Amy's Journey with...

Hypermobile Ehlers-Danlos syndrome (hEDS) ~ Postural Orthostatic Tachycardia Syndrome (POTS) ~ Focal Impaired Awareness (Complex Partial) Seizures ~ Fibromyalgia ~ Chronic Myofascial Pain (CMP) ~ Polycystic Ovarian Syndrome (PCOS) ~ TMJ Dysfunction ~ Bipolar Disorder Type I Rapid Cycling ~ Migraines ~ Gastroesophageal Reflux Disease (GERD) ~ Obsessive Compulsive Disorder (OCD) ~ Keratosis Pilaris (KP) ~ Complex-Post-Traumatic Stress Disorder (C-PTSD) ~ Panic Disorder ~ Generalized Anxiety Disorder (GAD) ~ Social Anxiety Disorder (SAD) ~ Nonsuicidal Self-Injury (Self-Harm) ~ Piezogenic Pedal Papules ~ Hashimoto's Thyroiditis ~ Irritable Bowel Syndrome (IBS) ~ Seasonal Affective Disorder (SAD) ~ Specific Phobias ~ Chronic Headaches

Monday, June 16, 2014

The last post ever

This is the last post for Please Tape Me Back Together: My life with Ehlers-Danlos Syndrome.  I enjoyed blogging here, but on the advice of another awesome blogger, I just can't continue to keep it up.  I am still blogging at Survivor: Living life with chronic pain, mental illness, and the ghosts of an abusive past and would love to have you visit me there.

Saturday, May 31, 2014

EDS Awareness Month: Ehlers-Danlos Syndrome Resources

The following are excellent sources to learn more about Ehlers-Danlos Syndrome.

Ehlers-Danlos National Foundation (EDNF) -- one of my favorite EDS charity and website

Ehlers-Danlos Syndrome Network C.A.R.E.S. Inc. -- another great EDS charity and website

National Organization of Rare Diseases' (NORD)'s page on EDS

Hypermobility Syndrome Association (HMSA) -- from the UK (for the hypermobility type)

Genetics of Ehlers-Danlos Syndrome on Medscape Research

Myths and Facts about EDS

Medline Plus page on EDS

Ehlers-Danlos Syndrome on Medicine Net

Wikipedia's Page on Ehlers-Danlos Syndrome

Website on EDS with lots of pictures

Typical facial features of someone with the vascular type of EDS

Urgent information on vascular EDS

I hope these help!  Are there any other websites on EDS that you'd like to add?

Sunday, May 25, 2014

EDS Awareness Month: Ehlers-Danlos Syndrome, Depression, and Anxiety

I don't see how you could have EDS, especially the things that go along with it, and not be depressed or anxious.  The isolation, the pain, and having friends abandon you are just a few of the problems people with EDS have to go through.  Anxiety about places you had bad things happen is common, too.  For instance, I had both my ankles sublux on the way to DH's dentist one time.  (That was back when he had a dentist.)  I was in so much pain, and they were so rude to me while I was in pain, that I get anxiety just thinking about that office.  I also find it depressing when I consider the things I used to be able to do, versus the things I can do now.  A few years ago I went jogging at least a mile, or walking at least three miles, every day.  It is so frustrating that now I can't do those things, or many other things I used to be able to do.

How do you get through it?  Well, I also have bipolar, with Complex-PTSD, Panic Disorder, Seasonal Affective Disorder, and social anxiety--plus I'm pretty sure I have Generalized Anxiety Disorder.  So, my depression and anxiety are treated by medication, though I still have trouble with them even on meds.  Some things that have worked for me include journaling, blogging, and meditation.

Have any other zebras found a method of decreasing depression and anxiety?

Saturday, May 24, 2014

EDS Awareness Month: Ehlers-Danlos Syndrome and POTS

Like so many others with EDS, I have POTS, a type of Dysautonomia.

The best source I've been able to find on dysautonomia has been The Dysautonomia Information Network.  According to them:
Dysautonomia literally means dysregulation of the autonomic nervous system. The autonomic nervous system is the master regulator of organ function throughout the body. It is involved in the control of heart rate, blood pressure, temperature, respiration, digestion and other vital functions. Dysregulation of the autonomic nervous system can produce the apparent malfunction of the organs it regulates. For this reason, dysautonomia patients often present with numerous, seemingly unrelated maladies.
Though there are many different types of Dysautonomia, the type most often associated with EDS is Postural Orthostatic Tachycardia Syndrome or POTS.  Postural orthostatic refers to changes in position.  A person with POTS has problems when their body changes positions, especially upon standing.  Tachacardia means an abnormally large increase in heart rate.  People who have POTS always feel fatigued.  I read once that just standing in place for someone with POTS is like running in place for someone with POTS; it's that exhausting.

What are the symptoms of POTS anyway?  The symptoms can vary widely, but most often are:

The hallmark symptom of POTS is an increase in heart rate from the supine to upright position of more than 30 beats per minute or to a heart rate greater than 120 beats per minute within 12 minutes of head-up tilt.

