Martha's Journal

I have a genetic medical condition called Ehlers-Danlos Syndrome, which is connective tissue disorder where the collagen is defective. This condition, along with some other medical conditions: dysautonomia, POTS, and vasovagal syndrome cause problems with my blood pressure, heart rate, dizziness, fainting, etc. I also have acid reflux. I am having trouble trying to figure out whether or not my aortic valve stenosis is causing symptoms. For years, I have been having problems while sleeping, with feeling like I am in the middle of a small earthquake. I figured out about a year ago, that earthquake is my heart--racing and pounding so hard my body is vibrating. This earthquake went away in April and returned again this week. The vasovagal symptoms returned 2 weeks ago. I believe all this, and more problems with my brain and brain stem, are because of a fall I had June 2. The symptoms that returned are due to my having major brain stem damage due to the compression of my brain and brain stem for 42 years. I had the decompression surgery for the Chiari malformation in January 2001. My first neurosurgeon also did 4 other procedures, including removing part of the base of my brain to make more room for my brain. My brain, brain stem, vagus nerve, and spinal cord in the neck had been very crowded for 42 years due to the deformity of the base of my skull and also in the vertebrates in my neck--which were also too small. Although my brain and brain stem had more room, there was still damage to the brain, brain stem, and spinal cord in the neck. In January 2002, I fell and broke the first two vertebrates in the neck, causing the skull to slip down, putting pressure on my brain stem and spinal cord. Needless to say, there has been quite a bit of damage to my brain stem, vagus nerve and spinal cord. I haven't had that many falls, but each one causes symptoms that had improved to return. My cardiologist told me March 9, that my aortic valve will need to be replaced. The valve is categorized as moderate. I have been to my vascular surgeon, who is very knowledgeable about the Ehlers-Danlos syndrome and the major problems it can cause with this valve and the surgery. I am going to see my electrophysiologist in September. Hoping the vascular physician and electrophysiologist will be able to help me sort out the symptoms to be able to tell which sympotms are from the valve (if there are symptoms now). I am gathering information about EDS and valve problems. EDS can cause valve problems. I have also been told my age is a factor as well. Have any of you been diagnosed with EDS and have had surgery by a surgeon who is extremely knowledgeable concerning doing surgery on an EDS patient? Stitching of any kind is done differently. I don't know what other differences in surgery there are for EDS patients. I am hoping there are some EDS valve patients who can share your experience and/or medical information with me. Thank you so much.

I have two heart murmurs since birth and was diagnosed in February 2010 with valve stenosis. Just found out in 2015 that it is my aortic valve. March 9, 2016, I found out it is now moderate and was told that it has to be replaced. I see my cardiologist again in November 2016. I also found out that I have TWO other valves going bad. I am noticing more problems with shortness of breath, unusual heart sensations, etc. I have other conditions that complicate the symptoms: Ehlers-Danlos syndrome, vasovagal syncope, dysautonomia, POTS, autonomic failure, fibromyalgia, Chiari malformation, and many other diagnoses. The first five I listed have similar symptoms as the aortic stenosis. I thought my worsening symptoms were due to the other conditions. On Tuesday, 07/19/16, I saw my vascular surgeon, who is monitoring a valve in my L leg that is not working properly. He is very knowledgeable about Ehlers-Danlos syndrome and he told me to see a geneticist again to find out what type of EDS I have, to try to rule out the vascular type, which makes the situation, possibly inoperable because my vessels would be too fragile. I will be scanned for an aortic aneurysm next week because any EDSer can have valve problems as well as aortic aneurysms and aortic dissections. I am also going to see the cardiologist/electrophysiologist about this. His office is about 2 1/2 hours away. I go to him for my dysautonomia/POTS conditions, that cause me to faint. After watching the main video Adam has done, I realize that time may be of the essence. I am gathering information and guidance from my vascular surgeon and electrophysiologist because of the Ehlers-Danlos syndrome--which can severely complicate the issue.