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Fibromuscular Dysplasia (FMD)

February 12, 2009
Dr. Gornik

Heather Gornik, MD
Staff Physician
Robert and Suzanne Tomsich Department of Cardiovascular Medicine: Clinical Cardiology and Vascular Medicine
Medical Director
, Non-Invasive Vascular Laboratory.

Description

Fibromuscular dysplasia (FMD) is a disease that causes one or more arteries in the body to have abnormal cell development in the artery wall. FMD is most commonly found in the arteries that supply the kidneys with blood (renal arteries). The second most common artery affected is the carotid, which is found in the neck and supplies the brain with blood.

More Information

Cleveland_Clinic_Host: Welcome Dr. Gornik, we are happy to have you us today. Also joining us is Pam Mace, thanks for being here. The response has been overwhelming and we are going to try to answer as many questions as possible in this hour. If we are unable to get to all the questions, we will try to answer questions to post in the transcripts.

Speaker_-_Dr__Heather_Gornik: Thank you for having me. I would like to acknowledge a few guests. Next to me we have Ms. Pamela Mace - President and CEO of the FMDSA organization - and a major advocate for raising awareness of this condition. Also - I have with me - Dr. Esther Kim - my colleague in cardiovascular medicine and partner in the Cleveland Clinic FMD clinic.


FMD: Medical Specialists, Diagnostic Testing and Follow Up

mhcalvillo: I was diagnosed with FMD by a neurologist and have continued to see him for FMD. I am also seeing a nephrologist and cardiologist , also for FMD. Should I be seeing a vascular doctor instead?

Speaker_-_Dr__Heather_Gornik: Great question. As long as your physicians have knowledge and experience in the care of FMD patients, I do not think you need an additional vascular doctor. Indeed, nephrologists and neurologists are important members of the FMD care team.

kaulrich: Most of us with FMD look healthy on the outside, but we feel like a train wreck on the inside. It seems difficult at times to have physicians take my symptoms seriously, they seem to base my health on me being young, and at a healthy weight. Any suggestions on communicating with the medical community effectively?

Speaker_-_Dr__Heather_Gornik: Great question. FMD is a problem that is not taught extensively in medical school. In fact, it was not until I did my vascular medicine fellowship (after 4 years of medical school and 5 years of post medical school training), that I really learned about FMD. In many cases, patients with FMD may be the first such patient their physician has treated --- and it is not the doctors’ fault! I think it is important to recognize this barrier in physician knowledge about FMD, but also have a positive attitude about the situation and realize that you can be an ambassador and advocate for your own health care. I have been really impressed with the efforts of the FMD Society of America to educate physicians about FMD, and they have a really great downloadable handout (available at www.fmdsa.org) that you can take to your physician to give him or her more information on this disorder. The FMDSA website is a great resource, and it even has scientific articles available for download for physicians.

Pam_Mace: You have to be your own advocate - you know your own body better than anyone. My personal experience was that my doctor did not listen to me and I found one that did - that is how I was diagnosed with FMD. Before that my symptoms were ignored.

kptocs: Is a positive ANA test related to FMD, and if some one with FMD has a positive ANA, what follow up should be done?

Speaker_-_Dr__Heather_Gornik: This is a very good question and relates to the last question. Young women are more likely to have a positive ANA test and are also more likely to have FMD.I do not think the positive ANA is directly related to the arterial disease of FMD, but I think both affect younger women and there may be common underlying risk factors.

Lotus_Blossom: What measures can you suggest be taken to get physicians outside of Cleveland Clinic to participate in education and research in order to provide the most appropriate and current treatment options available to patients?

Speaker_-_Dr__Heather_Gornik: Again, there have been a few case reports of finding FMD upon autopsy in very sad cases of sudden infant death syndrome. There are fewer than 10 reported cases of this in the world literature, so obviously it is quite rare. Nonetheless, I do think this a very important area that needs to be researched more in the future, and I also feel it is important to raise awareness of FMD among all health care professionals, so that they are aware of FMD as a potential cause of severe vascular disease.

ulrich: How often should one have there carotids and renals monitored if FMD has been stable? What test would you suggest for monitoring progression of FMD, ie MRI, MRA U/S?

