This person has classic NSAID-induced angioedema.
Samter's triad is perfect here; we learned the aspirin-asthma-nasal polyps triad in med school in the 70s.
In the susceptible person, NSAIDs induce airway edema that may be non-asthmatic (doesn't involve airway muscle contracture so doesn't respond to inhalers.) They cause the airways to directly swell due to blocking a controller molecule; in essence, allowing unchecked swelling. See images on your favorite search engine to understand why this disorder is one of the few that gets an ER doc's heart going.
This sensitivity is shared across all NSAIDs but not acetaminophen (because it's not an NSAID. That's a topic for another day.)
PAs know this; at least the ones I teach do!
An old ER doc
11
Does the failure of treatment short of Dupilumab suggest this patient suffered more than mere AERD? Rather, a more advanced, generalized Mast Cell ("Activation") Disorder -- sometimes referred to MCAD or MCAS? Might Dupilumab help in treating however many MCAD patients? Have doctors yet to even investigate this?
I have had asthma my whole life. My mother knew something was wrong when I was about 18 months old. We found a pediatrician that recognized the symptoms immediately. I am now 56 and have controlled my asthma very well by following a strict regimen my entire adult life. Nothing makes me crazier than when I read that a grown woman doesn't use the inhalers prescribed to her. People, take control of your health. Ask questions. Follow your doctor's instructions. Be your own advocate. Asthma can kill you if you don't take it seriously. And many adults I know suddenly develop asthma, or exercise induced asthma. We are never exactly sure why this happens. But pay attention to your body and for god's sake take your inhaler.
6
@Erin It might be because inhalers are around $300 each.
10
Really fascinating! And very well explained case. Thank you!
3
NSAIDs can alter human pheromone recognition and should be used sparingly or not be prescribed for anyone building a bond, parental or couple.
NSAIDs remarkable capabilities (some can be insecticidal) and origin as anti-herbivory agents all suggest their anti-pheromonal activity.
@Bubba Nicholson
Fascinating! Do you have a link to a research article or something like that about this?
8
This is what happens when medical care is so diluted that you have only minutes if that of a physical exam and the hospital CEOs out there think it’s cost effective to put PAs in the ER.
We should be rioting in the streets!
The only ones benefiting these days are the insurance companies! Look up Aetna CEO salary for example. Last I checked it was $20 MILLION PER YEAR!
19
Interesting story. I had sinus surgery just over 3 months ago to remove nasal polyps. Like the subject of this story, I never really had asthmatic symptoms, and I'm also a runner.
Following the surgery, I developed a sporadically recurring cough, along with asthmatic symptoms. It recently got so bad that I went to the doctor, who prescribed -- surprise, surprise -- a burst dose of prednisone and an albuterol inhaler to deal with more acute bouts of asthmatic symptoms.
The questions I'm seeking to solve are 1) why did this sporadic cough and these asthmatic symptoms suddenly start following sinus surgery, and 2), more importantly, how do I solve this problem?
14
The author neglects to mention the $46,000 annual cost of the new medication, Dupilumab. So, is hope really on the horizon?
20
Samter’s Triad was the creation of my physician... a dear friend of Hermann and Lisa Lisco... he fled Germany in 1937... was friend of Nobel James Franck who fled in 1933. My wife’s family with her 6 months of age fled in September 1936. They all came to Chicago. Max Samter was not associated with U Chicago... he and his wife Virginia were delightful company... and his command of asthma treatment was pretty good for that time. Care of asthma today respects the Triad.. but is also a function of the care given by Arthur Fost MD of Belleville, New Jersey... who treated me for a few years... today at nearly 81, I no longer have asthma created by allergy. I wish Max was around to diagnose what happens as we age. I was told never to live at a farm, never to raise cattle, or house dogs or cats... or live in an old dusty farmhouse. I have done all of those things... and I rarely use Adair... as it simply isn’t needed, most of the time. I cannot run the mile, but I never enjoyed running. I do swim in our huge man made pond... for all those allergens have no claim on me, and I take aspirin when needed, as well as Tylenol. I have a theory about allergy... that I will share with anyone... for free. I do not think it’s complicated. We need rest, exercise, good water, and black coffee... and we should not hesitate to get mad... Adrenalin helps. Sudden cold air can cause trouble. And there is more...
15
@S B Lewis You need to do a controlled study of a significantly large population and publish in a respected journal so your findings can be critiqued and replicated. Until then...
8
1. She was in the ER with unexplained chest complaints and had a history of asthma? The ER should have done a spirometry to document her FEV1 (asthma status) as recommended in the NIH asthma guidelines. That’s really basic. If she hadn’t been given the NSAID She would have gone home w likely uncontrolled asthma.
2. If the ER PA had properly entered the ibuprofen (NSAID) reaction into the hospital medical record system (EHR) and later typed in an order for ketorolac, an “allergy” alert would certainly pop up for the likely cross-reactivity between ibuprofen and ketorolac and would prevent ordering the second NSAID. this is one thing that EHRs do well.
Either the PA overrode the alert (she should not have) or retrieved and administered the injectable ketorolac somehow without first entering a medication order for it (this is a system failure and big no-no in any hospital system.)
Normally meds are locked down in a computer controlled dispensing cabinet and can’t be obtained by ER staff without first typing in the appropriate med order.
3. Where was the ER doc during all this? A PA is not an MD. The patient could have died. 3 days intubated in the ICU for the PAs error and the presumed system error for catching the likely cross reaction.
