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Understanding (FMD) Fibromuscular Dysplasia – (Dr. Gornik & Pam Mace, RN, FMDSA – 04-10-14)

Dr. Gornik

Heather Gornik, MD Cardiologist and Vascular Medicine Specialist,
Robert and Suzanne Tomsich Department of Cardiovascular Medicine,
Sections of Clinical Cardiology and Vascular Medicine

Thursday, April 10, 2014 - Noon

Description

Fibromuscular dysplasia (FMD) is an uncommon vascular disorder often found in women between ages 30 and 50. It can be difficult to diagnose, but while there is no cure, it can be treated effectively. Heather Gornik, MD and Pamela Mace, RN, President of the Fibromuscular Dysplasia Society of America (FMDSA) answer your questions about the diagnosis and treatment of FMD.

More Information


Symptoms of Fibromuscular Dysplasia (FMD)

Headache

Bross: I have recently diagnosed of both internal carotid FMD and my symptoms are diplopia, (atopic IV cranial par paresis) and chronic persistent headache. Is it Due to FMD?

Heather_Gornik,_MD: Dear Bross, headache is a common symptom among our FMD patients, but double vision is less common. I would definitely suggest that cervical artery (carotid or vertebral) dissection has been ruled out.

Pam Mace, RN: I agree with Dr. Gornik and it might also be helpful to be evaluated by a neuro ophthalmologist.

Jbugmimi: With FMD in carotid, vertebra, and renal arteries I experience many problems. The latest being severe pain in my right eye and always followed by a bad headache. I had a dissection in the right carotid in 1992 and was diagnosed with FMD. Is it possible for the eye pain to be related to FMD in the artery. One eye specialist believes the pain and vision problems are related to my vascular disorder. I have migraines and other headaches that seem to be different to migraine. I recently had MRI, MRA,CTA and Doppler done and my doctor wants an arteriogram done in four vessels. Where should I have this done? I'm afraid to have someone do the carotids Have had angioplasty twice In right renal.

Heather_Gornik,_MD: Eye pain is a very common symptom that goes along with headaches, including migraine headaches and some headaches related to prior dissection. I do think this symptom could be related to your FMD. I hope you can be seen by an FMD specialist. We generally have a very, very high threshold for any sort of intervention on the carotid arteries. This should really only be undertaken in very experienced centers and for very severe symptoms such as recurrent TIAs/mini stroke. I do hope you can be seen by an FMD expert. If not, perhaps a headache neurologist or cerebrovascular neurologist at a large medical center can help. Good luck.

Whooshing

Ronni H: Hi Dr. Gornik & Pam, thanks again for doing this. I won't be available on 4/10/14 so I wanted to share something which Pam already knows about. The PT in my left carotid was and is so loud it is affecting my quality of life so my neuro doc at Mayo did a carotid angioplasty on 12/31 which opened up blood flow but didn't help with the noise. I was wide awake during the procedure and could feel him going through the webbing but he could not go any higher nor place a stent due to possible dissection. Hearings aids and acupuncture don't work. The noise is getting louder in my right ear and I am seriously considering sign language in the event I won't be able to hear anymore. Is total hearing loss a real possibility without actually dissecting? I just wanted your opinion as I know I am not alone and I have to learn to live with it. Hopefully through the registry and research more answers will be available.

Heather_Gornik,_MD: Dear Ronni. Thank you for sharing your experience. I am sorry that the angioplasty did not help the pulsatile tinnitus. Pulsatile tinnitus is so common among FMD patients --- there was just a Registry abstract presented at the American College of Cardiology meeting by Dr. Redah Mahmood that ~ 1/3 of FMD patients have this most annoying symptom. We don’t have a lot of data on the natural history of this and whether it is associated with hearing loss. For all pulsatile tinnitus patients, I do suggest working with audiologists, hearing specialists. Our group here does offer a tinnitus management program where they talk about some behavioral maneuvers/therapies to control this annoying symptom.

Pam Mace, RN: Hi Ronnie, I know you are aware of Whooshers but for others who may not know there is a web site called whooshers.com. They have a lot of information on their web site and do hold meetings with some great speakers for those of you who live in NY and experience the pulsatile tinnitus. We will also have information available at the annual meeting from whooshers.com.

Dizziness

Minzmom: What might be the cause of the dizziness that is so common in FMD patients?

Heather_Gornik,_MD: Dizziness is one of the more common symptoms in our FMD patients... it's a really tricky one because for some patients dizziness is a vertigo type symptom, for others it's an imbalance, and for others it's a faintness. Some patients with very, very severe carotid FMD can have impaired blood flow to the brain, but that's quite rare. Would work with a neurologist to tease out what your "dizziness" really is.

Neck Pain

scottie: This question relates to FMD and neck pain. What is the mechanism of the chronic pain caused by FMD? I am aware that there are many causes of neck pain other than FMD but would like to understand how FMD causes the neck pain. (e.g. is it due to blood supply issues or pressure on vessels or something else?) Thanks for your help with my understanding of this.