Speaker_-_Dr__Heather_Gornik: As you know, it is important that patients with FMD have imaging follow-up of the vascular beds involved. In my practice, a patient with carotid FMD or renal FMD who is stable—that is no neurological symptoms, no dissection, and well controlled blood pressure—will have an imaging study performed every year. The type of study performed depends on the location of the disease and the expertise of the facility. In most cases at our institution, we can monitor renal and carotid FMD in stable patients with ultrasound. In some cases, MRI is necessary, such as patients with prior dissection, aneurysm, or carotid narrowings that is beyond the reach of the conventional ultrasound probe (such as disease in the blood vessels within the brain). In the case of patients with a carotid dissection, it is my practice to generally follow more closely in the first year after the dissection (for example at 1 month, 3-6 months, and then 12 months) if the patient is stable and then on a yearly basis if the dissections are stable and have healed.

ulrich: I recently had an arteriogram of my renal arteries which showed according to the IR showed a small web of FMD. No pressures were taken, and angioplasty was not needed. My blood pressure still remains 150-140/ 80 on three blood pressure meds. Could a small web cause high pressures, or would essential HTN be a better explanation?

Speaker_-_Dr__Heather_Gornik: Without seeing your angiograms and having a change to evaluate you in the office, it is difficult to give a definitive answer. I can tell you, however, that in many cases we have found that the degree of narrowing in an artery related to a “web” is not well appreciated by the pictures of an angiogram. Our interventional group has done renal artery pressure measurements, as you mention, and intravascular ultrasound (IVUS) to look inside the blood vessels and be sure that the “web” we see is not causing a severe narrowing of the artery.

Many people asked about finding a doctor in their geographic area:

Speaker_-_Dr__Heather_Gornik: Wow! These questions really emphasize the need to continue to raise awareness among health care providers regarding FMD. I am afraid I do now know of any specific FMD programs or clinics in the above geographical regions. I encourage you to consult with the FMDSA — they maintain a database of health care providers experienced in the care of FMD patients. In generally, vascular surgeons or vascular medical specialists, nephrologists, and neurologists will have some knowledge of FMD, as patients with FMD generally are cared for by these types of physicians. When you call to make an appointment with a specialist, do not be afraid to ask the doctor’s secretary or clerk answering the phone if the doctors has experience taking care of patients with FMD.

A couple participants asked about the program at University of Illinois:

Speaker_-_Dr__Heather_Gornik: I am delighted to know that there are more physicians taking a special interest in FMD. I had heard about the University of Illinois clinic via the FMDSA website, but I am afraid I do not have any additional information regarding their program.


Medical Care at the Cleveland Clinic

Lotus_Blossom: Since so many FMD patients from out of state visit Cleveland Clinic for consultation, diagnostics and treatment, what do you do to ensure that the prescribed course of care will be followed when patients return to the their local physicians?

Speaker_-_Dr__Heather_Gornik: When patients are seen in the FMD Clinic at Cleveland Clinic, it is our practice to send a detailed letter to the patient’s primary care physician (and any other physicians) back home. We also include the results of all consultations with additional specialists, reports of imaging studies, and even the hard copies of the imaging studies performed if requested. We are always available to discuss our recommendations with primary care physicians --- but we certainly cannot mandate that our recommendations be followed

Lotus_Blossom: Can you please address the specifics of the new FMD department at Cleveland Clinic? Including; How will the different specialties collaborate on patient care?; How many specialties are on board and will there be additions in the future?; Is there now a single location for the department, or will patients still need to travel to the various campus locations (ie: vascular, cardiology, nephrology etc)? How will the new department change the way patients are diagnosed and treated for FMD?

Speaker_-_Dr__Heather_Gornik: Thanks for your question. Last fall we proudly started a dedicated FMD Clinic within the vascular medicine section of the Cleveland Clinic. The clinic is staffed by vascular medicine specialists, internists and/or cardiologists with special advanced training in the care of patients with vascular disease. One of my partners in the clinic, Dr. Esther Kim, is here next to me now sitting in on the web chat. We have also assembled a team of specialists who have interest and expertise in the care of FMD patients, including neurologists, nephrologists, and vascular interventionalists (vascular surgeons and interventional vascular cardiologists). We also have a medical geneticists on the team, as there are some cases in which we have concerns there may be an inherited blood vessel problem related to the FMD. Patients are seen first either by myself or one of my partners in the FMD Vascular Medicine clinic, and we make recommendations for care and any additional testing or procedures that may be needed.

Our specialists see patients either in our area or in other adjacent buildings of the Cleveland Clinic. I think the establishment of an FMD team has already had a very positive impact on how we care for FMD patients here allowing for multidisciplinary discussion of the best way to care for each patient and also building up great expertise in the care of patients across the entire spectrum of FMD symptoms.

riqt88: How much time should an out of State patient put aside if visiting the Cleveland Clinic for a consultation?

