Tuesday, May 29, 2012

Diagnosed (Living With Cogan's Syndrome)

March 19th:

Today was my first appointment with the eye disease specialist, Dr. Russo. Now that I’m home, thinking back on the day, I feel exhausted and I’m doing my best to recuperate; however, I am also relieved.

Our road to discovery began as Dr. Russo’s assistant guided us into the exam room and we were seated. He was an older fellow—very friendly, but not too computer savvy—who was searching the keyboard for the correct letters before typing at an excruciatingly slow pace with only two fingers. Sarah and I smiled at each other, holding back our laughter. About fifteen minutes later, when he had finished asking us questions and entering the responses into a medical document, he performed a slit lamp examination on my eyes. When he was finished, he had me tilt my head back and released two different drops into my eyes.

“Max, have you been tested for…”

“Can you repeat that? I missed the last part,” I said.

He sat very close, looked me straight in the eyes and asked again.

“Have you been tested for an autoimmune disease or for Lyme Disease?”

“No, I haven’t,” I responded.

“Dr. Russo may want to test you,” he said. “The eye drops will take a few minutes to kick in, and then he’ll be in to see you. It was nice to meet you.” He shook my hand and then Sarah’s and walked out the door.
I looked over at Sarah and raised my eye brows.

“Well, looks like we’re on to something,” I said.

“Yeah,” she spoke with enthusiasm.

We waited for about twenty minutes, making silly faces at each other—what seems to have become a ritual at my appointments—until Dr. Russo glided in. I need to paint a picture of this man before moving on because that is exactly what it was like: a picture—Dr. Russo looked like a caricature. In walks this tall, thin man in his late thirties, holding a manila folder. He was wearing an open laboratory coat and underneath, he donned a white button-up shirt with a maroon vest. To top it off, he wore a large, maroon bowtie. He walked towards me with a perpetual smile—that lasted the entire appointment—staring straight into my eyes, arm extended, perfectly straight.

“I’m Dr. Russo,” he said, shaking my hand.

“Hi, I’m Max. It’s nice to meet you,” I answered, unable to keep my eyes off of his bowtie.

“It’s nice to meet you too,” he said, slowly turning around to go shake Sarah’s hand.

As he turned to place the folder on a table, I waved Sarah down, pointed to my neck and mouthed, “bowties are cool.”  She nodded in excitement, immediately understanding the reference to one of our favorite television shows, Doctor Who. There was a glimmer in her eye, and I wondered if she was thinking the same thing I was: If “The Doctor” were real, this guy would be totally believable as one of his incarnations!

“Tell me what happened, Max,” Dr. Russo spoke, but I couldn’t understand one word he said. He barely moved his mouth up and down from his permanent slight grin.

I shook my head, “I’m sorry?”

He repeated his question and gestured to me with his arm, giving me the floor.

“I woke up sick on Valentine’s Day and within a week I was beginning to lose hearing in my right ear,” I said. “My doctor couldn’t figure it out, she thought it was probably an ear infection but referred me to an ENT, who thought the same thing. About two weeks later, I woke up sick again and began to lose hearing in my left ear.”

Dr. Russo shook his head and in what seemed to be an unexplainable phenomenon, his smile turned into a frown without any real motion at all and somehow still vaguely resembled a grin.

“I went back to the ENT and then to another ENT who gave me a Prednisone injection in my right ear. He then referred me to Dr. Mazen, who referred me to you.”

“Wow,” he nodded, his smile returned. “Let me take a look at your eyes.”

He had me lean forward and used the same slit lamp machine that his assistant had used; only this time he turned on the most unnatural bright lights and shined them in my eyes. It was almost unbearable as my eyes began to water and ache. When he was finished, Dr. Russo pushed the machine away, leaned back in his chair, pointed at me and said, “You have Cogan’s Syndrome.” He paused, giving me a moment to ask him a question. So I did.

“Can you repeat that? I’m sorry I didn’t hear you.”

“You have Cogan’s Syndrome,” he said, this time spelling it out for me. I still didn’t understand, but I knew that I had finally been diagnosed. As he continued, he spoke in short, choppy sentences with pauses in between each one—not enough of a pause, however, for me to speak. “It’s an autoimmune disease. Very rare.  It affects your eyes; your hearing; your balance,” he swayed back and forth in demonstration.

