By Geraldine A. Collier
Courtesy of Worchester Telegram & Gazette
Doctors are taught in medical training to “look first for the horses, not the zebras.” In other words, look first for the typical causes of symptoms before exploring more exotic possibilities. But, former Worcester School Superintendent John Durkin wouldn’t be alive today if his doctors at UMass Memorial Medical Center hadn’t quickly found the exotica that was threatening his life. And after the problem was found, practically the entire staff of the medical center’s hematology service went further, holding off death while they fashioned a treatment where none existed.
Since he retired in 1992, Mr. Durkin had enjoyed excellent health, allowing him to take on the job of director of Worcester State University’s Center for Effective Instruction and spend plenty of time on the golf course. At the end of last year’s golfing season, however, Mr. Durkin was tiring easily; he cut his game back, walking six holes instead of nine. He also developed a limp and his hip started to bother him. Arthritis, he thought.
Winter passed uneventfully, but one day in mid-March, Mr. Durkin felt severe pain in his back and groin area. That night, Mr. Durkin slept in a chair downstairs, unable to make it upstairs to his bedroom. In the morning, he called his daughter and told his wife, Joan, that he needed to go to the hospital.
As he tried to get out of the chair, Mr. Durkin fell. “My leg had given out like a piece of wet spaghetti,” he recalled. At the emergency department on the Memorial campus, a primary care team found that Mr. Durkin was experiencing spontaneous bleeding into the psoas muscle, the muscle that joins the lower back and the inner thigh and is responsible for leg movement and spine flexibility. That explained Mr. Durkin’s symptoms, but not the ultimate cause.
“He had this bleed, but that by itself isn’t necessarily alarming,” said Dr. Bradley Switzer, a medical hematologist (blood doctor) at the University campus who was called in for consultation. A blood test, however, revealed an answer, but one so unusual that more tests were done, according to Dr. Switzer, “to see if it was for real.” It was, necessitating Mr. Durkin’s transfer to the University campus where hematologists saw a medical problem that rarely appears outside of a medical textbook.
“Mr. Durkin had acquired this one-in-a-million autoimmune phenomenon, where the body attacks its own proteins,” said Dr. Switzer. Now, the body has 12 clotting factors — numbered using Roman numerals through XII — that work together in sequence to keep your blood in a fluid state, without clotting and without bleeding. About one in 5,000 American men are born with a deficiency of Factor VIII, making them hemophiliacs. If they are cut or bruised — sometimes even spontaneously — they bleed longer than a normal person and can bleed to death.
Mr. Durkin was not born with this problem, but somewhere along the way as he grew older — he’s 79 now — he had acquired an inhibitor of Factor VIII. “His autoimmune system was attacking and chewing up Factor VIII,” said Dr. Switzer. “When it was all chewed up, it was like he was a hemophiliac; he just couldn’t stop bleeding.”
Daily bleeding caused Mr. Durkin’s right leg to swell up, from his knee to his hip, but that wasn’t the only area of trouble. The blood was “oozing anywhere, everywhere,” said Dr. Switzer. While loss of blood could be compensated for by large transfusions, the only chance for Mr. Durkin to survive was to stop the bleeding. But, according to Dr. Switzer, “his condition was so rare there aren’t any great treatments for it.”
Consulting at every turn with Dr. Doreen Brettler, medical director and adult hematologist for the Hemophilia Treatment Center of New England, Mr. Durkin’s doctors tried several drugs, looking for something whose previous use would suggest it might be effective. “We had to throw everything, including the kitchen sink at him,” said Dr. Switzer. “He almost bled to death on us several times, but because everyone here was fighting for him, doing everything we could, not holding anything back, it turned around.”
Doctors hit upon a drug called Rituxan (Rituximab), a chemotherapy weapon usually used to fight non-Hodgkin’s lymphoma and leukemia. It was worth a try, the doctors thought, since nothing else had worked and they were running out of options after nearly a month. And Mr. Durkin was running out of time. After the second grueling six-hour intravenous treatment, Mr. Durkin stopped bleeding and tests showed his blood coagulation was starting to normalize. “It literally saved his life,” said Dr. Switzer.
Mr. Durkin was well enough at this point to be transferred on April 16 to Fairlawn Hospital for physical rehabilitation, but while he was there, he would still need to return to the UMass University Campus on an outpatient basis for a third and fourth intravenous treatment with the Rituxan. Since Mr. Durkin had not finished his course of treatment yet, the prospect of his possibly starting to bleed again was very much on the minds of the Fairlawn staff.
“Everyone was definitely concerned and cautious about it,” said Dr. Trista Brown, physical medicine and rehabilitation specialist at Fairlawn, “but we wanted him to progress as much as possible, too, so you just have to take extra precautions.” An extra therapist was present at all rehabilitation sessions to steady Mr. Durkin should he begin to stumble or fall. The pace of the rehabilitation was slowed down to ensure safety. No orthopedic devices were used initially because there was worry about possible bruising. And his skin was frequently monitored for signs of trouble.
According to Dr. Brown, Mr. Durkin had a “pretty profound” weakness in his right leg because the nerves that travel through that area were damaged by the blood collection there. (That blood should eventually be reabsorbed by his body.) Because Mr. Durkin had trouble flexing his hip or extending his knee, an immobilizer brace — one that didn’t press anywhere — was put over his entire right leg to help his mobility. He had been on “complete bed rest” at UMass for more than a month, and his whole body had weakened.
“In rehab, we generally try to address the person as a whole and try to improve endurance, strength, range of motion, core functioning, etc.,” said Dr. Brown. The goal, according to Dr. Brown, was to have Mr. Durkin strong enough to be able to manage a wheelchair by the time he was discharged and to be able to do basic things for himself at home — for example, washing up and dressing. Mr. Durkin exceeded those goals. Instead of a wheelchair, he was able, using a walker, to walk over 100 feet down the Fairlawn corridors. And the leg brace was reduced to the knee, ankle and foot.
Besides the Fairlawn staff, Dr. Brown credits a great deal of Mr. Durkin’s success to the work he was willing to put into rehabilitation. “He was always motivated, always had a positive attitude, absolutely a pleasure to work with. He really made our job easy by wanting to work so hard.” He now returns for outpatient rehabilitation that focuses on walking and learning how to use the brace, according to Dr. Brown.
So what does the future hold for Mr. Durkin?
After the fourth intravenous treatment with Rituxan, Dr. Switzer was able to tell Mr. Durkin that there was no sign of the inhibitor in his blood. With the acquired inhibitor now completely gone, Mr. Durkin’s blood will clot normally if he gets a cut or a bruise. However, Dr. Switzer said, “there is a reasonable chance of it (the acquired inhibitor) coming back. Regretfully, there is no way for us to tell if he will be normal for the rest of his life or it will recur.” “I’m very pleased with the progress I have made so far,” said Mr. Durkin, who hopes for “a long time” with his wife Joan and their family of six children and 11 grandchildren.