Speaker_-_Dr__Heather_Gornik: I would recommend calling our appointment line to schedule your FMD appointment - it may take up to 2 - 3 days to schedule all your appointments.

ulrich: What is the best way to determine the degree of carotid stenosis? I have yearly MRI/MRA's and have been told Carotid Ultrasounds are not needed because MRI/MRA are better at showing the degree of stenosis.

Speaker_-_Dr__Heather_Gornik: See answer to question above. It depends on the location of the disease and also the expertise at your clinical center. We are fortunate here to have a very strong Vascular Ultrasound Lab with great experience in imaging arteries of patients with FMD, so we use vascular ultrasound for follow-up, in general, unless the carotid artery disease is beyond the area we can image with our probes (i.e., in the brain).

lecati: have bilateral renal artery FMD with aneurysms, one repaired, the others being observed. How often do you recommend follow up CT scans for aneurysms?

Speaker_-_Dr__Heather_Gornik: See above answers regarding follow-up for carotid dissection and imaging. This will vary depending upon the size of the aneurysm and the stability of the aneurysm (i.e., whether it has been the same size for many years). Generally every 6 to 12 months, most likely the later. More frequent CT scans are association with increased exposure to radiation over time. I might also consider imaging with MRI to limit the radiation exposure.


Women and FMD

ulrich: I seem to be symptomatic especially around my menstrual cycle, what role does hormones play?

Speaker_-_Dr__Heather_Gornik: There is likely some connection between FMD and hormones, as FMD is much more likely to occur in women than in men. That being said, the nature of this relationship is not at all clear scientifically and there is need for much more research in this area. I have heard anecdotally from a number of patients that their symptoms seem to be more prominent, particularly headaches and tinnitus (ringing or swishing in the ears), around the time of their periods. For others, there is no relationship. So I guess the answer to your question, is “stay tuned”.

wilhelmi: Is there any evidence that the progression of the disease slows down when women become post-menopausal?

Dr__Esther_Kim: That is a wonderful question. At this time, we don't know the exact answer - however these are the type of questions we would like to answer with our FMD registry.What we do know is that as women age, they are at increased risk for other arterial disease - namely atherosclerosis. It is important to receive regular care to screen for the development of atherosclerosis.

kptocs: How best can you determine if it is too risky to get pregnant once diagnosed with FMD in the renals - what type of provider is best to advise on this?

Speaker_-_Dr__Heather_Gornik: Thank you for this question. There have also been similar questions about pregnancy and FMD coming through. I think each individual case is very different with regard to risk of pregnancy. Issues that need to be considered include risk of dissection or tear in an artery, particularly if a patient has already had a dissection; and blood pressure control. I would recommend that you be cared for by a high risk obstetrician and a specialist in FMD. You may want to consult with these professionals before pregnancy.

Pam_Mace: When I was considering pregnancy, I met with a vascular specialist and high risk OB. Another consideration is that some medications are not safe during pregnancy for the fetus.

Speaker_-_Dr__Heather_Gornik: Great point Pam - thanks.


Abnormal Heart Beat and FMD

ulrich: Many of us with FMD have been told cardiac arrhythmias are not associated with FMD, yet we see in the media sudden death due to coronary fmd. Are there any tests to find FMD in the smaller arteries of the heart, such as the arteries that supply the SA node?

Speaker_-_Dr__Heather_Gornik: There have been a handful of case reports published in the medical literature of sudden cardiac death ultimately found to due to FMD within the heart. For the most part, these were patients who were not known to have FMD. There are also case reports of FMD involving the heart (coronary) arteries, but again this is rare. This is an area that clearly merits further research. However, I do not want to cause alarm. In my relatively large practice of FMD patients, sudden death has not been a major issue thankfully. At present, patients with FMD are not generally screened for heart involvement, unless there are other clinical symptoms that raise concern for heart disease, such as chest pain, palpitations, fainting spells, heart failure, or a family history of sudden death. This is an area that clearly needs more research, and I think the national FMD patient registry will be very helpful here.

ulrich: It feels like FMD is a symptom of a larger connective tissue disease, so many of us experience fatigue, palpitations, joint discomfort and skin irritations along with mood swings, any thoughts?