Heather_Gornik,_MD: Neck pain in FMD is really a very common symptom (in the “top 5” in the Registry), but causes are really variable and not well understood. In some patients, dissection of the carotid or vertebral arteries causes neck pain, in others the neck pain seems to occur along with headaches. In some other patients there seems to be some neck pain or “carotidynia” that occurs just related to the FMD. And in others, the cause of neck pain is not headache related or FMD related at all --- some patients might have severe spinal arthritis or bulging disks.

Leg Pain

klt01: Is there any research on young, healthy, nonsmoker females, early 30s, with FMD in the Iliac arteries and is there any relief for symptoms in these arteries after bypass/angioplasty (I've had both)? Symptoms are still happening during exercise and walking in extreme cold, but at rest all my PVRs are perfect. I seem to be the exception to the rule with my doctors and we are at a loss of what to do next. I've gone from running marathons and road cycling to barely being able to run. I exercise through the pain but I know something is still wrong. Thanks.

Heather_Gornik,_MD: So sorry about your symptoms. Need to make sure this is really a vascular problem. Your PVRs at rest, but they really need to do an exercise PVR with a much more rigorous treadmill protocol that would be done, for example, for an 80 year old with leg pain walking 1/2 block. You should be checked for FMD and also for a related condition in young women athletes with claudication called external iliac endofibrosis. I would suggest seeing an experienced vascular surgeon at a big medical center.

Fatigue

wontgivein: As I have come to understand, there are many different physical presentations of FMD with varying symptoms reported. However, so many of us have generalized pain and fatigue that are not attributable to anything else. On occasions the pain and fatigue become more prominent. Is it possible that this has anything to do with episodes of the FMD in some sort of "flair-up"?

Heather_Gornik,_MD: I really don't think of FMD as 'flaring up' like an inflammatory condition or an infection. Acute symptoms can develop if there is a new dissection. I know some patients with FMD have generalized pain and fatigue --- it is really variable and seems to relate to prior dissections, severity of headaches, how bad the pulsatile tinnitus/swooshing may be, and other conditions. FMD really has many faces and there is a wide spectrum of clinical symptoms and severity. I had a lot FMD patients in my practice who really have no fatigue.... so again, it's case by case and please make sure we are not missing something other than FMD. I've picked up thyroid disease, cancers, anemia, arthritic conditions, depression, and many other medical conditions in my practice when patients were told "it's just your FMD acting up."

dgraybosch: I was diagnosed with FMD about three years ago after having a dissecting aneurysm, left internal carotid artery; I later had to have an angioplasty for stenosis (90%) of my right renal artery. I was fortunate and have very little residual damage. I take two antihypertensives. My question is that I fatigue so easily. Do other FMDers experience the same phenomena? Are there any modalities to help reduce the fatigue? What causes the fatigue if it is indeed from the FMD?

Heather_Gornik,_MD: Fatigue is something I see in some of my FMD patients, but I also see it in a lot of my other patients with vascular disease, so I don’t think it’s necessarily specific to FMD. Be sure other causes of fatigue have been worked up, such as anemia, thyroid, sleep apnea, and depression. Talk to your doctor about your medications, some of which can cause fatigue.

kariulrich: Fatigue seems to be a common symptom in many of us with FMD. It is like my body has limited energy and daily tasks become difficult without frequent rest or breaks. I have heard that some patients have been given Adderal to help combat the fatigue and they have found it extremely helpful and it has improved their quality of life. What are your thoughts with this approach?

Heather_Gornik,_MD: I would not recommend Adderall in general - it can have some vaso-constrictive active properties that can affect blood pressure. Chronic fatigue believe it or not is not a common symptom in most of my FMD patients.

Pam Mace RN: I am aware of many patients who complain of fatigue and require more sleep - if you are not aware of the INSPIRE support group - you may be interested in some of the posts. Please see our INSPIRE group at https://www.inspire.com/groups/fibromuscular-dysplasia-society-of-america.


FMD and Blood Pressure

GeorgiaNana: Thank you Dr. Gornik for all the information we are all receiving from this site. I am scheduled to see you on April 16th and I'm really looking forward to meeting you. My question is: My son, who is 42 years old, has had uncontrolled high blood pressure while on four different medications. Even though he is a male, and FMD mostly affects females, would it be wise for him to be tested by ultrasounds. His weight is normal and he does exercise, so the high blood pressure is a concern since I have FMD. Thank you for your taking the time to answer our questions. Carol

Heather_Gornik,_MD: Dear Carol. Look forward to meeting you. It is rare to have a mom and son with FMD --- I have a few sisters in my practice and a few moms/daughters, but I don’t think I have met a mom/son yet. Would be good to check any patient with high blood pressure at a young age or high blood pressure that is resistant to medications for FMD, but I think the likelihood of finding FMD in your son’s case is not terribly high. Still would have him evaluated by a vascular specialist or nephrologist, though.

Carolinablue: Do you commonly see renal FMD patients who have perfectly normal blood pressure without the help of meds? Thank you so much for taking the time to answer our questions!

