Monday, May 23, 2016

The Website The Mighty Is Helping Me Explain A Little Bit About What Having EDS Feels Like

NO, This is not all in your head!!!  If you have or think that you might have Ehlers Danlos Syndrome then don't let those around you who are not inside your body and not feeling what your body is feeling tell you that the things going on with you are not real. Of course no one else can be inside your body and feel what you feel which is the point that I am trying to make to hopefully help you feel like you are not going crazy. So many things hurt and are out of balance and wack or whatever you want to call it when you have EDS and it seems unreal to those who are not suffering with it as a matter of fact, it seems unreal to those of us who are suffering it. I often find myself saying that if I lived on a planet full of people who had EDS that I would be completely normal but since I don't I have to look at things with different eyes than the average person. Knowing this, on some days, is more than half of the battle of just getting me through the day, and sometimes just through the hour.  Having others like myself to depend on helps in lots of ways and being able to describe how living with EDS feels so that others can know what I'm going through really really helps me feel part of a community these days. I often don't feel part of my regular community anymore and feeling isolated is once of the first steps to depression for me.

I was looking for something to help me explain to others and I found a great site called The Mighty its a great place to find support and information about being disabled and finding others who are dealing with some of the same issues that you do if you were to find yourself on that list.

Here is a post they have that has helped me feel better about myself and my situation.

29 People With Ehlers-Danlos Syndrome Explain What It Feels Like

Ehlers-Danlos syndrome (EDS) is a group of connective tissue gene disorders, and symptoms include skin that tears or bruises easily and unstable joints prone to frequent dislocations, among other issues.

EDS affects somewhere between 1 in 2,500 to 1 in 5,000 people in the United States, but understanding of the disorder tends to be limited among society and medical professionals. Some individuals with EDS remark that their doctors don’t even know how to spell it, and the most common analogy likens the body of someone with EDS to that of a house built with faulty materials.

“Our EDS community formed out of a need to understand ourselves even when medical professionals did not,” a spokesperson for the Ehlers-Danlos National Foundation told The Mighty. “Awareness about EDS leads to better lives. Although EDS is not curable, early diagnosis can limit long-term damage as problems can be treated as they arise, and sharing information in our communities about what has worked for each of us can help all of us.”

We teamed up with the Ehlers-Danlos National Foundation to ask their Facebook community how they would describe the disorder to someone who doesn’t have it.

Here’s what they had to say:

1. “It feels like I’m 80 when I’m 40.” —Kimberly A. Bates

2. “It feels like having the flu all the time.” —Linnie Lin

3. “I feel like an alien on a planet where I don’t belong.” —Yolanda Smith

4. “It feels like I’m slowly disintegrating into particles…” —Sarah-Marie Zeraphic-McFarlane

5. “It feels like my body is falling apart at every joint.” —Breanna Griggs-Meloy

6. “It’s like a big pair of knickers with no elastic left.” —Christine Di Ciacca

7. “It’s like having the flu, a hangover and getting hit by a bus all at the same time.” —Irene Beck

8. “I feel like a marionette and someone else is in charge of the way I move.” —Nicole Hess

9. “It feels like your body is trying to turn itself inside-out, piece by piece.” —Aria Eragon

10. “It feels like I’m made of rubber bands that are about to snap.” —Katie Thomson

11. “It feels like you’ve lifted a car off someone all day every day.”—Melissa Conder

12. “It’s like an old house, creaking, squeaking swaying, and popping in the night, the wooden walls and nails coming apart at the slightest breeze.” —London Elaine Ridenour

13. “It’s like riding a bicycle with very loose bolts. You have to hold it together yourself or else it will fall apart.” —Melissa Drennan

14. “EDS is living the day after a car accident in perpetuity.” —Sabrina Winchester

15. “The pain is like sand paper being wiped on the inside of your skin all the time.” —Tiffani Rinzel

16. “You cannot trust your body to do what it is supposed to do.” —Emma Stathopoulos

17. “It’s like trying to build a tower out of misshapen blocks, where the tower is your body and the blocks are faulty collagen.” —Courtney Simonds

18. “The hair on my skin hurts.” —Mary Carlson

19. “You feel like you are constantly disappointing people because you have to cancel plans at the last minute when you are in too much pain, too sick, or too depressed to leave your house.” —Lisa Allison

20. “A 3 on a pain scale of 1-10 is a good day.” —Laurie Bohanan

21. “I feel like I’m falling apart at the seams.” —Lisa Sinnott

22. “EDS is feeling ‘insane’ for years because people tell you there is nothing physically wrong when youknow there is.” —Sarah Elizabeth Erwin Bloom