Speaker_-_Dr__Heather_Gornik: This is a challenging issue in caring for patients with FMD because, particularly in regards to palpitations, FMD can occur in people who have other health problems.Women may commonly experience palpitations, in fact, this is one of the most common reasons why a woman would see a cardiologist. Women also are more likely to have FMD, so it is hard to say whether they are related causally or whether these are both problems that tend to affect women more. I think it is very important to be cared by a physician who has experience with fmd patients so they can determine what is caused by the fmd and what is caused by another problem.


Family History

ulrich: I have FMD of my Carotid and Renal arteries, I have one son with horners syndrome age 10 and another son age 13 who on several occasions has had documented HTN. I have been told not to worry about them having FMD, any suggestions?

Speaker_-_Dr__Heather_Gornik:FMD may run in families in some cases. In addition, high blood pressure in a thirteen year old is quite unusual.Many health care providers may not be familiar with FMD and may not know of the potential for FMD to cause hypertension in a child. I think it is wonderful that you are so knowledgeable about FMD and such a strong advocate for your children.I would recommend that you have you children evaluated by pediatric specialists in nephrology/hypertension (for your son with hypertension) and neurology (for your son with the Horner’s syndrome). These specialists should be more familiar with FMD and whether or not it could be related to your children’s issues. I wish you good luck.

gould4: If you are an FMD patient with siblings or children, are there any tests to screen them for FMD? Is it recommended that they be screened?

Speaker_-_Dr__Heather_Gornik: Most importantly, family members and the parents of affected family members need to tell their physicians about the family history. I do not recommend routine screening of asymptomatic family members, but would definitely consider screening if there were family members with high blood pressure especially at a young age, carotid artery dissection or bruits, or other clinical symptoms that suggest vascular disease. Right not there is no blood test available for screening for FMD. Screening would be done with imaging studies, generally of the renal and carotid arteries.


FMD and Dissection

perlini: I have FMD in the left internal carotid artery (string of Beads)and healed right internal carotid artery dissection near skull base with excellent distal flow most likely from a car accident arteries

Speaker_-_Dr__Heather_Gornik: Thank you for sharing your story and for your question. Because FMD is a relatively uncommon problem, there is not yet a consensus (agreement) among neurologists and vascular physicians as to how to optimally manage all patients. Because of this, I am not surprised two different doctors have given you two answers on how often to be imaged, and I might even give you a third answer. We don’t know the right answer at this time, and we need more research and meetings among FMD specialists to develop some clinical standards for issues such as follow-up that will allow for more standardization of how patients are managed. Without having access to your imaging studies, I cannot make a specific recommendation, but as a general guideline I do not feel ultrasound alone is adequate if there are narrowings or aneurysms located in the carotid or vertebral arteries within the skull. IN that case I would recommend an MRA.

provost: Do you feel if a patient is symptomatic that has experienced multiple dissections and frequently gets vasospasms is a good candidate for an angio?

Speaker_-_Dr__Heather_Gornik: Perhaps - but I would need to review all the clinical information in detail to make that decision. You should definitely be evaluated by a specialist. We would be happy to evaluate you here at the Cleveland Clinic.

Punkandgem: various members of the FMD Yahoo Group.Do dissections heal themselves or are there different results and why?

Speaker_-_Dr__Heather_Gornik: Yes, dissections often do heal themselves over time. If one takes another imaging study (CT scan or an MRA) months after a dissection, in some cases there may be no evidence of dissection, in other cases, there may be a small residual dissection (tear in the artery), and in some cases a small aneurysm will have developed at the site of the tear. This is called a pseudoaneurysm. In most cases, there is no treatment needed for pseudoaneurysms related to dissection, aside from medical therapy (coumadin often first, then aspirin or clopidogrel/Plavix). In rare cases, the pseudoaneurysms due to a dissection enlarge or at a location for which treatment is recommended (such as placement of a stent).

petersen: had a spontaneous carotid dissection 3 years ago, with no other incidences since. No other arteries are affected. What are my chances of another dissection and how often do you see patients with just FMD in just the carotid arteries? I keep waiting for the other shoe to drop.

Speaker_-_Dr__Heather_Gornik: We do not have exact statistics, but most patients who have a carotid dissection will not have another dissection. Nonetheless, careful follow-up and monitoring for symptoms of dissection is important (see below). I have seen patients with isolated carotid artery FMD, although sometimes we do look at the kidney arteries with ultrasound and find evidence of renal FMD that was not causing any symptoms.

sterritt: If you have a dissection, how do you know? AND What are symptoms of carotid artery dissection?

