Roger Beck cried for the first time in 22 years thanks to Randal Paniello, associate professor of otolaryngology. Paniello performed the first saliva gland transplant in the United States to restore Beck's ability to produce tears. Learn more about the procedure in this St. Louis Post-Dispatch feature.
Courtesy of St. Louis Post-Dispatch
(Republished with permission from the St. Louis Post-Dispatch. This article originally ran in the December 31, 2005, issue)
By Tina Hesman
St. Louis Post-Disptach
Dr. Randal C. Paniello brought a tear to Roger Beck's eye on Thursday.
It was the first time in 22 years Beck moistened up. Beck is not hard-hearted. A rare disease left him legally blind and unable to make tears.
Paniello, a head and neck surgeon at Washington University and Barnes-Jewish Hospital, transplanted a saliva gland from Beck's neck to a spot near his temple where it can bathe his eye with moisture.
Photo by Dawn Majors, St. Louis Post-Dispatch
Roger Beck, 29, of Sikeston, Mo., gets a kiss from 4-year-old son Denton Beck.
In other countries, the surgery has been done successfully more than 80 percent of the time for patients with a variety of dry-eye conditions. Paniello's is the first attempt in the United States. After nearly 12 hours of surgery, Paniello had connected the saliva gland.
"It's working. I'm seeing saliva coming out and it's just a thing of beauty," said Paniello, adding that the flow of liquid should increase over the next two to three months.
Thursday morning, Beck arrived at the hospital armed with Spider-man and a baby doll. His 4-year-old twins, Denton and Anna, lent him the dolls in case he needed a hug or a kiss.
"If I could have produced tears, I would have had one then," Beck said.
Paniello first read about the procedure in 1991. He tucked the idea into the back of his mind, waiting for just the right patient to try it on. When another doctor referred Beck to him, Paniello thought he could help.
In 1983, Beck was a normal 7-year-old until the day a mysterious illness caused his own immune system to turn on his skin and mucus membranes. On a Wednesday, Beck came down with a high fever. By Thursday morning his skin had begun to turn red and blister. His pediatrician gave him an antibiotic, but his fever kept climbing and he was hospitalized. On Friday morning, he was worse.
"His face, you couldn't see skin. It was blisters all the way down," said Rhonda Huffman, Beck's mother.
By the next morning, blisters covered the boy from head to toe, both inside and out. His pediatrician spent most of the night poring over medical books to diagnose the problem.
Beck, now 29, was eventually diagnosed with Stevens-Johnson syndrome, a severe disease that causes the immune system to attack the skin. It is usually caused by a reaction to drugs, especially anti-convulsant medications, such as those used to treat epilepsy; antibiotics such as sulfa drugs, penicillin or cephalosporin; or even anti-inflammatory drugs such as ibuprofen. In Beck's case, the disease may have been brought on by a viral infection. His mother blames a swim in a dirty pool.
The disease ravaged Beck's skin and mucus membranes and left him with diabetes. When he finally left the hospital after weeks hovering near consciousness, he was legally blind and unable to make tears. His lash line was split in two, causing one set of lashes to grow normally and the other to grow inward.
His eyes dried quickly. Every 10 minutes to 15 minutes he had to add drops to them to keep them moist, but sometimes his eyelids rasp across his eyes, scratching his corneas and dimming his sight.
"For 21 years he's suffered. Can you imagine sandpaper scratching your eye every day, all day long?" Huffman said.
But scratchy eyelids are nothing compared to inverted eyelashes. The lashes slice at his eyes like razor blades every time Beck blinks. He has suffered numerous surgeries to remove them. Severe infections led to the loss of his left eye when he was 12.
"I feel like Job every day. God told the devil, 'You can do anything to him except kill him.' I've been there," Beck said, referring to the biblical story of the long-suffering Job.
Doctors had warned Beck that he could lose his sight at any time. He credits divine grace for allowing him limited sight for so long. But Beck has run out of medical options to preserve his eye much longer, Paniello said.
So Thursday, Paniello and his assistants cut a flap of skin from Beck's temple and removed a small amount of a muscle to provide a cavity for the saliva gland to nest in.
The doctors removed a walnut-size submandibular salivary gland nestled under Beck's right jaw and the long salivary duct that connects the gland to the mouth. People have six major saliva glands. Removing one gland does not seem to cause problems for patients.
Saliva is mostly water, but it also contains a mild digestive enzyme called amylase and a protein called muscin. Muscin thickens saliva and other types of mucus. That could help the eye retain moisture better. The digestive enzyme is weak and doesn't appear to damage the eyes of people who have received such transplants, he said.
The surgeons connected the salivary gland's long duct to the upper inside part of the eyelid, close to the place where tear ducts normally release tears. Paniello also transplanted the facial artery and vein that feed the saliva gland, and a vein grafted from Beck's forearm, and connected them to blood vessels in the temple. Doctors in a Chinese study said fluid started to flow within 30 minutes of reconnecting the blood supply to the gland.
Paniello did not transplant the nerve that triggers salivary glands to work at the sight of food.
"You don't want the person to cry every time they get hungry," he said.
The gland will secrete a constant supply of liquid to bathe the surface of the eye. Later, another surgeon will attempt to reconstruct the inside of Beck's eyelid to stop it from scratching his cornea.
Copyright 2004 St. Louis Post-Dispatch, Inc.