4. It’s unfortunate the AERD was not diagnosed sooner but it would likely take a visit to allergy , ENT or pulm to get that properly diagnosed.
30
@Bernard Ramon PAs run the EDs at most NYC hospitals. They are supervised by a attending EM physician but there are limits to that supervision, given the hectic nature of NYC EDs (especially the one at Montefiore). The PA and NP lobbies have been pushing for independent provider status in many states, and have been partially successful (the buzzword the PA lobby uses is Optimal Team Practice -- see https://www.aapa.org/advocacy-central/optimal-team-practice/ ). My guess is that the PA overrode the alert (if you haven't worked in healthcare, then you should realize that the EHR throws up many alerts that don't totally make sense and are often ignored), didn't know about AERD (I doubt PAs would cover and memorize this disease in their school; although AERD is part of the standardized curriculum for medical students), and gave ketorolac (her thought process is that -- if the Pt had a allergy to ibuprofen, then since ketolorac is a different molecule, the pt shouldn't also have an allergy to ketolorac unless she has a generalized reaction to COX inhibition by NSAIDs in AERD).
5
@Bernard Ramon Bravo! You are so right.
Once the correct diagnosis is made, is it recorded properly? In the USA at least the answer in this case is almost certainly no. I have a patient with Samter's triad (also known as asthmatic triad) but this is not in my electronic medical record system's vocabulary, and nor is it in ICD-10 (the International Classification of Diseases) - so his diagnosis is not captured by any computer system or any of the widely-touted health analytic systems, and unless someone takes the time to read the free text I type into his chart they will not see his diagnosis.
21
https://www.nytimes.com/1999/02/15/us/dr-max-samter-90-immunologist-who-wrote-pioneering-textbook.html?searchResultPosition=1
Max Samter MD practiced on asthma patients when I was a teen asthmatic... in Chicago. He was a dear, dear friend of my wife’s family... his text on allergy was famous.
And his sense of humor was classic. His obituary in The New York Times captured him beautifully.
6
This is the problem with PA's in the ER. They simply do not know enough. I learned about this aspirin related malady in my first year of medical school. I think many good physicians would have recognized this. Instead, the PA prescribed the wrong medication, and she had a severe consequence. Limited knowledge has no place in an ER or office setting.
I think you are having a hard time finding mystery cases and are picking malpractice cases.
28
@Nancy Hooyman, MD
I used to think the random diseases I memorized for Step 1 was rare until I started working in the EDs in the Bronx. Nothing is truly rare.
9
How much is this patient paying for Dupilumab? As a monoclonal antibody, it can be assumed to be very pricey.
7
@r b It’s ~$46,000 per year. Insane!
Time for all diagnoses to be computer aided. Possibilities unknown to the physician are known to the computer.
7
Medical diagnostic is a challenge and most diagnosticians are worthy of respect -> a doctor who get it right 99 times out of 100 is underappreciated by the 99 and excoriated by the 100th. Especially admirable are dedicated veterinarians, whose patients cannot even speak.
26
I figured the situation out immediately, because I have Samter syndrome and have it since age 16 and now am 72. I was a participant in one of Max Samter study in the early 70’s.
14
This patient once again proves that many medical schools are teaching material that's out-of-date. The best advice for any doctor is to learn what's new in diagnostics...or see a specialist in the problem experienced by the patient.
@dbsweden It does not prove anything of the sort. I learned about this in medical school over 20 years ago.
3
@dbsweden
It proves nothing. Medical training (i.e., med school, residencies, fellowships) is the most difficult, grueling professional education, and is aptly described as trying to drink from a fire hose. Beyond the life and death responsibilities, exhausting work schedules, and voluminous details of thousands of diseases, and, in the US, mountains of debt, physicians sometimes have to put up with angry, ingrates who expect perfection that isn't possible. Unless you are a physician or close to one, you have no idea how hard it is. The system works best when there is compassion on both sides.
17
I am no doc - nor a medical professional - but folks below are too harsh in that young PA.
In hindsight, it all looks bad.
But the diagnosis as AERD only came later.
Another good article by the doctor - very educational.
I wish the good doc had told us a little more about asthma - how a person gets it and it's treatments.
8
A PA should not be expected to know everything the top of his/her head. That’s why there are protocols and systems.
That PA either:
Didn’t enter the original NSAID allergy
Or
Override the EHR “allergy” alert for the second NSAID. Without asking someone more qualified.
Or
Gave the second NSAID without entering the order first.
All these are failures to follow basic protocol and hospital policy and IMHO PA standard of care.
6
This is so typical. I was in an ER for hours with a virus turned into pneumonia. After a multitude of tests, a doctor told me that they could not determine the type of virus and could only give me cortisone. After telling him that I was allergic to cortisone (and a multitude of other medications), he insisted that I take it. I refused, told him to go read his medical books again and come back later. He did, gave me something else and the pneumonia was finally cured. This is not the first time this has happened and I have learned to question any medication suggested to me that may cause an allergic reaction. I am not a trained doctor but I have finally learned that a lot of professionals have no clue...
27
As a medical professional, I am absolutely horrified that a PA would give a patient that has a severe reaction to one NSAID (ibuprofen) another NSAID (ketorolac). I would give the patient ketorolac if I wished to kill her, but not if I wished to help her.