Speaker_-_Dr__Heather_Gornik: The most common symptoms of carotid dissection are headache and neck pain. Other symptoms, that are less common, are those of a stroke or mini-stroke, such as weakness on one side of the body, difficulty with speech, difficulty with walking.

kaulrich: Is it possible to have had a carotid dissection and not have it show up on scans?

Speaker_-_Dr__Heather_Gornik: The answer is yes, depending upon the type of study done. Some imaging modalities, such as ultrasound, might miss a small carotid dissection, particularly if it is located very high in the neck. The best non-invasive technique for diagnosis of carotid dissection is magnetic resonance angiography (MRA) in most medical centers. Angiography, however, remains the gold standard for diagnosis of dissection, but this is not needed in most cases.


Nutrition, vitamins and supplements

cprice1: I am a 52 year old woman who has had a stroke 4 years ago diagnosed with FMD in my carotid Artery. Please tell me about nutrition with this disease. What I can eat or any kind of nutritional pills like Fish Oil, I should be taking, and also any measures to take.

Speaker_-_Dr__Heather_Gornik: There have been questions regarding supplements and FMD - I have to say to begin that there is no substantive research on any nutritional regimens and FMD.We have a lot of literature on nutrition and atherosclerotic vascular disease, and I think this is where a lot of thoughts about fish oil, low fat diet, chocolate and pomegranates originate. To my knowledge there is no evidence that there is any dietary intervention can help prevent or treat FMD. What I do tell my patients though is that they already have one artery problem and we want to do whatever we can to prevent another, such as atherosclerosis or clogged arteries.

I would recommend a heart healthy diet (low in saturated fat, high in fiber, rich in fruits an vegetables) as I do for all of my patients.Dark chocolate has been shown to have some positive effects on blood vessel function - not in patients with FMD, so why not - especially as Valentine's Day approaches - if you are going to indulge - why not dark chocolate?


FMD and Symptoms

EnglishJean: Could you explain what type of symptoms are related to FMD

Speaker_-_Dr__Heather_Gornik: The symptoms that are caused by FMD depend on the pattern of the blood vessels that are involved. In addition, many patients with FMD may have no symptoms at all and are only diagnosed when an imaging test of the arteries is done for another reason (for example, a CAT scan of the abdomen for back pain). The most common symptom of renal FMD is high blood pressure. The most common symptom of carotid FMD is a bruit in the neck (not really a symptom, more of a sign), but also headaches, neck pain, and a ringing or swishing sound in the ears. If FMD involves the abdomen, a patient may have abdominal pain after eating or weight loss. If FMD involves the arteries of the legs, which is relatively rare, there may be pain in the legs with walking that goes away with rest. This is known as claudication.

Raynaud Symptoms

ctrc: Do you find that a lot of FMD patients have Raynauds-like symptoms?

Speaker_-_Dr__Heather_Gornik: See answer regarding ANA and palpitations - Raynauds is also common with younger women, so I think they are related to common risk factors but not the same disease process.

Headaches

There were several questions on Headaches:

Speaker_-_Dr__Heather_Gornik: The symptoms that are caused by FMD depend on the pattern of the blood vessels that are involved. In addition, many patients with FMD may have no symptoms at all and are only diagnosed when an imaging test of the arteries is done for another reason (for example, a CAT scan of the abdomen for back pain). The most common symptom of renal FMD is high blood pressure. The most common symptom of carotid FMD is a bruit in the neck (not really a symptom, more of a sign), but also headaches, neck pain, and a ringing or swishing sound in the ears. If FMD involves the abdomen, a patient may have abdominal pain after eating or weight loss. If FMD involves the arteries of the legs, which is relatively rare, there may be pain in the legs with walking that goes away with rest. This is known as claudication.

Caroline: Does FMD in your carotid arteries cause chronic headaches and why?

Speaker_-_Dr__Heather_Gornik: These are both great questions and this is an area in need of much more research. There does seem to be an association with carotid FMD and headaches that are like migraines, but as above, this is sometimes difficult to tease apart from the fact that younger women tend to be those who suffer from migraine headaches and are also those who are diagnosed with FMD. Patients with FMD who have suffered a dissection of one of the carotid arteries may experience a different type of headache or may experience intense pain in the neck. The national patient FMD registry will be collecting some information on headaches, including migraine headaches, and FMD, and I think this will be helpful.

emma: I have FMD in my carotid arteries, and suffered a stroke 3 years ago (at age 36). My condition is managed on aspirin. In the last week I have had low grade headaches for several days. Since the stroke I occasionally have headaches (maybe once a month) but not regularly. Should I be concerned about this? (Not sure if it is because it has been so hot here in Australia!)