Heather_Gornik,_MD: Absolutely. Having beading (FMD) in the renal arteries does not necessarily mean high blood pressure and if blood pressure is normal, no medication is needed! Sounds like your FMD was incidentally identified, such as during a test for another reason.

Cswann: Hi! My question is in regard to episodic blood pressure spikes and how to treat. I am currently on low dose aspirin and statin, had one VAD a year ago. Should I consider blood pressure med. and if so, can I take as needed or a dose each day. Blood pressure is ok most of time 125/75, but at night and when I'm tired I can feel my heart pulse from my heart to my brain. No tinnitus. Thanks!

Heather_Gornik,_MD: We do see labile blood pressure in some of our FMD patients and it can be a real challenge, but I fear I can't give specific recommendations without really reviewing your case.

camiswims99: What is the ideal blood pressure for an FMD patient to maintain? At what point do you like to see your patients on BP meds? Are there any natural alternatives to lower BP? I don't like taking beta- or calcium-channel blockers because my system feels so suppressed like it can't "get up and go" run, bike or swim.

Heather_Gornik,_MD: General guidelines < 140/90 mm Hg and ideally < 130/85 mm Hg. Low salt, high fruit/veggie diet (called DASH diet) is great for BP lowering. Avoid hidden salt sources like processed foods, soups, lunch meats, and even packaged breads. For renal FMD, we generally use ace inhibitors or angiotensin receptor blockers as first line agents. Good luck!

Pam Mace RN: Come join us as part of our running team in May in Cleveland at the Cleveland Marathon - we would love to have you!


Diagnostic Testing and Follow up for FMD

Jerrianne: How often should someone with FMD get their arteries checked for future problems?

Heather_Gornik,_MD: Great question. It depends on the vessels that are involved and whether there is just beading/narrowing or aneurysms/dissections. For most patients, we check “all” of the arteries from brain to pelvis once and then follow-up the vascular areas that are involved every 6-12 months. The program needs to be customized though to the individual patient.

scottie: Thank you for giving this opportunity today and for your work with FMD. I was told recently about PET-CT scanning which combines two scan techniques in one exam. I am aware of its uses in certain conditions and illnesses. Would this be a useful imaging tool in FMD? If so, how does it compare with MRA testing in terms of accuracy of results?

Heather_Gornik,_MD: In general Scottie, FMD is not a highly metabolically active process --- it is not an infection, cancer, or inflammation, so I don’t think this would be helpful.

Bruin: Hello. Can you please tell me if an ultrasound is a reliable diagnostic test for FMD? I'm a bit confused because one neurologist told me it isn't and another said it is. Thank you for clarifying this.

Heather_Gornik,_MD: Great question.. depends on the lab that does the test and the vessels being checked. For FMD in the carotid arteries in the neck, it does great in an experienced lab, but ultrasound will miss vertebral FMD or disease inside the brain even in the best labs.

Wendyll : What is the best way to Diagnose FMD CT or Duplex Ultrasound? Which one is more accurate?

Heather_Gornik,_MD: Great question. It really depends where FMD is being “looked for” and the local experience. At our center, our vascular lab does a great job assessing for renal artery FMD and carotid FMD, so I think I am confident in ultrasound for those types of FMD. But, for example, we don’t image the vertebral arteries well with ultrasound or the aorta and we can’t image the vessels inside the head with ultrasound to rule out aneurysms in the brain. For our patients who have definite FMD, we now recommend a one time check of the brain arteries with CTA or MRA and a one time check of the aorta and branches for aneurysm with CTA or MRA. Most of our follow up for renal and carotid FMD over time is done with ultrasound, and I still think ultrasound is a good place to start (in an experienced lab) to “screen” for renal or carotid FMD.

alekat: Is a high FIBRINOGEN (475) associated with FMD? What would this mean?

Heather_Gornik,_MD: The fibrinogen level is very minimally elevated... even a cold/viral syndrome can do this. I don't think this is related to FMD.

Curious: A lot of people are asking questions about imaging protocols for FMD patients. Can you explain?

Heather_Gornik,_MD: For our FMD patients, I generally make sure we've seen all of the arteries from head to thighs to rule out aneurysm and assess for involvement of the arteries. From then on, type of imaging and frequency of testing is determined by severity of symptoms. Some patients, for example, with new onset high blood pressure and renal FMD would be sent for an angiogram. Patients with just carotid FMD and pulsatile tinnitus and no other vessels involved and no aneurysms might just have an ultrasound once a year. There's no blanket recommendation --- program of imaging surveillance really needs to be customized to the individual patient by an experienced clinician in FMD.

camiswims99: Is there a particular protocol that should be followed for good ultrasound evaluation of FMD?

Heather_Gornik,_MD: See prior response.

Pam Mace RN: You can also review the scientific statement on FMD by AHA that was just published recently - it is the most up to date information available and the article is free. It can be found on the home page of our website and the Cleveland Clinic website. The link will provided at the end of this chat.


