26% could not activate the EpiPen. The researchers strongly recommended more training.9
Parental discomfort with epinephrine autoinjector has been shown as a factor in parents’ reluctance to use them. A survey of 165 parents of children who had been prescribed an EpiPen found that while 42% of children had had an anaphylactic episode, only 8% of parents administered the EpiPen. The researchers10 found that knowledge did not provide the essential degree of comfort. Fear was the most likely contributing factor that paralyzed and prevented parents from taking the appropriate action. The factors that enhanced comfort included
- prior administration of an EpiPen
- training in the use of the EpiPen
Parental feelings of empowerment increased the likelihood of appropriate use of the EpiPen. Hence repeated training is a requirement and should be given at every opportunity.
It is absolutely essential that patients at risk of anaphylaxis, such as those with severe or uncontrolled asthma,
- be educated as to the seriousness of the reaction
- be taught avoidance measures
- be prescribed an epinephrine autoinjector
- be taught how to use it
- engage in role playing with a placebo device in the presence of an educator
- have a written emergency plan
- obtain medical identification (bracelet, wallet card)
- be reminded to elevate the lower extremities or to lie down during an episode. (Sudden death can occur in a patient who stands or sits suddenly.)
Patients do need to be taught to use the epinephrine autoinjector when symptoms are severe and if they are in doubt as to the severity of the reaction. The slogan “When in doubt, DO” is a helpful reminder to use the epinephrine autoinjector and get professional medical assistance. The message and the training must be repeated at every opportunity to reinforce self-efficacy and to affirm the parent’s ability to take the requisite action when needed.
- Simons FER, Ardusso LRF, Bilo MB et al. International consensus on (ICON) anaphylaxis. World Allergy Organization Journal 2014, 7:9doi:10.1186/1939-4551-7-9
- Sicherer SH, Noone SA and Munoz-Furlong A. The impact of childhood food allergy on quality of life. Ann Allergy Asthma Immunol. 2001; 87: 461–464
- Simons FE, Ardusso LRF et al. World Allergy Organization Guidelines for the Assessment and Management of Anaphylaxis. Curr Opin Allergy Clin Immunol. 2012;12(4): 389-99
- Jerschow E, Lin RY et al. Fatal anaphylaxis in the United States, 1999-2010: Temporal patterns and demographic associations. JACI, 2014; 134(6):1318-1328.e7 DOI: 10.1016/j.jaci.2014.08.018
- Simons FE, Ebisawa M, et al. 2015 update of the evidence base: World Allergy Organization anaphylaxis guidelines. World Allergy Organ J. 2015 Oct 28;8(1):32. doi: 10.1186/s40413- 015-0080-1. eCollection 2015.
- Burstein M, Rubinow A, Shalit M. Cyclic anaphylaxis associated with menstruation. Ann Allergy. 1991 Jan;66(1):36-8.
- Webb LM, Lieberman P. Anaphylaxis: a review of 601 cases. Ann Allergy Asthma Immunol. 2006 Jul;97(1):39-43.
- Pouessel G, Deschildre A, et al. Parental knowledge and use of epinephrine auto-injector for children with food allergy. Pediatr Allergy Immunol. 2006 May;17(3):221-6.
- Brown J, Tuthill D,et al. A randomized maternal evaluation of epinephrine autoinjection devices. Pediatr Allergy Immunol. 2013 Mar;24(2):173-7. doi: 10.1111/pai.12048.
- Kim JS, Sinacore JM, Pongracic JA. Parental use of EpiPen for children with food allergies. JACI 2005; 116(1): 164-68DOI: http://dx.doi.org/10.1016/j.jaci.2005.03.039