Jose Suarez

Jose Suarez of Owatonna undergoes plasma exchange therapy at Mayo Clinic in Rochester. Doctors believe he is suffering from thrombotic thrombocytopenic purpura, or TTP, a rare blood disorder that affects 3 to 4 out of 1 million people. (Photo courtesy of Dianne Suarez)

OWATONNA — It’s not every day that breaking a hip would be looked upon as a godsend, but for Jose Suarez of Owatonna that broken hip might very well have saved his life.

But he’s still not out of the woods.

Suarez, 65, is in Mayo Clinic in Rochester where he’s been since mid-January, battling a rare blood disorder that was discovered quite by chance.

How rare?

The disorder — thrombotic thrombocytopenic purpura, or TTP for short — is a rare autoimmune blood disorder that affects about 3 or 4 people per million each year.

Since being diagnosed with TTP — actually, the doctors have said that Suarez has “presented TTP symptoms,” said his wife, Dianne — he has remained in the hospital undergoing daily plasma exchange therapy, the therapy of choice for those with TTP.

Though the therapy has stabilized him, his condition has not improved, prompting his medical team to look to more costly treatment options and finally to something much more intrusive — removal of his spleen, an operation he is slated to undergo on Friday.

Discovering the disorder

Jose Suarez’s medical odyssey began in his kitchen on Jan. 19, about 8:30 on that Monday night. Exactly how the journey began is not so certain.

Jose — who, while in the hospital, declined a personal interview, but spoke through his wife — said that he remembered having a muscle spasm and his knee buckling, causing him to fall and break his hip.

But medical personnel say it could have been that there was a tiny crack already in his hip that broke, causing him to fall.

No matter, Jose found himself on the kitchen floor, unable to get up.

With the help of neighbors and ambulance personnel, Dianne Suarez was able to get her husband to Owatonna where doctors planned to operate, if not that evening, then early the next morning, Dianne said.

But then, routine pre-operative blood work came back.

“They did the blood work and were seeing unusual blood level results,” Dianne said.

Specifically, they were told, Jose’s hemoglobin was low. Very low.

“They didn’t know how he was walking around,” Dianne said.

The doctors in Owatonna contacted the doctors at Mayo and it was agreed that he should be transported to Rochester that night. Immediately, in fact.

The only thing they weren’t certain of is whether he should be given a transfusion before making the trip.

On the one hand, Dianne said, the doctors at Mayo wanted to see exactly what the doctors here were seeing without that new blood in him.

On the other hand, they were concerned about his health and safety if he traveled that far without that transfusion.

In the end, Dianne said, “they gave him the blood.”

Facing tough odds

Jose Suarez is no stranger to facing difficult situations and tough odds. When he was just 12 years old, he was arrested, not because of anything he had done, but because of who he was, or, more specifically, who his family was.

Born in Cuba in 1949, a decade before Fidel Castro took power. Reared in the city of Cárdenas, where his family owned and operated a grocery store, his family was considered to be middle class, the bourgeoisie.

When Castro, a communist, nationalized all businesses shortly after coming to power, families like the Suarez family were persona non grata in Castro’s Cuba.

Jose was fortunate to have been part of Operation Peter Pan, an organized effort between 1960 and 1962 to bring more than 14,000 Cuban children to the United States. He first came to a Florida refugee camp, then moved to Toledo, Ohio, to live with a foster family.

“I left my parents behind,” he said in 2008 interview at the time that Fidel Castro announced his resignation as Cuba’s president. “But I was very, very fortunate that my parents were able to leave about a year later.”

His parents arrived in Florida on the last flight from Cuba on Oct. 13, 1962, just before the Cuban Missile Crisis. The family reunited in Toledo.

‘A rare, true medical emergency’

Once Jose arrived at Mayo that night in January, they were able to stabilize him and then fixed his hip the next morning.

“We were happy with the way that went,” his wife Dianne said.

That’s when the doctors went to work to try to find out what was going on with his blood. The initial diagnosis was TTP.

Thrombotic thrombocytopenic purpura is what Taryn Byrne, a spokesperson for the Answering TTP Foundation, has called a “rare, true medical emergency” — a condition that can cause heart attacks, kidney failure, brain damage and even death. And it all has to do with the platelets in the blood not performing their normal function, Byrne said.

Platelets are the part of the blood responsible for clotting so that when a person cuts himself, the blood is able to stop flowing.

But for a TTP patient, a problem develops with the platelets.

What happens is this: The platelets in the blood stream become sticky or spongy and start sticking together, causing clots in the blood that can travel to the heart, the kidneys, the brain and other vital organs.

But, Byrne said, that’s just part of it. Because the platelets are not performing their normal function, if a person with TTP gets cut or has a fall, he could have a difficult time stopping the bleeding, externally or internally.

What’s more TTP seems to impact the red blood cells so that they are unable to perform as they should as well. That means that the vital organs to a person’s body may not be getting oxygen and other things that the red blood cells are supposed to carry to those organs.