23. “You have to deal with the idea that your body is basically falling apart and there’s nothing you can do to stop it.” —Erin Geerlof

24. “EDS is the hypochondriac’s disease — always being questioned about whether or not you’re really in pain, or if everything wrong with you is really connected or not.“ —Sage Schultz

25. “Dislocations are like walking on glass, not knowing when you’re going to get cut but you know it’s inevitably going to happen. It sparks an unnatural fear that is hard to live with.” —Ariel Amberg

26. “The cruelest symptom of our illness is disbelief by medical personnel.” —Mary Carlson

27. “It’s like falling down the up escalator indefinitely while bystanders speculate about how you got there, what your injuries might be, and if you really even look hurt.” —Jess Elsen

28. “It’s like having everything in your body short circuit.” —EmJ Jackle-Hugh

29. “Day to day I feel like a stretchy rubber doll that’s working hard to stand up straight; bad days feel like I’ve been hit by a car, but the good days make me feel like I can still conquer the world!” —Beverly Wilson

Some answers have been edited for brevity and clarity.

Elisabeth Brentano

What If No One Had Ever Heard Of Cancer And Would You Treat Me With Concern If I Were Pushing Around An IV Pole?

Can you think back to a time when no one knew what cancer was, me either because as long as I've been alive awareness of cancer and a search for a cure has been a focus of the public? But can you imagine if your loved ones were sick with cancer and you had never heard of cancer, would you be afraid of them, would you abandon them, would you be frustrated with them and tell them it's all in their head, would you tell them they'll have to do things on their own and would you not pray for them? Of course not and the reason is because we have all heard of cancer and we know what it can do to a body.

People raise awareness for illnesses that don't have a cure so why don't all the illnesses in that category get awareness shoutouts? I happen to have four illnesses that have no cure or treatment. This is the case with patients that have Ehlers-Danlos Syndrome, it causes other illnesses that have no cure or treatment so dealing with everyday life becomes impossible at best on many days, however; we have some type of strength inside of us that we just keep on taking each day as it comes.

I have often been told that people don't understand what is wrong with me because they know I have something that they have never heard of before and because people are afraid of the unknown often times questions are not asked for better understanding. I have often also been told that if I were bald and pushing around an IV Pole that people would be bringing me potluck dinners and praying for me. Well, that's the truth as I have seen it first hand with family and friends who have had to fight through cancer.  Guess what?  I do push around and IV pole 18hrs a week while I get IV fluids to help my body think that it has more blood, since I have up to 15-20% less blood than the average person. Tricking my body into thinking that it has more blood help my heart not have to work so hard to pump blood through my body that basically flows backward because of EDS which has caused a condition called Postural Orthostatic Tachycardia Syndrome. I also get IV's three days a week because EDS and POTS cause me to be chronically dehydrated and the fluids only help me for about 24-36 hours before I need more. IV fluids also help me deal with Mast Cell Activation Disorder so I am able to put a band aide on three different problems that have not other treatment although the IV's are not really a treatment since treatment is something that will cause the problem to go away. 

Getting IV's puts me in an infusion room where I see all kinds of illness from infection to liver and kidney failure and cancer and myself with only one other person that I have met in three years with the same conditions as myself. I have seen 100's of people come and go through the infusion room in these past few years and I have made and lost many friends to cancer and other illnesses during this time. I by no way want to belittle the impact that cancer has on a person or a family. I have learned through these past few years something about cancer compared to all the other illnesses I have seen come through the infusion room. 

A few lessons that I have learned: 
1. Cancer has no age restriction, 
2. The majority of the cancer patients that I have met have never dealt with a lot of illness and lots of them are experiencing illness for the first time in their lives, 
3. For the most part, a diagnosis of cancer lets you know at the beginning of your treatment what your possible outcome will be and how well or not you might handle that treatment, 
4. People hear the C word and they come running out of the woodworks to help you out, cleaning for the patient or family, cooking for them, driving them to treatment, sitting with them through treatment, bringing them lunch at treatment and all sorts of things that are needed when you are stuck at the hospital getting treatments, I rarely ever see someone with cancer coming into the treatment room completely alone, 
5. Friends and family post all over their facebook pages to please pray for the patient and please spread the word to help to find a cure for cancer, 
6. Pastors sometimes come to the infusion room to pray with their patients, 
7. Nurses in hospital settings are more sympathetic and empathetic to patients with cancer than other illness that brings people into the infusion rooms, 
8. Cancer patients can have really bad days during their treatments where they feel just their worst, but so do the other patients in the infusion room, 
9. Cancer patients who have not dealt with illness have a hard time dealing with their cancer because they don't know what it's like to have their lives interrupted with doctors appointments and infusions and accepting people helping them with everyday life, 
10. Everyone in the infusion room wants to talk about their illness and once the cancer patient has shared about their illness they then ask me about mine and when I explain just the EDS part and not even getting to the 25+ other illnesses that I have because that's what EDS gives you,  they begin to feel better about their journey and they ask who helps me with life and I respond no one, then they are really encouraged to see me helping myself in the infusion room to the restroom, getting my own lunch and blankets and that fact that I am still smiling and telling jokes, they almost always respond by saying that if I can do what I do then they can make it through what they are going through. 

