Fibromuscular Dysplasia

On a hot day at the end of July, 37-year-old Pam Mace woke up with a left-sided headache that intensified as the day progressed. When she noticed that her pupils were unequal and her headache was so severe that she could not pick up her head, she went to the emergency room (ER). It wasn't until two more trips to the ER that she was told that she had a transient ischemic attack (TIA) or mini-stroke. For many months Pam suffered with an array of symptoms: severe headaches, a “swishing noise” in her ears, dizziness, and a feeling that she was about to faint. She also had trouble regulating her blood pressure, 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 arteries that supply blood to the kidneys (renal arteries), followed by the carotid arteries of the neck that supply blood to the brain. Fibromuscular dysplasia can also affect the arteries in 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.”
Many people with FMD do not have symptoms that can be detected during a general physical exam. The signs or symptoms that a person exhibits depend on which arteries are affected, and the degree of narrowing within them. Some patients with FMD have no symptoms at all. Fibromuscular dysplasia can be diagnosed through a number of methods including CT scan, MRI, ultrasound and angiogram. In the most common forms of FMD, a characteristic “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 Fibromuscular dysplasia, 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 couldn't work. In a follow-up MRI, a radiologist noticed that she had a carotid aneurysm.
After a second opinion, it was determined that Pam had suffered two aneurysms and had three arterial dissections or tears in the arteries of her neck. Renal FMD was also discovered.
“As an ER nurse, I knew something was wrong, but it was so frustrating that the 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 problems.”
There is no cure for FMD nor is there a set protocol for treating it. Depending which arteries are affected will determine the kind of treatment used. A procedure called angioplasty is the treatment most commonly used for severe narrowing due to FMD. Angioplasty is often performed at the same time as an arteriogram during which the radiologist, vascular surgeon, or cardiologist inserts a wire into or near the affected artery and injects contrast material or dye that can be detected by X-ray. If an angioplasty is performed, a catheter is extended into the affected artery and a small balloon is inflated that “stretches” open the artery. A metal stent is typically not required to keep the vessel open for patients with Fibromuscular dysplasia. “Right now, there is a great need for more research on FMD to determine which patients are at risk and the optimal treatment. The care of patients with FMD focuses on determining which blood vessels are involved and whether or not a procedure needs to be done to treat severe symptoms due to this disorder,” said Dr. Heather Gornik, a specialist in FMD at the Cleveland Clinic. “I also treat patients with carotid FMD with antiplatelet therapy, usually aspirin, and I work on controlling all other risk factors for vascular disease, such as smoking, cholesterol, body weight, and exercise. There is a great need to learn more about FMD, and the FMDSA will soon be sponsoring a national research study to learn more about this disorder.”
In Pam's case, she had two experimental stents placed in her arteries within a two-year period, which helped to minimize the swishing noise in her ears and her headaches. She currently 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 and does what her doctors tell her. She currently serves as executive director of the Fibromuscular Dysplasia Society of America, Inc. (FMDSA) and has made it her mission to help others afflicted with FMD. “My biggest frustration when I went through all of this was that I felt so alone and like no one was listening to me,” she said. “It was also difficult that it took so long to find the FMD, but I'm glad at least now I know what I'm dealing with and am grateful that I have an opportunity to help others affected by FMD.”

Email a Friend