I have discovered a few things that really help the symptoms of POTS and thought it would be good to share for others who may not know about them, they are simple but seem to help me a great deal and anything that helps has to be good!! #1: Firstly and its my savior, I have to say ginger really is a lifesaver!! I get really bad nausea most days, so bad im rendered sobbing into a bucket off the edge of the bed and it really is a pitiful sight! However, the hubby did his research while I was having a bad …
I had a great day yesterday….except for the severe hemiplegic migraine attack. The aura of this kind of migraine mimics a stroke. Terrifying…and this one was horrible. Feeling a bit better now, thankfully. For several hours, about six or so, at a best guess, I was totally incapacitated. It is like being locked inside your body while an alien controlls it like a herky-jerky puppet…you are aware but there are blackout spots. You can hear but things are alien and confusing. You cannot respond…either can’t speak at all, or it comes out all slurry, and you can’t really control what you say. You babble and the words and thoughts don’t match. One side of the body is pretty much paralyzed and the other is uncoordinated and jerky like with huntington’s chorea patients. There is numbness and tingling, tongue feels totally bizarre, there are freaky visual symptoms…it is hard to describe the terror of being trapped helplessly in a state like that. You can’t even let anyone know or ask for help. I was unable to let my husband know what was happening to me until the aura receded most of the way.
During the aura, people can tell something is wrong, but they have no way to know how bad it is. Nothing you manage to utter can be trusted because your words are not lined up with what you think and feel in your mind. Also, my husband missed the worst part, when I was jerking about as if having a seizure, so he only knew I was ‘off’, which could be any number of more frequent things that affect me. So even with my family right there, I was alone in this other place, with no means of contact…and no one but me knew this. I have not had one (HM attack) this bad in a long time. This was just horrifying beyond words.
We wound up eating our Christmas dinner quite late because of me…I had to get past the worst of the aura before I was able to get up and sit at the table. It was really nice having our dinner in the newly renovated sun porch, though.
I am totally honored that Dulcé Menéndez painted my portrait…you can see it here on Flickr. Thank you, Didi…it is so awesome! I am used to painting other people, so being the subject of such a talented artist is just fabulous to me…so cool it is sub zero!
Visual Snow….read about this interesting and rare condition (yes, I have it). I just completed my interview with Dr. Schankin yesterday for the research study. I can’t discuss the actual interviewvirtu I can tell you it was very thorough and quite interesting. I am looking forward to the findings of the study. If you have VS and want to participate, check the research page on the site I linked and send an email letting them know you’d like to participate.
I also have visual hallucinations…this is whenhype see swirIng/moving colors and shapes in the darkness or with closed eyes. I am not sure if either of these conditions have any relation to my Hemiplegic Migraines (migraines that exactly mimic a stroke…scary aura!) or optical migraine, but to me that would make sense.
I have had a terrible time with ‘blushing’ all my life…at least, that is what I believed it was for most of my life. It was embarrassing because people so often thought I was embarrassed when I was not embarrassed at all! It got worse as I got older, finally getting so bad that it looked like the classic Lupus butterfly facial rash. So far, I have tested negative for Lupus. By ruling out other likely causes of the flushing (and other MCAD symptoms), I now have a presumed diagnosis; a definitive diagnosis would require a painful bone marrow biopsy, a measure which at this point seems unnecessary since I am responding well to the MCAD therapies I have tried thus far (antihistamines, H1 H2 blockers, mast cell stabilizers).
I will swallow my vanity and show you two ghastly looking photos of a recent MCAD flushing episode. I was actually even redder in person…the camera actually toned down the redness somewhat. I did not adjust the images in any way. The pictured episode was triggered by activity and allergens. It lasted about six or seven hours.
MCAD (Mast Cell Activation Disease) flushing feels rather like a combination of sunburn and windburn, a sort of stinging sensation. I can feel a flushing episode before it becomes visible and also for a little while after it visibly fades. An episode can last a half hour or so for a very mild one to several days for a major one. During bad episodes, the reddened area is slightly swollen/raised.
