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Finding Fibromuscular Dysplasia: Trying to Diagnose an Illusive “String of Beads”

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Finding Fibromuscular Dysplasia: Trying to Diagnose an Illusive “String of Beads” SUMMER 2008 • VOL. 8 NO. 3 THE OFFICIAL PUBLICATION OF THE VASCULAR DISEASE FOUNDATION FIND OUT ABOUT... • Nutrition and Vascular Disease • Running Shoes vs. Walking Shoes • Renal Artery Stenosis • The Ankle-Brachial Index (ABI) • Frequently Asked Questions • Aneurysms • VDF HealthCasts • New Spanish Resources • New PAD Coalition and African Episcopal Church Partnership Finding Fibromuscular Dysplasia: Trying to Diagnose an Illusive “String of Beads” VDF is proud to announce the first annual On a hot day at the end of July, 37-year-old Pam Mace woke up with a left-sided headache that intensi- FOCUS ON VASCULAR DISEASE CONFERENCE! fied as the day progressed. When she noticed that her August 16th • Denver, CO pupils were unequal and her headache was so severe that she could not pick up her head, she went to the Top vascular medicine specialists will be presenting different emergency room (ER). It wasn't until two more trips topics on vascular disease and there will be educational to the ER that she was told that she had a transient ischemic attack (TIA) or mini-stroke. displays. Join us this summer in beautiful, colorful Colorado For many months Pam suffered with an array of for this exciting event! Visit us online at www.vdf.org for symptoms: severe headaches, a “swishing noise” in her more information. ears, dizziness, and a feeling that she was about to faint. She also had trouble regulating her blood pres- sure, and her pupils were unequal in size. Months later doctors were able to diagnose the cause of her TIA as fibromuscular dysplasia (FMD). FMD is an unusual blood vessel disease that leads to abnormal cell development in the artery wall that causes narrowing which decreases blood flow through the artery. FMD is most commonly found in the arter- ies that supply blood to the kidneys (renal arteries), followed by the carotid arteries of the neck that supply blood to the brain. FMD can also affect the arteries in Continued on Page 2 Finding Fibromuscular Dysplasia: Continued from Page 1 the abdomen (supplying blood to the liver, spleen and intestines) and extremities (arms and legs). In Pam's case, the arteries in the back of her neck were affected first and she experienced a dissection (a tear or rip) in one of her vertebral arteries, then she had another dissection in her left carotid artery in the front of her neck. “I had such a bad headache that nothing relieved the pain,” said Pam. “I spent over 11 days in the hospital and no one could seem to figure out what was wrong with me. I was very afraid that I was going to have a massive stroke.” An angiogram of a right internal carotid Many people with FMD do not have symptoms that can be detected during FMD with the “string a general physical exam. The signs or symptoms that a person exhibits depend of beads” shown. on which arteries are affected, and the degree of narrowing within them. Some Image use courtesy of patients with FMD have no symptoms at all. Cheryl Bailey FMD can be diagnosed through a number of methods including CT scan, MRI, ultrasound and angiogram. In the most common forms of FMD, a charac- teristic “string of beads” appearance is seen in images of the renal arteries that supply blood to the kidneys. This is caused by changes in the cellular tissue of the artery wall that causes the arteries to alternatively become narrow and dilate, or expand. In more aggressive forms of FMD, the vessel will narrow without the “string of beads” appearing. It was the “string of beads” that led to Pam's diagnosis of FMD. After a series of tests and many trips to the ER, she still had headaches that were so painful she could- Our Mission n't work. In a follow-up MRI, a radiologist noticed that she had a carotid aneurysm. To reduce death and After a second opinion, it was determined that Pam had suffered two aneurysms and disability from vascular had three arterial dissections or tears in the arteries of her neck. Renal FMD was also diseases and improve discovered. vascular health for all “As an ER nurse, I knew something was wrong, but it was so frustrating that the Americans doctors couldn't figure out what was causing my symptoms,” Pam said. “When the doctor saw the classic “string of beads” in my renal arteries during my angiogram, he indicated that I had FMD. I finally started to feel like I knew what was causing all of my Keeping In Circulation is published by the Vascular problems.” Disease Foundation. To receive a There is no free subscription or additional cure for FMD copies contact the VDF at 1075 South Yukon St., Ste. 320 nor is there a set Lakewood, CO 80226 protocol for treat- Call 888.VDF.4INFO ing it. Depending or Visit www.vdf.org which