Cathlin'story My BP has always had a tendency to increase when at the doctor’s office. It’s for this reason that I monitor my BP at home. I noticed that it was raised, varying between 160/100 and 145/95 (previously I was told over 140/80 was too high) so I took an appointment with my family doctor. My BP was 190/110 in her office. I was started on anti-hypertensive medication and I was referred to a cardiologist for a cardiac assessment. My BP was 185/110 in the cardiologist’s office, the nurse kept saying “it’s 185/110” which of course was why I was there! He explained it could be due to an uncommon, but possible cause of hypertension in younger women (I was 47 years old – so not that young!). I would need a kidney scan. He prescribed a beta blocker on top of the antihypertensive. I googled causes of hypertension in “younger” women and found fibromuscular dysplasia. Thanks to the FMDSA and the INSPRE site I was able to find information on the disease. The first thing I realized was how very different it can be for everyone depending on which arteries are involved. It was also clear that those within the FMD world were saying it is really important to be seen by a physician with FMD experience. Once my renal scan results where back, the cardiologist called to tell me I did in fact have a very important stenosis in my right renal artery. Which was suggestive of fibromuscular dysplasia, but that my kidney was a normal size which indicated no damage. He told me that either I would need to stay on the antihypertensive medications or they may consider a small procedure to try to dilate the narrowing. He wanted me to see a nephrologist. The nephrologist showed me the scan, one stenosis of the right renal artery (indicating a focal lesion) despite this the kidney was within normal size. He felt it would be best to refer me to an FMD experienced colleague in Brussels. He explained that they would usually check the carotid and vertebral arteries also – so I had an echo Doppler of those. I had searched and read as much as could find about renal artery FMD. Not being medically trained some of the published articles were heavy going! Following my appointment with the specialist, I had a scan of my carotid and vertebral arteries. It felt like a very long 3 weeks waiting for the results, every headache or twinge in my neck convinced me I would have it in other arteries. Luckily though, there was no further FMD detected. It was suggested I undergo a PTA (Percutaneous Transluminal Angioplasty) to improve the blood supply to the kidney. I had already read that in some cases (and more often in focal FMD or recent onset of hypertension) this could improve BP and for some it could mean normal BP without medication. It would be a 2-3 day stay in hospital. In November 2015 I had the angioplasty, the next day I was allowed home. I now had an antiplatelet and aspirin added to my medications. There was a bit of confusion as to whether I’d had a stent or not once back on the ward, as they were more used to cardiac procedures. I was sure there was no stent, it had been awkward and it was talked about during the procedure, but wasn’t needed, which I was relieved about as I remembered reading it wasn’t recommended in renal arteries unless there was a dissection. It was fascinating to be aware of what was going on during the angioplasty, but also a bit surreal if I’m honest! I chose not to have any medication to relax me beforehand. The next morning I was able to go home – when I asked about the BP medication, I was told to continue with it and if any problems contact my doctor. I had a few days taking things very slowly, my BP was lower, but not drastically, however every time I stood up or moved I felt dizzy – very dizzy. If I moved very slowly getting up was a bit better, so I had to try moving about slowly. My BP continued to drop and I was getting very cold fingers and toes, with numbness and pins and needles. My family doctor advised we halve the ace inhibitor meds to see if that helped. It did slightly. The circulation in my hands and feet improved a bit. She explained that it can take a while for your body to adjust to having a lower BP. This was not something I’d thought about prior to the procedure. After my first follow up appointment at the hospital I was able to stop more of the medication, and following a home 24 BP hour monitoring I was able to stop the remaining BP medication. I underestimated how long it would take for my body to adjust to the change in blood pressure and stopping the medications, and honestly it took almost 6 months until I felt really “back to normal” and up to exercising regularly again! Following my latest appointment, my blood pressure is still ok without medication. Follow up appointments will continue yearly, as for other FMD patients, and if I’m lucky that will be my FMD story. I keep my fingers crossed that my renal artery stenosis stays away, extra crossed if I’m honest as focal FMD does seem to have a higher risk of progression. I see from others experiences I have been fortunate, no dissections or aneurysms, and for now, no other artery involvement. The hardest thing is living with the fact that I have FMD and have no idea what that may involve in the future, but with the yearly follow up appointments we will hopefully get advance warning of any possible changes. I also realise I was lucky to have had a good experience with the health service here, something that is not always the case. I have seen and heard of others having to wait years for a confirmed diagnosis, and even when one is given the treating physicians may have little or no previous experience of FMD. When I was asked to be included in the European FMD registry I was very happy to have my details and DNA added, anything I can do to further the knowledge and subsequent treatments of this “rare/underdiagnosed” condition the better.
