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Rare disease hits young King George father

Nichole Langa was describing the way a rare disease changed her husband from a vibrant athlete, enrolled in a leadership program with the federal government, to a man who can barely walk or talk and has gauze stuffed in his mouth to absorb excess saliva.

At times, she stopped and looked into the dark eyes of Constantin, a Romanian by birth and American by choice. Because of his physical limitations, Constantin couldn’t hold his wife’s hand or put his arm around her.

He reached out in one of the few ways left to him.

As Nichole described the way she gets him up, dressed and into the bathroom each morning—just as she does their 3-year-old son—Constantin motioned in sign language. She smiled and clasped his hand, telling a visitor:

“He just made the sign for ‘I love you.’ ”

The lives of the Langas, who live in King George County, have been strained in every way imaginable since an ailment named Wilson’s disease swooped down on them three years ago.

But friends, family and even a specialist at Yale who’s treating Constantin praise the way the young couple has coped. Nichole is 32, and Constantin is 30.

“We have a very unique bond,” Nichole said, “and I think if we didn’t, we never would have made it.”

COPPER IS THE PROBLEM

Wilson’s patients have a defective gene that prevents them from processing copper—an element that’s needed for good health but can be poisonous in excess.

Normal readings, taken after a 24-hour urinalysis, are 10 to 20 micrograms of copper.

At his highest levels, Constantin had 2,534 micrograms.

Nichole was pregnant with their son, Traian, who was born in January 2010, when Constantin first showed symptoms.

He wondered if he was experiencing sympathy pains and asked her if she had excess saliva.

She said no, and little more was said.

About three months after Traian’s birth, his father starting drooling heavily. He also fell regularly, too. The young parents began fighting, which was unusual for them, and assumed they were overwhelmed by baby demands.

Constantin started shopping online, more uncharacteristic behavior because both Langas are frugal, Nichole said.

He was anxious and couldn’t sleep.

Their primary care doctor suggested a psychiatrist who verified signs of mental illness. But no one could determine why Constantin had problems speaking, and swallowing, as well as with physical coordination.

One doctor said he didn’t know what was happening and told the Langas to come back in three months.

‘LIKE KICKING A BEEHIVE’

The Langas ended up at Johns Hopkins Hospital in Baltimore and finally got a diagnosis by fall 2010. They had to wait another five weeks to see the right specialist.

Constantin was prescribed the safest of the limited drugs available, but a heavy dosage.

“Things got a thousand times worse when he started the medication,” Nichole said. “He went from falling to not walking at all.”

And he began to suffer incredible pain as the copper messed up signals from the brain. It directed his hands and feet to curl up and cramp constantly.

One of the few specialists on Wilson’s disease said the high dosage “was almost like kicking a beehive. You get them stirred up before they leave altogether,” said Dr.? Michael Schilsky, medical director of the Yale New Haven Transplantation Center in Connecticut.

He started seeing Constantin in August 2011. The heavy doses had been stopped, but they had caused the copper, stored throughout the body, to go into general circulation and cause problems to Constantin’s central nervous system.

Constantin’s mind was still as sharp as ever, said Micky Tingler, a college friend, but “he was trapped inside himself.”

For a time, he couldn’t even smile or grimace.

“It was like he had a dead face,” his wife said.

‘CONSTANTIN AGAIN’

Under Schilsky’s treatment, Constantin tried a different medicine that blocks absorption of any new copper and gradually pulls out what’s in his body.

A test last month showed he had 538 micrograms in his system—compared to 2,534 three years earlier.

His liver is scarred, but not to the point that he needs a transplant, his wife said. And the psychiatric symptoms he first displayed are gone.

The neurological damage remains, though Constantin is showing improvement.

His eyes twinkle, he smiles and he makes a grunting noise when he wants attention. Then, he either types on his iPad, which Nichole said has been a lifesaver, or uses sign language.

“I’m starting to see Constantin,” said Paula Van Alstine, his mother-in-law. “I’m in love with him again.”

Then, turning to him, she said, “Not that I didn’t love you before.”

“But we couldn’t see you in there,” Nichole said.

Van Alstine knew Constantin before her daughter did. She was a director at a fitness club where Constantin worked while earning his master’s degree from the University of Mary Washington. (He had a 4.0 average, she added.)

“I actually introduced Nichole and him in hopes they would hit it off,” Van Alstine said. When they “decided to marry is one of our happiest family moments.”

Photos from their honeymoon in Costa Rica in 2006 are in the living room of their home in Hopyard Farm subdivision.

