November 3, 2009
Heather Gornik, MD
Robert and Suzanne Tomsich Department of Cardiovascular Medicine: Clinical Cardiology and Vascular Medicine
Medical Director, Non-invasive Vascular Laboratory
Fibromuscular dysplasia (FMD) is a rare disorder characterized by abnormal cellular growth in the walls of medium and large arteries. FMD is most common in women between ages 30 and 50, but may also occur in children and the elderly. Treatment for FMD varies and can be tailored to treat different severities. Dr. Heather Gornik and Special Guest, Pamela Mace, RN, President FMDSA, answer your questions about FMD.
Cleveland_Clinic_Host: Welcome to our Online Health Chat with Dr. Heather Gornik. She is a vascular medicine specialist and leads The Fibromuscular Dysplasia Clinic at Cleveland Clinic. Joining us, we also have Pamela Mace, RN, who is the President of FMDSA. We are thrilled to have Dr. Gornik and Pamela Mace here today for this chat. Thank you for joining us Dr. Gornik and Pamela.
Speaker_-_Dr__Heather_Gornik: I am delighted to be here for our second FMD webchat - I am particularly pleased to have Pam Mace here next to me. Many of you know Pam and her role as Executive Director of FMDSA, an FMD patient and also a tremendous patient advocate. Let’s get going with the questions!
Symptoms of FMD
10 Most Common Symptoms
melva: Other than aneurysms, dissections and high blood pressure, what are you seeing as the 10 most common symptoms that the majority of us share?
Speaker_-_Dr__Heather_Gornik: Hi Melva. Nice to meet you again. The most common symptoms: bruit found by a doctor, headaches, swooshing or ringing in the ears, hypertension. Obviously there are the symptoms of very severe FMD, which include carotid dissection and renal artery dissection, mini stroke (TIA) and stroke, pains in the legs with walking, pain in the abdomen after eating.
Speaker_-_Pam_Mace_RN_-_Executive_Director FMDSA: Headache is very common. Vertigo and dizziness as well as neck pain can be there with FMD patients.
Speaker_-_Dr__Heather_Gornik: In terms of symptoms, sometimes it is hard to know if the symptoms are due to FMD or some other cause in the patient with FMD. Sometimes FMD gets “blamed” as the cause of all of the symptoms that bother a patient. It is tricky to tease apart what is vascular and what is not related.
cb: My daughter has Intimal FMD and suffers pains in her abdomen after eating and, at times, a stabbing, sharp pain in her right side. Is this FMD related?
Speaker_-_Dr__Heather_Gornik: It is certainly possible. Intimal FMD is the less common type of FMD where the narrowings in the arteries look less like a classic string of beads (that is the more common medial type, present in >90% of cases). Intimal FMD can affect the arteries of the intestines and can cause abdominal pain, but usually for this to be the cause there is extensive disease in multiple vessels of the abdomen. As always, we need to consider other causes of symptoms, as even for FMD patients, the FMD is not always the cause. I wish you and your daughter all the best.
Symptoms: Neurological symptoms
kptocs: Are the temporary neurological symptoms people with FMD get considered TIA’s? I am referring brief episodes of dizziness, migraines, visual disturbances, nausea.
Speaker-_Dr__Heather_Gornik: Good question. In general, a TIA is a temporary neurological symptom due to inadequate blood flow to the brain that resolves completely within a short period of time and has not caused a stroke on brain imaging. This has to be differentiated, however, from other temporary symptoms that are not due to inadequate brain blood flow. In most cases, for example, dizziness is not due to a TIA. Similarly, migraine headaches with vision changes (called aura) and nausea is also not a TIA. Even if these symptoms are not TIAs (which is a good thing), they may still potentially be related to FMD. Headaches are a common symptom among patients with carotid FMD and we are just learning more about this link.
carlar: I have terrible headaches off and on some are so bad I have to go to bed could FMD play a role in these headaches?
Speaker_-_Dr__Heather_Gornik: Headache is a very common symptom among patients with FMD, but is also a very common symptom among all adults and particularly women. There are many ways in which FMD can lead to headache. Patients who have had carotid dissection may have headaches related to this. Patients with FMD and brain aneurysms can also have headaches. Some patients with FMD who have some mild beading of their carotid arteries also have headaches, and these tend to be migraine-type headaches. It is hard to tell if the FMD is causing the headaches or if the fact that the link between migraines and women and migraines and FMD relates them.
Molly: I get ocular migraines about 1-2 times a month. I’ve tracked them and they are somewhat connected to stressors but not always. No pain, just the heat wave or broken glass affect in my vision. I used to get migraines but then had an aneurysm coiled and I no longer have traditional migraines. Now, I get these. My neurologist added verapamil (already on cozaar) to help. My question, are these “migraines” or something else?
Speaker_-_Dr__Heather_Gornik: Good question. Without reviewing your case it is difficult for me to say for sure. But I do want to pass along that it is very common for patients with carotid FMD to report migraine-type headaches, including ocular migraines, migraine with aura and even unusual types of migraines.
Symptoms: Peripheral Neuropathy
democrat: I have been diagnosed with FMD and also with Peripheral neuropathy. Is there a connection?
Speaker_-_Dr__Heather_Gornik: I have no knowledge of a connection, but I would be sure to really press your doctor to be sure that your symptoms are from neuropathy and not from the possibility of FMD in the arteries of your legs. A simple noninvasive test, like an ankle brachial test or leg artery ultrasound could help to sort it out.
Symptoms: Hypertension and FMD
MaureenW: Blood pressure medication; I hear a lot of confusion with patients regarding medication. Have heard and experienced myself blood pressure that is out of control. Extreme dipping and Insane Spiking with TIA’s.. I am controlled on Avapro, Toprol and Doxazosin. I have no “significant stenosis”. (Renal FMD; angioplasty 2002 & 2007, beginnings of carotid (bilateral bruits), mild to moderated subclavian. This medication combination calmed everything down and allowed me to return to work and my life. No one wants to have to take medication, me included, but I feel we have wicked vasospasms and sometimes we may need a more aggressive approach to control these arteries?