I tend to get such severe flushing that it has shocked several medical professionals. I say this because one does not have to look like these photos in order to have MCAD. It seems as though I am a medicinal overachiever of sorts, a habit I would really like to be rid of! Unluckily for me, it seems every medical thing with me has to be some kind of really extreme version of whatever it is. Many things are severe to the point of ridiculousness. But I digress.
MCAD is fairly rare, but more common with POTS and EDS patients such as myself. Though not curable, it is at least treatable. I believe it is likely the primary culprit of my voice loss issues, as well.
This is a great article to share with family and friends to help them understand what it is like to live with Dysautonomia
HELPING YOUR LOVED ONES UNDERSTAND DYSAUTONOMIA
What is Dysautonomia/POTS:
Dysautonomia is a malfunctioning of the Autonomic Nervous System, or the unconscious bodily functions – your heart rate, digestion, breathing, blood pressure, digestion, heartbeat, bladder function & narrowing or widening of the blood vessels, body temperature, sweating, bowel function & sleep patterns. Dysautonomia can also be referred to as Autonomic Neuropathy. Think of it this way – Dysautonomia can interrupt the inner workings of the human body. A patient with Dysautonomia is most often bedridden, because when they stand up, their brain forgets to send the correct signals to the heart to pump blood to the brain. This results in a significant drop in blood pressure upon standing, along with an increase in heart rate, because the patient’s heart must try to beat overtime to get blood to the brain so the patient doesn’t faint. Testing revealed that for me, when I stand up, my heart rate typically reaches between 120 & 145 beats per minute, without even having taken a few steps yet. This is often a target heart rate for someone that has been running on a treadmill for over 25 minutes. This is a hallmark characteristic of the condition.
The autonomic nervous system, also known as the involuntary nervous system, regulates those facets in the body that occur automatically, such as breathing, It is composed of two branches – the parasympathetic nervous system & the sympathetic nervous system.
Sympathetic – The sympathetic nervous system is also known as our stress response system, or the fight or flight system, & it is set into motion when we experience stress. It increases our heart rate & blood pressure, dilates pupils, restricts circulation, slows down digestion, relaxes the bladder, makes us more alert & aware & provides a boost in energy so that we are capable of dealing with the stressful situation effectively. It increases energy & is often referred to as the accelerator of the autonomic nervous system.
Parasympathetic – The job of the parasympathetic nervous system is the exact opposite. Once the stressful event is over, it brings the heart rate & blood pressure back to normal, constricts pupils, improves circulation, enhances digestion, calms us down, contracts the bladder & puts us into a state of rest & relaxation. It conserves energy & is often referred to as the breaks of the autonomic nervous system.
What Causes Dysautonomia or Autonomic Nervous System Dysfunction?
When the autonomic nervous system is functioning as it should, the sympathetic nervous system & the parasympathetic nervous system work in perfect harmony together to maintain balance in the body. The sympathetic nervous system provides us with the tools we need to respond to stress adequately & the parasympathetic nervous system restores us to our normal state of peace & tranquility.
Dysautonomia, or autonomic nervous system dysfunction, occurs when these two systems fail to work together in harmony. The most common scenario is the sympathetic nervous system remains dominant most of the time & the parasympathetic never turns on. When this occurs, then the body remains in a state of fight or flight at all times. The stress response system never turns off. If the body remains in a state of fight or flight at all times, then many degenerative processes begin to happen & result in a variety of chronic health conditions & overall poor health like those in our list above, because it is only supposed to be used for brief emergencies.
The stress response system was designed to deal with brief emergencies that threaten survival. It isn’t supposed to last very long because the body cannot sustain itself for very long in this state. The natural & preferred state of the mind & body is the parasympathetic state, because it is regenerative. However, it is willing to forgo its preferred parasympathetic state to deal with acute emergencies & will remain in that state if the emergency continues. If the brain & the body remain in the sympathetic fight or flight state for too long & too often, it is degenerative; it breaks us down. If this cycle continues, then eventually the system burns out. It is this cycle that results in Dysautonomia or autonomic nervous system dysfunction.
