Man waits for live liver donor

Paul Smoot, 36, of Strasburg, sits beside his wife Kutrina inside his mother's home n Stephens City recently. Paul Smoot was born with immune deficiency disorder and now needs a liver transplant from a live donor. Rich Cooley/Daily

Last September, Paul Smoot, of Strasburg, forgot how to use his cell phone.

Talking with him, his wife Kutrina couldn’t determine the problem. She asked if he felt all right, and he said he did. But then after trying several times to reach him at work that morning, she received a phone call from his boss at Howard Shockey & Sons’ precast group, where Smoot works as an estimator.

“You need to come and get him,” she recalled him saying.

Her husband had been trying to work at his computer — but his boss could see that something was wrong. Instead of typing, Smoot was staring at his computer’s screen saver pattern.

Born with an immune deficiency disorder, Smoot is more susceptible to illness. Last fall, the level of ammonia in Smoot’s blood was so high that his wife said he was in a sort of brain fog for days, unable to remember her name or the names of their four children. He had encephalopathy — a system of global brain dysfunctions that can have many causes.

His immunologist told the family the hepatitis that Smoot developed from an I.V. as a teenager contributed to the development of liver cirrhosis nearly 20 years later.

“Liver disease takes a really long time to show itself,” his wife said. But once his symptoms started, she said they progressed quickly.

This development followed adema that Smoot suffered two summers ago, when his legs and ankles swelled painfully.

Now 36, he needs a liver transplant from a live donor. The donation can be a partial liver, he said, since livers regenerate so easily. But the donor must have certain qualifications — someone in good shape between the ages of 21 and 50 who can pass a physical and has a matching O blood type. The donor must be a nonsmoker and agree to the stringent requirements for alcohol consumption.

The transplant will take place at the Hospital of the University of Pennsylvania, and the donor must have a friend or family member who can help in the recovery process.

Barbara Smoot, of Stephens City, said she would have gladly been a donor for her son, if she had fit the age requirements. His wife is a blood match and might have been a donor too, but then both husband and wife would require caregivers to help them recover.

She said it would be tough on her parents-in-law to care for the two of them for so long, in addition to finding care for their grandchildren.

Hoping for a miracle, the family explained Smoot’s immune deficiency disorder makes him a poor candidate for receiving a liver donation from a registry list. He’s too high risk, his wife explained. But, his surgeon told the family he feels good about the transplant process he has planned, should the family find a donor.

Smoot is the second of his three brothers identified with the disorder, though his mother said her first son, John W. “Jody” Smoot Jr., didn’t survive. He died at the age of 16 months.

Her second son, Scott, escaped the condition.

“I worried about him,” Barbara Smoot recalled. When he reached 14 months without incident, she said she was able breathe relief. “I thought I’d try for a girl, so I got Paul,” she said, laughing.

But Paul Smoot began experiencing illness at about 6 months and was diagnosed with the hereditary blood disorder agammaglobulinemia at 16 months. His body doesn’t make white blood cells like it should. His blood count per microliter is less than 100, when it should be between 5,000 and 10,000. For most of his life he has needed injections of gamma globulin.

His first round consisted of 11 shots into his leg muscles, his mother recalled, but the number of monthly shots kept increasing as he aged. It was a painful process and required a needle injection of the thick substance made from human plasma that contains antibodies to help fight disease.

Moving him to an intravenous method helped, his mother said. “He’s been getting that ever since.” For a while, home care came to administer the plasma. Now Smoot gives himself the weekly subcutaneous injections he said take about two hours to administer.

A 1997 graduate of Sherando High School in Stephens City, Smoot earned his associates degree in mechanical engineering from Lord Fairfax Community College in Middletown. This is his 16th year working for Shockey of Winchester.

The company has been awesome, his wife said — “The amount of support and whatnot that they’ve been giving, ’cause he’s been out so sporadically in the last year.”

But although he’s feeling better for now, the family knows he’ll need a transplant soon. And hoping for a miracle isn’t easy.

“You have to know the person good enough to want to be out of work for as long as it takes you to recover,” said Kutrina Smoot. “They’re not covering your bills. His insurance is only going to cover the procedure and the doctor time. All the transportation, all the food costs, I mean, this is all the way to Philadelphia.”

Self-employed as a part-time housekeeper, she said, “I’ll just be out of work if they accept me.”

Ideally, the donor should be a friend or family member.

“You’re going to have to like him a lot to want to do this for him.”

For information on donating a liver to Paul Smoot, call Linda Wood, a registered nurse and living donor coordinator of the Liver Transplant Program at the Penn Transplant Institute, at 215-662-6200, or email . Call 1-800-789-PENN or visit http://www.PennMedicine.org to learn about donor qualifications.

Contact staff writer Josette Keelor at 540-465-5137 ext. 176, or jkeelor@nvdaily.com