Renal Artery FMD

FMDSHANE: Dr. Gornik, recently many of the patients with renal FMD have been questioning whether or not hematuria is related to FMD? I also had painless hematuria (gross - it looked like a blood specimen) on and off for over seven years when I was in my late 30's. I was seen by urologists and a nephrologist and received no diagnosis (no infection. no kidney stones) except a rule out IgA Nephropathy or "thin wall membrane disease". I developed hypertension at that time as well but was not diagnosed with renal FMD until I was 66 years old. The nephrologist decided not to do an arteriography. He stated that it might have been related to exercise as I was running two or three miles a day. My kidney function studies were normal. He also declined to do a kidney biopsy to confirm a diagnosis. My right renal arteries had severe FMD when my first angiogram/angioplasty was done in 2005. Thank you so much for your support and many contributions to FMD patients! Barbara

Heather_Gornik,_MD: I don’t usually think of hematuria, especially gross hematuria, as being a manifestation of FMD, unless there is a renal artery dissection/infarction. Chronic hematuria related to FMD would be very uncommon, especially gross hematuria that you can see --- I would be very suspicious this is due to something else and encourage you to continue working with your urology team.

String of beads: I have two questions: I have bilateral FMD of the renal arteries diagnosed in 2004 by angiogram, however no hemodynamic measurements were done. I do have "hereditary" high cholesterol. I have been experiencing left mid back pain for about two years now, and I am curious if a more thorough angiogram should be done? (Especially since it has been ten yrs.). In November 2013, I had a sonogram that was very painful when the sonographer was looking at my left renal artery and my upper aorta, I have never experienced this much discomfort in the past with a sonogram and was very sore the day after. My provider ordered CT scan. After several calls to my provider, requesting my medical records, and months of waiting for my results, I was told that my celiac artery is dilated, does this mean this is an aneurysm? I have lost confidence in my provider due to a four month delay in getting my results. Thank you for your time.

Heather_Gornik,_MD: I am sorry you are having trouble. I generally would not do a renal angiogram for evaluation of back pain. Renal FMD usually does not cause back pain unless there has been a dissection or kidney infarct. I would generally start with non-invasive testing like at CTA, which was done. Mild dilatation of the celiac artery is very common and can be due to many things, one of which is a common physiological compression of the celiac artery by the diaphragm (called median arcuate ligament compression) --- hopefully this is not a true aneurysm.

Carolinablue: If an FMD patient has bilateral renal FMD but does not have high blood pressure, could this be because the beading or blockage is not severe enough to cause hypertension?

Heather_Gornik,_MD: Absolutely. And in fact, there are a lot of patients who have FMD, beading, in the renal arteries that are entirely asymptomatic and never develop high blood pressure! In those patients, we would definitely not recommend angioplasty or any other invasive treatment, just periodic follow up of blood pressure, kidney function, and periodic imaging of the renal arteries (such as a renal ultrasound).

Carolinablue: Is it very likely that someone with renal FMD without high blood pressure later develop hypertension? Since FMD is not thought to be progressive, could it be just as likely that their blood pressure remain normal?

Heather_Gornik,_MD: See answer to previous question. Absolutely. There have been some recent studies among healthy potential kidney transplant donors that up to 5% of healthy people without high blood pressure may have renal FMD!

fibaker62: Dr. Gornik, I have FMD in my kidney arteries. I had successful surgery in 1986 at Cleveland Clinic. I have one good kidney as a result of the disease. I have not been checked in my other arteries as for years we thought it was just in the kidneys. I am trying my hardest to get a doctor here in Houston to check my other arteries. It is like pulling teeth. They are simply not interested in helping me. I did have a bad reaction to the dye during a cat scan years ago on my sinuses. It was not allergic reaction, but my kidney was affected and I had a rash. They used the dye that is a little better for people with kidney problems. I had countless angiograms back in the late 1970's and 80's. I did not have any problems with the dye years ago. Is there some other was to see the arteries without the dye, and if so, is it available in other places besides Cleveland Clinic. I hope to one day go to the conference. My daughter is graduating from college that weekend.

Heather_Gornik,_MD: There are non-dye imaging studies to look at the blood vessels, especially ultrasound which can see the renal arteries pretty well depending on the lab. MRA studies use a different type of Dye. I wanted to mention there was just a state of the science paper published in the Journal Circulation last month on FMD. You may want to share this with your doctor. It now recommends one time brain to pelvis imaging of FMD patients.

Country girl: I am 59. While attempting to donate a kidney, it was discovered that I have FMD. I was immediately rejected but I know no more. I talked to my family doctor but she just brushed it off. I really don't think she knows much about it. Should I see a specialist in this field? Does FMD worsen with time? I've always had low blood pressure (BMI 20) but recently it is around 138/90. Could this be caused by the FMD? I've also recently developed ringing in my right ear. When I asked the doctor, once again she brushed it off. Can it occur in more than one area? Do I need to be concerned or just put my worries aside?