As much as I hate that anyone has to go to the infusion room for anything, I am glad that I can encourage others and I'm also glad that EDS patients are really good at hiding what is going on inside physically and emotionally. If it weren't for that strength or maybe sometimes it's a weakness, my gig would be up and I would not be encouraging anyone. 
The person with Ehlers-Danlos has lived their whole lives being strong and has multiple things wrong with them, not just one diagnosis, and the EDS patient is very apt at accepting help, if it is ever offered which doesn't happen much in the EDS world at all because people don't understand the pain that comes with it and the fatigue, and the EDS patient lives their lives around doctor's appointment schedules and does so alone, driving themselves and coming home so exhausted, not only from chronic fatigue but also chronic pain that never leaves, that they will often have to go straight to bed without the laundry getting done, the trash being taking out, or a meal prepared for them but yet EDSers get right back up the next day and do it all over again and again learning how to ignore what needs to be ignored, (those things that drive a person crazy like a messy room) and pay attention to the important things like how to walk across the room and not dislocate something.  

Many will say that at this point I am just complaining and to you it may seem so. To me, I am just stating the facts and telling the truth. I have learned a lesson in this as well, many people cannot handle hearing the truth so instead of saying something simple like I'm uncomfortable hearing about how hard your life is" or "I'm scared for you and don't know how to help you", they cut you off and tell you to get it together and stop complaining...... oh sorry, just a jogged memory there....   If you happen to be one that doesn't understand chronic illness then this may seem harsh of me to say or you do just think I am complaining then I would suggest that you need to take a long look at your day to day life and pay attention to the things that irritate you or that you get upset by in your life and imagine yourself going through your day with one foot and a hand tied behind your back and see how much you can still do and look at yourself in the mirror at the end of the day and see if you have a smile to offer to someone or an encouraging word. A life with Ehlers-Danlos Syndrome is not for the faint of heart or the weak in spirit. If nothing else then let those of us who have reasons to be patients in an infusion room encourage you to not take yourselves so seriously.   

 I have been posting a lot of things about Ehlers-Danlos syndrome this week and that's because it's Ehlers-Danlos awareness month. I'm doing this obviously to raise awareness because it is a rare disease that many people have never heard of, including doctors. I am not posting things about Ehlers-Danlos syndrome for someone to feel sorry for me but rather I want to raise my voice that Ehlers-Danlos is real and it changes your life and it can rob you of your future. I don't like to ask for help and I get embarrassed having so many illnesses. I come from a family of overcomers and you just don't let illness keep you from things, but that is impossible with EDS and so I am admitting that I am not an overcomer when it comes to EDS, and I do have to ask for help. So I am asking, could you please share this blog or the video in this blog or even just the name EHLERS DANLOS SYNDROME with someone in your life today to help spread awareness about this illness that destroys lives, it kills, it robs and it tears families and friends apart. It's not the type of illness that gets it's victims help and support and acceptance and world wide prayer chains started, and kindness, and home health care and disability coverage and national campaigns and so the list goes one but it is the type of illness that needs all of these things and much much more.
This woman's story is the story of all people with Ehlers-Danlos syndrome.

Most people with Ehlers-Danlos syndrome do have heart defects like she does, I myself have a hole in my heart along with four other heart defects.

Most people with Ehlers-Danlos syndrome have to give up activities and social events, I myself have to sit out many times when my friends gather.

Most people with Ehlers-Danlos syndrome cannot do what they used to anymore, I myself can no longer play the piano or play my saxophone as it hurts my hands and arms and back too much to sit with my instruments, this is very sad to me as my music was a way to express my feelings and to worship God with the talents he gave me, I myself can no longer hold a book to read, it hurts my hands they cramp and sometimes my wrists dislocate or a finger will dislocate while holding the book. My favorite book of all I can no longer hold which is my Bible so I don't carry it to church with me when I feel well enough to attend, I take an electronic Bible.

Most people with Ehlers-Danlos syndrome are not believed, I myself was told that it's just growing pains you will get over it or everybody hurts now and then, or it is impossible for someone to hurt that much, no one understands the pain that we are really in.