arteries are affected will deter- Newsletter Editors mine the kind of Sheryl Benjamin Christa Saracco treatment used. A procedure called Medical Editor angioplasty is the treat- Janice Nunnelee, PhD, RN ment most commonly Medical Advisory Board used for severe narrowing Heather Gornik, MD, Chair due to FMD. Angioplasty is Judith Fried, RVT often performed at the same Carolyn Robinson, RN time as an arteriogram during Anton Sidaway, MD which the radiologist, vascular surgeon, or cardiologist inserts a wire into or near the affected artery After suffering for months through an array of symptoms, Pam Mace and injects contrast material or dye (pictured above) was finally diagnosed with fibromuscular dysplasia or Continued on Page 3 “FMD”. Image use courtesy of Pam Mace. 2 Vascular Disease Foundation • www.vdf.org • 888-VDF-4INFO Board of Directors Mark A. Creager, MD, President that can be detected by X-ray. If an angioplasty is performed, a catheter is extended into the affect- Anton Sidawy, MD, President-Elect ed artery and a small balloon is inflated that “stretches” open the artery. A metal stent is typically Judith A. Fried, RVT, CVN, Secretary not required to keep the vessel open for patients with FMD. David Faxon, MD, Treasurer “Right now, there is a great need for more research on FMD to determine which patients are at Alain Drooz, MD, Past-President Alan T. Hirsch, MD, Past-President risk and the optimal treatment. The care of patients with FMD focuses on determining which John “Fritz” Angle, MD blood vessels are involved and whether or not a procedure needs to be done to treat severe symp- Richard H. Carmona, MD Kenneth Cherry, MD toms due to this disorder,” said Dr. Heather Gornik, a specialist in FMD at the Cleveland Clinic. “I Heather Gornik, MD also treat patients with carotid FMD with antiplatelet therapy, usually aspirin, and I work on con- Patricia S. Hofstra, JD Michael Jaff, DO trolling all other risk factors for vascular disease, such as smoking, cholesterol, body weight, and Marjorie L. King, MD, exercise. There is a great need to learn more about FMD, and the FMDSA will soon be sponsoring Marge B. Lovell, RN, CVN a national research study to learn more about this disorder.” Robert McLafferty, MD Suresh Vedantham, MD In Pam's case, she had two experimental stents placed in her arteries within a two-year peri- Jeffrey Weitz, MD od, which helped to minimize the swishing noise in her ears and her headaches. She currently Mary L. Yost Karen A. Zupko takes several medications, including a diuretic, blood pressure medication, and aspirin. Today, Pam continues to work as an ER nurse and remains active. She also watches her diet National Advisory Board Abbott Vascular and does what her doctors tell her. She currently serves as executive director of the Fibromuscular AngioDynamics, Inc. Dysplasia Society of America, Inc. (FMDSA) and has made it her mission to help others afflicted AstraZeneca Bard Peripheral Vascular with FMD. Bristol-Myers Squibb Company “My biggest frustration when I went through all of this was that I felt so alone and like no one Cook Medical, Inc. was listening to me,” she said. “It was also difficult that it took so long to find the FMD, but I'm Cordis Endovascular CV Therapeutics glad at least now I know what I'm dealing with and am grateful that I have an opportunity to help Endovascular Today others affected by FMD.” Sanofi-aventis W.L. Gore & Associates For more information about FMDSA, visit www.fmdsa.org. Scientific Advisory Board Mark Alberts, MD Meghal Antani, MD The Vascular Disease Foundation would like to thank FMDSA for contributing medical information to this article. Jerry Bartholomew, MD Carmel Celestin, MD Michael Dake, MD Marcelo P. Villa-Forte Gomes, MD Greg Guy, MD Diane Treat Jacobson, PhD Vicki Marx, MD David Meyerson, MD Wanted: Nominations for Jacobson Award for Physician Excellence Elizabeth Ratchford, MD John Rundback, MD Robert Schainfeld, MD Nominations for the 2009 Julius H. Jacobson II, MD Award for Physician JoAnne Spindel, MD Kerry Stewart, EdD Excellence are now being accepted. This prestigious annual award recognizes Christopher White, MD outstanding contributions to physician education, leadership, or patient care in Jean White, RVT vascular disease. Nominees for the 2009 award are being accepted through Friday, November 30th, 2008. For complete criteria, please contact VDF at [email protected] or 888.VDF.4INFO. “Right now, there is a great need for more research on FMD to determine which patients are at risk and the optimal treatment.”—Dr. Heather Gornik, Cleveland Clinic Keeping in Circulation • www.KeepingInCirculation.org • 888-VDF-4INFO 3 Nutrition and Vascular Disease Peripheral arterial disease (PAD) is a • How active you are • Fresh (1 medium piece) common form of vascular disease (disease • What and how much you eat • Frozen of the blood vessels).
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