During the summer of 2006, Maureen and Bill Merz' daughter Kristen was a perfectly healthy 17-year-old and recent high school graduate busy working with special needs children and preparing for college. But then one July morning Kristen started feeling weak and fatigued and began experiencing shortness of breath.

Figuring Kristen caught a bug from one of the children she comes into contact with at work, mom Maureen took her daughter to an acute care center for blood work. Results of tests conducted showed that Kristen's oxygen levels were well below normal while her creatinine level was at 7, well above the normal functioning kidney level of .08. Doctors immediately put Kristen on oxygen and transported her, via ambulance, to Winthrop-University Hospital. There, emergency room physicians told family members that there was uncontrollable hemorrhaging in Kristen's lungs, which was preventing her from absorbing oxygen, and that the teenager was in kidney failure.

Doctors explained that Kristen's kidneys were completely destroyed, with no hope of recovering. After several days in the pediatric intensive care unit (ICU) and a multitude of tests, Kristen was diagnosed with anti-glomerular basement antibody disease, also known as Goodpasture's Syndrome, a very rare autoimmune disease (1 in 2 million) that attacks the lungs and kidneys.

Doctors placed Kristen on a ventilator and into an induced coma while they began dialysis, inserting a catheter to remove the toxins from her blood. They then used the same catheter to employ a blood purification procedure known as plasmapheresis. Following the procedure, Kristen remained in ICU where she underwent approximately 21 days of plasma replacement, 17 transfusions and a kidney biopsy, making her the first Winthrop patient to undergo a kidney biopsy while on a ventilator.

After the biopsy, Kristen's blood pressure was unstable and it was nearly three weeks before the bleeding in her lungs stopped and she could be removed from the ventilator. Withdrawal from the coma-inducing drugs, however, began to cause seizures.

It was a month before Kristen could leave ICU and, by that time, she had spent so much time bedridden that her muscles had atrophied and physical therapy was needed. Kristen was transported to St. Charles Rehabilitation Center in Port Jefferson where she endured two weeks of grueling rehab to teach her how to walk again and regain coordination.

It was mid-September 2006, nearly two months after arriving at Winthrop's emergency room, when Kristen was finally able to return home, but her medical treatments were far from over. In addition to undergoing dialysis four hours a day, three days a week, doctors informed the Merz family that a kidney transplant was Kristen's only hope of resuming a normal life.

As a result, the Merz family was referred to Columbia Presbyterian Hospital in Manhattan and a search for a match began. While finding a suitable match is extremely rare and most people spend years waiting for a sutaible donor, tests confirmed that both Maureen and Kristen's aunt were matches and, in January 2007, a now 18-year-old Kristen went back into the hospital for transplant. Six hours later, Kristen's "new" kidney - donated by mom Maureen - was functioning properly.

"Its scary to think about how easy it was for me to get a kidney when there are thousands of people out there waiting for organ transplants without family members that can donate," said Kristen. "The average person spends at least three years on the 'waiting list.'"

When it came to donating an organ to her daughter, Maureen takes no credit. "I suppose, to some, donating an organ to save a life is very noble, but when it comes to donating an organ to your child it's not something you even need to consider," said Maureen. "There are people out there who donate their kidney to a friend or even someone they've never met. Now [to me] that's noble and I have tremendous admiration for those who do that."

April is designated Organ Donor Awareness Month and, for the Merz family, the importance of organ transplants and donation cannot be stressed enough. "Transplantation is one of the most remarkable success stories in the history of medicine but despite continuing advances in medicine and technology, the need for organs and tissue is vastly greater than the number available for transplantation," Maureen said. "Organ and tissue transplants offer patients a new chance at healthy, productive, normal lives and return them to their families, friends and communities. You have the power to change someone's world by being a donor."

"One single person can save up to 10 people upon death by being an organ donor. Many people die everyday waiting for that 'gift of life,'" said Kristen.

Despite the surgery's resounding success, Kristen is not permanently out of the woods. During the first year after the transplant, Kristen had to medically withdraw from college because she was sick a lot, suffering from various infections as well as bronchitis and shingles. Because her immune system is artificially suppressed by anti-rejection medications, Kristen must be more cautious than the average person when it comes to being exposed to germs.

Immediately after the transplant Kristen had follow-up doctors' appointments every other day but now sees her nephrologist at Columbia Presbyterian Hospital once every three months and her nephrologist at Winthrop-University Hospital once every two months. She will be required, however, to take immunosuppressive drugs the rest of her life to avoid rejection but that number has been significantly reduced from some 10 immediately after the transplant to just two a day now.

"When I first got sick, I wasn't able to do much except sleep and go to dialysis. I couldn't even walk for long periods of time," said Kristen. "Eventually, I gained my strength back but not until almost three months after the transplant. I spent six months on dialysis. I got sick a lot up until the one-year anniversary [of the transplant], which was this past Jan. 19.

Although she has to stay away from sick people and has to be careful when in crowds of people due to the fact that she can get sick so easily, Kristen said like if "finally getting back to 'normal' now." And as a part of that normalcy, Kristen plans to return to Nassau Community College in the fall.

According to Merz family, they could not have gotten through the past year without all the "tremendous support received from family and friends." "First and foremost on my mind during the ordeal was to stay strong by focusing on the present, taking it one step at a time and dealing with the future as it comes," said Maureen.

Goodpasture's syndrome or anti-glomerular basement antibody disease, is a rare autoimmune disease by which the body's defense system reacts against some part of the body itself. When the immune system is working normally, antibodies to fight off germs are created while the immune system of a person affected with Goodpasture's syndrome makes antibodies that attack the lungs and kidneys.

First signs of Goodpasture's syndrome are usually vague and can include fatigue, nausea, difficulty breathing or paleness as well as coughing up blood or burning sensations when urinating. These preliminary symptoms are typically followed by kidney involvement, represented first by small amounts of blood in the urine, protein in the urine and other clinical and laboratory findings. The disease is diagnosable through a blood test; a kidney or lung biopsy may be necessary to check for the presence of the harmful antibodies.

Goodpasture's syndrome may last only a few weeks or as long as two years. Bleeding in the lungs can be very serious and even fatal in some cases but Goodpasture's syndrome does not usually lead to permanent lung damage. Damage to the kidneys, however, may be long-lasting. If the kidneys fail, dialysis to remove waste products and extra fluid from the blood, or kidney transplantation, may become necessary. Treatment includes oral immunosuppressive drugs to keep the immune system from making antibodies; corticosteroid drugs given intravenously to control bleeding in the lungs, if necessary; and a process called plasmapheresis, which may be helpful and necessary to remove the harmful antibodies from the blood.

For more information on Goodpasture's syndrome, contact the American Kidney Found_ation 1-800-638-8299; email helpline@kidneyfund.org_; or visit www.kidneyfund.org.