Heather_Gornik,_MD: Thanks for this question... it is very timely. There are many published case series which show up to ~ 6% of kidney donors may have FMD in the kidney artery(ies) on the prop testing. I would recommend you have your renal arteries assessed, especially if you now have HTN, and again we now do a one time check of the vessels from brain to pelvis to look for asymptomatic aneurysms that can be present in up to 20% of FMD patients according to the FMSDA funded US FMD patient Registry.

DebNsatx : I have another question regarding blood pressure meds, as I am currently on Toprol xl- 50mg twice a day, Norvasc-5mg once a day and have carotid FMD. I was checked approx. five years or more for renal FMD and I was fine but they advised it was a baseline for me. The vascular tech advised my kidneys were fine but they were on the lower end of normal at the time. Now, they put me on Diovan-80mg once a day, but I'm reluctant to take it because of the chance of renal FMD as I don't want to put a lot more pressure on my kidneys if I don't have to. My Physician Assistant said recently that my renin, bun and creatine levels were all OK but he couldn't speak about my vascular side for the kidneys. So my question is whether or not I should be concerned about taking this new blood pressure med or not? Thank You for your response!

Heather_Gornik,_MD: I am afraid it is hard for me to give you specific medical advice without evaluating your cases. If patients do have carotid FMD, we sometimes do recheck the renal arteries many years after they were first evaluated to be sure FMD has not developed, though in general this is not likely.

SunnyGirl: When there is decreased kidney perfusion in one kidney (6%) due to renal artery FMD in an otherwise healthy, active young women (20 yrs.) is it likely that kidney will improve with treatment (meds or procedures) or does kidney function stay the same? At what intervals is it recommended that function retested?

Heather_Gornik,_MD: It depends... sometimes if there is a renal artery blockage that can be opened it can restore function to the kidney, but other times the damage may be done, or sometimes the kidney damage/loss of function is not due to a vascular problem but other issues (e.g., chronic scarring from urological problems and infection).


Carotid Artery FMD and Dissection

FinnKa: How long do carotid dissections take to heal? When do you consider other options if they are not healing? Do the headaches and neck pain go away with time? When can you exercise again. I have been told to keep my heart rate at 110 or below. Doesn't take much to get the heart rate up. Will the full feeling and lightheadedness ever go away. I am not able to drive right now; so I am anxious to know when I will be able to drive. How distinguish serious symptoms of possible stroke from FMD symptoms? Have neck pain, headaches, and numbness in hands. Scans show no change.

Heather_Gornik,_MD: You have a lot of great questions in there, not sure if I can get to all of them. Healing of dissections is variable. In some cases the arteries heal within a few days or weeks, but in others, the artery can remain blocked long term or heal with a pseudoaneurysm or bulge in the vessel wall. Most dissections are managed with medical therapy, but sometimes interventional procedures are needed to re-open the artery or treat a pseudoaneurysm. I think it is important to be evaluated by a neurology specialist with experience in dissection management. Distinguishing stroke from FMD symptoms can sometimes be challenging. Many FMD patients have headaches and whooshing noise in the ears. Having true neurological ischemia symptoms like loss of vision or weakness on one side of the body or speech problems is very uncommon. What can sometimes be tricky though is that migraine headaches which many FMD patients have can sometimes present with symptoms that act like a mini stroke/stroke. When in doubt, for any neurological symptoms, would seek immediate medical attention as these could be signs of TIA/stroke.

Pam Mace, RN: I agree with Dr. Gornik and it might be helpful for you to read some of the stories on the FMDSA web site where patients share their experience. I also share my story on the website and I dissected three of my arteries. www.fmdsa.org.

FinnKa: Can you fly if the carotid dissections have not healed? Is fatigue common with the dissections?

Heather_Gornik,_MD: Check with your doctor first, but in general, most patients can do commercial airline travel once cleared by their doctor (I am assuming you don't mean piloting the plane). As for fatigue, I do think the dissections seem to be a risk factor for fatigue in FMD patients. I have some FMD patients after dissection who have really struggled with headaches, fatigue, and there was even a paper a patient actually alerted me to which showed a prevalence of post-traumatic stress like symptoms in patients with prior arterial dissections. However, I also know many dissection patients who have fully recovered and are doing great (... like Pam Mace to my left for example).

lenar1982: Hello! I’m 32 years old and lives in Israel. Week after birth (c/s) of my second child, had a severe headache after CTA and angio found out that I have dissections in both carotid and vertebral arteries, didn’t check my renal arteries yet (BP 140 systolic + tachycardia around 100). Not symptomatic except of one short episode of weakness in the left hand after beta blockers). No brain damage , etc. Now I’m only on Aspirin 300 mg, do you think i need stents or other intervention? What about the blood pressure? If it’s getting low (120). I feel very bad, weakness and afraid of weakness in my hand again. Thank you for your help. In Israel, all FMD is a mystery. The doctors don’t have a lot of information. Be glad for any help.