Speaker_-_Dr__Heather_Gornik: Thanks for your input. I do want to clarify that FMD is a problem of arteries that is present at all times and does not just come or go/reverse. Vasospasm is a reversible process. I also would clarify that patients with FMD, like all patients and >75% of the older American population, can have essential hypertension too. Thus, even when there is a successful opening of a severely narrowed artery, high blood pressure (sadly) may not be cured
fmd_hubby: I was diagnosed with hypertension in my early 50’s. My BP was always fine before that sudden onset. My wife has been dx with FMD (carotid) in the last year or so and is concerned my HTN may be related to undiagnosed FMD. Is this a valid concern as FMD is so underdiagnosed?
Speaker_-_Dr__Heather_Gornik: Dear FMD hubby, it is always possible, but quite unlikely. First of all, 19 out of 20 of my patients are women, and I am always suspicious of the diagnosis of FMD in a male patient until we truly confirm things. Second of all, you would be the first married couple in the history of the world medical literature to have FMD. Nonetheless, bring it up to your doctor and see what he or she has to say.
MaureenW: Anxiety/Panic is a common symptom with my renal FMD. It is something I never felt before until diagnosed in 2002. Only had it in 2002 and 2007. It is my red flag that “okay may need another angioplasty.” That activation of the renin - angiotensin system is not a fun ride. I am a nurse and understand the compensatory mechanism as to what is happening. Could you briefly explain to patients what and why this happens.
Speaker_-_Dr__Heather_Gornik: Great question. Many patients with severe high blood pressure feel unwell during blood pressure spikes. It is hard to know whether it is the blood pressure alone or some other mechanism. Usually in severe renal FMD, the renin and aldosterone levels are high at all times, not necessarily spiking intermittently.
Speaker_-_Pam_Mace_RN_-_Executive_Director: When I experience blood pressure spikes, I become very emotional/anxious. My renal blood work has always been within normal limits. When this happens to me, I try to relax until the episode is over.
Speaker_-_Dr__Heather_Gornik: If you were my patient, this is the type of symptom I would want to know about in between office visits. Sometimes we do ambulatory blood pressure monitors to monitor a patient over a time period to catch these spikes.
NHCC: Do you find Cozaar to be a good drug of choice for blood pressure control?
Speaker_-_Dr__Heather_Gornik: For patients with renal FMD, I generally use an ACE-inhibitor (like lisinopril or captopril) or an angiotensin receptor blocker (like Cozaar [Losartan]) as first-line treatment for high blood pressure. These drugs cannot be used, though, if there are very severe narrowings in both renal arteries (which is the case in only a small percentage of cases). There is some recent literature on potential benefits of Cozaar in patients with a certain connective tissue abnormality known as the mutation of the TGF beta receptor 1 or 2 gene, and sometimes referred to as Loeys-Dietz syndrome. Thus, for patients with FMD and aortic aneurysms we would consider this drug as first-line. I know of no specific data on Cozaar and FMD, however.
Symptom: Tachycardia and FMD
NHCC: What would be a good drug with FMD that could lower the heart rate? (tachycardia)
Speaker_-_Dr__Heather_Gornik: There are two main classes of drugs (maybe 3) that lower the heart rate. These are beta blockers (metoprolol, atenolol) and calcium channel blockers (diltiazem, verapamil). These drugs also lower the blood pressure. Neither is used specifically in FMD patients.
Renal Arteries and FMD
gmandmax: I had CT w/contrast which showed FMD in right renal and celiac arteries. No intervention is being considered for celiac as superior mesenteric is clear. In consultation w/radiologist, we agreed to have carotid ultrasound prior to angioplasty of the renal artery. He discussed measuring pressure in the artery prior to inserting and opening a “balloon”; noted diminished size of the kidney, unremarkable history of hypertension, other than that it has increased significantly over four to five years. My question is: does it necessarily follow that the carotids will, eventually, be effected by FMD, if they are not at this time? I am also interested in knowing if it is advisable for my adult children to be screened for this condition?
Speaker_-_Dr__Heather_Gornik: Thank you for your questions. There are many patients with FMD who have only renal artery disease, and carotid FMD never develops. I will generally check the carotid ultrasounds in all patients with renal FMD at least once. If there is no disease there, I do not check again unless there are compelling symptoms that suggest carotid disease has developed. In terms of screening children, I recommend that my FMD patients tell their kids and siblings to bring up FMD at their next office visit. Most cases with FMD are not familial/inherited, but some are. Certainly, if a family member also has hypertension at a young age, severe headaches or swooshing in the ears, or dissection, they need to be evaluated for FMD.
EdnaO: What are the indications that an renal artery bypass is needed instead of an angioplasty for FMD of the renal arteries that is causing very high blood pressure?
Speaker_-_Dr__Heather_Gornik: Most renal FMD can be successfully treated with balloon angioplasty to disrupt the webs/narrowings that occur in the vessels. We recommend that this be done with guidance by intravascular ultrasound (an ultrasound picture is taken inside the artery to identify the narrowings/webs) and assessment of pressures across the narrowings to be sure the lesion is successfully treated. In a small percentage of cases, the narrowings are too extensive to treat with angioplasty alone or there is disease in very small branches that is not amenable to angioplasty. In these cases, an aortorenal bypass or other open surgical procedure is recommended. I would recommend that all angioplasty and surgical procedures for patients with FMD be performed by specialists who have expertise in the management of FMD of the renal arteries, as this disease is very different from the more common atherosclerosis.
annkeith: I’m a 47 yr old woman recently diagnosed with FMD. Last March I had angioplasty on my right renal artery. Blood pressure isn’t much better and now they recommend they put in a stent. How long do they last and should I just have both arteries done now? I’m in good health otherwise and I’m not overweight. 115 lbs. no other health issue’s right now I’m not a smoker but I do exercise!
Speaker_-_Dr__Heather_Gornik: It is tough for me to give you specific recommendations without reviewing those renal angiograms in detail. It would be very important that all of the webs were treated, and we generally use an ultrasound catheter inside the renal artery (IVUS) and an assessment of pressures across the kidneys before and after intervention to be sure the narrowings were treated.
MaureenW: Current Literature indicates renal FMD as being most common findings though I have noticed that at the past two annual conferences and with talking with new patients diagnosed that there appears to be more involvement with carotid and vertebral. Your thoughts??