Most people with Ehlers-Danlos syndrome go to many doctors and spend a large portion of their life never knowing what's wrong with them, I myself see over 19 doctors and that's because all the doctors specialize in just one thing, and I was not diagnosed until I was 44 years old. Once you receive the diagnosis of Ehlers-Danlos syndrome you have to have every body part checked, every organ checked, because our collagen is weak, it's possible to die from what would be a simple problem for most people if it's not taken care of. Once diagnosed you also find out if there are many other diseases that you have because of Ehlers-Danlos syndrome.

Most people with Ehlers-Danlos syndrome are very sick even though they look just fine, I myself am very sick and some days I cannot even take care of my basic needs although I look perfectly healthy. But if you really pay attention you can see the fatigue on my face, my red cheeks from poor blood circulation, or a fever, the straining, and caution with my movements.

Most people with Ehlers-Danlos syndrome have to go to the emergency room often, I myself sometimes have to go several times a month and then there are times when I might not go for a month or two. For us, it is very frustrating to go to the ER because many of the doctors that you see in the ER don't know what Ehlers-Danlos is and you are treated as if you are a freak or someone just looking for attention.

Most people with Ehlers-Danlos syndrome are often abandoned by their friends and forgotten about, I myself have some relationships that have suffered because very few of my friends have researched or ask me what Ehlers-Danlos is. Because I don't have a disease that people know about others are uncomfortable talking about it, they don't know what to expect from my disease or how to support me. I can think of many ways to help me.

Most people with Ehlers-Danlos syndrome are afraid of the future, I myself am afraid that I will be left alone since I have no husband or children. I myself am afraid of how I'm going to take care of myself, I'm afraid that I will lose the ability to walk as I have already lost the ability to cook and clean like I want to.

Most people with Ehlers-Danlos syndrome have anxiety and depression, I myself have lots of depression and I often feel suicidal because I am afraid of my future and my pain on most days is just too much to handle, and I have lots of anxiety about what's going to happen to me and also if what I'm doing is going to dislocated joints and cause more pain. Sometimes I have anxiety about going to the doctor's appointments alone and understanding everything they tell me if they're even going to know what's wrong with me in the first place.

Most people with Ehlers-Danlos syndrome have chronic fatigue, I myself have chronic fatigue, there are days when I cannot wake up no matter what, and then there are days that I'm so exhausted that I cannot go to sleep because my pain is too great to rest.

Most people with Ehlers-Danlos syndrome just need someone to understand, I myself need someone to understand that I cannot stop thinking about Ehlers-Danlos syndrome as it affects every single second of my life no matter what I'm doing I am in constant pain, there is never a break from pain, I need someone to understand that I need to sometimes talk about what is going on with me and my body, and that I need to talk about my fears and my concerns, and I need someone to understand that I'm not being negative when I'm depressed and I'm not being negative when I talk about my health issues. I need someone to understand that even though my voice sounds happy or my voice sounds like I feel good that I really don't feel good, but I can still feel happy and not feel good. I need someone to understand that I can't change my disease or how I'm feeling by suggestions that are made to better my health, I have tried lots and lots of things and believe me if those things helped I would be doing them. I need someone to understand that I don't need to be fixed but instead I need someone to say I'm sorry this is happening to you and I'll pray for you.

Sunday, May 15, 2016

If You Have Ehlers Danlos Syndrome You Will Totally Get It, If You Don't Have EDS Check Out This Article To See If You Can Find An Answer To Your Chronic Illness Here.

Blogging Blogging Blogging! How important it is and how much I'm preaching to myself here. I need to be more disciplined about sending out information on Ehlers Danlos Syndrome so that others may find people in their lives, or themselves, or maybe even medical professionals may find some of their patients in these pages. Well, that is a big goal that I have set for myself since I hardly ever feel liking blogging. I don't like to blog when I don't feel well because I know I won't be at my best but, thank goodness there are others with EDS that blog as well. Together we all share the need to send a bright shining light into the medical community that EDS is alive and well and missed when all of the MANY illness that it causes shows up in the offices of doctors.

I ran across a blog post from who had a great article about what EDS looks like.

Here is a list of some signs of EDS and some of the things that EDS causes in the EDS patient. I'm going to highlight the ones that I deal with just so you can see some of what I deal with on a daily basis. This is not a complete list by any means but hopefully it will give you the reality of why finding out information about EDS is so important for those who suffer with it.

 Other signs of Ehlers-Danlos include:
-Easily visible veins, especially on thighs, chest, or abdomen.
-“Transluscent” skin that can be described as especially soft, or “velvety.”
-Skin that bruises easily
-Blue sclera - that is when the whites of your eyes have a blue tent to them.   Yes, these are my eyes...... They are blue sometimes and green at other times. You can see the blue sclera in the eye on the left side of the picture, just in the outer edge.

