Sticky Blood: Tragedy and Hope

My friend, *Ann, and her husband, *Don, suffered through several miscarriages. One pregnancy miraculously resulted in the birth of *Ken, a perfectly healthy baby boy. They were also blessed with the ultimate miraculous joy of having a son and a daughter through adoptions. Ken grew up, finished high school with honors, and began his college career with a generous scholarship. He was quickly elected as an officer of a prestigious fraternity, while holding a part-time job and fulfilling his university coursework obligations.

As he walked his usual several miles to class one day, he collapsed, and passers-by called 911. Family and friends prayed, as University Medical Center's (UMC) staff scrambled to save Ken's life and diagnose his problem.. Ann and Don took turns at UMC in a round-the-clock vigil with their son, in Tucson, Arizona. While one was at UMC, the other was about twenty miles away, comforting their younger daughter and son waiting at home.

Ken had not been ill. He had not been in an accident. He was not a drug user. He was not a habitual smoker or drinker. He lived a healthy lifestyle. In fact, he had recently conquered the steep trails of the 14 mile round-trip Grand Canyon hike, as well as accomplished grueling hikes in the extreme altitudes of Colorado's national forests. The question, "Why was this young man at death's doorstep?" burned on everyone's lips.

Frustrations mounted as hours turned to days and days to weeks, but Ken's health responded and answers slowly emerged. The collapse was caused by blood clots in his lungs. A combination of blood tests, computed tomography (CT) scans, and magnetic resonance imaging (MRI) eventually revealed the clotting's extent. The diagnosis took longer, but the final report was clear: Ken had Antiphospholipid Syndrome, or APS, also known as "sticky blood." Simply stated, Ken's young, twenty-something-year-old body was fighting itself. His antibodies declared war on his own plasma proteins, or phospholipids, by producing blood clots. The blood clots blocked arteries and veins, cutting off blood supply to his lungs.

Consequently, Ken, family, and friends were immediately immersed in a new language of medical terminology and the shocking realization of how tragic this event could have been. Ann and Don could not type an Internet search on the computer fast enough. They hurriedly gathered information about "sticky blood" and how to help Ken win the war against it. They shared their additional knowledge with eager friends and family, as their connections and prayers entwined with the doctors' vigilance to increase Ken's strength and health. All were anxious for Ken to return to his wonderful twenty-something life as quickly as possible.

As Ken's recovery quickened, he had new habits to make. He reluctantly succumbed to the necessity of always wearing a medic alert emblem, because of medications necessary to surviving and dealing with "sticky blood." He learned diligence, as he had never imagined before, in maintaining additional healthy living behaviors. At school and work, immediate and long-term needs, as well as learning to self-advocate, took their toll on him. It would be a long road, but with the right professional guidance along the way, as well as family and friends ready with listening ears and supportive patience, it would happen.

Fraternity brothers, who smoked, quickly realized that they must never smoke near him again. Smoking was relegated to outside, if anywhere around the frat house. The occasional hookah bar outing became a thing of the past. During a visit to Ken's hospital room, Ann and I shared this thought, as we watched some of his frat brothers leaving: "Wouldn't it be a wonderful side effect if the smokers of the group quit because of this?"

As Ann and Don continued studying resource after resource for why and how this happened to their son, they made three amazing discoveries:

1. "Sticky blood" is not as rare as one might think. Many times it goes undiagnosed because a victim of it is unaware - and their first 'event' is their last. Strokes, heart attacks, and blood clots in the lungs take a person's life before diagnosis is possible. Ken was a living miracle, yet again.

2. Pregnant women who have 'sticky blood' often suffer from multiple miscarriages or premature births. Ann and Don endured such losses after Ken's birth. Could Ann also be an unknowing victim of "sticky blood?" Yes, research showed it is a genetic autoimmune disorder. Ann's doctor ordered blood tests, but surprising results showed she had Anticardiolipin Syndrome and ANA antibodies, a condition similar, but not exactly like APS. She and Ken found they shared grave risk for stroke, heart attack, and life-threatening blood clots, in spite of the differences in their specific syndromes.

3. They learned why the babies following Ken's birth did not survive. Both syndromes can cause miscarriages. The revelation provided closure for the previously unexplained tragedies. Many parents of lost pre-term babies never have that luxury.

So, what does this mean to others? Who should be tested for "sticky blood?" Women with history of miscarriages, stillbirth, retarded fetal growth, toxemia (high blood pressure in pregnancy), or unexplained placental abruption should be tested for antiphospholipid antibodies along with their other routine medical tests. Anyone with a history of blood clots of any kind, heart attack, stroke, or other autoimmune disorders, including lupus should ask their doctors about the possible need for testing. APS statistics show "15 - 20 percent of deep vein thrombosis cases, one-third of strokes in people under age 50, and 5 to 15 percent of recurrent miscarriages" (Kugler par.4) are due to APS.

How is antiphospholipid syndrome treated? Patients can receive injection(s) of the blood thinner, Heparin, to prevent blood clots. They may have long-term prescription protocol, including the blood thinner, Coumadin, as well as baby doses of aspirin. Some people are prescribed Prednisone, but it is important to note the physician and patient decide which protocol best suits the individual patients' needs, risks, and potential side effects of their treatment.

Pregnancies are at particular risk. Pregnant women known to have the antiphospholipid antibodies are monitored on a monthly basis through a variety of methods, which may include ultrasound, non-stress tests, and others which may be determined by the physician and patient team.

Is there hope on the horizon? Yes! Scientific research continues to move forward on this, as well as other less known blood disorders. One exciting development began in 2001, with the formation of a national registry. Eight locations across America began collecting data on symptomatic APS patients, as well as people who were not exhibiting symptoms - even though they had APS antibodies. Robert A. S. Roubey, M.D. published findings on these clinical trials in July of 2003. Warfarin was recently reported as a promising new drug weapon in the battle against APS. The Antiphospholipid Syndrome Foundation of America, Inc. remains a good resource in watching for more new trends and additional hope for winning the war on APS.

* Names changed for privacy rights considerations.

Bibliography:
Personal account as told to Herstory, by *Ann, Tucson, Arizona, September 2006
Antiphospholipid Syndrome, by Mary Kugler, APS Foundation of America, Inc, at www.apsfa.org
National Institute of Arthritis and Musculosketal and Skin Diseases, at www.niams.nih.gov
WebMD Health Search, at www.webmd.com
National Library of Medicine, NCBI, PubMed, at www.pubmed.com