42 PERSONAL ESSAY SEPTEMBER 2007

worsening. The ER was backed up with slowly moved all the way down my face. My Experience with trauma cases, so I approached the triage I was scared to death. nurse to ask how long the wait would The nurse then took me right back. be. I explained that I was a registered My blood pressure was 210/130. I had Fibromuscular nurse and that my headache was getting never had high blood pressure. After worse. I asked her to just look at my being sent for a CT scan that came out pupils. She started explaining that they negative, a young resident presented Dysplasia and were backed up because of all of the me with discharge papers and a diag- traumas and as I again told her I was a nosis of unequal pupils. Mind you, my Pam Mace, RN nurse (which wasn’t getting me any- blood pressure was still elevated and President, The Fibromuscular Dysplasia Society of America where), my face started to go numb. The my pupils were unequal and I just had numbness started at my left temple and a TIA (small stroke).

A Brief Review of Fibromuscular Dysplasia Jeffrey W. Olin, DO Professor of Medicine, Mount Sinai School of Medicine New York, New York

What is Fibromuscular Dysplasia? Fibromuscular dysplasia (FMD) is a disease that causes one or more in the body to have abnormal cell development in the wall. The disease typically occurs in young women and most commonly presents with , transient ischemic attack, stroke, or an asymptomatic cervical . In 60% to 75% of cases, the is affected. The carotid and vertebral arteries are affect- ed in about 25% to 30% of cases. Although renal, carotid and vertebral arteries are most affected, FMD can be present in any arterial bed. FMD is a nonatherosclerotic and noninflammatory disease. There are several different types of FMD, but medial fibroplasia, producing the appearance of a “string of beads,” is the most common type encountered. Many physicians consider fibromuscular dysplasia to be rare, but it is not that uncommon; it is often simply overlooked or misdiagnosed.1 y name is Pam Mace and at the age of 37, I had a tran- Possible Causes of FMD Msient ischemic attack (TIA). The cause of fibromuscular dysplasia remains unknown. A variety of genetic, mechanical and hor- It started with a headache. I woke monal factors have been proposed, but further study is required to better understand the cause of this one morning with a dull headache. condition. Over the course of the day, my head became tender to touch. I thought I Diagnosing FMD might feel better after going for a run. The most important aspect is recognition of the that may occur with fibromus- As I went to leave, my granddaughter, cular dysplasia. The most common presentations are hypertension or an asymptomatic carotid bruit in Ashley, asked if she could ride her bike a young person, usually a woman. Other not uncommon presentations include neck pain, Horner’s syn- alongside, and although I knew it drome, transient ischemic attack, stroke, headache, pulsitile , , mesenteric ischemia would slow me down, I agreed. Thank and renal insufficiency. Patients with FMD are more likely to experience a of an artery. God, I did! Since Ashley was with me, While the gold standard for diagnosis remains catheter-based , duplex ultrasound, CT I had to stop at every intersection, gas angiography and MR angiography have all been used with some success. station and fast food restaurant we The “string of beads” appearance as visualized on the angiogram and MR angiogram (in the accom- came upon to help her with her bike. panying article by Pam Mace, RN) is characteristic of the medial type of FMD. Intimal disease, peri- After returning home and shower- medial disease and other less common types have a different angiographic appearance. ing, I noticed my pupils were unequal. Patients with carotid artery FMD should undergo a MRA of the intracranial circulation because about My headache wasn’t any worse but 7-12% of such patients have an associated intracranial . my left pupil was very tiny and my right was huge. Being an emergency Treatment of FMD room nurse, I concluded that these Treatment depends on the artery affected and the associated signs or symptoms. In a young per- were neurological symptoms. If some- son with FMD of the renal arteries and hypertension, the treatment of choice is percutaneous balloon one arrived at the ER looking as I did, . implantation is not necessary as the results with angioplasty alone are excellent. I would think something bad was FMD of the carotid arteries likewise responds very well to angioplasty. Angioplasty should be reserved going on, like a stroke or brain tumor. for patients with symptoms (TIA or stroke). Asymptomatic carotid FMD (detected by hearing a bruit) I called a close friend of mine, an should be treated with 81 mg daily. No intervention is required or recommended. If a dissection ER physician with whom I’ve worked, occurs, the patient should initially be treated with anticoagulation. If that fails, or blood supply is seri- and described what was happening to ously compromised, a stent should be placed. me. She urged me to get to the ER right away. I felt really stupid. I References wouldn’t describe it as the worst 1. Olin JW. Recognizing and Managing Fibromuscular Dysplasia. Cleve Clinic J Med 2007;74:273-282. headache of my life… at least not yet. 2. Slovut DP, Olin JW. Fibromuscular Dysplasia. N Engl J Med 2004;350:1862-1871. After arriving at the emergency room, the pressure in my head was More information is also available at www.fmdsa.org. SEPTEMBER 2007 PERSONAL ESSAY 43