42
@Bobby McGee
Well, PAs are just nurses with a bit of advanced medical training. Nursing education provides very little, if any, chemistry, so PAs ability to recognize chemical structure similarities, and, therefore, potential physiological response similarities, between two chemicals with different common names, may be nonexistent.
12
@Total Socialist Well, no, Tot. PAs are doctors' technicians with just 2 years of training beyond a generic bachelors.
Nurse practitioners are ADVANCED PRACTICE NURSES who have 2 or more years of education beyond their NURSING baccalaureate degrees and experience.
You're welcome.
16
@Leslied1 Actually Nurse Practitioners have an MSN (Master's of Science in Nursing).
4
It was completely irresponsible for the person in the ER to give her Ketorolac if it was already known that she was allergic to ibuprofen. The shared allergic reaction to this class of compounds is widely known and taught in 1st year med school pharmacology.
25
@Larry Figdill Agreed! We learned that in nursing school!
How do physicians acquire comprehensive contemporary medical knowledge such that their level of expertise is at least equivalent to the current cohort of medical students? I imagine a yearly standardized compendium of the most salient journal articles within their specialty along with an updated medical text.
This would help set a continuing education standard as well as suggest how to identify accessible specialists with a particular expertise. Doctors’ referrals to experts are as essential as their knowledge of what best therapies/pharmaceuticals to prescribe.
Here’s an article on AERD that would be included in the yearly compendium/comprehensive medical update text.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6313255/
If I had AERD, I would consider seeing Dr. Laidlaw at Brigham and Women’s Hospital in Boston or ask via phone/email if she could recommend a local expert.
The aforementioned article refers to new organizations focused on developing expertise in understanding, supporting, and successfully treating patients with AERD/Samter’s Triad.
What is the current actual state of the continuing education licensing requirements for physicians?
6
@Joel H
Excellent advice on Dr. Laidlaw!
I was only labeled AERD and desensitized to aspirin after I watched her speech on YouTube. After thoughtfully responding to my email, she knew a doctor in Chicago to treat me.
It only took me 7 years, multiple surgeries and the frustration of a lifetime getting there.
8
@Ron Goldberg Can you please tell me the name of the physician in Chicago? I developed asthma in my late 30s after years of endurance training. More recently I've basically lost my sense of smell.
1
@Joel H 50 hours over 2 years.
1
What I took away from this article was that the P.A. almost killed the patient. Where was the ED doc? Obviously, the P.A. didn't have the education, knowledge about the risks of the medication, or experience to handle this. I routinely as part of my job read deposition transcripts from lawsuits where the P.A. missed the diagnosis, administered the wrong medication, did not order the right test, and the list goes on. Fortunately, this patient survived. Many don't.
22
But PAs cut costs. Isn’t that more important?
8
I’m glad the patient was sorted out. A good
general physician might have considered
the possibility of nasal polyps, aspirin allergy
and asthma after a thorough history and
physical. Ketorolac certainly proved to
be a definitive stress test and in my opinion
should never have been given to someone
with a known problem with NSAIDs.
Asthma is so common and so dangerous
that discussing cases like this helps medical
professionals and patients.
26
This was me, only in a somewhat milder form in that I never had to be incubated. Asthma out of nowhere - in great shape and also a runner. Completely loss sense of smell (though no scans ever indicated polyps.) Was coughing so hard that I was mis-diagnosed as bronchitis at several points. Asthma that was poorly controlled by pretty much all available medicines (reduced to 50% lung capacity) and my previous go-to pain medicine of ibuprofen seemed to be a trigger. Long story short, after a year of being a dutiful patient, I changed my diet to paleo and was off of medicines within 2 weeks, and have been off them 8+ years. People like to make fun of the paleo diet, but it saved me. Longterm, low-level food sensitivities had ramped up my inflammation levels to a point were everything was overly sensitive. It's really nice to know I'm not stuck taking longterm prescriptions too - because they all have side effects, some of them worse than the illness they treat. And getting your sense of smell back after not having it for years is just magical.
50
@DL It was probably stopping the ibuprofen that caused the problem, not your diet!
13
@Tasha that's not necessarily true. My ex husband was well on his way to a colostomy due to ulcerative colitis. His gastroenterologist at the time (this was @2001) had him on large doses of a drug called Asacol and prednisone. As his weight plummeted down to 168 from 210 (6'3") I decided to do some research on an alternative to the ineffective drugs. We discovered the Specific Carbohydrate Diet. Within two days on the diet, he started to get better. He was able to wean himself off the drugs and hasn't had a recurrence since. His now former gastroenterologist told him he "couldn't accept" that the diet had anything to do with his recovery. Don't always assume that diet has nothing to do with effecting one's health!
11
@Tasha No, I didn't notice the ibuprofen as a trigger until a couple years later when I took some and had a reoccurrence of the asthma. The entire experience had convinced me to stop taking pretty much everything - Rx and OTC meds - and only a really bad and painful cold has me grab some meds for relief. Totally wasn't worth it.
3
A fine example - says this solo practice MD of 50 years - why one never, ever - and I mean never ever - gets treated solely by some PA in an office or hospital setting.
From my standpoint they know little and many of them I've encountered are staggeringly hubristic.
Their introduction and permeation into the health care system was, in my opinion, one of the worst things that ever happened - and it will never be repaired.
It's interesting that when I talk to patients about PA's they virtually always say the same thing "but he/she was so young and so nice", but never do they mention anything about their competence or a sense of trust. They are seen by such because they are usually very convenient.
