“Is there a recommended protocol patients should work with their primary physicians to set up in case of an emergency?”

Mar 22, 2016

Dr. C: Happy Spring to everyone. This is a topic that is critical to consider for all patients with HAE. As in all things we need to “hope for the best, plan for the worst”. As all patients can experience unpredictable attacks of swelling it is recommended that everyone have two doses of effective therapy for on demand treatment. All patients are also at risk of a life threatening laryngeal attack and need to have a plan of action. We recommend that a written emergency plan be constructed in consultation between the patient and their doctor. The basic outline should include what and how medication is to be taken, who will administer, who to contact in the event of an emergency and where to go. We give all of our patients an emergency card to have with them and take to the ED which includes information on HAE, what are and are not effective therapies for HAE and what the on demand treatment is, and if relevant the prophylactic program for the individual patient. The HAEA is also a valuable resource here with 24-hour call in to speak with someone to assist with coordination of emergency care. Marc, what approach do you recommend for our reader?


Dr. R: Establishing an effective acute treatment plan for managing HAE attacks is the “first rule” of overall HAE treatment. However, there’s no specific protocol that can be recommended as each person’s situation has unique factors to consider. HAE treatment is best individualized so sitting down with your HAE specialist to discuss these details is important. The basic steps to cover include: 1) Selecting an acute medication to treat attacks. 2) Ensuring you have reliable access to that medication – usually this means having the medication in your possession, though occasionally medication will be stored at a treatment site such as a hospital or clinic. 3) Knowing how to administer the medication – this may be self-administration in many cases, but might also involve receiving treatment assistance from your physician, a nurse, or a family member/friend. 4) Knowing exactly where to go in case of any emergency such as an airway attack, difficulty giving the medication, or lack of an adequate response to the medication. This “back-up” plan is important to develop ahead of time. 5) Knowing who to contact if you have questions or need advice about your treatment plan. 6) Knowing how to refill your medications efficiently once they are used to treat an attack. It’s also a good idea to have a letter or wallet card from your HAE physician that can be shared with other physicians (such as Emergency staff) outlining the basics of your condition and recommended treatment plan as well as HAE specialist contact info. This can sometimes facilitate better communication and avoid misdiagnoses and ineffective treatments in the event of an emergency.


Dr C: Thank you Marc. I think that the key message here is to be prepared and think things through in advance. Bruce, do you have any additional thoughts on how to approach developing these strategies?


Dr Z: I think that you have covered this ground well. I’d like to reinforce the point that every HAE patient is at risk of having a severe attack. It’s human nature to become complacent when things are going well; however, HAE is an unpredictable disease. Being prepared can make the difference between an attack being a nuisance versus it becoming a catastrophe. It’s also worth reiterating that it is a good idea to have a physician who is knowledgeable and experienced in managing HAE be involved in your care. Our philosophy at the US HAEA Angioedema Center at UC San Diego is that medical care can be layered, allowing expert HAE physicians to work with primary physicians to deliver the best possible care for patients.


Dr. C: Thank you Bruce and Marc—I hope that our discussion was helpful for our reader and followers with HAE. We look forward to hearing from you and our next “Question of the Week”.


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