The next day, I ended up in another The rest of my story involves decided they should do an angiogram carotid. Since the second stent, I have ER where I was given a spinal tap and almost being sent home again. Being a to look at the arteries. It showed areas been doing great. The really an MRI. The MRI showed that I had nurse, I challenged the physician’s of and a “classic string of made the difference for me. dissected my left vertebral artery. After knowledge and he re-evaluated my beads” appearance, which is character- (Unfortunately, I learned an FMD being stabilized, I was sent back to the case. Had I been sent home, I would istic for fibromuscular dysplasia patient who was stented the same day first hospital I had been to because have had a massive stroke and died. (FMD). My physician also looked at and who received two stents in her left that is where my primary physician Even after that I was misdiagnosed my renal arteries. My right renal artery carotid artery had an episode last July. was on staff. By this time I had the again. If it weren’t for my nursing was also affected, which explains the Both her carotid arteries collapsed and “worst headache of my life.” training, I most likely would not have sudden onset of hypertension. she had a severe stroke.) I go in for survived this ordeal. From the dissections, I formed yearly checkups on my carotid and I ended up dissecting my left verte- carotid and needed to have renal arteries. My carotid arteries look bral and bilateral carotid arteries. stents placed in my carotid arteries. In great and it appears my right renal Months later, I continued to be sympto- 2001, a Multi-Link Tetra stent was artery has more stenosis now than a matic and went to see a vascular doctor placed in my right carotid and a year year ago. For now, I will continue tak- at The Cleveland Clinic. My physician later, I received a JOSTENT in my left ing blood pressure medication and

Beading on carotid ultrasound Courtesy of Dr. Jeffrey Olin

Medial Fibroplasia Courtesy of Dr. Jeffrey Olin

Medial Courtesy of Dr. Jeffrey Olin 44 PERSONAL ESSAY SEPTEMBER 2007

diuretics. At some point, I will need to have a angio- I survived with no permanent disabilities — I am has also gotten involved with the FMDSA organ- plasty on the renal artery. one of the lucky ones! I ization and is working out the details of a patient As a result of my experience, I have changed my registry for fibromuscular dysplasia. focus and am doing everything I can to raise aware- Pam Mace is a registered nurse with 14 years Pam can be contacted at pam.mace@ ness of FMD. FMD is more common in women and of experience. She currently lives in Milton, MA fmdsa.org. More information is available at The is a cause of stroke. It was only last year that the and works in the emergency room at Milton Fibromuscular Dysplasia Society of America National Stroke Association listed FMD as a cause Hospital. Pam has also worked as the Clinical (online at www. fmdsa.org). of stroke. FMD can appear in any arterial bed, but Director of Emergency Services at Lincoln Park is more common in the renal and carotid arteries. I Hospital in Chicago. She has worked in the car- know of several patients with mesenteric and subcla- diac cath lab, critical care transport, ICU, and vian FMD. Most of my physicians were not familiar CCU. She is currently the President of FMDSA Letter to the Editor with FMD. Everywhere I go, I need to educate my and is dedicated to raising awareness of fibro- physicians, but this is changing. muscular dysplasia. The University of Michigan Dear Cath Lab Digest, My name is Dave Hartman. Although I have been a licensed paramedic for 25 years, I have worked as an invasive special proce- dures technologist for St. Joseph Mercy Hospital in Ann Arbor, Michigan for the past 7 years. Dr. Mansoor Qureshi of the Michigan Heart and Vascular Institute is planning a project to initiate cath lab services in Rabwah, Pakistan in November 2007. I plan on accompanying him to assist with medical procedures and training of staff. The project will take place at the Tahir Heart Institute of Fazl-e-Omar hospital. F.e.O. is a 90-bed hospital with complete Toshiba 5-Axis digital angiography system, but as of yet, no trained staff or materials to perform diagnostic or interventional procedures. A consulting cardiologist will arrive in August to begin seeing patients. We plan to arrive the last week of October and stay for 4 weeks. In addition to performing patient procedures, we plan to train resident staff in BLS CPR, sterile technique and invasive procedure assistance. On our departure, staff will be capable to assist visiting interventional cardiologists. Eventually the plan is for a permanent interventional capability. Fazl-e-Omar and Tahir Heart Institute are both non-profit organizations that were paid for with donations and built by volunteers. Rabwah currently has a population of around 11 million within a 60-kilometer radius. There is 1 CCU bed per 70,000 people. There are no cardi- ology services available. We hope to initiate the infrastructure to bring better health care to the people of Pakistan. While I have not worked in this part of the world before, I have done projects in Central and South America. The largest was design and implementa- tion of BERT, the NGO designated by the govern- ment of Belize as responsible for Emergency Medical Services. I feel confident that we can bring the Pakistan project to fruition. Our project costs are estimated at $10,000 U.S. Accordingly, we are seeking grant monies and donations. Any and all gifts will be gratefully accepted and acknowledged. Any excess funds will be left in place for material acquisition. Please feel free to contact me at [email protected] with any and all questions. Thank you for your time. Sincerely, W.D. (Dave) Hartman BS EMTP I/C. St. Joseph Mercy Hospital Ann Arbor, Michigan [email protected] I