It’s not the volcano or blackened rocks that attract the eye: It’s his arms and chest muscles. He is ripped.

Nichole believes his prime condition—and the fact that he didn’t poison his liver with alcohol in college—kept him from further deterioration.

Schilsky added that Constantin’s focus on nutrition and his being “extremely motivated” have helped also.

Longtime friend Jon Friedrich, who’s known Constantin since they were teenagers, agrees.

“He’s been nothing but positive through the whole thing,” Friedrich said. “He’s very religious, and he believes everything happens for a reason.”

CONSTANTIN’S VERSION

As for Constantin’s prognosis, Schilsky said he’s hopeful that he will continue to improve. “Time is going to be the final arbiter,” the doctor said.

Doctors used to believe patients would make all the recovery they would within the first year of treatment, but studies have shown that Wilson’s patients can improve for up to three years.

“It’s a slow process,” Schilsky said. “For some people, it takes time to pull [the copper] out.”

The Langas have been told that Constantin never will be 100 percent again. Constantin said he can live with that.

“He says he’ll be 99 percent with 1 percent room for improvement,” Nichole said. “That’s Constantin’s version of it.”

 

A LOVING FAMILY MAKES THE DIFFERENCE

The doctor treating Constantin Langa said his patient has quite

a resource in the fight against Wilson’s disease.

“He has a saint for a wife,

a woman who is extraordinarily engaged and caring, loving and extremely intelligent, trying to seek out every which way she can to support him,” said Michael Schilsky, medical director of the Yale New Haven Transplantation Center in Connecticut.

Nichole Langa has juggled the jobs of being Constantin’s advocate while she works full time as a financial analyst at the Navy base in Dahlgren, raises their 3-year-old son and schedules the caregivers who are with Constantin 18 hours a day.

In 2012, the Navy base acknowledged the challenges Nichole faces. She received the Paul J. Martini Award, one of the highest civilian honors given by the Naval Surface Warfare Dahlgren Division.

Nichole is quick to point out that their extended families help with everything from child care to mowing the lawn. Constantin’s mother, Daniela Langa, helps with groceries and takes him to local appointments. When his father, Romeo Langa, isn’t driving a truck cross-country, he’s in King George, helping with home maintenance, car repairs or putting up Christmas lights.

Nichole’s mother, Paula Van Alstine, is her “sounding board” and support system, and her father, Dan, provides meals and financial help.

Two of Constantin’s friends, Micky Tingler of Richmond and Jon Friedrich of Fredericksburg, usually visit once a week. They stay with Constantin and his son, Traian, and give Nichole a break.

Others in the King George community, as well as the Nativity of the Theotokos Greek Orthodox Church in Fredericksburg, which the Langas attend, and some practitioners of alternative medicine, have helped with donations, food and moral support.

Still, the juggling is an incredible burden, physically and financially. Nichole pays for the five caregivers, who stay with Constantin or provide therapy, out

of her salary. She takes off work for at least two days to take Constantin to Connecticut to see his specialist. And she monitors his diet to make sure he avoids foods that contain copper, such as chocolate, shellfish, beans and mushrooms. Everything he eats has to be puréed.

It’s been an eye-opener for the 32-year-old to learn about everything from Social Security disability to the cost of private caregivers.

“We’re in a group of people that’s overlooked,” she said. “We’re not on welfare, we’re not old-age, we’re not the typical patient.”

ABOUT THE DISEASE

About one in 40,000 people get Wilson’s disease, according to the National Digestive Diseases Information Clearinghouse. It’s a genetic disorder that causes copper to build up in the liver, brain, eyes and other organs.

One of the telltale signs, which Constantin Langa had, are Kayser–Fleischer rings in the eyes. They are rusty–brown rings around the edge of the iris and in the rim of the cornea.

Two drugs—d-penicillamine and trientine hydrochloride—are used in the treatment, and Langa had both. Both have the major side effect of worsening neurological symptoms, which Langa experienced. He started with the trientine, which is typically the milder of the two, but had high doses of it, which caused more problems.

He’s currently taking d-penicillamine, which his body is accepting.

Wilson’s disease requires lifelong treatment. After Langa was diagnosed, his wife, Nichole, was screened. She doesn’t have the gene and is not a carrier for it, which means their son, Traian, doesn’t have it either.

Cathy Dyson: 540/374-5425

cdyson@freelancestar.com

Permalink: http://news.fredericksburg.com/newsdesk/2013/06/15/rare-disease-hits-young-king-george-father/

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