QOTW “With the currently available therapies for HAE do I need to have pain medication on hand as part of my management program?”

Jan 19, 2016

Dr. C: With the exception of children with HAE we unfortunately all remember when pain medication was one of the mainstays of treatment to ease the suffering from swelling. For abdominal attacks narcotics and fluid support were all we had for acute care. Fresh Frozen Plasma was the only therapy with the potential to arrest an attack but had the potential to unpredictably accelerate the swelling. One of the many dark sides for patients with HAE was being accused of “drug seeking behavior” when they would present for care. Some patients would also become addicted to the medications as a consequence of frequent use. Fortunately the current treatment options have largely erased the need to treat with pain medications. The approved on demand treatments such as Kalbitor, Firazyr, plasma derived or recombinant C1 inhibitor can arrest an attack and prevent pain. As we have emphasized in the past it is important to treat early. There can be instances however where either the drugs were not available or administered too late. These situations are best handled in a setting where additional measures such as fluids are available and if needed pain medication. In answer to our reader’s question, I do not think that patients need to have pain medication on hand—all should have effective on demand treatment. If the treatment is not working this is best handled in a medical facility. Marc, what is your view on this topic?


Dr. R: There was a time not long ago when pain medication was a mainstay of treatment for HAE. This was before the availability of proven, effective HAE-specific medications that stop the swelling process. During these many years without better treatment, we saw the numerous difficulties with using pain medications repeatedly for HAE attacks, particularly the group of medications known as opioids or narcotics. Some of the more commonly prescribed drugs in this class are morphine, hydromorphone (dilaudid), oxycodone (percocet or oxycontin), and fentanyl. These drugs don’t change the underlying swelling process or shorten the duration of the attack – they just cover up the symptoms for a few hours at a time. They also have substantial short and long term adverse side effects. In the short run: sedation, constipation, low blood pressure, seizures, respiratory depression, and allergic reactions. These things are pretty common with some of the more potent pain meds. In the long run, tolerance to opioids can develop over time, which means that more and more pain medication is needed to accomplish the same level of pain relief. In addition, physical dependency on opioids can develop with frequent use – this is a biochemical process but can lead to complications of addictive behaviors. This latter issue isn’t that common in HAE but it’s very real and even today we unfortunately see it occurring occasionally in individuals with HAE. With all these issues of the past in mind, concerted efforts have been made to generally move away from pain medications as a mainstay of HAE treatment, and fortunately advances in medical treatment have improved the options. There may still be a role for pain medication used sparingly in some situations, such as when the use of HAE-specific medication is delayed for some unexpected reason. But we have to be very careful and judicious in these situations, and relying heavily on pain medication as a routine part of an HAE-management plan is risky and typically unnecessary. An optimized acute management plan with the reliable early use of HAE medication should allow for quick relief of the HAE-associated swelling and pain such that pain meds aren’t usually necessary. Each person is different and each treatment plan should be individualized accordingly with their HAE-specialist. For now, HAE is a lifelong chronic condition and we have to keep that long view in mind for each person including the potential long term side effects of medications used regularly – narcotics may have a real down side in this regard.


Dr C: Thank you Marc. It sounds like we are in agreement on this point. Bruce, do you have some thoughts here? I would be particularly interested in cases of HAE-nl-C1 inhibitor without a factor XII mutation. As you know we have no diagnostic test only clinical criteria. I have found it to be helpful in the process of establishing the diagnosis to determine the response to available on demand therapies. For patients that are not getting reliable relief I am always concerned that we need to look for alternative or comorbid conditions that may provide an explanation. The use of pain medications may obscure symptoms from other causes of abdominal pain in particular and thereby jeopardize the health of the patient.


Dr. Z: First, let me agree with both of you that pain medicines ought to be treatment of last resort for HAE attacks. The goal of treatment should be to prevent swelling from causing any significant problem, including pain. This can be accomplished either by on-demand treatment given early in an attack or by prophylactic treatment. In most cases, therefore, the need for opioid pain medicines in the management of HAE represents a failure of our treatment plan. On the other hand, failure of the newer on-demand medicines to stop an HAE attack should alert the physician to the possibility that the diagnosis of HAE might be incorrect. I intentionally hedge my language here because we recognize that there is considerable individual variability in the response to a given drug. Some HAE patients may not respond well to one on-demand drug but will respond very well to another. The issue we face with HAE-nl-C1INH is, as usual, more complicated. In this case, we are much less certain that the on-demand medicines will work quickly and well. Nevertheless, as you indicated, we do use the response to these on-demand medicines as one small part of the data we analyze to help us determine whether the diagnosis is likely to be HAE-nl-C1INH without a F12 mutation. Administering opioids will obscure the impact of the on-demand medicine, and make it much harder to evaluate the response to the medicine. Like you, I always worry that we could be covering up a serious but unrelated problem when we have to give opioid pain medicines.


Dr. C:Thank you Bruce and Marc—I hope 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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