Speaker_-_Dr__Heather_Gornik: Good question, Maureen. In previously published case studies, renal FMD is the most common site, followed by FMD in the carotid and vertebral arteries. I think part of the reason why we are diagnosing more carotid FMD is because we have better noninvasive tests to see the carotid arteries.
bjeanh1: I learned that I have FMD while being screened as a kidney donor. The cat scan found 50% stenosis of the renal artery. When I consulted with a specialist after learning of this, I was told my condition is not serious and probably never will be. Does that mean that FMD does not progress? I.e., I will always have 50% stenosis and it will not get worse?
Speaker-_Dr__Heather_Gornik: We follow many patients with asymptomatic renal FMD that does not progress at all over time, and there is a good chance that this would be the case. Nonetheless, I would recommend a careful follow-up program with close monitoring for high blood pressure and annual check of your kidney function (BUN and creatinine). At our institution, we would also likely obtain a renal ultrasound every year to reassess the size of your kidneys (decreasing size may be a marker of worsening disease) and the degree of narrowing on the ultrasound.
Jodi: My 18 year old daughter had angioplasty on right renal artery recently. Other arteries checked at that time and looked good. Is it possible for stenosis to occur at a different artery at a later time? She is taking baby aspirin should she continue this indefinitely? Do you think Provera is safe for her to take? Also should she see a nephrologist and neurologist for annual exams? Thanks for your time.
Speaker_-_Dr__Heather_Gornik: Many good questions. There can be renarrowing of the renal arteries at the site of an angioplasty. Also, it is possible that there was a web or narrowing of an artery that was not treated in the first place. There could be narrowings in other vessels, but these can be asymptomatic for many years.
I generally recommend continuing antiplatelet therapy (aspirin) in patients with carotid, and FMD to a lesser extent, renal FMD. In terms of the specialty of the physician who takes care of an FMD patient, I don’t think their particular discipline is as important as their knowledge and experience of caring for FMD. In many cases, nephrologists and neurologists are the most experienced doctors in an area that follows patients with FMD. Don’t be afraid to ask your doctors about their experience in caring for patients with FMD.
Speaker_-_Pam_Mace, RN: What I recommend, if you can’t be seen at Cleveland Clinic or another clinic that specializes in FMD, is to be seen by the specialist for the area involved and your vascular doctor.
LindaS: RE: FMD : FMD dx 1990, RAS. Has progressed. Dx. July last yr with atrial fibb and L atrial clot. Clot not dissolving/ dissipating with 1st Heparin and Coumadin and now with Coumadin. Was flailing in circulation and hasn’t broken lose. Second TEE shows no change. Is this condition common with FMD. Was determined that had atrial fibb because of prolonged high BP with Pheo and RAS, both better controlled but have to monitor BP continually. ? address FMD and Pheo. Thank you, Linda
Speaker_-_Dr__Heather_Gornik: I know of no association between FMD and atrial fibrillation, other than the fact that atrial fibrillation is the most common significant heart rhythm disturbance in a middle aged/elderly population. Similarly, I know of no documented association of pheochromocytoma and FMD, although they are both on the list of things that can cause difficult-to-control hypertension.
BL: Hi Dr Gornik and all...First of all, I must join in w/all rest of my fellow “FMD’ers” in thanking you, Cleveland Clinic, FMDSA and...” well, here I go...(brevity not being one of my virtues), only beginning of my post and I find myself using too many words! With this problem of mine in mind, I proceed to questions (especially since yesterday’s GREAT chat!) 1) 65 yr old, history of bilateral renal artery FMD with aneurysm, one large enough to be removed in late 1990’s...follow up CT’s, angiograms showed condition/aneurysms fairly “stable” (one notable exception, last CT scan, about 3 years ago, showed one of two arteries supplying right kidney, seemed to show NO SIGNS of FMD on one of those two arteries, previously diagnosed w/it...done by same hospital, read by same Radiologists.3) Now in an “FMD Crisis” for first time blood results have come back (two tests) abnormal Creatinine 1.3 but most concerning (to me) is GHF of 44...OK...I should delete this...but, something is keeping me from it...maybe it’s all “my sister FMD’ers” who stepped forward yesterday to ask the questions we’ve all been posting about for so long...to thank you for being here for us, to take your time to put an “official ear” to all our fears...There! I KNEW my heart would put out the reason for this rambling email...to THANK YOU...(and Dr Naz McDonnell) for...helping, so much, in my case...do what I’ve been advised to do by other FMD’ers: Be proactive. I hope to meet you and Dr McDonnell Naz soon...
Speaker_-_Dr__Heather_Gornik: Dear Ms. BL, Thank you for your heartfelt and enthusiastic letter. Taking care of FMD patients is one of the most rewarding things I get to do in my life here at Cleveland Clinic. I would most definitely recommend repeat imaging of your renal arteries to be sure there is no renarrowing. Also, be aware that the GFR number is based on your creatinine value, primarily, and that GFR also tends to decline with age and could decline on the basis of age alone (albeit a little bit) in the 10 plus years since you’ve been living with FMD. Please let me know if we can help here at Cleveland Clinic.
cb: My daughter has had a vein used from her leg for a renal artery bypass, as she had no other suitable arteries. How many times can veins be taken from a patient for bypasses?
Speaker_-_Dr__Heather_Gornik: Hello! Great question. Believe it or not, there is actually quite a lot of vein in the legs, and in most cases, this vein is adequate for any future bypasses (and I hope your daughter does not need another). There are two major superficial veins in each leg that can be harvested for leg bypass. One runs from the groin to the ankle, and the other from the knee to the ankle. In the arms, there are also superficial veins running from the wrist to the upper arm that can be harvested.
Carotid Artery and FMD
Pat: I was having TIA’s frequently to find I had the beaded effect in both carotids last year. After trying meds as a lesser of the evil, endarterectomy, which I wound up having done one in August and another in December,. I am back to the same situation beginning June of this year with frequent TIA’s with the beaded effect back again. I was surprised it returned this quickly, I guess this is what I can expect for life? 2) My Vascular Dr. had me on Plavix, Aspirin 81 mg and Coumadin, my other Dr. feel this is a harsh mix that I shouldn’t be on all 3 blood thinners, what is your insight?