The two pictures below I know are gross looking, I was feeling bad this day so the blood vessels in my eyes were bright red which I have noticed happens when I don't feel well. The blue sclera can be clearly seen when I turn my eyes all the way to the outside of my eye socket.  Here is the right eye                                   and here is the left eye. 

-Antimongoloid slant - this is a downward slanting of outside corner of the eyes.   My eyes are almost half moon shaped. 

-Pectus Excavatum - also known as sunken or funnel chest
-Flat feet
-Scoliosis (congenital or developed) - for now mine is mild in my lower back
-Marfanoid Habitus- a constellation of symptoms resembling those of Marfan syndrome, including long limbs, with an arm span that exceeds the height of the individual, and a crowded oral maxilla, sometimes with a high arch in the palate, arachnodactyly - condition in which the fingers and toes are abnormally long and slender, in comparison to the palm , and hyperlaxity- In such conditions as Ehlers Danlos or Marfan's syndrome, the connective tissue, which is the basic building block for ligaments, joint capsule and tendons, is abnormal.
-Mitral Valve Prolapse (though the especial frequency of this in EDS has been disputed in recent literature)
-Myopia - A condition in which close objects appear clearly, but far ones don't.This is not just a condition of getting older, and at times for me I can't see anything close up or far away without it all being very blurry.
-Abnormal orthodontics (severe overcrowding, losing baby teeth at late age.)
-Temporomandibular Joint Disorder
-Loss of proprioception - The unconscious perception of movement and spatial orientation arising from stimuli within the body itself. In humans, these stimuli are detected by nerves within the body itself, as well as by the semicircular canals of the inner ear. The American Heritage® Science Dictionary.  This one is tough for me because I am always running into things and misjudging where my arms and legs are and when I am standing up I have trouble knowing where my feet are on the floor. I often just kind of tip over or stumble as I walk and stand. I've gotten really good at catching myself from falling. 

Conditions that Frequently Co-exist or are caused by Ehlers Danlos:
Adrenal Insufficiency - a condition in which the adrenal glands do not produce adequate amounts of steroid hormones, primarily cortisol
Anxiety Disorders and Panic Attacks (result of Dysautonomia and the dysfunction of the para/sympathetic nervous system, and/or Adrenal Insufficiency)
Autoimmune Disorders (Esp. Thyroid related)
Carpal Tunnel Syndrome

Celiac Disease
Chiari Malformation -A condition in which brain tissue extends into the spinal canal that can cause problems with balance and coordination. It happens in people with EDS because the fluid from the brain doesn't drain properly so there is extra pressure on the brain forcing it downward so that the cerebellum rests on the spinal column. Chiari can happen in people without a visual sign of the cerebellum resting on the spinal column which is called Chiari Zero.
Chronic Cerebrospinal Venous Insufficiency - a condition where people have obstructed blood flow in the veins that drain the central nervous system (the brain and spinal cord). This can cause Chiari in EDS patients. Research indicates that CCSVI is significantly correlated with multiple sclerosis. As a result of these venous abnormalities, the blood flow rate through the central nervous system back toward the heart may become slowed, and blood may reflux back toward the brain and spine.
Chronic Fatigue (and Immune Deficiency) Syndrome
Chronic regional pain syndrome
Chron’s disease
Craniocervical Instability or Craniovertebral instability
Dysatuonimia - a malfunction of the Autonomic Nervous System
Early onset herniated disks or spinal degeneration
Fibromyalgia Syndrome
Fixed dystonia - abnormal posture even at rest
Gastroparesis (delayed gastric emptying)
Headache associated with cerebrospinal fluid leakage
Heart Murmur
Hiatus hernia
Immunological Deficiencies
Insufficient REM sleep (related to insomnia, breathing difficulties, etc.)
Irratible Bowel Syndrome