Okay, calling all POTSies and Zebras out there…this post is a sort of plea for opinions and advice. First, you might want to peek at my last post and listen to the BlipMe link (recording of my voice so you can hear how raspy and hoarse my voice gets). I am not sure what to do about this combination of factors:
Frequent loss of voice or extreme hoarseness
Intermittent trouble swallowing, even my own saliva; aspirating things
Asthma seems worse (same symptoms as VCD)
MCAD (Mast Cell Activation Disease) worse
I know that is vague, but I wanted to quickly hit the high points. Now I will ‘splain some more, or try. It is very difficult to tell the difference between VCD (Vocal Chord Disorder) and asthma. The symptoms list is pretty much identical. MCAD is basically an overload of mast cells in the body that release a crap load of histamines, so it gives you allergic type reactions, often affecting the skin. I am wondering if the MCAD is affecting the tissue in my throat and airway. It feels sort of damaged, which is mostly from what the doc said was (borderline pneumonia) severe bronchitis/sinusitis that began like over three months ago. Did the z-pack of antibiotics. Doing the Albuteral inhaler, which helps a little bit, but not nearly enough. The thing that has actually helped the most is NasalCrom spray, which is a mast cell stabilizer and prevents my MCAD caused constant drippy nose, and actually does help my throat a bit. There is a point for MCAD.
What has been happening is I will have a dry coughing jag, and on exhale, it feels like my throat and airway/trachea is sticking to itself, and I can only suck in hard won tiny gasps of air…it all slaps shut between each bitty gasp of air. I guess I need to find out whether a rescue inhaler will help this? I have never had to use one.
Because of my fatigue, I need to keep my outings to a minimum, and I don’t want to waste time going to a half dozen different doctors who don’t know what to do with me (yes, that has happened a LOT). So any thoughts or advice on approach, what kind of doctor would be best to see…having EDS (connective tissue disorder) complicates things a bit.
If anyone has been through this, I am interested in your experiences. I should add that I have been losing my voice and having trouble swallowing for a good 10-15 years, but it has gotten increasingly worse over the past several years. So it is this thing I have lived with so long that it doesn’t seem like any sort of urgent thing. It is the airway closing up thing that is freaking me out. I am not sure if I have a floppy trachea and throat because of EDS? My throat has always had issues…recurring strep and tonsillitis, had the tonsils removed at age 14. I dunno…just really frustrated with all this. It seems like if it were simple asthma, the inhaler would be doing more for me.
I have this recurring problem with losing my voice. It has been happening for a good decade or so, getting worse in recent years. Typically, it will happen with exertion, in the afternoon and evening, and when I speak often. Mostly, it is just obnoxious, I suppose. I also have difficulty swallowing and have worsening asthma…whether these are related, I am not certain. It could be an aspect of my MCAD (mast cell activation disease), the CFS/ME, and/one EDS. In all likelihood, it is an overlapping thing. Wanna hear how I often sound?
This is how I roll…I am cleaning my weapons, readying my survival gear…putting on my best Sarah Connor attitude…so don’t look for self pity in this daunting read. This is war.
I am struggling pretty hard at the moment, and I’ve been bashed over the head with some daunting realizations. This is it. It is ALWAYS going to be a roller coaster, and I am not ever going to achieve some stable result, some place with a nice view from which I can enjoy the grandeur of achievement. This is it. This is how I roll, now, for better or worse. And even this is subject to change.
I do not say this to be depressing or exhibit self pity. I feel disappointment and frustration, but very little self pity. I say this to be pragmatic. I say this because, in order to continue my fight, I have to assess and the odds and the lay of the battlefield. I have to know my enemy, and what my enemy wants from me. Without this knowledge, it is that much harder (not to mention highly unlikely) to kick the ass of my enemy…or should I say enemies. After all. I am blessed with more than one incurable rare POS condition. POS is piece of $#*+, by the way, not some new medical acronym.
I came to these nasty realizations after being knocked yet again back to square one, bottom of the tall mountain, looking up dismally at the altitude I must find a way to attain once again. And I may not get there. I might, I might not. But I am gonna fight like a bushido warrior to get there. Again. And again. Rinse and repeat. Therein lies the realization rub; it will continue to be this kind of battle. There is not going to be a victory point at which my enemies surrender and sign the peace treaty. This is my own personal never ending war on terror. It is a battle that has no ending; no defined rules of engagement. And it will screw with me every way it can.
I am oddly calmed by this…knowledge. I was tempted to say acceptance, but I suck at acceptance, and I just don’t see a reason to change now. I don’t want to accept it; I will instead fight back, get creative, look for ways to beat the unbeatable, James Tiberius Kirk-style. Eff acceptance. That’s for sheeple.
Just because I am oddly calmed does not diminish my dismay, my frustration, or my “holy crap, this sucks balls” realization; a realization I would very much like to trade in for some other. I can’t do that…at least not yet.
All this comes in the wake of a severe battle with (borderline pneumonia) bronchitis and sinusitis, which I am not entirely sure I can quite get rid of. The cough lingers…three months later. I feel changes inside my airway, trachea, throat…there is tissue damage, which I hope will heal. My asthma is in overdrive. That ticks me off, because I need to start training again, and this is going to be a bitch of an ascent. I have to literally climb the mountain I live on…start hiking again.
My battle begins lying in bed, barely able to sit up many days. I have been here before, minus the effects of the bronchitis and worsened asthma. I know how hard the beginning is; now daunting; how physically miserable. But I WILL do this. Again. And whether I reach the summit, itself, it WILL reach a personal summit. Maybe, just maybe, I will exceed all my hopes…hope is a fabulous four letter word. But in the meantime, it is gonna be pretty excruciating (mentally, physically, emotionally). So I am readying for this battle. I am the 300. I will push through the misery. Again. And I will prevail.
The part that sucks most is really understanding that this cycle IS my reality. I don’t get to do it one time, two times, three…(I have already done it far in excess of that…a dozen times, perhaps). I get to keep doing this, probably for the rest of my life.
So why bother? Because I CAN. Because I want a life, and to feel strong, able, fit, battle ready…even if it is a part time gig. Part beats none, yo? And no way am I rolling over in some ditch and letting a bunch of cruel enemies chortle over a badly won victory. Nuh uh. Not this girl. Not on your life, or mine.
I do think it sucks monkey crotch that I have to keep doing this. Especially the initial part (that really miserable beginning) of each new battle. I don’t want to go trough this crap over and over. I really don’t. I’d honestly rather eat dirty worms. But what I do want is what I have experienced on the other side of each fight. I am not willing to give that part up, just because it sucks REEEEAAAALLLYYY bad getting there. So there you have it.
Rinse. Repeat.
oh, and when I start whining and moaning like a total sissy, please link me back here…tell me to STFU and re-read this, and get off my self pitying ass and start climbing, okay? I’d really appreciate that!
As you probably know by now, painting is a huge part of my therapy and art is my only means of income these days. I’ve been doing portrait art again, as I once did in the French Quarter at Jackson Square, in New Orleans just about twenty years ago. This painting, still in progress, is the beautiful daughter of a friend.
Bella
If you are interested in having a portrait done from a photograph, please get in touch. I also paint animals.
Rachel Phillips was a ballet dancer who performed with the Royal Ballet in London, the Kirov in St. Petersburg, Russia and other major companies in the US and abroad. Her world came to an abrupt halt when she began to have complications from a rare connective tissue disorder. With her airways now over 90% collapsed, her only option is to regrow her trachea using her own stem cells.
This life-saving therapy is not yet approved to be performed in the United States, nor will it be covered by Rachel’s health insurance. In addition to the fight of their lives, Rachel and her husband Steven face mountains of medical and other related expenses. They must raise the funds necessary, not only for this life-saving treatment, but also to travel overseas and stay there through Rachel’s recovery, care for Rachel’s service dog Siena, and so on.
The clock is ticking. We need your help NOW in bringing awareness of Rachel’s life-threatening situation to a wider audience for the purpose of knocking down regulatory barriers standing in her way and to raise the funds necessary to pay for this necessary treatment.
I would like for you to please watch this video as it explains exactly what Rachel is facing and experiencing in her own words……http://www.youtube.com/watch?v=waCOXBkVNbw&feature=share
Many many efforts are in the works to help Rachel get to Europe;
The website with the most information (including all efforts and past media coverage) is www.helprachelbreathe.com
A store featuring the artwork of Dar WcWizard and pictures of Rachel has been created and is open for business @ www.zazzle.com/helprachelbreathe.com
A store @ cafe press with computer art work by Michaela Oteri, Angel Oteri and myself at www.cafepress.com/zebrasneedzebras
A FB page supporting Rachel was also recently created @ https://www.facebook.com/groups/198660840207194/
Rachel Phillips also has a CAUSE page on FB to help with donations and to share her story.
Cliff Phillips (Rachel’s father-in-law) raised $5,000 to help with the cause by shaving his head.
Efforts continue world wide amongst the ‘zebra’ (EDS) community, as members of the herd offer creative fundraising, moral support, and spread the word far and wide. You can be part of these efforts!
As an admirer, friend and fellow zebra, I implore you to help Rachel in any way possible…even spreading this message is a huge help!
If your have questions or would like to help in any way, please get in touch with me. I have all of the contact numbers for Steven….however I would ask that you first contact me so that I may relay the information to Rachel and Steven Phillips since Steven is now Rachel’s caretaker as she waits at home to make her trip overseas to have her life saving surgery.
I thank you for taking the time to read this…please explore some of the links below, as well. There is a message in Rachel’s own words, information about the procedure she needs in order to stay alive, information about Ehlers-Danlos Syndrome, and beautiful video clips of Rachel dancing before her illness took away her breath.
Wikipedia has a clear definition of Ehlers-Danlos Syndrome in Layman’s words @- http://en.wikipedia.org/wiki/Ehlers-Danlos_syndrome
(KHQA/Quincy, Il to run a two part series October 28th & 29th once it airs it will be added to the websites)
Rachel in her own words:
http://www.youtube.com/watch?feature=player_embedded&v=waCOXBkVNbw (rachel talking with traceostomy)
http://www.youtube.com/watch?v=hyXUvSgzyr4&feature=player_embedded (This is a short clip of one of Rachel’s coughing jags that last for hours… and it isn’t as bad as they get)
MEDICAL VIDEOS
http://www.youtube.com/watch?v=SehlJen0xIs&feature=player_embedded(Film of bronchoscopy showing collapse of the trachea, both main bronchi, and lower airways. It was done in late October, five weeks after Rachel’s tracheobronchoplasty.)
http://www.youtube.com/watch?v=HH0vas1wFq8&feature=player_embedded (Bronchoscopy 11-22-2010 showing metal airway (tracheal) stent encased in cemented secretions. Acetylcysteine (Mucomyst) was used in an attempt to loosen the secretions.)
http://www.youtube.com/watch?v=TI-xaA6CP2Q&feature=player_embedded (Bronchoscopy 11-22-2010 showing metal airway (tracheal) stent encased in cemented secretions. Acetylcysteine (Mucomyst) was used in an attempt to loosen the secretions.
http://www.youtube.com/watch?feature=player_embedded&v=252UbpvMDZA (Bronchoscopy to look at stent treating tracheobronchomalacia. Mucomyst and Pulmozyme were used to break up secretions, but shortly after this, Rachel was rushed to her airway specialist to get it removed as the granulation tissue was growing in way too much.)
http://www.youtube.com/watch?v=M4Li9Rr56Oc&feature=player_embedded (Bronchoscopy showing metal tracheal stent with granulation tissue at the distal end. This requires it to come out as soon as possible and they will have to debride the tissue in order to remove the stent.)
http://www.youtube.com/watch?v=1rejUgAF6Mw&feature=player_embedded (Bronch to check vocal chords and the healing progress of granulation tissue in the airway
This is about living with Ehlers Danlos Syndrome, Dysautonomia (Postural Orthostatic Tachycardia Syndrome, Neurocardiogenic Syncope), spine disorders and a host of other gripes; this blog is about coping, healing, observations, rants, discoveries, creative adaptations, and living life on my own terms despite chronic illness/disability.
Rachel Phillips
Grand Jete Photo
Rachel and her service dog Siena
Rachel and her beautiful smile
