Dr. Kohrt, who was known as Brook, had no family history of hemophilia — a disorder in which blood does not clot normally — but his parents became alarmed shortly after he was born when he developed mysterious bruises and bled profusely after his circumcision.
Child protection workers investigated, wondering whether he was being abused. Doctors discovered that his gene for a clotting protein known as Factor 8 was not functional.
Dr. Kohrt wore a helmet to guard his head against injury until he was 7. He began giving himself infusions about the same time, injecting himself with a lifesaving blood-clotting factor. (His second-grade teacher fainted when he demonstrated the process at show and tell one day, he said.)
Classmates ridiculed him. People with religious objections to transfusions spat at him. “To this day, I’ve never encountered so much bias and irrationally steadfast antagonism about a person doing what he needs to do to stay alive,” Dr. Kohrt said.
When he was 8, he began attending a summer camp specially equipped for hemophiliacs.
“About 80 percent of these kids got H.I.V.,” he told Stanford Medicine in 2013, referring to the human immunodeficiency virus, the virus that causes AIDS. “As a result, there are about 50 percent fewer hemophiliacs alive today than there would have been without H.I.V.”
The camp disbanded after eight years because only he and one other camper were left. (Since then, doctors have found ways to purify donated blood and to engineer the clotting factor genetically without depending on human donors.)
Holbrook suffered a painful and enigmatic infancy marked by unexplained bruises, swelling, and uncontrolled bleeding. He was eventually diagnosed with Severe Hemophilia, a rare genetic disorder caused by missing or defective Factor VIII, a clotting protein needed to stop external and internal bleeding. “Treatment” for the disease often consists of prevention of accidents, and Holbrook wore a helmet during his waking hours until he was seven years old to prevent him from a possibly fatal head injury. Combined with his armory of slings, splints, crutches, and mobility aids, his disease was not to be an invisible one, and thus he quickly learned to be a spokesman for the disease in his small town.
Brook’s lifesaving treatment carried with it high risks for infection, especially as a child in the ‘80’s, when HIV/AIDS became a pernicious and stigmatizing global epidemic. Around 80% of individuals with severe hemophilia contracted HIV during that time period. Holbrook described the effect of HIV on his childhood and perception of mortality:
“From when I was about 10 until I was about 15 or 16, I was very aware that my risk of contracting HIV and other pathogens increased with each transfusion. I was also very aware, though, that without the transfusion, I would die. I watched some of my best friends become infected in this way, and saw them go through the process of dying from AIDS and the stigma the disease carried at that time. The whole experience was very shaping.” (from In His Blood by Krista Conger, Science Writer at Stanford University. April 2013)
Holbrook was one of the lucky ones who didn’t contract HIV, though he did acquire, and eventually recover from, Hepatitis C. From the time Holbrook was old enough to understand his disease, he was mature enough to understand the implications, and he lived his life with a sense of foreshortened future.
He became extremely achievement-oriented, pushing hard to accomplish quickly, wholly, and unapologetically. He had his younger siblings carry encyclopedias in their backpacks when they were with him so he could be constantly learning, and he spent hospital stays as a teenager immersed in news articles and applications for leadership programs. He graduated valedictorian from Wallenpaupack Area High School and went on to study Molecular Biology at Muhlenberg College, where he graduated Summa Cum Laude and valedictorian. He attended Stanford University Medical School as a Baxter Foundation Scholar, Howard Hughes Scholar, and American Society of Hematology Research Fellow. During that time, he developed and implemented a system for predicting risk for early stage breast cancer. He completed a Ph.D. in clinical trial design and tumor immunology at Stanford, and conducted his dissertation on the use of an agonistic monoclonal antibody capable of enhancing the efficacy of tumor-targeting therapeutics…
Brook lived his life at a dizzying pace, achieving and experiencing with a blinding vigor. Everything he did was at full-speed: he drove fast, he loved hard, he sang loud, and he danced with every part of his often-aching body...
“Oh, yes, but it’s more philosophical than physical. I realized early on that I have to do everything I want to do as soon as possible because I didn’t know what the future could be. That’s been useful in terms of the research and the science. I have the stamina and the commitment to keep trying things. It’s not been so good in terms of personal relationships. I’ve been married twice. But that knowledge forces me to take the time I have to give the maximum to science and to my patients. Research requires great tenacity. When you’ve had a serious illness since infancy, you know to make the most of every single day.”...
Over the past three years, Holbrook’s hemophilia had become increasingly complicated. His body built up a resistance to the clotting factor that he injected into his body, almost daily, to prevent bleeds. He had chronic arthritis in his joints from recurrent bleeding, which limited his mobility and led to decreased bone density and increasingly severe muscle atrophy. As his disease progressed, he rallied his intellectual and social resources in an attempt to discover a treatment for the life threatening resistance he had developed to his medication. He relocated to Europe in 2015 for experimental hemophilia treatments…
In February of 2016, with his hemophilia treatments showing promise, Holbrook flew to the Caribbean for a retreat aimed at refocusing personal and professional priorities. He reached out to his familiy and friends to express his desire to build on his relationships. While there, he developed a severe infection and was flown to Jackson Memorial Hospital in Miami. A number of complications related to his hemophilia, severe sepsis, and a lifetime of attempting to manage intense pain converged on February 22nd when he suffered a catastrophic bleed into his brain. Friends and family from around the world came to be with him during his final hours. He passed away on the morning of February 24th, with his mother and younger brother, Barret, at his bedside.
Courtesy of a colleague
It’s not the years in your life, it’s the life in your years.