Kyphosis- especially of cervical Spine
Mast Cell Activation Syndrome - a condition with signs and symptoms involving the skin, gastrointestinal, cardiovascular, respiratory, and neurologic systems. Earlier proposed criteria for the diagnosis of MCAS included episodic symptoms consistent with mast cell mediator release affecting two or more organ systems with urticaria, angioedema, flushing, nausea, vomiting, diarrhea, abdominal cramping, hypotensive syncope or near syncope, tachycardia, wheezing, conjunctival injection, pruritus, and nasal stuffiness. This is kind of like being allergic to anything and everything but not everytime nor do you know when that time may be or not. 
Mitral Valve Prolapse
Multiple Sclerosis - highly suspected but not yet confirmed for me. 
Osteoporosis (or osteopenia)
Postural Orthostatic Tachycardia Syndrome (POTS)- 
a condition in which a change from the supine position to an upright position causes an abnormally large increase in heart rate, called tachycardia. Other symptoms of an orthostatic nature — occurring in response to upright posture — may accompany the tachycardia. EDS patients, or at least how it was explained to me about myself, do not retain fluid they take in. I was told that the cells in my body, because of the EDS are the wrong shape to hold in salt which causes me to not retain fluid so therefore, I am always dehydrated and I will always be dehydrated no matter how much water I drink. I have ended the week of getting IV's and ended up in the ER and tested for dehydration and was show to be dehydrated. It happens everytime I get my blood checked. The saline IV's help me feel a little better for about 24-36 hours but after that I feel myself sinking so to speak and my mood changes, not for the better and I become more dizzy and tired. Patients with POTS are known to have up to 20% less blood volume than the normal person causing the heart to overwork to compensate. POTS is very tiring to the body and is compared to using as much energy as it would take to run on a treadmill 24 hours a day 7 days a week non-stop or like someone who has congestive heart failure. For the EDS patient once you have POTS you will have it for life. There is no cure for POTS although there are some instances a person can develop POTS and then they will cease to have it. The only help for POTS is to try and control the symptoms, which you never know when they will hit, with saline IV's and certain heart medications that may slow the symptoms. I spend 18 hrs a week at the hospital getting saline IV's to help my heart not have to work so hard which below you will read about my heart and how important that is for me. Saline IV's are also said to help support patients with Mast Cell Activation Disorder.
Scoliosis, usually congenital
Temporomandibular Joint Disorder

Tethered Cord Syndrome
Vitamin Deficiencies (malabsorption) (Esp. B, and D)  

These were not on the list that I found on the blog post below but I included them because the are all caused from EDS. The heart stuff gets pretty scary if you ask me.
Arterial Fibrillation
PFO grade III - A patent foramen ovale (PFO) is a persistent, usually flap-like opening
Tricuspid Regurgitation - a disorder in which the heart's tricuspidvalve does not close properly, causing blood to flow backward (leak) into the right upper heart chamber (atrium) when the right lower heart chamber (ventricle) contracts
Mitral Regurgitation-leakage of blood backward through the mitral valve each time the left ventricle contracts. A leaking mitral valve allows blood to flow in two directions during the contraction.
ASD -Atrial Septal Defect - a hole in the heart
ASA - Atrial Septal Aneurysm - a rare but well recognized and localized saccular deformity of the atrial septum that bulges into the right or left atrium with uncertain clinical significance.

This sounds like a lot doesn't it? Well, ask anyone with Ehlers Danlos and they will tell you that this just scratches the surface. Having so many things wrong with our bodies is because this is a collagen disorder and since the human body is made up of about 80% collagen, when you make faulty collagen it messes with every single system of the human body. Another reason that I blog about EDS is because I want my friends to understand that when I say I don't feel good, I don't mean that I'm a little tired or that my stomach hurts, I mean at least 90% of my body doesn't feel good at any given moment. 

There are some great facts and info in the blog chronicilnessproblems and you can look for yourself there or someone you may know who seems to be chronically ill but yet never finds an answer to what is causing the problems.

Giving all the credit to Chronicilnessproblems here is the blog post copied and pasted here.

Thinking You Might Have Ehlers-Danlos Syndrome?

Unlike many heritable connective tissue disorders (HCTD,) there isn’t a genetic test for most types of EDS. There is a test for Vascular type, but it isn’t 100% accurate, and there is a test for Classic, but it’s only about 50% accurate. So the diagnosis for Ehlers-Danlos Syndrome is generally made by the Beighton, Brigton, and sometimes the Villefranches criteria, and by history and family history questioning done by a geneticist.

Decades ago, Ehlers-Danlos was estimated to affect 1 in 10,000 people, for the most common type. Now, it is estimated that Ehlers-Danlos Hypermobility Type is at least twice that common, and that still three-quarters of cases go undiagnosed, due to poor education and awareness about the Syndrome. Many doctors who do diagnose EDS fail to recognize the less common types, and some with the other types are misdiagnosed as Hypermobility type.

Beighton and Brighton Criteria for EDS Hypermobility Type:

The Beighton Scale:
The Beighton scale is scored from 1-9, and measures hypermobility. As is pictured, you get one point for each: pinky that can bend back past 90 degrees, each thumb that can touch your wrist, each elbow that bends backwards, knee that bends backwards, and one point if you can press the palms of your hands to the floor without bending your knees.
(0-2 points = not significantly hypermobile, 3-4 points = moderately hypermobile, 5-9 points = distinctly hypermobile)
A score of 4 is generally considered hypermobile in practice. Many people can do one to a few of these, which isn’t abnormal, but 4 will be classified as hypermobile.