Words of very sound advice - make sure that an MD evaluates you if you have been seen by some PA!!!!!!
70
@Bob I agree. My husband was recently treated by a very good PA since our doctor was over-booked. PA prescribed a Z pack for his illness.
Z pack was not particularly effective. She prescribed a second Z pack. Worse, when his oncologist found out that he had been prescribed a Z pack, her comment was: why would you prescribe a medication with so many bad side effects when doxycycline would have worked better?
Sigh.
22
@Bob I had a PA in Montana tell me that my urine was too yellow, it should be clear. This was in the course of my annual check-up. She delivered this news like she was telling me I had only days to live. When I asked my doctor about this, she just sighed and shook her head.
14
@Bob
50 years in practice. Hummmm...judging from that and your comments, you likely believe all nurses are women and all women in medicine are nurses too.
Don’t be so quick to generalize. This Penn, Hopkins trained NP has seen many an error by a doc and saved many a patient from a doc. The Ketorolac was a seriously poor choice agreed.
20
The patient should also try a salicylate-free diet to see if that helps her.
7
I’m a very-retired doc who saw tons of wheezing, some of it nearly lethal. I like the term “united airway disease” — it highlights the inter-connectivity of our bodily systems.
While severe wheezing is the final common pathway, “asthma” has MANY causes, components, and, thus, treatments.
Memo to docs: You can’t diagnose what you don’t think of. Learn the pathophysiologies involved.
Memo to patients: Never accept the knee jerk “wheeze/inhaler/good-bye.” Demand appropriate explanation. Especially for serious attacks.
Asthma kills 3,500 Americans a year, most of them adults. Dying of air hunger is a horrible death.
84
Sorry, but did the author of this article seriously glaze over the fact that the patient seemingly physically assaulted the PA treating her? Am I reading that properly? Wow.
5
The PA almost killed her, after being told of the patient’s reaction to Ibuprofen and suggesting that there was little danger. I would cut the patient some slack.
44
@Alyssa
Try to understand the circumstances. She was obviously in a panic. This wasn’t some random assault on the street.
37
@Alyssa You have obviously never been in the situation of truly fearing for your life due to air hunger. All you feel anymore is pure panic, reason shuts down, the body does everything it can to survive.
33
But of course there is absolutely NO discussion of botanical medicines and/or diet in this article. Yet there are several comments saying that the chemical medications are "very expensive". This is the USA's 'disease fighting' modus operandi masquerading as a 'health care system'. And tragically our government, which has been bought by Big Pharma, will not provide funds for Naturopathic research. No surprise here.
There are numerous plant medicines that could be extremely useful here-- Baical skullcap (Scutellaria baicalensis) for severe inflammation; Nettles for allergic response modification; Lobelia inflata for bronchial spasms; Mullein, Pleurisy root, Elecampane are the "Three Musketeers of the Lungs"; and many more...
The effective medicines are here, now ! But you will not hear of them unless you think outside of the Medical Pandora's Box.
And I will be very surprised if this newspaper even publishes this comment. But IF you receive this-- "thanks for listening".
A medical herbalist from Cape Cod.
17
I wish I had all the money I spent on alternative, holistic medicine, salicylate free diet, herbs and supplements. None of it helped and it delayed getting the treatment that eventually helped me and I got my sense of smell back. Allopathic medicine is often the answer.
25
@Audie Absolutely. Audie. I have had asthma all of my life and I am now 74. BUT, since incorporating plant medicines into my health protocol I have almost completely eliminated the asthma and use of inhalers. Pharmaceutical medicine is ACUTE medicine. Plants and diet are medicine for CHRONIC conditions. Huge difference. Big Pharma and Big Med know LITTLE about how to treat chronic (and auto-immune) conditions. I.M.H.O. Stephan
8
@stephan brown That you call it "BIg Med" indicates you do not trust allopathic medicine, and so it is good that plants and diet worked for your asthma. They didn't work for me, though I would have preferred that route. This condition, Samter's Triad, can be managed, but like many auto-immune disorders, cannot be cured.
3
Years ago I developed this. On a whim I decided to use Advil for my occasional headaches, instead of aspirin or some other pain killer. Over the next couple of years I started getting asthma. (I'd had it sometimes as a child, so I just thought it was coming back.) At the same time, I started getting migraines. I took Advil for the migraines. Also I started getting horrible, sudden attacks of sneezing and congestion. It was like someone opened the top of my head and poured a quart of liquid into my sinuses. I could barely breathe. I went to an allergist, who prescribed many different kinds of inhalers and antihistamines. Things got worse and worse. Some of the inhalers made me gag; others caused me to develop cataracts. The allergist never asked what I was taking for the migraines. Finally, a friend said, "don't you know about asthma and Tylenol? Take Tylenol!" In no time the migraines, asthma, and congestion attacks stopped. And, um, the allergist never looked up my nose. I have a nasal polyp.
19
All these articles leave out one important fact: the patients either have insurance or the means to pay for treatment. I would like to see just one that shows a uninsured person with a difficult case where the problem was solved. Just one.
27
@Paulie
It happens, mon cher, all the time at big city teaching hospitals, and has for many decades!!!!
23
@Paulie @bob is right. Many of the patients featured in this column haven't had insurance. These were often patients seen at academic medical centers. I only have 1400 words to tell a story and when the presence or absence of insurance doesn't have an impact on diagnosis or treatment, I usually don't discuss it.
