September 26th is AERD Awareness Day
“I don’t normally sound like this. I’ve been sick for a while, but it’s not contagious — I promise,” is what I said on my first date with my girlfriend, Amy. It was August 2016 and since April 2016, my day-to-day life consisted of chronic sinus congestion. My pharmacist advised, “Ragweed is really bad this year,” so I purchased Claritin and was still congested. A colleague recommended I try Afrin to decrease inflammation. It didn’t work. I tried Mucinex. That too did not work. I could have opened my own pharmacy given my medicine cabinet overfilled with nonsteroidal anti-inflammatory medicine, netti-pots, sinus rinses, herbal teas, hot compresses all because I got a cold and it never went away.
My colleague at work suggested I check out Harvard Vanguard Medical in Cambridge, Massachusetts. Once there, I saw an NP that specialized in Pulmonology. She evaluated my symptoms, gave me a round of prednisone and sent me on my way. In case you don’t know, prednisone is a magical corticosteroid drug that tricks the body into thinking it is better and decreases inflammation almost imediately. It’s commonly used for patients with gout, arthritis or asthma and it worked — albeit temporarily. In less than 24 hours, I could smell, taste, breathe, and — despite the stomach aches as a side effect — felt pretty great … until I ran out.
(Spirometry graph example, see below. My results look like the blue line — yours are probably red if you don’t have asthmas or COPD.)
I was back in my NP’s office in less than three weeks. She was perplexed and, after another physical, ordered a spirometry exam. The spirometry exam tests the ability for lungs to function. My exam results indicated I had asthma and my NP sent me home with a Flovent inhaler, Albuterol rescue inhaler, and more prednisone! The thought was my congestion issues were connected to my asthma issues and by curing the asthma symptoms, that would cure my congestion.
It didn’t work.
I was back in the NP’s office less than a month later still complaining about my cold-like symptoms and asking for more prednisone.
My NP wanted to rule out allergies. I was referred to an allergist who tested me and the results? Negative, but I got more prednisone so at least I could breathe!
Next, I was referred to an ENT at Mass Eye and Ear, named Dr. Ianuzzi. He’s a terrific doctor with more than 30 years as a surgeon and likes Amazon, which we bonded on. After an initial endoscopy exam, he concluded I had a deviated septum and if I had a septoplasty and turbinate reduction, that would open up my nose so I could breathe normally. I agreed and, despite knowing my coworkers would probably say I got a nose job, scheduled the operation.
The date was May 18th, 2017 — my girlfriend’s birthday. She was obviously thrilled in needing to take care of me instead of going out but understood that after a year of chronic breathing issues, asthma issues and snoring (which adversely affected her) it was best for both of us to get this procedure done. My dad also flew out for the operation which was incredibly caring considering how busy he is.
I had the surgery and it went well. Dr. Ianuzzi remarked my results were some of the best he has ever seen. I had limited pain in the recovery, and I was back to work the next Monday ready for nose-job jokes.
All was well. I could breathe, sleep well, smell, and taste — for about three months. Then in September 2017, like a slowly eroding beach, but with no beautiful sunsets in sight, the runny nose, post nasal drip, sneezing, coughing, and wheezing slowly crept back.
I went back to Dr. Ianuzzi and underwent another endoscopy.Dr. Ianuzzi indicated that I was still healing and that’s probably what the swelling was from and my symptoms. He ordered me to take another round of prednisone. At this point, my bones were probably atrophying from the repeated doses of steroids, but at least they worked in alleviating symptoms!
The final straw occurred during Christmas vacation in 2017. I was in Holden, Massachusetts celebrating the holiday with my girlfriend’s family and my symptoms were unbearable. I had the worst cold-like symptoms, couldn’t sleep, couldn’t taste, was blowing my nose every five minutes resulting in untold embarrassment and basically was single-handedly skyrocketing the price of Kleenex stock in value. I called my ENT and immediately asked for more prednisone to cure the symptoms. They balked and said they couldn’t take anymore because I had received too many doses and was at risk of hurting myself. I was incredulous, said I didn’t care, and wanted to speak to someone who could authorize the prescription.
It was December 23rd and, after a brief conversation with Dr. Ianuzzi, he ordered a CT scan to find out exactly what is going on in my sinuses.
On January 15th, I arrived at the Harvard Vanguard Kenmore Radiology department to undergo a CT scan. The results were evaluated and, on January 16th, I was ordered to take an additional MRI.
(Side note: MRI machines are the single most menacing sounding machines in existence. It’s like being in the middle of a jackhammer.)
In any case, at 6:00 AM on Thursday, January 18th — I awoke to a call on my cell. The voice on the other line said, “This is Dr. Bleier’s office at Mass Eye and Ear. Don’t eat anything this morning, we want to operate on you today.
The reason for this sudden surgical course of action was the photo above. As you can see, the left side of my brain looks (surprisingly)normal with some swelling in the frontal sinuses. The right side, however, has completely bulged and my right frontal sinus was encroaching on territory that was normally reserved for my brain. I asked Dr. Bleier if the surgery was an emergency because my sales kick off was the next week in Seattle and I didn’t want to miss it. He said, it was not an emergency, but given we know about the issue, it was ‘emergent.’ He added that I was one medium to low trauma impact away from going septic in my brain.
That’s all I needed to hear. I elected to operate and six hours plus a bilateral maxillary antrostomy, sphenoidotomy, total ethmoidectomy, and frontal sinusotomy with Draf III drill out procedure later, I was in the recovery room with my girlfriend.
It’s Still Not Over
I was fine, for about 90 days, then the swelling, lack of smelling, tasting, post nasal drip all returned — my cold was back. The fortunate news was that Dr. Bleier had a sneaking suspicion of what my actual illness was: Samter’s Triad or AERD (Asprin Exacerbated Respiratory Disease). The disease is characterized by three symptoms: adult-onset asthma, reoccurring nasal polyp growth, and aspirin allergy. After taking a low dosage aspirin, I confirmed I had all three.
There’s no cure to AERD and, after seeing the two premier researchers of AERD in the world — Dr. Laidlaw and Dr. Buchheit — at Brigham and Women’s hospital within the last 90 days, they were able to enroll me in a trial for an experimental drug that may be a cure for AERD patients. Additionally, I went through an aspirin desensitization which should extend my need to have a sinus-clearing surgery every 5–10 years instead of every 2–3 years.
I’m writing this because it’s AERD Awareness Day on September 26th and barely anyone knows about AERD or Samter’s triad. There are no known cures and the typical patient diagnosis time from onset of symptoms to treatment is ten years!
Can you imagine having a cold for ten years?
I was one of the lucky ones because my journey to treatment was only two years, but if you know someone who got a cold and it never went away, please feel free to reach out to me — firstname.lastname@example.org - or the incredible professionals at Brigham and Women’s Boston. There is symptom relief in sight and, if we can get about 40 more patients to participate in the drug trial at Brigham and Women’s, maybe a cure down the road too!