World's 51st case of disorder is in Michigan
Roger Schmidt believed he was certain of the day he was going to die.
Then, almost by mistake, on the day Schmidt thought would be his last, doctors discovered what was killing him — and arrested it in that 11th hour.
He has a disorder so rare that many doctors have never heard of it. There were perhaps 50 cases in the world.
"I was No. 51," Schmidt says.
The suburban Grand Rapids resident did not die that day. The last-minute discovery saved his life, for now.
This is the story of how a soldier who pointed howitzers at North Korea, who was in the advance team in the Desert Storm invasion, is shedding new light on a little known disorder with a benign name: CLIPPERS syndrome.
This is the story of No 51.
It is, perhaps, 200 steps from the parking lot pedestrian bridge to the hospital room where Schmidt nearly died. Hallway walls are beige, hung with fine art. The sixth-floor tiles are three shades of brown.
The one window in Schmidt's semiprivate room faces north toward a gleaming cancer hospital. A single beige curtain separates the beds.
In other words, it could be any room in any hospital. This is Spectrum Health in Grand Rapids, set atop the east bluff overlooking the Grand River Valley.
On Dec. 4, Schmidt was weak and incoherent. He was confused and did not know the current year or his birth year. He would stop in the middle of a sentence, and finish with an unrelated thought. He was dragging his left foot.
"It was bizarre, really bizarre," Diane, his wife, recalls. "He said, 'I really don't know where I'm at.' I said, "You're home!' It was scary to me, to put it mildly."
She made an appointment to meet Dr. Anjon Chakrabarty, an internal medicine specialist.
Schmidt, 52, barely made it into the building. He suffered what appeared to be a stroke in the strip mall parking lot, where Chakrabarty shares offices with three similar specialists and a physician assistant.
Chakrabarty immediately called 911. An ambulance took Schmidt directly to the hospital. His health declined as test after test came back negative.
Why was a special weapons sergeant who worked with the 3rd Armored Cavalry at the Korean demilitarized zone dying? Why was an ammo sergeant, who was in the advance team for Desert Storm and who exercised with his family at 6 a.m. daily at a local club, dwindling before them?
By Feb. 24, Schmidt and his wife were preparing for the inevitable. They were certain this was the last day for the former auto mechanic and master technician for Sears, more recently doing maintenance at Porter Hills Retirement Home.
His heart was failing. So were his kidneys. He lost strength and 42 pounds. He limped on his left side.
A close call
Diane, 49, is sitting at home on a couch with a maroon slip cover. Behind her are pictures of their family. Wall hangings read "Live, Laugh, Love," and "Faith starts here." Nearby is a novel-length 400 pages of medical records.
Was Diane prepared that February day for life without Roger?
Her blue eyes are brimming now. Roger has his right arm around her shoulders. She recalls when they met at a small white clapboard church, better suited to a New England setting than hard up against railroad tracks in Comstock Park, a community of about 7,000 people just north of Grand Rapids.
"He is my rock," she says, collecting herself. "We have been through so much together. I'd never have done it by myself."
CLIPPERS' official name does not roll off the tongue: chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids.
Translation: Lesions near the brain stem produce myriad life-threatening complications. The afflicted part of the brain stem is a message station between several areas of the brain.
Diagnosing it is like addition by subtraction. Doctors test for more common diseases first. Start at the top, work your way down. When all tests are negative, it could be CLIPPERS. Tests can confirm it — if doctors even know about it.
With so few diagnoses worldwide, it is hard to tell exactly how many there are at given time. CLIPPERS is not even listed on the Rare Disease Registries in Europe, though cases there are known.
Still, medical literature suggests Schmidt is near accurate in his estimate of the number of cases — perhaps spot on.
That February day, as Schmidt's health became precarious,Dr. Daniel Legault happened by his room with a trainee. He asked if he could see Schmidt's reports.
Legault had read an obscure medical article a few years earlier and said, "I think I know what this is." Within hours, No. 51 had his diagnosis and began to respond to treatment. He was discharged within a week.
"It was very last minute," said Diane Schmidt, a cook at Heather Hills retirement home. "He would not be here, in my mind."
Legault did not return calls seeking comment.
Schmidt's discharge papers, dated Feb. 25, list various diagnoses: heart disease, kidney failure. And there it is: "CLIPPERS Syndrome."
CLIPPERS is a chameleon. Among reasons it is difficult to identify is its symptoms shift, perhaps depending on where lesions form. Few people experience precisely the same symptoms as others.
The prognosis is unknown. What is known is that strong doses of steroids will arrest the disease, especially if caught early. But steroids cause their own health problems over time. The disease is almost certain to return if steroids are discontinued.
There is hope No. 51 will provide more insight.
Schmidt was to travel to the Mayo Clinic, which first reported on the disorder in 2010.
"We raise the possibility that CLIPPERS syndrome may be more common than realized," doctors wrote for the Journal of the American Academy of Neurology in 2013. "We surmise that careful review of perplexing MRI imaging on difficult cases could reveal CLIPPERS as the diagnosis for many more potentially treatable cases."
Doctors want to learn from No. 51. Hidden cancer remains a concern.
Regardless, Schmidt is not expected to recover heart and kidney functions, but remains optimistic yet realistic.
"If we have a relapse, we know that will probably be the end of it," Diane says.
No. 51 smiles that quick, wide smile, white teeth, hazel-eyes crinkling. Friendly.
CLIPPERS syndrome is among the rarest neural disorders in the world, with only a handful of diagnosed cases. It's official name is chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids.
■Identified in 2010, by doctors at the Mayo Clinic in Rochester, Minn. There were eight known cases.
■Mimics multiple sclerosis and other disorders, but is separate and difficult to identify.
■Symptoms vary from person to person, making it hard to diagnosis.
■What it is: Lesions near the brain stem produce myriad life-threatening complications. The afflicted part of the brain stem is a message station between several areas of the brain.
■Strong doses of steroids will arrest the disorder, if caught. But steroids cause their own health problems over time. The disease is almost certain to return if steroids are discontinued.
■Kent County's Roger Schmidt returned to the hospital on May 2 with a failing heart. He was released May 12.