Speaker_-_Dr__Heather_Gornik: I am sorry you have been having such a difficult time. Unfortunately, in order to give you recommendations, I would need to see and examine you and, very importantly, review your imaging studies to be sure the narrowings in your arteries were adequately treated. Also, it is hard for me to know the risks versus benefits to you of being on aspirin, Plavix, and coumadin. There is a higher risk of bleeding for patients on all three medications at once, but in some cases this is needed. We would be happy to see you here in Cleveland if you are able to travel.
RonnieH: Hi Dr. Gornik and Pam, Thank you so much for having this chat again. I just visited my cardiologist who checked out my heart and legs and I’m still having discomfort when I’m on my feet too long. Any ideas? I am thinking about the possibility of work on my carotids by you but I am nervous. How dangerous is this procedure, the noise is so loud but I’m worried about the possibility of a stroke while you are performing the procedure. Thanks for listening.
Speaker_-_Dr__Heather_Gornik: Hi Ronnie. Great to hear from you. Thank you for your support of the FMD community. You are referring to an angioplasty procedure of some of the carotid artery narrowing/webs to treat a severe narrowings that are symptomatic. Every case is different, but, in experienced centers, the risk of stroke with angioplasty is low. In general, when we do an angioplasty we use a special protection device that decreases the likelihood of embolic material going to the brain. Each case is different though, and I recommend that you touch base with your physician about the risk and benefits of this procedure.
Speaker_-_Pam_Mace, RN: Hi Ronnie - if you would like, I can discuss my experience with this procedure. I have had no complications.
judyc: I am a 61 year old female and was diagnosed with FMD about 4 years ago. Both carotid arteries are effected. The right side is the worst. What type of mental changes should I look for if any (memory, vocabulary, etc.)? Would these be more gradual or sudden? Thank you Judy
Speaker_-_Dr__Heather_Gornik: In general, I do not think of FMD causing memory loss or dementia. Symptoms to watch out for that are commonly reported in FMD patients include headache, a ringing or other sound in the ears. In severe cases, we would be watching out for carotid dissection or stroke or mini stroke (TIA). This would be sudden onset of severe intense headache, loss of vision or ability to speak, weakness or loss of sensation on one side of the body.
A large percentage of patients with FMD in the carotid arteries have no symptoms at all, or maybe their doctor heard a bruit in the neck. I don’t want you to feel as if you are doomed to have symptoms of FMD, as many patients have none at all.
CPetersen: When I have my yearly I had a carotid dissection 5 years ago. Healed on its own with no surgery. When I have follow up MRA’s to measure blood flow in the those arteries, my doctors say the blood flow is very good and they show me some numbers. Are they saying blood flow is good for someone with FMD? Can my blood flow be the same as a non-fmd patient? I still have dizziness, so isn’t that an indication of some blood flow problem?
Speaker_-_Dr__Heather_Gornik: Good question. Believe it or not, sometimes dissections heal completely and there is no significant narrowing of a blood vessel. In addition, many patients have some of the beads in their carotid arteries due to FMD but do not have limitation of blood flow to the brain.
Pat C: I have beaded effect in my carotids, how often can this reocurr since they have been removed 8 months ago. Is this something that can go on for the rest of my life?
Speaker_-_Dr__Heather_Gornik: Unfortunately, FMD is generally a chronic condition and there is no true cure. I should clarify that, presuming you had a carotid angioplasty, the beads are not actually removed. In FMD, there are narrowings of tissue growth or webs in arteries followed by areas of mild enlargement of the artery. The angioplasty balloons actually open up the artery. The good news is that, for most patients with FMD, the success rate for an angiplasty procedure is high. You still need to have regular follow-up, though.
DeniseG: Diagnosed with FMD left carotid artery 2005, dissected in 2007, right carotid dissection diagnosed 2009, recent hypertension, three tia’s over last six months, my questions: since this has occurred in both carotids is it likely that I may have FMD elsewhere now or in the future? What symptoms should I look for? Should I be concerned as my doctors don’t seem to be as long as the blood is flowing through the arteries. Currently on Plavix and aspirin but recently had a tia, not as severe as the original one prior to meds-are these symptoms likely to continue? What direction should my care be going in? Controlling hypertension, finding cause? Treating symptoms for now or preventing stroke in the future? What does the future hold for me-is a stroke inevitable? I heard that some dissections heal on their own-how long would that take? I have a vascular surgeon and a family physician that I see-should I be seeing any other specialists?
Speaker_-_Dr__Heather_Gornik: I am sorry to hear you have been having such a difficult time. I would recommend assessment of your renal arteries to rule out renal FMD if you are having new and hard-to-control high blood pressure. This can be assessed with a duplex ultrasound in vascular labs experienced with this test or a dye-based CT scan or MRI. Without reviewing your specific history, your scans and examining you, it is not possible for me to tell you the direction in which your care should be going. We would be delighted to see you here at Cleveland Clinic for a second opinion. In general, vascular surgeons do have experience taking care of patients with FMD, but this also depends on the particular specialist. You might also consider asking your family physician if you should see a nephrologist/hypertension expert.
NHCC: If an person has confirmed FMD of the inner carotid arteries and a patent foramen ovule and an atrial septal aneurysm diagnosed recently, would this be an added consideration as an extra risk associated with FMD?
Speaker_-_Dr__Heather_Gornik: I know of no clear link between FMD and patent foramen ovale and atrial septal aneurysm, other than the fact that both are diagnosed in younger patients and that they have some other common features. PFO/atrial septal aneurysm is a communication between the top chambers of the heart and is something that is present at birth. We are not sure about the exact epidemiology of FMD in terms of specifically when it develops and if some features of it are present in childhood. Both FMD and PFO have been associated with migraine headaches in young patients, and both could potentially lead to a stroke in young patients, though due to different mechanisms (for FMD, can be due to dissection; for PFO, can be due to a clot traveling to the brain). The other thing that both FMD and PFO have in common is that many patients have these disorders, but are completely fine, healthy and asymptomatic.
FMD in Multiple Vessels
maryP: FMD WEB CHAT When I was first diagnosed with FMD (1996) I was told by several doctors it would probably never go beyond the renals. Now I have it in multiple areas. Is current research showing an increase of FMD involvement in patients as they age?