The Brighton criteria:
Major Criteria-
-A Beighton score of 4/9 or greater (either currently or historically.)
-Arthralgia (joint pain) for longer than 3 months in 4 or more joints.

Minor Criteria-
-A Beighton score of 1, 2 or 3. (0, 1, 2, or 3 if aged 50+)
-Arthralgia for more than three months in one to three joints or back pain for more than three months, and/or spondylosis/spondylolysis/spondylolisthesis
-Dislocation or subluxation in more than one joint, or in one joint more than once
-soft tissue rheumatism. greater than 3 soft tissue lesions (bursitis, etc.)
-Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactyly (positive Steinberg/wrist signs)
-Abnormal skin, striae, hyper extensibility, thin skin, papyraceous scarring.
-Eye signs: drooping eyelids or myopia or antimongoloid slant
-Varicose veins or hernia or uterine/rectal prolapse

Ehlers-Danlos (or Joint Hypermobility Syndrome) is diagnosed in the presence of:
-Two major criteria OR
-One major and two minor criteria OR
-Four minor criteria OR
-Two minor criteria IF a first-degree relative is unequivocally affected

This is what doctors and researchers use to classify Ehlers-Danlos Syndrome. They will also, especially in types other than Type 3- Hypermobility type, look at family history of hypermobility of sudden death due to organ or vessel rupture, at scoliosis, presence of skin fragility or elastic skin, organ fragility, or related conditions.

The Beighton and Brighton generally are used to diagnose the Hypermobility type, and in 1997, a group of experts met in Villefranche and developed the Villefranche criteria. This criteria also includes important aspects for the other types of Ehlers Danlos.


Other signs of Ehlers-Danlos include:
-Easily visible veins, especially on thighs, chest, or abdomen.
-“Transluscent” skin that can be described as especially soft, or “velvety.”
-Skin that bruises easily
-Blue sclera
-Antimongoloid slant
-Pectus Excavatum
-Flat feet
-Scoliosis (congenital or developed)
-Marfanoid Habitus
-Mitral Valve Prolapse (though the especial frequency of this in EDS has been disputed in recent literature)
-Abnormal orthodontics (severe overcrowding, losing baby teeth at late age.)
-Temporomandibular Joint Disorder
-Loss of proprioception

Conditions that Frequently Co-exist or are caused by Ehlers Danlos:
Adrenal Insufficiency
Anxiety Disorders and Panic Attacks (result of Dysautonomia and the dysfunction of the para/sympathetic nervous system, and/or Adrenal Insufficiency)
Autoimmune Disorders (Esp. Thyroid related)
Carpal Tunnel Syndrome
Celiac Disease
Chiari Malformation
Chronic Cerebrospinal Venous Insufficiency
Chronic Fatigue (and Immune Deficiency) Syndrome
Chronic regional pain syndrome
Chron’s disease
Craniocervical Instability or Craniovertebral instability
Early onset herniated disks or spinal degeneration
Fibromyalgia Syndrome
Fixed dystonia
Gastroparesis (delayed gastric emptying)
Headache associated with cerebrospinal fluid leakage
Heart Murmur
Hiatus hernia
Immunological Deficiencies
Insufficient REM sleep (related to insomnia, breathing difficulties, etc.)
Irratible Bowel Syndrome
Kyphosis- especially of cervical Spine
Mast Cell Activation Syndrome
Mitral Valve Prolapse
Multiple Sclerosis
Osteoporosis (or osteopenia)
Postural Orthostatic Tachycardia Syndrome
Scoliosis, usually congenital
Temporomandibular Joint Disorder
Tethered Cord Syndrome
Vitamin Deficiencies (malabsorption) (Esp. B, and D)

If these criteria fit you, you may have Ehlers-Danlos Syndrome. Even if you’re close to the criteria and seeking a diagnosis, or if you have a family history of similar issues, it’s definitely worth looking into and being evaluated by a geneticist, because if not EDS, there are similar HCTDs. Geneticists are generally the best doctors to see for EDS, but rheumatologists can also be experienced in treating and diagnosing Ehlers-Danlos.

The Ehlers-Danlos National Foundation- EDNF- has a list of specialists by area here.
The biggest names are Dr. Clair Francomano and Dr. Brad Tinkle, and also in the Maryland area the Johns Hopkins doctors are good- Dr. Howard Levy is great, and Dr. Dietz is also good, especially for Vascular type, though his specialty is other HCTD, (Like the one he helped name, Loyez-Dietz Syndrome.)
Even a doctor who isn’t considered an “expert” can help diagnose Ehlers-Danlos and start the treatment process, but always be sure to do your own research and don’t believe everything a single doctor says.
(For example that EDS is “just a label” or that EDS “can’t really be treated anyway.”)