Heather_Gornik,_MD: I am so sorry to hear about your health issues. Unfortunately, I can’t make these recommendations without reviewing your case, examining you, and reviewing you imaging in detail. Cleveland Clinic does have a mechanism for electronic formal consultation called e-consult, and we can perhaps provide you details regarding this?

lenar1982: 1) Do memory problems have something to do with cerebral FMD? 2) Can there be improvement in the artery after an acute phase (after pregnancy)- few months later? 3) Does having low blood pressure dangerous for the brain perfusion in FMD of the carotid arteries?

Heather_Gornik,_MD: I don’t know of any data on memory issues and FMD, and I am fortunately not seeing dementia/major memory loss in our patients with FMD. Many patients with dissections do have significant improvement in their symptoms over time. In general lower blood pressure is a good thing, but some patients with very severe narrowing in the arteries can become more symptomatic with low blood pressure.

alekat: I have been diagnosed with FMD in both carotids. The doctor says I have type B? What would this be considered? I have a M1 chronic occlusion and they see a pattern of moyamoya? He made the comment that I may have the rarest of rare disease. Does this make sense?

Heather_Gornik,_MD: I have not heard of Type B nomenclature for FMD... not sure.

Ronni Herrick: I can't remember if I asked you this. Mom's neurologist said she has vascular dementia and I asked if it's FMD related and he said yes because she had more white matter than most people her age. Just wondering. Thanks and so sorry I can't listen in. See you in May.

Heather_Gornik,_MD: Not sure... need to see if there is FMD in the arteries and how severe. White matter changes on MRIs are "nonspecific" and not necessarily due to FMD. Older patients can also have FMD plus atherosclerotic disease plus effects of longstanding high blood pressure/diabetes and other things that affect the vasculature. See you in May... always a treat.

Jerrianne: My son's left carotid artery became 100% blocked due to FMD causing a massive stroke at the age of 19. How concerned should we be that the same thing will happen to his other carotid?

Heather_Gornik,_MD: I am so sorry to hear about your son. We would really need to see him and review his data to answer your question.

Pam Mace RN: I agree - I think your son should be evaluated by a specialty center such as Cleveland Clinic and if you are unable to travel, they are the only center I know that offers MyConsult online second opinion. Feel free to contact me - we do have many physicians throughout the US and outside the US and could possibly help you find someone locally.


Coronary FMD

kariulrich: Dr. Gornik, I had a cardiac echo stress test that showed inducible cardiac ischemia with near-global hypokinesis at high workloads. It is believed to be caused by chronic hypertension, I do have a small FMD web in one of my renal arteries. Unfortunately because of the bends in my renal arteries they were unable to continue with IVUS during my angiogram so I do not know the pressures. I was told the small web is of little concern. My question is, can hypokinesis of the heart be reversed if hypertension is controlled? How do physicians know that the hypokinesis is caused by hypertension vs. coronary FMD? Does hypokinesis cause symptoms such as angina and shortness of breath. I continue to have a multitude of symptoms despite the meds, including Nitro. I realize you may not be able to answer my questions on this format. I am surprised that in less than a decade my echocardiogram results could change so drastically. Thank you for your dedication to FMD. Kari Ulrich

Heather_Gornik,_MD: I have not seen this issue come up previously in our large FMD practice or in the published literature. I would definitely need to evaluate your special circumstances, clinical information individually to comment.

hunter16: I have bilateral carotid and vertebral FMD. In Nov., I went to ER for chest pressure, troponin levels elevated and had cardiac cath done. Was told I had NSTEMI with 80% blockage, DES stent placed and now on Plavix and low dose ASA. Went to see Dr. Olin in March who reviewed my cd of cath and he informed me that I had FMD of mid LAD, said I was doing good and to follow up with him in a year. My question is this, would the treatment have been the same if FMD was diagnosed at the time of the cath? I also have MVP with moderate to severe mitral regurgitation that I have to follow up with yearly ECHO.

Heather_Gornik,_MD: I’m afraid it’s not possible for me to answer this without reviewing you case and angio in detail. I will say for others on the chat that in the FMD Registry, heart attack is not common among FMD patients fortunately (< 5% in the 2012 Circulation publication on the first 447 patients).


Iliac and Femoral FMD

Linda-Lou: Hi Dr. Gornik and Pam and thanks again. In studies how many people have iliac and possibly femoral FMD and what symptoms do they present with. Depending on symptoms, how much diagnostics and/or procedures are done. Second question, have seen a few people with hypercoagulable states and is this related to FMD? Third, with SMA, where is the pain usually located? Thanks so much again. Sincerely, Linda

Heather_Gornik,_MD: Iliac FMD most commonly manifests as femoral bruits, but sometimes can present with leg claudication or acute limb ischemia due to dissection (very, very rare). I generally get ankle brachial indices before/after exercise for patients with FMD with femoral bruits or claudication and we image the external iliacs with CTA or ulrasound. As for hypercoag states, I don't think it's directly related to FMD, though few patients have both (Factor V Leiden for example if very common in the general population). FMD of the mesenteric arteries can be asymptomatic, can present with abdominal bruit, and less commonly can manifest as dissection or abdominal pain.