Speaker_-_Dr__Heather_Gornik: Most likely, the FMD was present in the other vessels (carotids, vessels of the arms or legs) at the time it was diagnosed in 1996, but it did not cause symptoms. It is very common to have asymptomatic FMD in other vascular areas. If we see a patient with renal FMD, we do a very careful physical examination and also generally image the carotid arteries (with an ultrasound in most cases).
lindalwml: I have FMD in multiple arteries. My dad died of an abdominal aortic aneurysm and his nephew of a brain aneurysm at age 42. Although we don’t know if they had FMD, should my adult sons be checked and what test could done to se I they have FMD. Would ultrasound be effective in diagnosing?
Speaker_-_Dr__Heather_Gornik: In your family’s case, I would be very concerned about familial FMD or other familial connective tissue disorders. I would recommend you and your family be evaluated in a vascular program with a medical genetics clinic. We would be happy to see you here in Cleveland. We have a genetics counselor every Friday with us in Clinic.
Pearl Effect in Arteries and FMD
Pearl: Does anyone know the cause or origin of the pearl effect that one can have in their arteries? Is it so that it will reoccur for life?
Speaker_-_Dr__Heather_Gornik: I think the pearl effect you are referring to may be the beading we see in arteries that is generally due to fibromuscular dysplasia. The beads are formed by narrowings in the vessel and areas of dilation/widening after the narrowings.
FMD and Women, Hormones, Menstrual Cycle
maryP: Thank you Cleveland Clinic for your support and compassion for those of us with FMD! Having been told there would never be research in my lifetime (I was 39 at the time and now 53), I am so thankful to your fine institution! My question is that myself and quite a few of my fmd friends have noticed an increase of our symptoms prior to our period. Has your observance of female patients with FMD noticed this? Could it be the surge of hormones during this time frame increases our symptoms (headaches/BP/fatigue etc.).? Thank you again for your interest in FMD!
Speaker_-_Pam_Mace, RN: My symptoms are also worse around my periods. Once my period starts, they tend to resolve.
Speaker_-_Dr__Heather_Gornik: Other female patients with FMD have reported this to me. I am not yet sure, scientifically, what to make of this, and I definitely think this needs to be explored further as far as any hormonal influence on FMD symptoms. One thing that is tricky to tease apart are what symptoms are due to the actual FMD vascular disease and what symptoms may occur because FMD occurs more commonly in female patients.
Sterk: Many post menopausal FMD females suffer from various prolapses (rectal, bladder, etc) but also dry and very thin skin in the vagina. My questions are 1. Do you recommend repair of bladder prolapses that protrude into the vagina wall, or given the nature of our stretchy skin and weak connective tissue, to leave them alone and not repair...... 2. To combat the dry thin skin in the vagina, which seems to accompany many females as they age, do you recommend estrogen creams to combat this. I am not sure that FMD’ers should be taking any hormone creams (oestriol 1mg) or (vagifem 25mcg) for example, despite them being a low dosage. I would be pleased for your comments on these questions thanks. Thank you!
Speaker_-_Dr__Heather_Gornik: The use of systemic hormones in patients with FMD is somewhat controversial and we really need more research in this area. In my own practice, if patients are severely symptomatic and need hormone replacement therapy, I work with the patient’s gynecologist to have them on the lowest dose possible. I do think topical estrogen is probably lower risk than oral hormone replacement therapy. The international FMD patient registry will be collecting information on hormone replacement therapy and menopause, so this may be useful in the future.
Mimi: I was wondering if there has been any connection found between FMD and polycystic ovary syndrome?
Speaker_-_Dr__Heather_Gornik: Not to my knowledge, but they are both disorders that involve women, primarily (for PCOS that’s obvious!!).
NHCC: If FMD of the Inner carotid arteries have been diagnosed, when testing/scanning should the entire brain also be included? circle of willis??
Speaker_-_Dr__Heather_Gornik: See prior answer regarding carotid testing. Definitely if there is carotid FMD on an ultrasound study, we recommend that the patient have brain imaging at least once to rule out FMD in the brain and brain aneurysms. We generally do this with an MRI scan.
LynnW: I have FMD in 8 arteries (iliacs, renals, carotids, vertebrals). The iliacs were discovered incidentally during angioplasty, the carotids and vertebrals were diagnosed only because I insisted on being scanned. How important do you feel it is for a FMD patient to be scanned from top to bottom?
Speaker_-_Dr__Heather_Gornik: Good question. Here is my approach. We always image the carotid and renal arteries - and the brain arteries if there is carotid FMD. I do a comprehensive vascular exam for all FMD patients, feeling all the pulses of the arms and legs and listening to bruits. If there are any bruits or abnormal pulses in the arms or legs, we get imaging studies of those vessels. Any patient with a family history of aortic aneurysm or dissection or abnormal findings on exam will also get imaging of the entire aorta.
NHCC: What is the best way to monitor FMD of both bilateral internal carotid arteries? Best scans or tests and how often should be done?
Speaker_-_Dr__Heather_Gornik: There is some institutional variation in how this is done and also on the location of disease. At our institution we have a strong vascular lab with experience in assessment for FMD, and we generally monitor carotid narrowings with ultrasound. At some institutions, follow-up may be with ultrasound, MRA or CTA. If there is disease in the brain (brain aneurysms) or prior dissection, we generally use MRA or CTA for follow-up. We try to limit the number of CT scans (CAT scans) over time, though, because CAT scans require radiation exposure and many of our FMD patients are young and will need follow-up for many decades
FMD and Coronary Arteries
mimi: Does FMD develop in the arteries of the heart?
Speaker_-_Dr__Heather_Gornik: There have been case reports of FMD involving the heart (coronary) arteries, but these are very, very rare. I have taken care of a few FMD patients who have had typical coronary artery events, such as heart attacks and unstable angina. I have also taken care of a few patients who have had unusual coronary artery events, such as a coronary artery spasm or a dissection. None of these angiograms have shown typical “beading” of the vessel like we see in the renal and carotid arteries, although a few of the angiograms have had somewhat bending or tortuous coronary arteries. This is an area where we definitely need much more research, and it will be included in the FMD registry to a certain extent. I do not recommend “screening” the coronary arteries in most cases of FMD, but every case varies, and we have done stress tests and other cardiac testing for patients with compelling symptoms.