This tachycardic response is sometimes accompanied by a decrease in blood pressure and a wide variety of symptoms associated with hypotension. Low blood pressure of any kind may promote the following:
  • lightheadedness, sometimes called pre-syncope (pre-fainting) dizziness (but not vertigo, which is also called dizziness)
  • exercise intolerance
  • extreme fatigue
  • syncope (fainting)
  • Excessive thirst (polydipsia) '

Chronic or acute hypoperfusion of tissues and organs in the upper parts of the body are thought to cause the following symptoms:
  • cold extremities
  • chest pain and discomfort
  • disorientation
  • tinnitus
  • dyspnea
  • headache
  • muscle weakness
  • tremulousness
  • visual disturbances
Autonomic dysfunction is thought to cause additional gastrointestinal symptoms:
  • abdominal pain or discomfort
  • bloating
  • constipation
  • diarrhea
  • nausea
  • vomiting
Cerebral hypoperfusion, when present, can cause cognitive and emotive difficulties. Symptoms that persist in the supine (recumbent) state are difficult to attribute to "cerebral hypoperfusion"
  • brain fog
  • burnout
  • decreased mental stamina
  • depression
  • difficulty finding the right word
  • impaired concentration
  • sleep disorders
Inappropriate levels of epinephrine and norepinephrine lead to anxiety-like symptoms:
  • chills
  • feelings of fear
  • flushing
  • overheating
  • nervousness
  • over-stimulation
This is what DINET has to say about the increased risk that people with Ehlers-Danlos Syndrome have for developing POTS:
Ehlers-Danlos Syndrome (EDS), a connective tissue disorder, is found in some POTS patients. Physicians propose that these syndromes occur together due to abnormal connective tissue in dependent blood vessels in those with EDS, which permits veins to distend excessively in response to ordinary hydrostatic pressures (Rowe, Barron, Calkins, Maumenee, Tong & Geraghty, 1999). Simply put, this connective tissue abnormality allows excessive amounts of blood to pool in these patients' lower limbs when they stand up.   
There are a variety of types of Ehlers-Danlos syndrome. Classical and type III EDS were originally reported in orthostatic intolerance patients (Rowe et al., 1999). Many POTS patients with EDS have type III (Grubb, 2002).
The classical form of Ehlers-Danlos syndrome (types I and II) is characterized by soft,hyperextensible skin; easy bruising; poor wound healing; thin, atrophic scars; hypermobile joints; varicose veins and prematurity of affected newborns (Wenstrup & Hoechstetter, 2001). Mutations in type V collagen are a major cause of the classical type of Ehlers-Danlos syndrome.
Clinical features associated with EDS III include soft skin and large and small joint hypermobility (Wenstrup & Hoechstetter, 2001). Skin may be stretchy but scarring tends to be normal. POTS patients with EDS III are often hypermoblie/hyperflexable, double jointed, pale, female and tend to have blond hair and blue eyes (Grubb, 2002). The genetic basis for EDS III is unknown.
A wide variety of medical complications may occur with the classical and hypermobile types of EDS. Mitral valve prolapse can occur in all types of EDS and delayed gastric emptying has been observed in type III (personal observations, Wenstrup & Hoechstetter, 2001). A significant number of individuals with both the classical form and the hypermobile form have dilation and/or rupture of the ascending aorta (Wenstrup & Hoechstetter, 2001). Hiatal hernia has been widely reported in adults with EDS (Steinman, Royce & Superti-Furga, 1993). As previously stated, premature rupture of the membranes in pregnancy (primarily classical type) and poor wound healing (particularly with severe classical type) may occur. Other complications include a mild to moderate increase in peripartum bleeding, joint dislocations, chronic pain (most common in the hypermobile type), surgical complications and intraoperative problems (more common in the classical than hypermobile type), diverticulitis, problems associated with fragile skin (particularly with the classical type) and motor delay (Wenstrup & Hoechstetter, 2001).
One study on patients with "joint hypermobility syndrome", a disorder similar if not identical to EDS III, showed that 78% had signs of dysautonomia, such as orthostatic hypotension, postural orthostatic tachycardia syndrome and uncategorized orthostatic intolerance (Gazit, Nahir, Grahame, & Jacob, 2003). These patients also had evidence of a-adrenergic and B-adrenergic hyperresponsiveness. The authors of this study note that patients with the joint hypermobility syndrome have apparently intact vagal control of heart rate with disturbed sympathetic function. They further state that "the sympathetic dysregulation associated with joint hypermobility syndrome may have several explanations, such as peripheral neuropathy, blood pooling in the lower limbs, impaired central sympathetic control, or deconditioning due to muscle disuse through pain or fear of pain".
Another study of one hundred and seventy women with joint hypermobility syndrome concluded that non-musculoskeletal symptoms are common in patients with joint hypermobility syndrome, and that individuals with these symptoms may express more fatigue, anxiety, migraine, flushing, night sweats, and poor sleep than their peers (Hakim & Grahame, 2004).
People with Chiari also have an increased risk for POTS.
Chiari malformation is a condition in which the cerebellar tonsils protrude down into the spinal cord. This can restrict the flow of cerebral spinal fluid. The symptoms of Chiari are similar to those of POTS. A number of Chiari patients have reported being diagnosed with POTS. Some of these patients proclaim a decrease or resolution in POTS symptoms after undergoing surgical correction of their Chiari malformation. Other POTS patients with Chiari malformation have not experienced any benefits from corrective surgery. Some physicians are convinced that Chiari malformation is a cause of POTS, others doubt the relationship.
Physicians from the NIH and The Chiari Institute believe their is a connection between Ehlers-Danlos syndrome, POTS and Chiari 1 malformation.
Dysautomonia  has a specific test that is usually used, called the Tilt Table Test (TTT).  I couldn't finished the TTT because I was in too much pain.  The TTT sounds weird and it kind of is.  You lay down on a table which you are strapped to and you get an IV.  They put a bunch of electrodes on you to measure heart rate, they test your oxygen levels, and they have a blood pressure cuff on each arm.  Then as the "table" tilts upwards until you are standing and they watch to see if your heart rate increases too fast or if you faint.  If you faint you won't fall because you are strapped to the table.  The IV is there as a precaution, because though it is very rare, some people have had their heart stop during the procedure.  The test can take quite awhile, but I don't know how long as mine had to be stopped.  I can't stand for very long.  The day I had the TTT done was a good pain day until I stood for 10 and was crying in pain.  I suppose in 10 minutes they would have caught any changes that were to happen.