51
I have insurance. I am a retired, female MD. I have been blown off more times than I care to go into. I have long term problems tied to rushed care. I fought to give each pt the TIME required ... and as a result, in the few bad outcomes I had, parents understood I had done MY best.
Rushing through care is like speeding through a school zone.
9
Good reminder how much is left to learn, even for the smartest doctors. Whole health approaches that go beyond the physical are helping many.
8
simply extraordinary to read this. It explains my experience for last 6 months which involved doses of ketorlac, asthma, and diagnosed polyps. Not one ENT brought up Samter's Triad.
The ketorlac came through eye drops after eye surgery.
I had never put the eye drops into the mix of possibles since I did not think that they entered the system. However, another eye drop, prednisone made my blood sugar numbers spike, so it apparently the drops do pass into the system. I still don't understand why dr's. do not have an app [beyond their diagnostic education] that takes in all the meds and generates a decision tree.... possible explanations. I seem to be allergic to many things and maybe that explains my susceptiblity to this trifecta.
39
@eloi
Again this is why biology need to be taught in school.
7
I was also diagnosed w Samter’s Triad. For me it was uncontrollable sneezing w asthma that was initial alert to aspirin allergy. Also, I avoid food dye yellow 5, which also brings on uncontrollable sneezing and asthma.
8
Interesting how this disorder appears in many runners. I'm wondering if chronic exposure to air pollutants and environmental chemicals is a contributing factor ???
46
@DB
Or could it be that running exacerbates the symptoms that might not be quite as noticeable in a more sedate person? I don't know, I'm just asking.
2
@jim
Good point. There was a study many years ago i think suggesting that runners had evidence of suppressed immune systems and tended to get more colds.
1
Thanks for this article. I recognized myself into the second sentence. I had a classic case of this disease (onset beginning in early 30's with sudden severe NSAID allergy, worsening asthma and finally nasal/sinus polyps). I was on my fourth sinus surgery when my new ENT told me I had Samter's Triad. She was the first of 5 or 6 ENT/allergists to recognize the syndrome. I've elected not to do the aspirin desensitization, but just had my fifth sinus surgery (balloon surgery this time, so no general anaesthesia and not too much recovery necessary).
8
I had AERD undiagnosed for 7 years. My nasal polyps were so many in number and large in size that I had multiple years of 100% blockage in both nostrils in addition to late onset asthma and chronic sinus infections. Breathing exclusively out of my mouth was incredibly challenging. When I was desensitized to Aspirin (which is not mentioned in the article) it was like a running faucet was turned off. So immediate we’re my improvements that my goal was to promote knowledge of this trying disease. It saddened me to see that still today the disease is not being recognized despite some great work by doctors. Taking over 1000 mgs of aspirin every day has certainly slowed the regrowth of polyps, but certainly not cured the disease
26
I was never allergic to anything, and had no asthma, when I started wheezing after a morning run when I was 46. Soon after I landed in the ER with a serious asthma attack after taking two aspirin for a headache. The next morning the doctor made an urgent call to warn me to no longer take aspirin, that I must have AERD. I subsequently lost my sense of smell (and taste), and had four surgeries to remove sinus polyps over ten years. After the fourth surgery, and aspirin desensitization at Scripps in San Diego, I have regained my sense of smell and with my small daily dose of aspirin and (very expensive) asthma inhaler, I am good. My ENT doc at the U of M has many patients with AERD and is excellent, I'm lucky to have access to good medical care.
49
@Audie
Is your inhaler a ventolin inhaler, advair discus, or something different. My sense of smell has never returned
My inhaler is Flovent, which is steroid. I use albuterol inhaler very occasionally as needed. What has kept polyps away for me is the aspirin desensitization, I had it 6 weeks after my last surgery, before the polyps had a chance to grow back. Yes, they came back very quickly after my previous three surgeries. Hope this helps.
10
@Audie
Try getting Flovent from Canada pharmacy, much less $
3
Dupilumab, for its anti allergy effects, seem remarkably well suited for this particular Tough Mudder patient. However, one care I'd be concerned about is the long term effects that Dupilumab may have on the body's natural protective mechanisms that comes about with an allergy response. Inflammation, igE responses, uticaria, diaherra and a host of other allergy responses are a human's typically normal defense mechanisms. They should be thought of as signals regarding what that allergic human should avoid! The risk of eliminating those naturally beneficial 'signals', i.e., not allowing the allergic response to rid the body of toxins or signal the body to stay away from agents that trigger the allergy could eventually can cause cancer for the person taking the allergy suppressant. There are studies that show humans with allergic responses are more effective at reducing the advent of cancer compared to humans that cannot mount an allergic response. Dupilumab might be a temporary benefit to this Tough Mudder, but a steady application of this drug for years could be equally deleterious. It is important that patients consider the long term effects of this drug versus the long term benefits of expressing an allergy, and using the allergy response to determine the root cause of the trigger and specifically elimination the trigger ( e.g., NSAIDs in this case).
21
I had a very scary reaction to dupilimab, and my allergist said she had several patients who had bad reactions to it.
Biologics are not benign.
16
@Jan Shellman: allergy, like auto-immune disease, represents a failure of regulation within the immune system, not some kind of natural function designed to rid the body of unspecified “toxins”. You come across as someone who has learned a few terms out of medical science but fundamentally distrusts the same science in favour of a “natural” approach. Attempting to link this to cancer is particularly unkind: “suffer your allergy or die of cancer!”