Aspirin Resistance and FMD
KariU: Question for Dr. Heather Gornik’s FMD web chat: Could you tell us about Aspirin Resistance and, is this something that should be checked for fmd patients? What test is used to check for aspirin resistance?
Speaker_-_Dr__Heather_Gornik: Hello there KariU! Great to hear from you, and thanks for your continued support of the FMD community. Great question. Aspirin resistance is a phenomenon when a small percentage of patients will require a higher dose of aspirin to inhibit the platelets (which are the clotting cells that aspirin target). There is a lot of interest in the area of aspirin resistance among patients with coronary artery disease and specifically those that undergo coronary stent placement.
I don’t know of any specific data on aspirin resistance in FMD patients, nor is my sense that this is a major problem in the FMD population. Aspirin resistance tests can be done by seeing how platelets clump together in the test tube when exposed to different pro-clotting chemicals. If aspirin is working well, as it should, it should inhibit the platelets from clumping together (we call this aggregation). It is a special blood test that requires special equipment and chemical reagents. That being said, I rarely ever order an aspirin resistance test unless I am worried that a patient has had a clotting type event while on aspirin or if she can only take a very, very low dose of aspirin and I want to be sure it is effective.
Ibuprofen and FMD
maryP: Has there been any adverse affects noticed from the drug ibuprofen on those with FMD?
Speaker_-_Dr__Heather_Gornik: Non-steroidal anti-inflammatory drugs (NSAIDs) have the potential to effect the abilities of the kidneys to filter the blood effectively. This affect can be particularly prominent in patients with significant kidney disease. So while I know of no specific studies on FMD and ibuprofen, I would have the recommendations I use for all of my patients with cardiovascular disease, hypertension, and renal disease, which would be very sparing use.
Physician Follow-Up for FMD Patients
TerriW: Questions for FMD Webinar What kind of followup should I have? (Just diagnosed with FMD in both Renal arteries - had angioplasty) How closely should the rest of my body be monitored? Do I need to have low intensity workouts? Should I follow a specific diet?
Speaker_-_Dr__Heather_Gornik: Many very good questions. FMD is a chronic health condition that requires periodic follow- up with a health care provider who is familiar with this problem. Even after angioplasty, a patient needs careful follow-up that depends on the vascular areas involved (e.g., carotid, renal) and how the patient is doing clinically. I see stable FMD patients every 6-12 months, and patients with active issues more frequently. The specific exercise prescription for a patient (i.e., The amount of exercise they can do) depends on the individual’s pattern of FMD (carotid vs. renal; prior dissection or aneurysms) and also how well the blood pressure is controlled. There is no specific FMD diet; however, I recommend a heart-healthy diet, maintaining a normal body weight, and losing weight if you are overweight. While we do not have a cure for FMD, there are many other cardiovascular risk factors that can interact with FMD, leading to uncontrolled hypertension, for example, that we can control. For blood pressure control, a low-salt diet rich in fruits and vegetables is very important.
jenw: How often would you recommend a patient diagnosed with FMD (carotid/renal) see a specialist & what kind of specialist would you suggest. I have seen a cardiovascular doctor for a dissected carotid artery that has since healed - doctor suggested I don’t need to see him unless I feel things have changed.
Speaker_-_Dr__Heather_Gornik: In my practice, I think it is important to follow FMD patients long term and not just through an acute problem. After all, this is a chronic problem and it is important to have a relationship and alliance with your FMD doctor.
Speaker_-_Dr__Heather_Gornik: For patients who live locally or can travel to Cleveland Clinic, I see them every 6 to 12 months - more frequently if problems are not under good control, and annually if completely stable.
Speaker_-_Dr__Heather_Gornik: I want to emphasize that FMD is not a problem we cure with a single angioplasty. It is a chronic health problem we need to manage long term.
Connective Tissue Disease, Ehlers-Danlos Syndrome and FMD
NHCC: Are you finding more patients with Ehlers-Danlos Syndrome and FMD?
Speaker_-_Dr__Heather_Gornik: The link between Ehlers-Danlos syndrome (EDS), an inherited disorder of collagen production (which is an important protein of the blood vessel wall and other tissues such as bones and ligaments), and FMD is an area of active clinical research. Dr. Nazli McDonnell at the National Institutes of Health is conducting research on a potential link between the two. I have seen a few rare cases of patients with both FMD changes in their blood vessels and confirmed EDS. More commonly, though, we are doing some testing for EDS when patients are referred to us for possible FMD and it is clear that they do not have classic FMD but mainly have a problem of dissections in their blood vessels without a known cause. In these cases, we have been testing for EDS and some other connective tissue abnormalities.
Susan_Florida: I have renal FMD with a right renal stent, mild carotid and some mild brain FMD but I’m troubled more with the connective tissue side of things. My spine continues to degrade and I’m having lots of fatigue, muscle weakness etc. (more EDS symptoms) From what you are seeing in your study base am I more the exception than the rule? Any suggestions on how to slow down the connective tissue problems? Thank You Thank You for all you are doing for FMD.
Speaker_-_Dr__Heather_Gornik: So nice to hear from you. We have seen some FMD patients who also have musculoskeletal problems such as scoliosis and some features suggestive of a connective tissue problem, such as joint laxity and scoliosis of the spine. At this point, I do not have firm evidence for you. I know this is an area of active research by Dr. Nazli McDonnell at the National Institutes of Health. The website for her laboratory is: www.grc.nia.nih.gov/branches/irp/nmcdonnell.htm.
Sandy1. Can FMD be found in an artery previously checked via CT Angio for FMD or other imaging methods? 2. Could you please discuss the connective tissue overlap symptoms common in FMD patients and in relation to Dr. McDonnells 1st Abstract noting FMD may be a milder variant of EDS Vascular? 3. Why do published articles on FMD not include the overlap connective tissue symptoms prevalent among FMD patients like joint pains and fatigue so that Physicians who only treat one or two patients with FMD have a reference to validate their patients non vascular symptoms? This would be very beneficial to all patients and especially for those who cannot advocate well. 3. Could you please explain how a dissection heals and the degree of risk for stroke or other complications from the time of dissection until healed. Approx. time a dissection takes to heal? 4. Since the last web chat have you learned any additional information from your clinic that is relevant to the questions asked last time? Specifically have you had any patients with FMD in the coronary arteries?