Véronique'story. My clinical story began in 2007. I was 48 years old, with 3 grown up children and a position of physician in a medical school . At the beginning of January I began to suffer with extreme anxiety and depression. I was unable to sleep, often ended the day in tears, was exhausted and irritable. A general medical examination failed to highlight any physical problems. I went on to consult a psychiatrist who diagnosed anxiety and depression as a consequence of my stressful professional role. I was prescribed a short period of rest and some sessions of supportive psychotherapy. In 2009 I realized my blood pressure was regularly reaching 200/100 – 210/110. I consulted a internist who after a full examination and 24hr BP monitoring (ABPM) concluded all was normal and the raises in BP must be due to stress. At this time I accepted the findings and got on with life – work, family and therapy. I continued to have raises in my BP, and I was exhausted. Despite more medical visits no answers were found other than work related stress. By 2011 it was impossible for me to continue to work, I was totally at the end of my tether both physically and mentally. It was interfering with every aspect of my life and I took the difficult decision to leave my job at the university. By 2012, I had consulted a hypertension specialist. He gave me two diagnoses’ that would explain the raises in BP. Fibromuscular Dysplasia in the renal arteries accompanied with multiple visceral aneurysms and chronic glomerulonephritis (proteinuria of nearly 2grs/24h and a microscopic haematuria with altered RBC’s) Treatment of Candesartan EG was started and things improved steadily. The spikes in my BP subsided and the renal abnormalities corrected themselves. The severe fatigue I had suffered began to lift. By 2014 I felt well enough to be able to volunteer for an humanitarian organization. Since 2014, my medical follow-up include : - A daily medication for hypertension ; - A annual check-up (1) of the kidney function and (2) of the aneuryms in my abdominal arteries (angio-CT or angio-RMN) Now, things are going well ! The hardest thing is living with the fact that I have FMD and have no real idea what will happen in the future… but it’s the life and I am optimistic. It’s very important for me to discuss with other FMD patients : I find that the sharing of experience is a important way to minimize the fear of unknown future. Furthermore, the fact that I have a medical doctor who knows well FMD and is open to listen to me and my questions help me very much.
Paola'story My FMD story started in Rome, where I was paying my parents a visit in October 2017. Well, it probably started a long time before that, but I was living in blissful ignorance until then. One morning, I felt a bit fuzzy-headed. My chest, jaw, and upper arms felt tight. But I put it down to recent stress and a lot of long-distance travelling. And sure enough, half an hour later the tightness was gone. A few days later I was back in Accra, Ghana, where I was living at the time. I had gone to bed early one evening, when a startlingly familiar feeling swept over me: tight chest, tight jaw, tight upper arms. And once again, I thought it was probably due to a lot of tiring travelling. This time the tightness lasted for about 45 minutes. I decided that I would go to our local clinic and get myself checked the following morning. I am surprised that no alarm bells rang: I had twice over the years followed Red Cross first aid courses. I think I assumed that because I swim regularly, practice a lot of yoga, have a healthy diet and have never smoked, I was immune to heart problems. However, I had been taking medication for hypertension for about fifteen years, and my high blood pressure had not really been under control for the last few years. The next morning I felt tired, and a little queasy, but not worryingly so. I went to the clinic, and had an ECG and a blood test. The ECG showed nothing untoward, but the blood test showed that huge amounts of troponins (enzymes I had never heard of) had been released into my blood, indicating damage to the heart. The doctor decided I should be taken by ambulance to Korle-Bo Hospital to have further checks. At Korle-Bo I had another ECG and a blood test, as well as a heart scan. The blood test confirmed high levels of troponins, and I was given blood thinners, statins and anticoagulants. The cardiologist I saw said I needed a coronography. It was decided that I should be taken to Brussels, in case any complicated follow-up became necessary. I spent one night at the hospital, while arrangements were made for my trip, and the next night I was on a plane to Brussels. During the flight I was given Nitrolingual spray as I had chest pains. From Zaventem Airport I was whisked off by ambulance into A&E at St Luc where my blood pressure was checked every fifteen minutes for about six hours. Then I was moved into intensive care, where my BP was measured every hour. Blood samples were taken every six hours. The following day I had a coronography, which revealed that I have fibromuscular dysplasia, a condition I had never heard of, in my two renal arteries. I was told that the chest pain episodes were spontaneous coronary artery dissections, or SCADs – another term which was new to me. I learnt that there is a link between FMD and SCAD. The next day I was discharged, with a prescription for statins, cardioaspirin, blood pressure tablets and betablockers, and a recommendation to take things easy, and measure my blood pressure regularly. A month later I had my follow-up visit. My blood pressure was still high, so the cardiologist changed my medication. He told me that due to my age (I am 64), an angioplasty would be very unlikely to help. Since then I have had another scan of my arteries, which showed that I have an aneurism in my spleen, which is not of too much concern at the moment, but will be monitored. I had a MAPA (I was connected to a blood pressure machine for 24 hours, just doing the things I would normally be doing), which showed that my BP was still a bit on the high side, so my medication was slightly increased. I will have another MAPA in a few months. Seven months after my SCADs, I have left Accra and am living in Brussels. How do I feel? I am grateful for my speedy and efficient medical evacuation, and for the care I received both in Accra and in Brussels. Happy and reassured to have met Cathlin and Véronique, from the Belgian FMD support group. Relieved to be alive and well. Emotionally much more settled. With each day, the fear of a recurrence fades. I am less anxious and tired than before my diagnosis. Physically I feel better than I have in years: I sleep well, practice a lot of yoga, and walk and cycle regularly. I realize now that over the years I had spiraled into a state of chronic stress and fatigue, and now I see that this is absolutely not necessary: it’s quite possible to live life in a simpler, more relaxed way, and it’s a lot more fun!