FMD and Median Arcuate Ligament Syndrome (MALS)

kariulrich: What is Cleveland Clinic recommending for treatment with FMD/Mals patients? I had my celiac plexus removed with my bypass and cannot find information if these nerves tend to grow back? I had three good years, now my symptoms have returned and are worse. My arteries are flowing, so I am wondering if scar tissue is playing a role. How is scar tissue found, is it only through surgery?

Heather_Gornik,_MD: Symptomatic median arcuate compression syndrome comes up in some of our FMD patients, but I also see this in patients without FMD. It's a tricky diagnosis to make because in many patients, the diaphragm and related structures and compress the celiac artery and not cause symptoms. I have not sent many FMD patients for MALS related procedures, but we do have a group here that has recently published on their experience with laparoscopic median arcuate ligament release in our surgical departments.


Genetics and FMD

andersm9: Recently I have been diagnosed of FMD through a carotid arteriography, in January 2014. I wonder if this disease - FMD- can be genetic or hereditary because I have three daughters, and if it would be progressive course, and what is the best treatment for my symptoms: headache and double vision. I would also like to know if there is any study that recruited patients for genetic studies, possible treatments and prognosis in USA or Spain.

Heather_Gornik,_MD: The genetics of FMD are currently not known... this is an active area of research interest in the field, and we are having a scientific meeting here next month where a group of interested genetics researchers will strategize on how to sort this out. In my practice, most FMD patients don't have an affected family member.

Pam Mace RN: As far as your treatment with headaches and double vision, I would make sure you are being followed by a neurologist and would also suggest being seen by a neuro-ophthalmologist to discuss treatment options. I am aware of some patients with severe headaches where botox treatments seem to be very helpful when other options have failed. As far as participating in a research study - Dr. Santhi Ganesh from University of Michigan has a study that anyone in the world can participate in. The information can be found at www.fmdsa.org under research. I agree with Dr. Gornik there are not a lot of families with multiple family members affected. That said, if your daughters would start to develop symptoms consistent with FMD, then they should be evaluated.


Types of Physicians for Treatment of FMD

WhatsFMD: I've been diagnosed with carotid FMD in both arteries and have had a stroke, some doctors (neurologist, vascular and cardiologist) say they have seen patients with FMD. Should I be seen by a FMD specialist?

Heather_Gornik,_MD: Like all rare diseases, you are best off seeing a doctor who has seen a lot of your condition. We follow > 400 patients with FMD here at this point. I have a totally different point of view on this disease than I did in 2005 when I first got started seeing a few FMD patients.

Pam Mace RN: I do recommend being seen by a FMD specialist at least once. I understand many patients do not have the ability to travel but think it is in your best interest as these physicians have a better understanding of the disease and how to treat it. They can also work with your local physicians.

Wendyll: Would it be a waste of time seeing a Vascular Surgeon for a Tortuous Carotid Artery that's 70% occluded due to Tortuous and string of bead in the other Carotid?

Heather_Gornik,_MD: Even if no intervention or surgery is to be done (which for carotid FMD is generally the case if you are not having TIAs or other neurological symptoms), it is important for FMD patients to be evaluated and followed by a doctor who is experienced with FMD. Medical therapy, screening for aneurysms, and surveillance are important. I might suggest looking into one of the clinical centers for the FMD patient registry, as these sites have experience with FMD, or finding a vascular surgeon or cerebrovascular neurologist in your area who is experienced with FMD if that is not possible.


QUESTIONS

FMD and Medications

Minzmom: What factors are considered when prescribing a daily 325 mg aspirin versus Plavix? Is the aspirin as effective for thinning the blood as Plavix? Thanks so much for addressing our questions!

Heather_Gornik,_MD: Unfortunately, there are zero clinical studies on the optimal antiplatelet agent for FMD patients. Most patients are on aspirin unless there are side effects or if there are recurrent symptoms on aspirin.

camiswims99: Does the benefit of anticoagulation (aspirin, clopidigril, coumadin or heparin) therapy outweigh the risk of bleeding with dissection or aneurysm rupture in patients with known FMD, dissection history and aneurysm?

Heather_Gornik,_MD: It's really case by case depending on location of dissection, thrombus presence, symptoms and timing. There is a randomized trial going on in Europe looking at anti-platelets vs. anticoagulation for dissection. Many patients are on anticoagulation just initially (e.g., first three months) then transitioned over to anti-platelets like aspirin/Plavix.

Pam Mace RN: These topics will be discussed at the FMDSA meeting by Dr. Stephanie DeBette who is leading research on dissections and also Dr. Hussain will be discussing treatment options for dissections and aneurysms.

EliC: Have many FMDers in your experience had problems with medications? If most of us are, is there anything to be done to get Food and Drug Administration to test meds on women as the majority of us are female? Why do we have to be used [at our own expense] to test drugs they have only tested on men and male mice? It seems bad enough to have a rare disease but thanks to Drs. Gornik, Kim, Olin, and now a number of others plus Pam Mace and other FMD staff it is getting better.