Speaker_-_Dr__Heather_Gornik: Wow, many great questions. Some answers:
- It is unusual for FMD to progress so rapidly, but not impossible. Most likely, there might be a difference in the techniques of the CTA scans or the interpretation. It is always helpful to have the scans performed in the same facility and interpreted by the same radiologists.
- Connective tissue disease overlap is an emerging area in FMD, but there is little published, peer-reviewed data on this topic, and at this time, I feel it is too early to comment upon this in a general FMD forum. We hope the registry will help to define the nature of FMD and symptoms from which patients suffer. Unfortunately, even in FMD patients, not all symptoms are actually due to FMD, but there may be other common risk factors or environmental factors which cause these symptoms to seem more common among FMD patients (such as gender, age, other medical conditions, perhaps connective tissue abnormalities). Please stay tuned and continue to be such a motivated FMD patient!
- The time it takes for a dissection, or tear, in the vessel wall to heal is variable. The concern in the early stage of an arterial dissection is that a clot may form on one side of the tear due to low blood flow, and a piece of the clot can break off (embolize) to the brain or kidney. That is why anticoagulation (or antiplatelet medication in some cases) is generally recommended for dissection.
- We’ve had some interesting new approaches to treatment. We have seen a few cases in which balloon angioplasty has been helpful or treatment of severe, life-limiting swooshing in the ear or severe headaches. In these cases, patients have had severe narrowings of the arteries. We also continue to learn the importance of using hemodynamic assessments (intravascular ultrasound and pressure/flow wire) to assess how severe lesions are in the renal arteries, as sometimes the angiogram does not tell us “how tight” the narrowings are.
carlauss: My complicated FMD seems to be stable now. Is there hope that it will continue?
Speaker_-_Dr__Heather_Gornik: Yes - very much so. I care for many patients who had a very difficult period of time with dissection or other problems that led to their FMD diagnosis but are now stable. I think there is hope.
Speaker_-_Pam_Mace_RN_-_Executive_Director: In my case, I dissected both carotid arteries and a vertebral artery, which was a difficult time in my life physically and mentally. After being treated at the Cleveland Clinic, I have been doing well for years now without complications.
H1N1 and FMD
w: Should there be any concern about getting the H1N1 vaccination if diagnosed with FMD?
Speaker_-_Dr__Heather_Gornik: WE have received many timely questions about H1N1 vaccine and FMD. I don’t know of a specific relationship between the two. It has been generally recommended by the CDC that patients with heart and vascular disease get vaccinated. This is particularly important for our younger FMD patients. I would recommend both the regular influenza patients and the H1N1 if available. Always talk to your doctor first.
Kidney and Liver Cysts
carlar: I was diagnosed with a suspect liver and kidney cyst are those common for people with fmd?
Speaker_-_Dr__Heather_Gornik: Kidney and liver cysts are very common findings among all patients. I do not think they are more common among FMD patients than patients in general, but I do think they are more likely to be diagnosed in a patient with FMD because of the fact that patients with FMD tend to get ultrasound, CT, or MRI scans of the belly. This leads to what we call an “incidental finding” of the kidney and liver cyst that has always been there.
FMD and Exercise
mimi: On the TV show, Mysterious Diseases, the physician said that Pam’s running raised her blood pressure and may have contributed to the dissection of her carotid artery. If that is true, is it OK for those of us who have FMD in the carotids to do weight lifting or other exercise that might raise our blood pressure?
Speaker_-_Dr__Heather_Gornik: Exercise and FMD is an area where we have no hard science, but we do have common sense guidelines. Patients with FMD seem to be at increased risk for dissection or tears in the arteries, may have aneurysms, and may have severe high blood pressure. Certain types of strenuous exercise are not advisable under these conditions. Depending on the location of your disease and severity, your doctor may limit your exercise to a certain extent. As a rule, I recommend against contact sports; boxing, SCUBA diving, sky diving, heavy weight training. I also recommend avoiding chiropractic manipulation of the neck. In most cases, fitness walking, low-impact aerobics, pilates, yoga are fine, but you may want to talk to your instructor and your doctor about your disease.
Speaker_-_Pam_Mace, RN: It depends on the type of exercise, location and severity of the disease. Regarding my personal experience with running, after my event, running triggered neurological symptoms, so I no longer run, and walk instead. If an activity triggers symptoms, you probably should not be doing it.
KateP: Do you recommend Pilates for FMD patients? We are all aware that H1N1 Flu can cause excessive coughing and possible vomiting. With FMD in our carotid arteries does this put us at greater risk for a dissection and do you recommend we get the vaccine as soon as available and do you think we should we be considered in the High Risk Group? Thank you.
Speaker_-_Dr__Heather_Gornik: Please see prior answer regarding H1N1 vaccine. The CDC has recommended the vaccine for patients with vascular disease, if it is available to you. In terms of pilates, I think this is probably OK (there is no scientific literature about safety of these types of exercise), but please tell your instructor about your condition - that sudden jerking movements or heavy pressure on the neck are not recommended.
Caitlynn: What are your current recommendations regarding exercise?
Speaker_-_Dr__Heather_Gornik: Aerobic exercise is highly recommended. Please see the previous answer. I generally recommend against any contact sports, heavy lifting, SCUBA or sky diving. Jogging is OK in some cases, but there are some patients for whom this may not be advisable. The exercise prescription should be customized to the individual patient.
Massage and FMD
Mimi: Because I have a lot of muscular pain and have been diagnosed with migraine headaches, a physical therapist has recommended that I have deep tissue massages. I have FMD in my renal arteries and carotid arteries. It is OK for me to have massages of the back of my neck and my scalp?
Speaker_-_Dr__Heather_Gornik: Massage and FMD is a bit controversial and there is not a lot of data here. I generally tell my patients with carotid FMD to avoid any intense manipulation of the neck, such as chiropractic adjustments. Massage is generally OK, but I am not sure about deep tissue. I recently had a deep-tissue type chair massage and was honestly surprised and a bit alarmed by the intensity of it, and I do not have FMD. I would probably stick to traditional relaxation or European massage only and be sure to tell the therapist about your FMD.