14
@Annette Dexter
Hey, most folks might agree, but the data is overwhelming: Allergies have an evolutionary benefit that should NOT be taken as a mal-adaptive response. For example, Peanut allergies can get their igE response from a toxin that mounted to a protein in an early period of exposure. The toxin is often a fungi
toxin, e.g., due to storage of the peanuts for a long period of time. Ingestion of the peanut may then recognize the protein that once bonded with the fungi, signally the body to reject the 'peanut' because of its prior association with the fungi. Allergies are signals, and one can be a detective to start eliminating exposure to foods, or car exhaust, fire or smoke (often a trigger for asthma). To say our evolved IgE responses are maladaptive is a bit naive. Scholar Google allergies and cancer studies. There is a meta study I think you might lie to read. Or click onto
https://medicineworld.org/cancer/lead/10-2008/the-upside-to-allergies-cancer-prevention.html
1
I have episodic asthma (seasonal allergies and cold viruses linger and usually trigger asthma), since my 40s, and have discovered that after 2 or 3 days of nsaids use for arthritis I am wheezing and my asthma has been triggered. No surprise here. I avoid nsaids.
10
Amazing to me how "accepting" everyone here is of our broken medical system.
A person with a chronic condition poorly handled (by the patient) uses an ER for her medical care. A new drug, touted in the article, costs over $3000. per month, is mentioned without irony or comment on the rapacity of the pharmaceutical system....
As a retired RN with over 30 years spent in medical centers, this is sad, and unbelievable.
168
@RLiss I don't agree. She tried primary care - the asthma meds they prescribed didn't help. Same thing happened to my child. It wasn't until we went to a specialist in a big children's hospital that we were told the asthma meds should stop the coughing almost immediately. If they don't, they are doing more harm then good and we were to stop giving them. He figured out what was wrong and he arranged the right treatment. You complain about the patient being given an expensive drug but it sounds from the article that she had been under the care of a specialist who had tried other modalities. It's not like she was given that drug day one. If after years of suffering this drug helps why shouldn't she take it?
59
She was started on the expensive drug by an immunologist. Presumably she was referred to her after a number of visits to the ER where she required nebuliser treatment and IV corticosteroids because she having a severe asthma attack - you know, an emergency.
21
Thank you, this is a great series. Also representative of the opportunity offered by artificial intelligence (AI) in medicine, the ability to sift through what might be an unrelated group of symptoms and suggest diagnosis' without having to rely on the serendipity of coming across a knowledgeable specialist able to identify a zebra.
19
@Medhat
quit with the AI will solve all our problems thing, found in comments after each of these articles!
AI is programmed by humans.....no, it won't ever be "better" than a good human doctor who can make intuitive leaps.
10
But it will be better than a doctor that’s never heard of AERD.
11
@RLiss What percent of human doctors will be better than an AI developed by a skilled team of doctors?
3
Thank you for this article! I developed salicylate sensitivity after decades on NSAIDs for pain relief from arthritis. The hives and asthma-like symptoms mystified my doctors, and I finally sleuthed out the answer on the Web: I'd crossed the threshold of NSAID tolerance. I began taking SAM-e, which controls my daily pain without ill effects. Having been off NSAIDs for three years, I can now take an occasional ibuprophen for more severe pain, but for no more than a day or two. While my symptoms were far less severe than those described in this article, I think everyone who routinely pops NSAIDs should be aware of this hazard.
17
I have cough-variant asthma, sporadically badly controlled and hypersensitivity to NSAIDs (which has been getting worse and covers more and more different NSAIDs) but I do not have nasal polyps. I see from comments that the other two components of the triangle seem more common; do we know anything about the NSAID-asthma connection?
5
The mechanism was outlined in the article. The solution to your problem is simple - don't ever take NSAIDS again.
3
Thankfully, I have none of the problems discussed in this article.
What I find fascinating is that after all these years how aspirin works or doesn't work for many people is still not completely understood.
34
Would be interested in the similar symptoms but different diagnosis of Samter Syndrome where there is an allergy to aspirin.
2
Thank you for this article! I think I have this too and had never heard of it before. I developed an allergy to Advil recently (I had my first reaction when given toredol after giving birth, then another when I took a couple Advil months later for back pain). In the past year I’ve also had my first asthma attack. My ent told me I have nasal polyps but they don’t really bother me that much. Wow, good to put the piece together - if a bit scary, especially as a new mom.
36
Would the diagnosis have been made earlier if the patient had been seeing a primary care physician regularly rather than going to the emergency room?
39
Depends. My primary care physician had never heard of it when I told her about it. I discovered I had AERD when I took 2 Advils and I almost died from an Asthma attack. In the emergency room I was incorrectly diagnosed with a blood clot in the lungs and was put on Coumadin . So the ER docs failed to correctly diagnose my AERD. Only when I insisted that they call in a pumonologist was it diagnosed correctly and that's when I learned for the first time that my then current lung issues were really Asthma and I had AERD.
30
@KMM :
I asked that too....and I think not..... ER's are for emergencies, not to routinely monitor or treat chronic conditions!