That’s just part of the picture. The rest of the picture can get a bit fuzzy.

“There’s still a lot we don’t understand about TTP,” Byrne said.

Chief among the mysteries is what causes the disorder — a disorder that Byrne called “not discriminatory, affected the very young and the very old, the chronically ill and those who are otherwise very healthy. They simply don’t know what causes it.

“That’s one of the million-dollar questions we’re trying to answer,” Byrne said.

In Jose Suarez’s case, the doctors at Mayo have looked for possible causes, testing him for a number of maladies that may — or may not — be associated with TTP, including hepatitis, leukemia, bone marrow cancer, organ damage, HIV and other cancers, but all the tests have come up negative.

And, in fact, it’s not even certain that Jose has TTP, though the doctors at Mayo say he presents TTP symptoms. Still, even the most definitive tests have come back inconclusive.

“But all the symptoms say he’s got it,” Dianne Suarez said. “They’re calling it atypical TTP.”

Treating TTP

If all goes according to plan — and plans have been changing at a rapid pace — Jose Suarez will have his spleen removed on Friday morning.

The doctors will try the less-invasive laparoscopic surgery, but with Jose’s spleen — enlarged — they may have to resort to traditional surgery, his wife said.

About the size of a fist, the spleen acts as a filter for the blood, recycling old red blood cells and storing both white blood cells and platelets.

What doctors have discovered is that Jose’s spleen is enlarged — a condition that can be caused by liver disease, blood cancers and mononucleosis, among other things.

Or, Dianne Suarez said, the doctors are thinking that perhaps, just perhaps, the enlarged spleen may be related to his TTP. It could be causing the TTP, or the TTP could be causing the spleen to enlarge. Or perhaps they are not related at all, Dianne said.

But the doctors have told them that there have been some TTP patients who have had their spleens removed, have recovered from the disorder and have gone on to live long, happy and healthy lives.

Some, but not all.

It wasn’t the first treatment that Jose received.

When he was first admitted to ICU, he received plasma exchange therapy, considered to be the “therapy of choice” and the “first line of treatment” for TTP patients, said Byrne.

The plasma exchange therapy, sometimes called PLEX, has proved effective in 80 percent to 90 percent of the TTP patients who receive the treatment right away. But 90 percent to 95 percent of the patients who don’t receive the therapy will die.

The therapy is done using a machine that replaces the patient’s plasma with donor plasma, thus both removing antibodies and replenishing normal plasma proteins. The process takes three to four hours each day and uses up to 20 bags of plasma a day.

Jose has been undergoing plasma exchange every day since Jan. 21, with the exception of two days, his wife said.

“Most people would respond to plasma therapy,” Dianne said. “He didn’t budge.”

He was stable, she said, but not improving.

So the doctors took him off the therapy for two days. But when they did, his condition worsened.

“He’s been on ever since,” Dianne said.

The doctors then added prednisone, a steroid that’s been used to slow the immune system and, therefore, slow the progression of TTP, an autoimmune disorder.

Still nothing.

Then last Friday, the doctors broached moving to an even more aggressive treatment, what Dianne Suarez called “jumping to the big gun.” That big gun is the drug Eculizumab, which goes by the trade name of Solaris and is used for the treatment of blood disorders.

But there were a few catches.

For one, the drug is very expensive — $20,000 per dose, which, with their co-pay, would still cost the Suarez family $4,000 a dose. The drug would have to be given at a minimum of once a week for a month, then every other week for a month, and then once a month until remission is achieved. That’s at a minimum seven doses.

Not only is it expensive, but it’s also difficult to get.

“They only have three doses in the hospital, and they keep that for emergency use,” Dianne said.

There’s also the fact that TTP is not on the list of ailments for which the drug is approved, meaning that the insurance company could say no.

And even if the drug is approved and is used, there’s no guarantee that Jose won’t experience a relapse. Relapses with the disease are not uncommon — about 30 percent.

Byrne said the founder of the foundation has experienced relapses with the disease six times.

That’s when the doctors at Mayo approached Jose and spoke about removing his spleen, Dianne said.

“They said it was worth a try. It might do the trick,” Dianne said. “But there’s no guarantee.”

And if it doesn’t work?

“If it doesn’t help, it’s back to the drug,” she said.

Longing for home

In the midst of his treatment, Jose Suarez is still contending with recovering from the broken hip, a process that is not progressing as quickly as it might normally because of how tired he is.

“He’s not up and moving as much as he should,” his wife said.

Yet, he remains in good spirits, eating fine and reading the newspaper with Dianne every day. But it’s not easy for either of them.

“It’s wearing on him,” Dianne said. “He really wants to come home.”

Reach Managing Editor Jeffrey Jackson at 444-2371, or follow him on Twitter.com @OPPJeffrey

Jeffrey Jackson is the managing editor of the Owatonna People's Press. He can be reached at 507-444-2371 or via email at jjackson@owatonna.com

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