The Tilt Table Test

Thursday, May 15, 2014

Celebrating EDS Awareness Month

My body seems to be celebrating EDS Awareness month; much to my dismay.  I've had a four day flare that ended yesterday, brought on by ten minutes of using my recumbent stationary bike.  I end up in elevated pain, or even a flare, for three or so days after using the bike.  It makes it hard to get used to, and hard to want to do, when it ends with me in so much pain.  When it's not in use I put foam over all the handles, hardcover books on the seat, and a basket over the seat.  It looks horrible, but I do it in an effort to keep the cats from destroying it.  Katya has already put little claw holes in my seat, even with all the precautions I'm taking.  It takes less than a minute to take off the foam, books, and basket.  It still is embarrassing when someone comes over and they see what I've done to keep it safe from the kitties.  Well, I hope everyone is having a nice May, and your bodies aren't celebrating, either.

Wednesday, May 7, 2014

EDS Awarness Month: Ehlers-Danlos Syndrome and Fatigue

For me, this is how a bad fatigue morning goes with Ehlers-Danlos Syndrome.  Imagine fatigue so bad that you can barely lift your hand from the bed.  Rolling over requires more energy than you have, and getting out of bed to pee in the morning isn't possible.  So you lie there, in the weird position you woke up in, and hold your pee, waiting to have the energy to walk the few steps to the bathroom.

This is how a normal fatigue morning goes with EDS, at least for me.  Waking up in the morning you feel exhausted, like you've just ran three miles.  You sometimes have to sit in bed, gathering the energy to get to the bathroom, but other times you are able to walk there upon waking.  Some mornings you have to use a cane to walk the seven steps to the bathroom from your bed.  You go sit on the bed afterwards, waiting to gather enough energy to get dressed.  Once you get dressed, you walk to the living room and sit on the couch for awhile.  At this point most of the time you are too exhausted to make your own breakfast, and your husband has to make it for you, even if it's only milk and cereal.

EDS causes severe fatigue in most patients.  Often the fatigue is severe enough to be disabling.  Some EDSers are in wheelchairs not because of dislocations or pain, but because they are too fatigued and dizzy to walk on their own.  Some EDSers are bed bound from fatigue.

If you have EDS and fatigue, it may seem very frustrating to try to get things done.  I hate it that between the pain and fatigue I am unable to keep my house clean.  That really bothers and frustrates me more than anything.  What is your biggest frustration when it comes to fatigue caused by Ehlers-Danlos Syndrome?

Sunday, May 4, 2014

EDS Awarness Month: Ehlers-Danlos Syndrome Vascular Type

Ehlers-Danlos Syndrome, Vascular Type (formerly type IV)

  • This type affects approximately 1 in 250,000 people. Signs and symptoms include:
  • One fourth of individuals experience a serious medical problem by age 20
  • 80% of individuals experience a serious medical problem by age 40
  • The median age of death is 48 years
  • Passed down as an  autosomal dominant gene 
  • 50% of passing down C-EDS to children
  • Estimated prevalence is guessed at 1 in 250,000
  • There is no way of knowing how many people are undiagnosed with EDS
  • V-EDS is most often diagnosed on autopsy

Saturday, May 3, 2014

EDS Awarness Month: Ehlers-Danlos Syndrome Classic Type

Ehlers-Danlos Syndrome, Classical Type (formerly types I & II)
  • This type affects approximately 1 in 20,000 people. Signs and symptoms include:


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