6
Found this article interesting. I don't have any of these symptoms but have had sinusitis on and off for years and have always had a poor sense of smell and taste - I'm now 76. A couple of years ago I began to have severe sinusitis all the time and nothing seemed to cure it. My GP suggested that, before we tried surgery, I should try cutting out dairy products and to my surprise it worked. It turns out that those of a Northern European heritage are about the only people who do not develop a lactose intolerance in later life - or so my internet research tells me.
9
@GinaSwifte Hmmm... I am of Northern European heritage and I have lactose intolerance. Lactose intolerance can have different reasons - not just genetic ones. Bacterial imbalances in the gut caused by antibiotics is a common reason for lactose intolerance.
@GinaSwifte
Some Irish-descent people do have lactose intolerance too. I am one of them. Discovered this by accident, never knew what was causing my stomach problems, which disappeared after I stopped drinking milk.
4
These patients are often sensitive to natural salicylates - I find that changing their diet works. Being strict at the beginning and liberalizing as tolerated. Dr Feingold was the first to make the connection (though he was studying ADHD). The Feingold diet has worked for my patients with the triad and certainly is worth a try!
22
A low salicylic diet was once recommended, but it’s not actually the same compound as what those with AERD react to, just similar. Experts now recommend a diet focused on omega 3 and omega 6 consumption.
6
@Mary Negro it has worked for many of my patients salicylates ARE what are causing the inflammation - that is why the aspirin does not work in many cases. But hey I just have an MD from Yale and 25 years experience behind me.
3
Yes, I’m aware they cause inflammation. They do not cause AERD inflammation. Only one diet is recommended by AERD specialists, and it’s an omega diet.
2
I am a 74 year old male who until the age of 67 ran in over 400 road races. I was first diagnosed with exercise induced asthma while in my 50’s. I was prescribed an inhaler that helped but continued to suffer on and off for years. When I was 60 I had surgery for a deviated septum. Some help but not much. I then began to lose my sense of smell, had a Cat Scan that showed my sinuses loaded with polyps. Another surgery. I never took aspirin or ibuprofen very often but had some knee issues so took two Advil. I had an asthma attack but attributed it to allergies. Several weeks later I took Advil again and had a serious attack. My most recent regiment consists of Pulmicort for the asthma, once a day nasal flush with a budesonide ampule and also a direct application with budesonide as well. It has been successful up to this point but continue to avoid NSAIDS.
28
@JohnCamelio
But do you think you will ever be able to run? How is your quality of life? Your story is scary because you are doing the best to take care of your health and then you hit a road block.
2
@Rana
I don’t race anymore for other reasons but do the elliptical, row, kayak, play Pickleball, and bike. The asthma is under control and the budesonide regimen has kept me polyp free for 2 years. After exercise I do cough up some junk. Also, I didn’t mention that I try to be as salicylic free as possible, especially red wine and consume more omega 3’s.
4
Thank you for this article - it is eye-opening. Out of nowhere, I was diagnosed with asthma a few years ago. Prior to that, I had to have sinus surgery which included the removal of nasal polyps. Prior to that, I was someone who simply had "mild seasonal allergies." This has been a downward progression over the years for me in terms of quality of life and I would say that with the surgery and asthma medications I feel better, but not all the way better. I will be looking for more information on AERD and will print this article out to take into my next doctor's appointment.
37
@Louis Smith -not all doctors are familiar with AERD and it is often misdiagnosed. If you join the Samters Triad-AERD Facebook group there is a list of recommended doctors in the files.
21
You should see a board certified pulmonologist. Asthma like symptoms, nasal polyps and a downward progression in the quality of life could be many things but one disease that I have family experience with is adult onset Cystic Fibrosis. As I said this could be many things but if your quality of life is decreasing you should certainly consult a specialist to find out why you do not feel healthy.
3
Thanks for publishing this article, and thanks to all who commented.
32
As a patient, I appreciate seeing an article about this. AERD typically starts with chronic congestion and then progresses to asthma and aspirin sensitivity. The article reads like a product placement for Dupliumab. This drug is very expensive and even with excellent private insurance, my insurance company would not approve it. The list price is $3019.50 a month.
98
@e.w. I've heard multiple appeals can be helpful. With plenty of documentation of treatment failure.
17
@e.w. :
congrats on living in the U.S. where the bottom line is king.
13
This doesn’t sound like product placement at all. It sounds like a wonder drug. If your clinician prescribed it, your insurance company cannot turn it down.
2
This case triggered my looking for further information on this condition and its treatment, which I am submitting below.
Several reviews appeared on this condition in the past two years, including one this year, from the ENT Service at Montefiore itself.
The reviews I read state that the asthma and rhinosinusitis are present without provocation by NSAIDS. That’s why the NSAID is considered an exacerbant, rather than the cause.
I didn’t see anything in my brief literature review indicating that the asthma component is progressive, so I wonder what the term “progressive” used in the article refers to. (Obviously, it is bad enough to have severe asthma and life threatening exacerbations WITHOUT it causing progressive lung damage.)
The reviews I read stressed the use of aspirin desensitization as a “standard of care”. It wasn’t clear to me if the patient underwent this or was just advised to take some dose of aspirin. A routine daily regimen of 325 to 650 mg of aspirin twice a day is the standard maintenance dose after desensitization.
The monoclonal antibody mentioned in the article is being studied in current clinical trials. Was it specifically approved for use in AERD? I read that it costs $46000/yr, but I don’t know if that varies with the indication.
There are other agents also used successfully in such patients, I read, including Zileuton and montelukast. I wonder if they are equally efficacious and less expensive.