Speaker_-_Dr__Heather_Gornik: I am so sorry you had a stroke at such a young age. i would need to know whether the stroke was due to narrowing of the artery or dissection (tear) in order to give you recommendations for treatment.In general I would say you need regular follow up with a neurologist. And your neurologist needs to know you are having headaches now. Also - as your headaches are worse now and in a different pattern - you should discuss this with your primary care physicians right away.

Pedsnurse: I WAS DIAGNOSED WITH FMD IN 2007, BOTH RENAL AND CAROTID ARTERIES. DURING RIGHT RENAL ANGIOPLASTY, I WAS INFORMED THAT THE CAROTID BLOCK WAS EXTERIOR AND THEREFORE NOT NECESSARY TO BALLOON. IS IT POSSIBLE THAT THE CAROTID FMD IS WHAT HAS BEEN CAUSING MY DEBILITATING HEADACHES? IS IT KNOWN WHETHER FMD GETS PASSED FROM MOTHER TO CHILD, OR IF IT SKIPS A GENERATION?

Speaker_-_Dr__Heather_Gornik: See above, we are learning more about the association of carotid FMD and headaches in people who have had dissection and those who have not had dissection but who have significant narrowing of the arteries. I would recommend evaluation by a neurologist or vascular specialist with experience in the care of patients with FMD. As for your second question, inherited cases of FMD have definitely been reported, although most patients with FMD I have seen do not have a family history

Breathing problems

Swilliam57: Can fmd cause breathing restrictions

Speaker_-_Dr__Heather_Gornik: Shortness of breath would be an unusual symptom of FMD, and I would be concerned about other possible diagnoses. A thorough evaluation by a physician who is familiar with FMD should be helpful to tease apart which symptoms could be due to FMD and which are entirely unrelated.

Flank pain

Jenw: Hello Dr. Gornik - My name is Jennifer and I was diagnosed with FMD two 1/2 years ago following a TIA where my left carotid artery dissected. It has since reopened but I continue to have other symptoms related to the FMD. I have heard about "flange pain" and was wondering if you could describe it for me. I have been waking up with pain on my left side of my back daily for a few weeks and am beginning to wonder if this is more than a bad night's sleep

Speaker_-_Dr__Heather_Gornik: I think you are referring to flank pain, which is pain along the side of the body in the low back area. Flank pain is most likely due to muscle irritation or strain, but can also be due to kidney problems, such as a kidney infection, or rarely renal artery narrowing or a tear. I would recommend discussing this symptom with your primary physician and letting her/him know that you are a patient who has had a carotid dissection in the past due to FMD.


FMD Registry

Kasper39: What is the age range of FMD patients in the national registry?

Speaker_-_Dr__Heather_Gornik: Hello there! I am glad you asked about the registry. The FMDSA is sponsoring an international registry of FMD patients that is being coordinated by the University of Michigan. There are 9 sites worldwide. Information about the registry is posted at The Fibromuscular Dysplasia Society of America website. We are collecting information on FMD regardless of age or gender. We are proud to have enrolled the first patient worldwide at the Cleveland Clinic 10 days ago.


FMD and other Vascular Conditions

mariael: I have 40 years of age, I am Argentina and live in Ecuador. 14 Years Ago I had a total colectomy; I have Hypothyroidism, and 10 Years Ago I was diagnosed with Autonomic dysfunction and POTS syndrome in the USA. ( Vanderbilt University). And recently (in November) in the Johns Hopkins Hospital I was diagnosed with FMD. 3 Years ago I started to feel weakness in the hip, legs and arms, and claudicating when I walked. In the last 4 months I have had Episodes of fainting, arrhythmias and angina, and very severe pain in my abdomen. I went to the Johns Hopkins Hospital in January 2008 and this last November they gave me this diagnosis: "An atypical distribution fibromuscular dysplasia" I went to the cardiologist but after some test they couldn’t determine the cause of fainting and angina, the arrhythmias was related to the pots. I do not know any specialist in FMD and my medication at this time is: For FMD Trental (pentoxifylline) 500 mg morning and evening, and Plavix 45 mg Lunch; For my Heart condition Dilatrend (Carvedilol) 6,25 mg morning,Dilatrend (Carvedilol) 12,50 mg evening,Ciliren (Diltiazem Clorhidrato) 30 mg morning and 60 mg evening; For my Hypothyroidism Synthroid 75 mg per day; For my POTS 3 Sandostatin (Octreotide) injections (subcutaneous) 0,50 cc each every day. At this time my questions are, 1/ if I have this atypical distribution fibro muscular dysplasia, do you have any treatment for this? it is possible that I have autonomic dysfunction + Pots syndrome + atypical distribution fibro muscular dysplasia? if I have this atypical distribution fibro muscular dysplasia, do you have any treatment for this? my heart condition have any relation with some of these diseases, or specifically with FMD? my abdominal pain, have any relation with FMD? I have had stones in my kidneys and in my gallbladder (they removed the gallbladder), Perhaps any relation with FMD? my headaches, dizziness, vertigo, pressure, noise and ringing in the ears, are more related to FMD or to POTS? normally when I wake up every morning my hands are asleep (numbness), this could be by FMD?

Speaker_-_Dr__Heather_Gornik: Thank you for sharing your story, and I am so sorry that that you have been through so much during these past few years. Without being able to fully interview or examine you, it sounds as if you have many health issues. Your situation is one I find with some of the patients I see in the FMD clinic. There is evidence of FMD or beading in some of the arteries, but it is not clear that all of the symptoms are caused by FMD, as opposed to another health condition. In these situations, I think it is important to be evaluated by a physician with experience in the care of patients with FMD so that the specific symptoms you are having can be correlated with the FMD, or to determine if the FMD is not the only health issue. While FMD can cause dizziness, headaches, and high blood pressure, some of the problems you describe would be unusual for FMD, and I would be concerned that they are not due to FMD but another diagnosis.I would recommend seeing a specialist in your area with experience in the care of patients with FMD to help sort this out. If you would like to be seen here, we can help facilitate this, as well.

Koala1787: Can FMD "spread" to other arteries in the body?

Dr__Esther_Kim: This is a good question. FMD can affect any artery in the body. However, it is not a disease that "spreads" rather affected areas that were previously involved are identifies when imaged.


FMD and Mortality

noah: How many people with FMD die in a year?

Speaker_-_Dr__Heather_Gornik: Although I do not have specific statistics for you - my sense is that sudden death and death at a young age is extremely unusual in FMD. I do not have a single patient in my relatively large FMD practice who has had a heart attack or died due to complications of FMD.

kptocs: What is known about life expectancy for people diagnosed with FMD?

Speaker_-_Dr__Heather_Gornik: Unfortunately I do not have scientific data to present to you. I think the FMD patient registry will be absolutely critical for answering this question

karibaby_2: Is there a connection between Sudden Infant Death and FMD? Would this be known from an autopsy and would the pathologist HAVE to be looking for it? What about a connection between children and teenagers who suddenly drop dead? Are they performing autopsies and looking into possible FMD as a cause of death? Another question: If a metal stent is required due to the stenosis of the vessel, is there an increased risk of weakness or vessel failure above and/or below the stent due to the already compromised situation? Thank you for all the research that is being done. It is so important to get the information out there to primary care physicians and to pathologists to be looking for this.

Speaker_-_Dr__Heather_Gornik: Again, there have been a few case reports of finding FMD upon autopsy in very sad cases of sudden infant death syndrome. There are fewer than 10 reported cases of this in the world literature, so obviously it is quite rare. Nonetheless, I do think this a very important area that needs to be researched more in the future, and I also feel it is important to raise awareness of FMD among all health care professionals, so that they are aware of FMD as a potential cause of severe vascular disease.


FMD and Medications

ctrc: What is the best blood pressure medication to take if you have FMD (both Inner Carotids) , Ehler's Danlos Syndrome (Hypermobility), Chiari 1 with a small syrinx and recently identified with an Atrial Septal aneurysm(not life threatening). I currently take 10 mg of Norvasc, but can't get it down to 120/70. My diastolic is always in the 80-90's. Heart rate is always high--80's and 90's. Three years ago , it was always around 70.

Speaker_-_Dr__Heather_Gornik: There is no specific blood pressure agent that has been studied extensively in FMD patients. In patients with renal FMD, I generally use an ace inhibitor or angiotensin blocker, such as Losartan, applying experience with renal artery disease from other causes.

melva: I have aortic root dilation with my FMD illness. I have seen it greatly reduced after using Cozaar. I saw the John Hopkins study. Should it be routinely prescribed for all aneurysms associated with FMD?

