Aortic aneurysmal disease is deadly

Anne Marie and Takahisa Miyashita

I recently lost my best friend from high school, Anne Marie (Giordano) Miyashita to what the medical examiner determined to be a "spontaneous dissection of an aneurysm of the ascending aorta." She collapsed and died Christmas Day 2002 after entertaining her brothers’ families for the holidays.

Although we lived on opposite coasts and corresponded by letter about twice a year, the life history and fun we shared from our high school years into our late twenties when I moved West, forged a bond undiminished by time or distance.

I’d open a letter from Anne Marie and an origami crane crafted of shiny gold paper would tumble out, or a dried flower from her garden, or yet another goofy picture of a frog which was such a long-running gag between us that I can no longer recall the origins of the joke.

Though a Moroccan by birth and a native French speaker who learned English on the fly when plopped into an American elementary school after her parents and brothers emigrated to the U.S., Anne Marie became enamored of things Japanese. At a Japanese language class she met Takahisa (Taka) Miyashita, shy and sweet with a very tenuous command of English. Somehow they communicated, fell in love, married and shared a life in a house on Robert Street in Hicksville, New York. I danced at their wedding. With Taka, Anne Marie finally achieved her most exciting dream—to visit Japan.

The pale blue envelope that brought news of Anne Marie’s sudden passing is soaked with my tears. Her origami crane sits on my nightstand, reflecting light. In my grief I turned to the Internet to research and understand what had taken her life. While I’ve learned that, for Anne Marie, there was likely no chance of survival because of the nature and location of the aneurysm in her aorta (the "main trunk of the arterial system of the body"), for others there is hope.

An aneurysm, from the Greek meaning "a widening," is a "localized abnormal dilatation [the condition of being stretched beyond normal dimensions] of a blood vessel, usually an artery. Due to congenital defect or weakness of the wall of the vessel," according to Taber’s Cyclopedic Medical Dictionary. In basic lay terms, it may be considered a balloon-like growth.

It may also be described in lay terms as an internal time bomb. Only great skill and precision can "defuse" (repair or remove) it safely, and even then there may be significant perils during and after the arduous and delicate surgery. While the medical community generally assigns a certain size of growth or expansion (about 5 cm.) to an aneurysm before surgery is indicated, the risk of a rupture may exist both before and during surgery. (There’s currently no drug known to "shrink" or "dissolve" aneurysms.)

Aneurysms generally exhibit a slow rate of growth. A person may live with aneurysmal disease much of his life without symptoms, and be informed that an aneurysm of significant size has been detected due to abnormalities noted during a physical or while testing for other medical conditions.

Medications such as beta-blockers and blood thinners and lifestyle adjustments including no strenuous activity or heavy lifting, no smoking and keeping weight/blood pressure under control, are advised for those who are not yet surgical candidates.

Among the Websites I discovered were two that shared accounts from individuals who had survived aneurysmal disease. Their stories described a commonality of symptoms and treatments and post-op prognoses. The accounts are full of terror, courage, medical "miracles" and a remarkable life-affirming optimism (and even humor) in face of what—to those stricken—must have felt like the full force of a volcanic eruption contained within their chests.

One Website, Aneurysm Outreach, Inc. at www.alink.org, stresses the critical importance of tracking family history and is hoping that "Any family history of aneurysms?" will become a standard question printed on medical forms that patients are required to fill out. Many aneurysmal patients have reported losing close relatives to aneurysms, which may also occur in the brain, abdomen, iliac and other arteries. There is also a connective tissue disease, Marfan’s syndrome (a hereditary condition) whose sufferers are at greater risk of developing aortic aneurysms than the general population.

Described unanimously by survivors as "severe, excruciating," the intense chest and back pain associated with aortic aneurysm rupture or dissection can mimic a heart attack—or indigestion. (In some cases, circulation may be cut off to a limb as well, producing numbness and clamminess in the affected area. There can also be head pain, temporary loss of vision and a sudden sense of extreme weakness.) That’s why family history is so important as well as a thorough, comprehensive exam. Better to be armed with knowledge and suggestions for additional tests that perhaps will make you seem irksome to a doctor than to be sent home with Pepcid or pain pills. A number of survivors report this surreal scenario, as do—tragically—the loved ones of those who failed to receive an accurate diagnosis and/or timely treatment.

Luck is a tremendous component in surviving a life-threatening illness. Many survivors’ tales pivot on getting "mercy flights" to major medical centers for treatment unavailable at their hometown hospital; coming under the care of an experienced cardiologist and highly competent surgeon and attending staff; having devoted family, friends and/or their faith to help them endure both the physical and psychological trauma.

In our "worst nightmares," there is no luck, no relief, no hope. In reality for people affected by or at risk for aneurysms—who receive timely and attentive medical care—the future may yet hold a wealth of dreams.

Helpful sources to contact for further information include Aneurysm Outreach Inc., a nonprofit organization "dedicated to mobilizing people and resources to eradicate aneurysms" at (225) 622-1577 or 17222 Highway 929, Prairiesville, LA 70769; and the Aneurysm and AVM (Arteriovenous Malformation) Support Page provided by the State University of West Georgia, Carrollton, GA 30118, at http://www.westga.edu/`wmaples/aneurysm.html. You can also search for this site by typing in Aneurysm and AVM Support.

If you are aware of or suspect a family history of aneurysmal disease, don’t wait for symptoms to strike—consult your physician immediately for testing, and have your children tested, too.