Stephen Rinsler, MD
48
@Stephen Rinsler As a patient I can answer some of your questions. Dupilmab was not approved specifically for AERD. It was approved for asthma but is being prescribed to patients with AERD. You are correct the symptoms are present without NSAIDs. Some patients who have not been desensitized to aspirin avoid all NSAIDs. Some patients are not able to be desensitized. For some reason, it doesn't work for them. There is also research on a diet that is low Omega 6 and high Omega 3 that seems to be helping some people.
30
Dupilumab is FDA labeled to treat nasal polyps and severe asthma. I see it used all the time for these cases and patients report it is life changing, able to resume exercise they could not before. Although not specifically labeled for AERD it has been successfully studied in the condition and these studies can be found in UpToDate. The drug is expensive but accessible if you have a good team who can navigate the paperwork.
24
This case triggered my looking for further information on this condition and its treatment, which I am submitting below.
Several reviews appeared on this condition in the past two years, including one this year, from the ENT Service at Montefiore itself.
The reviews I read state that the asthma and rhinosinusitis are present without provocation by NSAIDS. That’s why the NSAID is considered an exacerbant, rather than the cause.
I didn’t see anything in my brief literature review indicating that the asthma component is progressive, so I wonder what the term “progressive” used in the article refers to. (Obviously, it is bad enough to have severe asthma and life threatening exacerbations WITHOUT it causing progressive lung damage.)
The reviews I read stressed the use of aspirin desensitization as a “standard of care”. It wasn’t clear to me if the patient underwent this or was just advised to take some dose of aspirin. A routine daily regimen of 325 to 650 mg of aspirin twice a day is the standard maintenance dose after desensitization.
The monoclonal antibody mentioned in the article is being studied in current clinical trials. Was it specifically approved for use in AERD? I read that it costs $46000/yr, but I don’t know if that varies with the indication.
There are other agents also used successfully in such patients, I read, including Zileuton and montelukast. I wonder if they are equally efficacious and less expensive.
Stephen Rinsler, MD
(2nd try)
11
@Stephen Rinsler many of us with this disease have not found any significant benefit to taking either Zileuton or montelukast. The polyps still grow out of control even with large amounts of steroid sprays and budesonide soaks. The dupixent drug has been approved for treatment of nasal polyps since July. I have yet to try it as my insurance keeps denying me. Many have found great success doing a low salicylates diet. I personally am quite nervous to try dupixent anyway because of side effects and long term effects. I’m looking for alternative ways to heal my body from this awful, awful disease.
1
@DK ,
There are allergist/immunologists apparently who have “significant” experience with AERD. If possible, you might benefit with a consultation with one of them.
That doesn’t include me .
1
Thank you for writing this article. I first started showing signs of AERD when I was 19. It took a total of six years for me to get diagnosed. Over that time period I saw several doctors and specialists, but no one was able to explain what was happening to me. Similar to the woman in your article, I loved to run, but began to no longer be able to because of what my body was experiencing. Thank you for sharing this story, raising awareness, and contributing to a world where less people have to suffer in the shadows.
69
Tiffany Could the vigorous breathing of polluted air while running have anything to so with this syndrome?
1
Thank you for writing this. AERD frequently goes undiagnosed and this can have devastating consequences for patients. There is an incredible lack of awareness of this disease in the medical community - aside from doctors who specialize in the disease, few seem to know much about it. The lack of awareness is remarkable considering that the disease isn't all that rare and can have life threatening consequences. Thank you!
52
Thank you for bringing attention to AERD. Many of us go through years of misery before receiving a correct diagnosis. It was roughly eight years after my first anaphylactic reaction to NSAIDS that I finally saw a doctor who put all the pieces together. My quality of life has since improved considerably.
74
Thank you so much for publishing this article. I have AERD and it’s so hard to feel seen and heard about a disease that no one seems to understand. The pain and distrust you begin to have with your own body is one of the hardest things I’ve ever dealt with in my life. If there are more updates to this story we would love to see them.
116
I also have it. Just like this patient, I was diagnosed by a severe reaction to ibuprofen, which I had then safely all my life. Like this patient, I have no sense of smell, which sounds perhaps like a small thing, but which leads to depression in many patients. It’s not the worst disease you can have but it’s not an easy one either. Sufferers can’t drink alcohol, have limited enjoyment of food, must be hyper vigilant in medical situations since common pain treatments can be deadly, and have severe symptoms (polyps) that can impact a life significantly. And all of this comes on suddenly in your mid thirties ... it’s kind of strange.
35
I, too, had these symptoms. Loss of smell. And taste. Polyps. Allergic to ibuprofen. Adult onset Asthma. But mine went deeper. Woke up one morning having difficulty walking. All downhill from there. Two week stay in the hospital. Released with no diagnosis. Took months to figure out. Turns out I have an auto immune disease called EPGA. If you have these symptoms, please ask or find a doctor to help you. In my case, it was too late. I would hate anyone else to go through what those of us with this disease go through.
24
@Kevin Walsh
@ Kevin Walsh,
Sorry to hear of your illness.
Mepolizumab (brand name Nucala) is a biologic drug that is approved for both eosinophilic asthma and EPGA (formerly Churg-Strauss Syndrome.
A recent retrospective study of patients with AERD suggested benefits from mepolizumab.
Have you received that as a treatment? If so, has it helped?
10