Heather_Gornik,_MD: I am afraid I don’t have any data to give you. There are no specific medication/drugs that are indicated specifically for FMD. It is mainly anti-platelet type blood thinning medications and medications for blood pressure control. I do agree we need more research on specific medications for FMD patients --- hopefully in the years to come there will be some, but we really need to have a better understanding of the mechanisms of FMD first.


FMD and Pregnancy

katzesoup: I'm 31 and was diagnosed with FMD in my carotids in 2012 after my baby was born. An MRI picked it up when I went in for stroke symptoms (was Bell's palsy). I'm missing a vertebral artery and have a small 2x3 mm aneurysm in my carotid artery outside the brain. I would love to have another baby someday and my neurologist thinks I'll be okay as long as I deliver via caesarean section and keep BP down. We're nervous considering I only have one vertebral artery now and haven't been scanned elsewhere in the body for FMD. BP has always been pretty low and blood work comes back fine and I'm an active swimmer. What should I be concerned about? Should I hassle my general practitioner to do more tests first? I have been having abdominal pain for about three years now but she thinks it might be IBS.

Heather_Gornik,_MD: Would definitely want you to be evaluated in an FMD center and also by a high risk OB before pregnancy is contemplated. This is important to get all of the information about risks of pregnancy and options before becoming pregnant.

Pam Mace RN: I agree with Dr. Gornik and have spoken to many patients contemplating pregnancy. Most are considered high risk and it may be helpful to review some of the patient stories on our website of successful pregnancies and what they experienced.

SunnyGirl: What are reproductive and lifestyle considerations for young women (20s) who are being medicated for hypertension vs. angioplasty or stent with renal FMD? Are the "ideal" candidates for each of these approaches?

Heather_Gornik,_MD: We generally try to avoid stents for the renals in FMD when possible. Stenting is less problematic in the carotids. Pregnancy risks with renal FMD are mainly related to HTN, but need to be sure with pre-pregnancy evaluation that there is no carotid FMD or brain aneurysms, which are much more of a challenge to manage.


MoyaMoya and FMD

alekat: Has MoyaMoya been associated with FMD

Heather_Gornik,_MD: For those on the chat not familiar... Moya Moya is severe narrowing of the arteries INSIDE the brain. This is uncommon in FMD, but we have seen this in a few cases and it is reported in the literature, especially in the non-string of beads type of FMD and in younger/pediatric patients.

Pam Mace RN: There is actually a story on the website of a child who was diagnosed with MoyaMoya and FMD that you may find informative.


Antiphospholipd Syndrome and FMD

DebNsatx : Thank You, Dr. Gornik and Pam Mace for doing these web chats as I have learned a lot about FMD from reading all the web chats. Also thank you both for educating the medical community and others about FMD. My question is whether or not you have ever seen patients that have both FMD of the carotids along with Antiphospholipid syndrome/Hughes syndrome or another name for it is Lupus Anticoagulant syndrome? I just know that others with FMD have had positive ANA's and I was wondering if there is any correlation that either of you know between the two diseases. Thanks Again! Deb

Heather_Gornik,_MD: I have a few patients who have + Anti-nuclear Antibodies (ANAs) and anti PL antibody syndrome, but this is a few in more than 400, and I don’t think they are directly linked. ANAs in particular are common among women, and of course FMD is a disease for which >90% of our patients are women.


Ehlers-Danlos and FMD

kariulrich: How many patients in the patient registry have been diagnosed with both Ehlers-Danlos and FMD? Do you know the percentage?

Heather_Gornik,_MD: Hi Kari. There is a field on the FMD Registry form which was added a few years ago which is collecting this data, but it has not been analyzed at this time. We previously published in Vascular Medicine Journal on testing for Col3A1/vascular EDS in our FMD patients (Poloskey, et al. 2012), but we did not have a single patient test positive.

Pam Mace RN: To my knowledge of the patients we have spoken with and have shared their medical history with me I am only aware of one patient who has both Ehlers-Danlos and FMD.


General Questions

camiswims99: How common is it to have both medial and intimal FMD? Does this ever occur in the same vascular bed? Is intimal FMD a progression of medial?

Heather_Gornik,_MD: Yes, you can see lesions that look like both in FMD. See the new AHA Scientific Statement. I think you might like the new classification system for FMD described in that paper.


Closing

Heather_Gornik,_MD: Thanks for the great questions everyone. Always lively. Please consider telling your health care providers about our FMD scientific meeting in May: www.clevelandclinic.org/fmdsymposium ...and see many of you at the FMSDA meeting.

Pam Mace, RN: Thank you for participating everyone. It is great to see so many of you being involved and being your own advocates. I hope to see many of you in May. See our website for more information about the meeting at www.fmdsa.org and the Health Hub at http://health.clevelandclinic.org/2014/04/new-fmd-guidelines-pave-the-way-for-more-research-video/

Reviewed: 04/14

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