“How do I persuade my partner to treat early with C1 inhibitor in case of abdominal pain? I know it is a HAE symptom but he always wants to wait and see.” 

Jul 20, 2016

Dr. C: This is a deceptively simple question. It is difficult to provide a precise answer lacking some additional background information. How often is your partner correct to wait? Are there certain symptoms that predictable for an HAE attack? Are there other confounding health conditions?
How do we convince a loved one to do anything even if it involves their best interest? Facts are often a helpful starting point. As we have emphasized before in discussions of treatment the medications that we have are effective in arresting the attack of swelling. They do not accelerate the resolution. This translates into the longer fluid is leaking out the longer it will take for an attack to resolve. With abdominal attacks delayed treatment equals more pain and misery for the patient. This is compounded by disruption of the attack in every other sector of their life such as work, school or recreation. The other concern is what are the barriers for using the C1 inhibitor? Does your partner not feel comfortable infusing? Are they worried whether they will be able to get sufficient amounts of the drug to have on hand if they have more severe or life threatening symptoms? Have they been made to feel guilty over using such an expensive medication?
I would encourage you to have your partner discuss their treatment plan with an HAE specialist who would be equipped to address these issues.
Marc, what is your advice for our reader?

Dr R: Treating HAE attacks early with effective medication is one of the most important steps in optimizing the acute treatment plan. I hear from patients that abdominal attacks are usually the most difficult to judge, as early on, it can sometimes be difficult to tell whether mild abdominal discomfort is the early stages of angioedema or some other condition such as acid reflux or an upset stomach from a meal. There’s many possible causes for abdominal symptoms, making this more difficult to judge compared to skin swelling or airway symptoms. That challenge recognized, we have good clinical studies showing the clear benefit of treating HAE attacks early – the earlier we treat, the faster people get better, and the less time suffered with angioedema symptoms. So while it’s quite important and ‘easy’ to recommend treatment early in an attack, the difficult part is for each person with HAE to be in tune with her/his body and symptoms such that they can best judge when an angioedema attack is starting. As soon as one strongly suspects the symptoms are HAE, that’s the time to treat with medication. Waiting to see how severe any attack might get will often lead to problems – once the attack becomes advanced, the medications are unlikely to work as quickly leading to hours or more of debilitating symptoms. The other point I’d emphasize is that IF abdominal pain and symptoms DON’T respond to the FDA-approved HAE medications, then it’s very important to get evaluated at the hospital. The modern acute HAE medications are so effective, that if they don’t provide relief, then we need to be concerned that some other non-HAE process is occurring. Every once in awhile, other serious intra-abdominal medical problems occur – appendicitis, gall bladder issues, pancreatitis, etc. – and we don’t want to miss those things. It’s important to avoid ‘tunnel vision’ whereby we attribute any and all issues to HAE. We become most concerned about these other conditions when symptoms are resistant to the early use of HAE-specific medications.

Dr C: Thank you Marc. Anything to add, Bruce?

Dr Z: The three of us are in complete agreement on this topic. Once the swelling starts, treat as soon as possible. I’ll venture into an area, however, where some physicians do have a disagreement. This relates to whether to treat when you are experiencing a prodrome rather than an actual attack. As much as we encourage patients to treat early in an attack, I’m very reluctant to give treatment during a prodrome. Most prodromal symptoms are relatively nonspecific and we don’t have information about the probability that the symptom will be followed by an attack. People remember when a symptom was followed by an attack, but they don’t remember the times they experienced a transient symptom that was not followed by an attack. Having said that, we recognize that there are always exceptions. If a patient experiences a prodromal symptom that she/he knows with complete certainty will lead to an attack (such as erythema marginatum in some patients), then a good argument can be made for treating at the time of the symptom.

Dr C: Thank you Bruce and Marc. I hope that this discussion provided some help for our reader and followers with HAE. We look forward to hearing from you and our next QOTW.

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