“Wow. Okay, then. So that’s that,” I said.

Dr. Russo introduced us to another assistant, Hope, as he began to explain all of the medication I would need to be on and what sort of treatment would be necessary. Hope scribbled down all of the information for us.

“It’s treatable,” he said, his smile growing, obviously relaying an important message.

“I’m sorry?”

“It’s treatable.”

I shook my head, not comprehending.

“Treatable,” he repeated again.

“Oh. Treatable,” I nodded. Thank God.

“You’ll need to do a Remicade treatment here at the hospital. Once a month. For two years. It’s a long process. You’ll start with a pre-treatment this week: you’ll need to go across the street where the infusions take place.  You’ll start with a steroid directly injected into your bloodstream for three consecutive days.  I’m also going to put you on Methotrexate. You’ll take it once a week. I’m going to prescribe you more Prednisone. You’ll take it daily,” he continued on with his fragmented speech through a plastered on smile but I lost track somewhere at the beginning.

Dr. Russo then pointed at me and for the first time I heard him clearly, "You are going to get your life back." That sure felt good to hear.

His assistant gave Sarah the written copy explaining everything I had missed. Sarah spoke and motioned to the paper, asking questions until she seemed satisfied she had a grasp on the correct course of action. The first two items on the list were to get an X-Ray of my chest and have blood work done right away. It was unlikely, but I gathered from the discussion that there was a chance that this disease could impact my heart. My blood would now need to be tested every two weeks as Methotrexate can affect the liver.

Phew. We spent twenty minutes in Dr. Russo’s third assistant’s office after that. Trudy’s presence is infectiously enthusiastic; one can’t help but feel a little bit happier when one is sharing a room with her. She spoke with Sarah for several minutes, got on the phone and made appointments for us, faxed documents to our primary care doctor, and printed out information we would need.

We took the elevator to another floor to get my chest x-ray and blood work done.  We decided it was better to do it then than to wait until later. In the waiting room, Sarah got my attention and started to delineate the gist of all that had happened in Dr. Russo’s office.

“Honey, I’m not sure what you could or couldn’t hear. Do you have any questions about what just happened?”

 “Can you tell me what I have?”

“Oh, you missed that?” she asked.

“Yup. He hardly moved his lips.”

“I know,” she laughed, “it was almost as if his lips were frozen in a continuous smile.”

“I know!” We couldn’t help but try and mimic the expression: moving our lips ever so slightly with the rest of our faces completely statuesque. We have done this many times since.

“Okay, you have Cogan’s Syndrome,” Sarah steered the conversation back.

“Hogan’s Syndrome?”

“No,” she laughed again, “Cogan’s. With a ‘C.’”

“Oh, Cogan’s  Syndrome,” I said, finally understanding.

Sarah relayed the explanation in its entirety, speaking slowly and annunciating her words so that I could comprehend what she was saying. She told me that Dr. Russo had indicated I should be getting at least some of my hearing back with the treatment. She went on to express her conversation with Trudy.

“Trudy is amazing. I’m sure you saw that she handled her interaction with us like we were her first and only priority. She also explained where you will be going to get your treatments,” she hesitated to make sure I was following her. “You are going to the cancer center to get your infusions. She explained that you would be in a room with patients receiving chemotherapy and that she didn’t want us to get there and be shocked by it all.”


“Yeah. You are getting the steroid injections on Wednesday, Thursday and Friday, and then you will start Remicade on Monday. They moved some things around to make sure you could get in as soon as possible.”

“Busy week. How are you going to be able to fit this in with your work schedule?” At this point, I hadn’t driven in about three weeks due to my vertigo.

“We’ll figure it out,” she assured me. “How do you feel about today?”

“Really good,” I said. “We finally know what I have and that it’s treatable. It’s a bummer that it’s going to be such a long recovery process, but I am thankful that I’ve finally been diagnosed.”

“Yeah, and Dr. Russo seems really good. He immediately knew just by looking at your eyes and hearing your story. It was amazing,” she said.

“His bowtie was amazing.”


  1. We love Dr. Who! So glad he's incarnated as YOUR doctor!

  2. Yes! We should just start calling him "The Doctor."