FMD and Venous Disease
Mary: HI have FMD in my renal, carotid, and abdominal arteries; I also have had swelling in my ankles, weak legs, varicose veins and broken veins. The peripheral pulses in my legs are good; therefore, I would like to know if I could have FMD in the veins that lead to my lower extremities. Whenever FMD is discussed it always seems to be related to the arteries; however, I saw a recent article that revealed beading in the renal veins. Are we missing something here- can FMD affect the entire vascular system including the veins?
Speaker_-_Dr__Heather_Gornik: Great question. I have never seen a case of FMD of the veins... but I do know that varicose veins are a VERY common ailment that primarily afflicts women. For your venous disease and leg swelling, I generally recommend graduated elastic compression stockings. You may also want to see a vascular specialist, as there are treatments available for varicose veins, too. Good luck.
additional reply: To my knowledge, FMD of the veins of the legs (saphenous veins) has not been reported in the literature. Most likely, you have two problems that are separate (though both involve the blood vessels): arterial FMD and varicose vein and venous insufficiency. Both problems, unfortunately, tend to affect women. There are many treatments available for varicose veins/venous insufficiency, including compression stockings, sclerotherapy, and vein ablation procedures. A comprehensive vascular specialist should be able to help you.
Restless Leg Syndrome and FMD
BonnieW: I have FMD and Restless Leg Syndrome. Is it possible that they are related? I also experience extreme sleepiness (go to sleep while driving); could that be related to FMD? Thanks for your consideration.
Speaker_-_Dr__Heather_Gornik: I do not know of anything that links FMD to restless leg syndrome or extremely sleepiness. I would discuss both symptoms with your doctor. For the daytime sleepiness, you may need to be evaluated for something called obstructive sleep apnea, which is entirely unrelated to FMD.
Raynauds and FMD
ssvilar: Is there a connection between Raynauds Phenomenon (vasospastic attacks that cause the blood vessels in the fingers and toes to constrict) and FMD?
Speaker_-_Dr__Heather_Gornik: Hello there. The only connection I can think of is that both tend to affect primarily women.
Speaker_-_Pam_Mace, RN: I also have Raynaud’s, and we have several members who have contacted us who are also diagnosed with both.
Research and Latest Information
mimi: I was unable to attend the conference in June. Is there any new information about FMD that was discussed at that time and if so, where could I get access to it?
Speaker_-_Pam_Mace, RN: We put updates on the Web site when we have them. We hope we will have information to share from our patient registry at our upcoming conference in May 2010. Information on how to register for the conference is on our Web site.
csterritt: Are research studies going on at the Cleveland Clinic? If so what does it entail to participate?
Speaker_-_Dr__Heather_Gornik: Right now, our primary focus in terms of FMD research is active participation in the international FMD patient registry, which has been supported by the FMD society of America. I cannot overemphasize how important this registry is for this disease. We have enrolled approximately 60 patients in the registry since February at Cleveland Clinic, and we are only one of 9 sites enrolling patients in the United States and Europe. We are in the planning stages for other research programs related to FMD which will focus on FMD genetics and also quality of life and care satisfaction issues among FMD patients.
Speaker_-_Pam_Mace_RN_-_Executive_Director: To participate in the registry,you have to be seen at a participating center. The centers are listed on our Web site - http://www.fmdsa.org. We are looking at adding additional centers next year.
melva: Has the clinic at Cleveland Clinic uncovered any new info that was not previously known about FMD treatments or symptoms?
Speaker_-_Dr__Heather_Gornik: Thank you for this question. We have been running our dedicated weekly FMD clinic for just over 15 months at this point. We are participating in the international FMD patient registry, sponsored by the FMD Society of America, which will hopefully yield some very important new information about this disease within the next two years (stay tuned). In terms of new FMD treatments, we do not have any “magic bullet” for treating or curing FMD. Unfortunately, this is a chronic problem. We have, however, had a number of patients in the past year with severe symptoms that we have been able to help with balloon angioplasty guided by intravascular ultrasound along with surgical bypass techniques. These are not new treatments (balloon angioplasty has been done for FMD for years), but we are using new techniques such as flow wire assessment and intravascular ultrasound to determine who may benefit from angioplasty.
FMD Clinic at Cleveland Clinic
Melva: I have FMD in renals, mesenteric, femoral and carotids. I also have ruptured and unruptured brain annies. How is the first visit to CC set up to include all the areas?
Speaker_-_Dr__Heather_Gornik: Hi there. Our FMD clinic, which is staffed by vascular medicine specialists and a genetics counselor, is the first point of care for FMD patients. We contact you in advance to arrange for review of your medical records. Consultation with additional specialists (interventionalists, surgeons, nephrologists, neurologists) is arranged as needed, primarily if there is a need for an opinion regarding interventional therapy. In many cases, there is no need for additional consultations outside of our FMD vascular expert. We would generally perform a dedicated carotid duplex and renal ultrasound. If you have leg or arm symptoms or disease we would perform blood pressures studies on the arms and/or legs and possibly an ultrasound. We may have you see our genetics counselor if you have not already undergone evaluation and if your history raises concerns regards a familial element to FMD. You would be offered participation in the national FMD patient registry during your visit and given a packet of educational materials. In your case, particularly with your brain aneurysms, we would arrange for neurointerventional consultation with the specialists in our FMD program. We hope to have the opportunity to meet you.
Cleveland_Clinic_Host: I’m sorry to say that our time with Dr. Heather Gornik and Pamela Mace, is now over. Thank you again Dr. Gornik and Pamela for taking the time to answer our questions about Fibromuscular dysplasia.
Speaker_-_Dr__Heather_Gornik: Thank you for having me today. I will work on answering all the unanswered questions offline. I want to once again acknowledge the wonderful job FMD patients do in terms of spreading the word on their problem and serving as self advocates. I would also like to acknowledge the wonderful work done by FMDSA on behalf of patients with this disorder. See you next time.
Speaker_-_Pam_Mace, RN: I would just like to thank everyone for participating today and for your continued support of FMDSA.
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