You are referring to research done on a disorder called Loeys-Dietz Syndrome, which is another genetic disorder affecting blood vessels.Use of this medication has not been studied in FMD specifically, however, we are learning more every year about the genetics of FMD and overlap with other vascular disorders.

Dr__Esther_Kim: Aortic aneurysm in general is unusual in patients with isolated FMD. The presence of aortic aneurysm in a patient with FMD would make me wonder about another diagnosis or an overlap syndrome.

Speaker_-_Dr__Heather_Gornik: You would be the sort of patient who we would send to the genetist on the FMD team for an evaluation.

lindatemp: It has been published, by the FDA, that Plavix has been found to be less effective in some people, possibly genetic differences. It has been stated that some PPI's may make Plavix less effective. I am on Prilosec, and Plavix. Are you recommending that Plavix users change to a H2 blocker such as Zantac, Pepcid, Tagamet and Axid, to help avoid this problem?

Speaker_-_Dr__Heather_Gornik: You are correct. There recently has been some concerns about a drug interaction of clopidogrel (Plavix) and proton pump inhibitors (such as omeprazole), which decreases the effectiveness of Plavix in terms of blocking the platelets. This may be more of a concern with cardiac patients, but of course the information could apply to vascular disease patients, including those with FMD. The Food and Drug Administration is currently seeking more data on this issue, and the American College of Cardiology, American Heart Association, and American College of Gastroenterology came out with a statement for patients, which I agree with, which says: “In the interest of patient safety, the AHA/ACC and the American College of Gastroenterology (ACG) advise that patients who are currently taking these medications should not change their medication regimen unless advised by their healthcare provider.” So, I think the bottom line is that I would discuss this specific issue with your doctor.


FMD, Activity and Massage

perlin: Scuba diving - do you recommend it be stopped?

Speaker_-_Dr__Heather_Gornik: There are case reports of SCUBA diving leading to carotid dissection (mainly in patients without FMD). As you know, SCUBA diving is associated with significant stress to the heart and vascular system. I would generally recommend against SCUBA diving in a patient who has previously suffered a vascular dissection and among patients with evidence of cerebrovascular FMD. Sorry about this

kasper: Are massages out of the question with the carotid involved?

Speaker_-_Dr__Heather_Gornik: General therapeutic massage (swedish, light tissue or relaxation) in FMD. I would recommend telling your massage therapist you have fibromuscular dysplasia and are at risk for carotid dissection. Avoid deep tissue massage and extensive manipulation of the neck.

ssvilar: Are there any activities that should be avoided by patients with carotid FMD (chiropractic adjustments, massage, weight lifting, yoga, etc.)

Speaker_-_Dr__Heather_Gornik: This is another area where we do not have standardized guidelines for patients. I do not restrict general exercise - however I think recommendations need to be tailored to the location of the disease and whether dissections have occurred.I would discuss your exercise prescription with your doctor who will have more details about your disease.


FMD and High Cholesterol

botha_2: Is there any connection between FMD and high cholesterol

Dr__Esther_Kim: Currently there is no evidence to suggest an association between high cholesterol and FMD. However, because FMD patients have arterial disease, we aim to have LDL cholesterol less than 100 mg/dl to prevent atherosclerotic arterial disease.


Social Security


Melva and Punkandgem asked about social security and FMD:

Speaker - Pam_Mace: Though we have no official affiliation with Social Security, I can say that several of our members/patients have received disability.


Resources:

Speaker_-_Dr__Heather_Gornik: Thank you for having me, Dr. Kim and Pam Mace from FMDSA. The questions were excellent. I want to acknowledge all of you as FMD patients who serve as advocates for your own health. I look forward to meeting you at the June FMDSA annual meeting in Cleveland.

I have added additional comments to the transcript but I am sorry I did not get to all of your great questions.

Cleveland_Clinic_Host: Given the overwhelming response, we hope to host another chat soon. We will let you know when it is scheduled.

Technology for web chats paid in part by an educational grant from AT&T Ohio and the AT&T Foundation (formerly SBC).

Reviewed: 02/09

This information is provided by Cleveland Clinic as a convenience service only and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. Please remember that this information, in the absence of a visit with a health care professional, must be considered as an educational service only and is not designed to replace a physician's independent judgment about the appropriateness or risks of a procedure for a given patient. The views and opinions expressed by an individual in this forum are not necessarily the views of the Cleveland Clinic institution or other Cleveland Clinic physicians.

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