Research can be found through EDNF, NCBI Pubmed Studies (and other scientific websites like Journal of the American Osteopathic Assiciation,) and Pretty Ill, and any medical websites. Ones aimed at medical students or published medical studies and journals are better for information than discussion boards, but discussion boards can be helpful in their way, especially for psychosocial type support from the more positive ones.

Lastly, there is the question over differences between EDS Hypermobility type, Joint Hypermobility Syndrome, and Benign Joint Hypermobility Syndrome.
Benign JHS versus non-benign JHS is basically just asymptomatic (benign) versus Symptomatic. The only diagnostic criteria for Benign Hypermobility Syndrome is the Beighton Score of 4 or higher, but without any joint pain (arthralgia.)
There is much recent debate in literature and research over whether there is a difference in EDS Hypermobility Type (HT) and JHS. Some say that they are the same illness, since they are often seen in the same family (one member will be diagnosed with JHS and another with EDS,) indicating that the genotype for EDS HT and JHS is the same and hereditary. Others dispute this, saying that JHS is simply the joint hypermobility due to looser joints, and that JHS does not present with the same symptoms as EDS HT, such as skin involvement, appearance (translucent skin, blue sclera, high palate, pectus excavatum, etc.,) or the other systematic involvement that is frequently present in EDS like the autonomic dysfunction, gastroparesis, etc. This is frequently disputed, and likely will be until we discover the genes behind EDS HT and behind JHS, and are able to tell whether or not they are the same illness with a wide range of presentation, or different illnesses altogether.

Friday, May 13, 2016

Tips For Helping Reduce Pain For The EDS Patient In The Kitchen

Cooking! I don't care to do, nor do I need to that much. I am single and live alone, I have no children so cooking is not a must do activity for me. I used to like to bake but now that my EDS has progressed so far I really can't enjoy baking because the pain way out does the pleasure of the outcome of the baking. That's okay because that is less clean up for me.

I do have to eat and at times carry a lunch with me to places and doing so can be a challenge for me as well as for any EDS patient. I found a great article on tips of reducing pain in the kitchen. Here is the article  and the link:

Reducing Pain and Effort in the Kitchen
Jul 14, 2012

EDSers are often frustrated with kitchen implements – they may be ineffective, difficult to use, or downright painful! Opening jars, bottles and cans are high on the list of complaints. Slicing, peeling, and cutting with knives is often painful. Lifting heavy pots, especially those with long handles, create torque on the wrist that makes it prone to dislocations.

To address kitchen challenges, here are some tips and product suggestions presented by an occupational therapist at the 2011 EDS conference.

Try these helpful hints to reduce EDS pain in the kitchen:
• Use spring loaded scissors to cut open bags or to cut food
• Use ‘zipper’ style baggies
• Buy reusable containers that are easy to open/close
• Buy food pre-chopped or use a spiked cutting board
• Use large well sharpened knives and the right knife for the job
• Use oven mitts to help you carry heavy pots/pans with 2 hands
• Use tongs to lift foods instead of forks
• Put groceries in paper bags for 2 hand carry, or in reusable bags for carrying over shoulder or pack light and carry over forearms
• Use plastic cups and plates
• Use electric whenever possible for opening cans or jars, mixing, blending, chopping
• Use built up kitchen tools
• Use paper towels to avoid having to wring; or press sponge with palm rather than squeezing
• Use an ‘L’ shaped knife or pizza cutting wheel
• Put dycem or wet dishrag under bowls when stirring
• Drag pots filled with liquid to sink or stove. Use extensible kitchen faucet to fill pots
• Use stainless steel colander in pots to avoid having to tip out liquid
• Buy lightweight pots/containers

List Source: Joint Protection of the Hands, 2011 EDNF Conference Presentation by Romina Astifidis MS, PT, CHT. Excerpt from Helpful Hints, page 5.

As a fellow EDSer, I have a few tips of my own. A good option for lightweight cookware is stainless steel pans. Revere Ware is my personal favorite. They are copper bottom with a stainless steel inside, which is fairly easy to clean when you coat the bottom with olive oil. Also they are aluminum-free and Teflon-free, which is healthier.

I have a lot of trouble with scissors. My favorite scissors are Black & Decker electric scissors. I like them because they do not require me to open and close my hand. They have a large grip and maneuver easily. They work well for both the kitchen and office – cutting both plastic and paper with ease. They even cut material! They are cordless and rechargeable.

- See more at: