Published on May 14, 2006 By Katie E. Leslie  News-Post Staff MYERSVILLE — Judy Ziegler cries without tears.

Photo by Bill Green

Because of a serious eye disease she developed years after an allergic reaction caused her skin to blister and slough off, Judy Ziegler was once going blind. After several operations largely restored her vision, she is able to tolerate small amounts of sunlight. She watches her son Tucker's baseball game from inside a tent and wears thick black sunglasses indoors and outdoors to protect her eyes.

You can tell she is crying because her face reddens and facial muscles tug her beautiful smile into a grimace. But her tear ducts, one clogged with scar tissue and the other surgically closed, offer no release.

Judy hasn't been able to shed tears in more than a decade. Eleven years ago, she developed a rare illness that caused her skin to blister and fall off. Though she survived, her eyes were marred by dry scar tissue that began to cause blindness.

Unable to see clearly for years and living in constant pain from the scar tissue, Judy said knowing her three children needed their mother kept her going. While she endured painful surgeries to restore sight, her husband, Bob, shouldered the demands of raising their young family — Judd, 12, Tucker, 8 and Becca, 5.

"Without my children, there were days I might not have gotten out of bed," Judy said. "I kind of just push the pain down inside me to get through the things I need to do."

A recent operation restored much of her sight in her left eye. For the first time in five years, Judy is able to see her husband and children on Mother's Day.

Unexplained, deadly reaction

It began with a stomach flu.

Judy remembers falling ill on March 2, 1995, with what seemed like an intense, yet run-of-the-mill stomach bug. She was a 29-year-old math teacher at Catoctin High School. When her nausea and fatigue worsened on the third day of illness, she called her sister and physician, Dr. Mary Howell, for help.

Her sister determined the stomach illness and 103-plus temperature should have been improving, and prescribed an antibiotic.

Judy had taken Cipro, or ciprofloxacin, once before with no problems, she said. She had also taken over-the-counter pain killers along with Cipro.

When she awoke the next morning, she and her husband, Bob, also a teacher, noticed a small, scarlet rash on her neck and cheeks.

When they realized the rash was spreading and red bumps were growing, Bob took her to Frederick Memorial Hospital. Her sister, an internist who had been working at Frederick Memorial Hospital for a few months, immediately began researching the cause of Judy's illness.

"I've lived a life with allergies, but nothing that I thought would take my life," said Judy, who lives with her family in Myersville. Since she was a young girl, Judy battled food allergies with certain fruits and shellfish.

She was too weak to get out of her hospital bed on March 6. The hot, itchy bumps had spread all over her body, except for her hands and below her knees.

Dr. Anusha Belani, an infectious disease specialist, examined her later that day. When Dr. Belani pressed down on Judy's face to examine her eyes, a layer of skin sloughed off beneath her fingertips.

"I could feel the skin move under her fingers, but I was too in fever-land to

realize what was going on," Judy recalled.

FMH staff members knew they were unable to treat her condition and a helicopter took Judy to the University of Maryland hospital.

"I didn't really feel emotional about it until I saw my in-laws when I was leaving the hospital. They were really emotional," she said. "Then I thought, Oh my goodness, something must really be wrong with me."

Once she reached the University of Maryland hospital, doctors began a series of tests, which confirmed Judy had toxic epidermal necrolysis syndrome, or T.E.N.S., a rare, life-threatening skin disorder in which the top layers of skin blister and peel off in sheets.

A disorder that is estimated to affect approximately one in a million people annually, T.E.N.S. has a 30 to 50 percent survival rate, according to the "British Journal of Dermatology." The majority of cases involve recent drug ingestion, according to the journal, as well as a study of T.E.N.S. published in the March 2002 "Annals of Burns and Fire Disasters."

The recovery of patients who develop T.E.N.S. greatly depends on how quickly they are transferred to a burn unit, where specialists are trained to treat raw skin and prevent infection, a major cause of death in T.E.N.S. patients.

No one knows why Judy developed T.E.N.S. Some doctors believe it was an allergic reaction to taking Cipro, but because Judy fell ill days before taking the medication, others have said it was an autoimmune reaction.

"Nothing that she took or anybody did had anything to do with the illness. When we look back on the years, she's always been such an allergic person," Dr. Howell said. "Having something like this is like getting hit by lightning. It's rare."

In an attempt to salvage her deteriorating skin, doctors and nurses piled bandages on her. When nurses lifted her forward to bandage her back, her skin slid off in several large pieces, she said.

By the evening of March 7, Judy was unable to breathe properly. Tissue sloughing off in her throat, trachea and bronchial tubes was clogging her airways, Dr. Howell explained.

Dr. Howell remembers physicians asking her whether Judy should be resuscitated if she stopped breathing.

"That was one of the most unbelievable moments for me," Dr. Howell said. "I told them, 'She's 29 years old with a 1-year-old child.' Of course you put her on life support and you better get busy doing it.'"

After hours of labored breathing, Judy was put on a ventilator. She remembers her husband standing at the foot of her hospital bed before she was sedated.

"I told him 'I love you and make sure Judd knows I love him,'" Judy recalled. "I didn't think I was going to live at that point."

Judy wouldn't open her blistering eyes again for two weeks.

Two weeks of waiting

The day following her intubation, a groggy, sedated Judy was transferred to the Baltimore Regional Burn Center at the Johns Hopkins Bayview Medical Center, where doctors explained the complications of T.E.N.S. to her husband.

If she could live through the first 14 days of skin deterioration and infection, doctors said, she would likely make it. But the outlook was grim.

"I remember telling my brother I was going to be a single parent," Bob said. "I was preparing for the worst."

Horrified by what was happening to his wife, he declined the hospital's request to have her illness photographed for research purposes.

"Looking back, we should have," Judy said.

When word of her condition spread among members of the Zieglers' church, Brook Hill United Methodist Church, letters of support began pouring in from not just Frederick County, but around the world, including people the Zieglers had never heard of, she said.

The Rev. Conrad O. Link, pastor of Brook Hill UMC, spent several days at the hospital with the Zieglers, monitoring Judy's progress. He said what started as a prayer chain evolved into a massive prayer experience that went beyond the Frederick County community.

Judy was on a ventilator and had a feeding tube for 10 days. While her family waited to see if she would survive, Bob read letters from well-wishers to his gauze-covered wife. A morphine drip managed her pain.

Three times a day, doctors would sweep under her eyelids with a glass rod to prevent her raw skin from fusing to her eyes. The tissue of her gums and inside her cheeks also attached in places.

Two weeks passed and Judy continued to improve, largely because before her illness she was healthy, doctors told Bob.

Almost all of Judy's body began to heal, from her legs to back to throat to eyes.

"For the first 14 days, she was one big scab," Bob said.

Once off ventilation, she felt the pain of healing — having her bandages replaced daily was excruciating, she said.

While in the hospital, Judy insisted on listening to the popular children's shows "Barney & Friends" and "Lamb Chop's Play-Along," because she knew her infant son Judd was watching them in a Frederick County day care center.

"That was my time with my son," Judy said.

She endured daily sessions in the hospital whirlpool to prevent bacterial infections.

"I remember waking up in the whirlpool and seeing this very reddish pink mutilated skin and little patches of skin floating in the water, but yet it was attached to me," she said.

Judy refers to the 45 minutes a nurse spent peeling decayed skin from her face as the best facial she's ever had.

Healing ahead

Judy returned to Myersville to recuperate on March 31. Despite the "facial," she was horrified by her appearance.

"I looked like something out of Star Wars," she said, describing the dry skin that hung from her bright pink cheeks.

A nurse from FMH visited daily to change the bandages, but Judy and her nurse were unable to keep the raw skin from fusing to the gauze.

"Every day, my nipples would be ripped off and bleed ..." she recalled. "Just four months prior to that, I was nursing my son."

Though her skin healed within a few months, it remained hypersensitive. Her husband wasn't able to hug her for months without causing tremendous pain, she said. Judy was also unable to sweat for roughly a year, and couldn't be outside for more than a few minutes or exercise at all.

Determined to resume her life, Judy returned to the classroom in June for the final days of the school year, but realized she wasn't hearing as well as before. She went to an otolaryngologist for a consultation where she learned a pencil eraser-sized piece of dry skin was clogging her ear like a cork.

"(Having the plug pulled) was more pain than birthing all of my children," Judy said.

T.E.N.S. also left Judy with other health problems. After not having a menstrual period for three months, she went to her gynecologist to see if the illness had damaged her reproductive organs. An examination revealed her vaginal opening had fused shut, a surprising condition quickly corrected with surgery.

Though it wouldn't happen for several years, Judy's greatest battle with T.E.N.S. was in her eyes. Her doctor at the Bayview Medical Center warned she would likely suffer from eye problems, but until 2000, they were minimal.

She was seeing as well as she did prior to T.E.N.S., though the thick scar tissue that framed her eyes was a daily reminder of her illness. To this day, Judy must keep track of how many eyelashes she has to make certain no follicles are becoming clogged or growing inward. She has 48 above her left eye, 25 above her right.

Judy credits her survival to the power of prayer.

"I can honestly say I had thousands of people praying for me — that's all it could be," she said. "Even though I was in this hospital, freezing cold, skin falling off, I was at peace. I was OK."

With the exception of scar tissue in her eyes and a chronic cough from scarring in her lungs, life seemed to return to normal by 1997, the year Judy gave birth to Tucker. She had no problems with her pregnancy, but breast-feeding was nearly impossible because of scar tissue in her breasts, Judy said.

"That was the only major problem in five years, until I had Becca."

After surviving toxic epidermal necrolysis syndrome in 1995, which causes the skin to blister and slough off in sheets, Judy Ziegler's eyes are encased in scar tissue. The Myersville woman began going blind in 2000 because of a serious eye disease she developed as a result of T.E.N.S.

After surviving toxic epidermal necrolysis syndrome in 1995, which causes the skin to blister and slough off in sheets Judy Ziegler’s eyes are encased in scar tissue. The Merryville’s woman began going blind in 2000 because of a serious eye disease she developed as a result of T.E.N.S.

When Judy Ziegler returned home from the hospital in January, she saw her 5-year-old daughter's smile for the first time since Becca was born.

After cataract surgery to her left eye, her fifth major eye surgery in less than three years, Judy could see Becca's lips, nose and light brown hair.

"I was so used to relying on my right eye to see her. I cried when I saw Becca with both of my eyes together," Judy said.

Two weeks after giving birth to Becca, Judy began to feel severe pain and burning in her eyes. It was July 2000, more than five years since Judy had suffered an allergic reaction that caused her skin to blister and slough off. She was among the estimated one in a million people globally who develop toxic epidermal necrolysis syndrome each year.

T.E.N.S. left Judy with severe scarring in her eyes, as it does many survivors. Though she was warned by her doctors in 1995 that she would likely have eye problems, she suffered minimal eye pain until Becca was born.

Doctors believe that after her third pregnancy, Judy's immune system went into overdrive and began attacking the cells in her eyes.

Judy's vision began to deteriorate. As months passed, seeing the faces of her children ----Judd and Tucker, now 12 and 8, and Becca ----became impossible.

"Becca was very blurry. I strained myself to try and see her," Judy said. "I could tell her body. I know her arms and her legs. But I couldn't tell anything about her facial features at all. As far as her early years of being a baby, that's a blank."

Battling blindness

Judy has ocular surface disease, a condition characterized by chronic dry eye syndrome, pain, blurred vision and burning. Her condition is worsened by the pieces of scar tissue that fused between her eyelid and eye when she had T.E.N.S.

To control the agony in her eyes, doctors put Judy on steroids. She had just given birth to Becca and the medication caused her weight to balloon to more than 180 pounds, said Judy, who is 5 feet 4 inches tall.

Unable to tolerate light or wind, she was quickly relegated to the darkest rooms in her home. She stopped driving because of her blurred vision. Perforations in her cornea made it seem like she was seeing the world through crinkled plastic wrap, she said.

But much worse was having to give up teaching after 15 years, she said. Judy was a math teacher at Catoctin High School.

At the urging of her primary ophthalmologist, Judy and her sister, Dr. Mary Howell, a physician, sought opinions from several sources.

One doctor recommended Judy begin immunosuppressant drugs, believing she had systemic active inflammatory process, or chronic inflammation. However, a physician at the Wilmer Eye Institute at Johns Hopkins told the sisters that nothing could be done.

Judy also sought advice from the National Eye Institute of the National Institutes of Health to no avail.

"I guess I was frustrated, but I knew that I was in God's hands," Judy said of the years she spent without an answer. "I knew these places I was sent were not part of the plan."

She underwent chemotherapy for roughly five months to suppress her overactive immune system, but it did not help. By December of 2002, Judy's primary ophthalmologist, Dr. Marwa Adi, had recommended she learn to read Braille and get a seeing eye dog.

"I kind of swallowed hard and realized some hope was being dashed," Judy said. "I knew I was in pain and I knew things were bad, but I thought -- this is way more serious than I ever expected."

Then Dr. Adi told Judy and her sister to consult Dr. Edward J. Holland, who in the early 1990s pioneered a procedure using stem-cell and cornea transplants to restore vision.

Dr. Holland is the director of cornea and external disease at Cinncinnati Eye Institute and an expert on ocular surface disease. He immediately accepted Judy's case, and a stem-cell transplant in her left eye was scheduled for January 2003.

"Within the first five minutes of meeting him, I knew I was in the right place and he was the miracle worker God sent in my path," Judy said.

Hope restored

Judy's sister was first considered as a stem cell donor, but because she had worn contact lenses for years, she was deemed ineligible. The stem cells came from a cadaver.

The goal of the surgery was to strengthen the thin cornea in Judy's left eye, which was badly damaged from T.E.N.S. One year later, she underwent another partial transplant.

Her vision improved slightly, though she still could not see facial details, much less read.

"I did not have a clear view of any of my children's faces. I could tell their sizes, but even simple things like cutting their fingernails, cleaning their ears or checking a boo-boo, I couldn't do," she said.

In August 2004, Dr. Adi surgically placed a patch over the weakest part of her cornea. The surgery seemed successful, until a day in November 2004 when a single tear rolled down Judy's left cheek.

"I knew something was wrong, because I can't cry (with tears)," Judy said. Her tear ducts are closed, one clogged with scar tissue from T.E.N.S. and the other surgically closed to help retain all natural moisture in her eyes.

Judy's cornea had ruptured.

She and her sister rushed to Cincinnati to see Dr. Holland, and Judy was scheduled for a corneal transplant.

Though the surgery was successful and Judy's vision began to improve, a major problem remained. The steroids Judy had taken for years caused cataracts to form in both of her eyes.

"Try putting a piece of tape over the lens of your glasses ----(that's a cataract)," Dr. Howell said.

Judy would need another surgery to restore vision in her left eye.

She underwent cataract surgery this past January. For the first time since July 2000, she could see the faces of people whose voices she has known for years.

One of the most profound moments after regaining vision in her left eye was going to church.

"I could see a big cross in front of the church. I remember being really emotional about that," Judy said.

When her vision was poor, friends would approach her and identify themselves by name, she said. Now she is able to greet them first.

Since the surgery, Judy has also been able to see her own reflection.

"I hadn't looked at myself in years," she said.

She is still sensitive to light and wind, and must wear thick, black sunglasses wherever she goes. She is known in Myersville for her shades.

"Sometimes I think I wear them just because when I take them off, it's very obvious something is wrong with me," Judy said. "(Before T.E.N.S.), I really had a great complexion and I had great eyelashes.

"My husband is still so wonderful. He still tells me I'm beautiful, even though I know I'm É"

Judy paused.

"Whether I was 180 pounds on prednisone or 130 like now, or with a scarred face, he stills tells me I'm the most beautiful person in the world to him, which blows my mind."

It takes a village

During Judy's struggles, Bob assumed the major duties at home, including driving the family, packing the children's lunches and cooking. Many of their friends and family pitched in to help take care of the children, especially when Becca was an infant.

"Can you imagine trying to change diapers without seeing?" Dr. Howell said.

In many ways, Judy's experiences with T.E.N.S. and her subsequent eye condition have strengthened her marriage and family, she said.

"Each of these hurdles have made us stronger as people, better parents and stronger Christians," she said. "We matured as a couple and our faith multiplied."

Bob said Judy's challenges make their family unique.

"I knew when she was in the hospital, our life was going to be different," he said. "I don't think we're a normal family."

Because Judy can't drive and their children are too young to stay home alone, the family travels together when Judy needs to run an errand. In fact, they do everything together, he said.

A teacher at Myersville Elementary School, Bob coaches his sons in wrestling and baseball. During sports seasons, the family piles into their red pick-up truck and traverses Frederick County for games and meets.

Even after Judy's most recent surgery, the boys need to help their mother by leading her when sunshine or wind gusts blind her, or by reading to her or Becca. Judy is still unable to see fine print.

"I think they've learned a lot of patience," Bob said. "I'm hoping my boys become better men because of it."

Judy's relationship with her older sister also strengthened through their years of commuting to doctor's visits together. During the critical stages of Judy's illness, her sister's medical expertise was invaluable.

"My sister is the best thing my parents gave me. My education is the second," Dr. Howell said.

Though the vision in Judy's left eye is not perfect, it's vastly improved. She must undergo the same surgeries in her right eye down the road.

But even with successful surgery, her sight is not guaranteed.

"I know it's not going to last forever, but I'm so much further than I was before," said Judy, 40. "It's hard to think of a day I won't see."

Moving forward

Judy said she does not blame anyone for her illness.

"It's easy to ask 'Why God? Why do I have to endure this?'" she said. "But everybody has something they have to endure in life. It's all about how you choose to handle it."

Her doctors hold differing theories as to why Judy developed T.E.N.S. Dr. Howell is among those who believe the T.E.N.S. was an autoimmune reaction to the stomach flu Judy had in 1995, but Judy said she thinks it was caused by the Cipro. They've only once discussed the possibility the Cipro, which Dr. Howell prescribed, caused her illness, Judy said.

"I told her I don't blame her for anything that has happened to me. I only hold her responsible for things that improved my life," she said.

Though Cipro is documented to have caused the deadly reaction in other cases, Judy and her family never once considered pursuing legal action against the pharmaceutical company.

"I am just not that kind of person," she said. "I move forward and I'm not looking back."