What Is POTS??
Showing posts with label Autoimmune Disease. Show all posts
Showing posts with label Autoimmune Disease. Show all posts
Friday, January 17, 2020
High Burden of Premature Arteriosclerosis on Renal Biopsies in Incident Lupus Nephritis
IN THE NEWS
"Cardiovascular disease (CVD) is accelerated in patients with systemic lupus erythematosus (SLE) and lupus nephritis (LN). Despite the literature suggesting renal arteriosclerosis predicts CVD in other glomerulonephritis diseases, arteriosclerosis grading and reporting might be particularly overlooked in LN biopsies."
How does lupus affect white blood cells?
IN THE NEWS
How does lupus affect white blood cells?
"Leukopenia and neutropenia are very common in active lupus, "
"Leukopenia and neutropenia are very common in active lupus, "
US NEWS &WORLD REPORT--Signs of Lupus in Women
IN THE NEWS
Signs of Lupus in Women
"ANYONE CAN HAVE LUPUS, but the fact is that women have more than their fair share of this autoimmune condition. In the U.S., an estimated 1 million to 1.5 million people have lupus, and women account for about 90% of lupus diagnoses."
Signs of Lupus in Women
"ANYONE CAN HAVE LUPUS, but the fact is that women have more than their fair share of this autoimmune condition. In the U.S., an estimated 1 million to 1.5 million people have lupus, and women account for about 90% of lupus diagnoses."
Lupus News Today--Lupus Lessons: What Currencies Run Your Life?
IN THE NEWS
Lupus Lessons: What Currencies Run Your Life?
"Lupus is the giver of many lessons, and one of the most important lessons it taught me was how to value the things in my life."
"Lupus is the giver of many lessons, and one of the most important lessons it taught me was how to value the things in my life."
MedPage Today--Sjogren's Phenotype: It's Not Just One Thing Study details different presentations, influential factors
IN THE NEWS
Sjogren's Phenotype: It's Not Just One Thing Study details different presentations, influential factors
"The systemic manifestations of Sjogren's syndrome at the time of diagnosis differ according to individual patient characteristics such as sex, age, and ethnicity, a large international study found."
"This analysis confirmed that individual patient factors including age, sex, ethnicity, and residence strongly affect the systemic manifestations of Sjogren's syndrome. "Both the type of organ affected and the severity of the involvement are modulated by these geoepidemiological factors, which should be considered as critical when a personalized follow-up is planned for a patient newly diagnosed with Sjogren's syndrome," the researchers concluded."
First Potential Biomarker Identified for Postural Orthostatic Tachycardia Syndrome
IN THE NEWS
First Potential Biomarker Identified for Postural Orthostatic Tachycardia Syndrome
"New research from The University of Toledo College of Medicine and Life Sciences strongly suggests postural orthostatic tachycardia syndrome, or POTS, is an autoimmune disorder and may help pave the way for a simple blood test that could help physicians diagnose the condition."
"In the largest study of POTS patients to date, published Sept. 9 in the Journal of the American Heart Association, Grubb and UToledo research collaborators found 89 percent of patients they examined had elevated levels of autoantibodies against the adrenergic alpha 1 receptor."
First Potential Biomarker Identified for Postural Orthostatic Tachycardia Syndrome
"New research from The University of Toledo College of Medicine and Life Sciences strongly suggests postural orthostatic tachycardia syndrome, or POTS, is an autoimmune disorder and may help pave the way for a simple blood test that could help physicians diagnose the condition."
"In the largest study of POTS patients to date, published Sept. 9 in the Journal of the American Heart Association, Grubb and UToledo research collaborators found 89 percent of patients they examined had elevated levels of autoantibodies against the adrenergic alpha 1 receptor."
Sunday, April 30, 2017
SKIN CANCER AWARENESS IN MAY
May is Skin Cancer Awareness Month. People who have POTS often have autoimmune disease as an underlying cause. And people with autoimmune disease have a higher risk for all types of cancer including skin cancer and lymphoma.
I can tell you from personal experience, it's not fun to have chunks carved out of you and then have to wear makeup to cover the scars. I have a couple of scars that are bad enough that I tell people I have been in a knife fight.
Wear your sunblock people.
Skin cancer risk in autoimmune connective tissue diseases
https://www.ncbi.nlm.nih.gov/pubmed/24975951
Skin cancers associated with autoimmune conditions among elderly adults
Merkel Cell Carcinomas--Autoimmune Disease
New England Journal of Medicine--Risk for Nonmelanoma Skin Cancer Associated with Immunosuppressive Treatment of Autoimmune Disease
Diagnosis and Treatment of Basal Cell and Squamous Cell Carcinoma
Basal Cell Carcinoma--The Most Commonly Occurring Form Of Skin Cancer
Monday, April 17, 2017
WHAT YOU SHOULD LOOK FOR IN A BODY LOTION
WHAT YOU SHOULD LOOK FOR IN A BODY LOTION
People with autoimmune diseases have a particularly hard time keeping their skin moisturized and protected. And people with Sjogren's Syndrome have an even harder time because their body doesn't produce moisture to start with.
All of the options in the moisturizer aisle can seem overwhelming. They all make claims about what they will do for you. But there are particular ingredients you need to look for. I don't like to just go by advertising or word of mouth. I want to know what is in any products I use and they science behind it. I suppose that is because my Father was a chemist who worked until he retired at Proctor and Gamble. He often discussed with me the products that they made and why they were superior to others at the time. When I was a teenager, I actually tested out some of their products before they were on the market as a volunteer.
The following information on the difference between creams, lotions, and ointments come from the website of the National Eczema Association. They have a list of approved moisturizers there.
OINTMENTS
Ointments are semi-solid greases that help to hydrate the skin by preventing water loss. Petroleum jelly has no additional ingredients, whereas other ointments contain a small proportion of water or other ingredients to make the ointment more spreadable. Ointments are very good at helping the skin retain moisture but they are often disliked because of their greasiness.
CREAMS
Creams are thick mixtures of greases in water or another liquid. They contain a lower proportion of grease than ointments, making them less greasy. A warning: creams often contain stabilizers and preservatives to prevent separation of their main ingredients, and these additives can cause skin irritation or even allergic reactions for some people.
LOTIONS
Lotions are mixtures of oil and water, with water being the main ingredient. Most lotions do not function well as moisturizers for people with dry skin conditions because the water in the lotion evaporates quickly.
Ingredients
Ingredients fall into three categories. Humectants such as glycerin and urea absorb water from the air and hold moisture in the skin. Other humectant ingredients to look for are hyaluronic acid, alpha-hydroxy acids, sorbitol, propylene glycerol, and sodium lactate. Emollients like mineral oil, lanolin and petrolatum fill spaces between skin cells to replace lipids and smooth and lubricate the rough skin. Other emollients to look for are jojoba oil, isopropyl palmitate, propylene glycol linoleate, squalene and glycerol stearates.Occlusives such as petroleum jelly and cocoa butter, are oily substances and they form a film on the top of skin which seals in cracks and prevents moisture from escaping.
Water: This is the main ingredient of moisturizers, and it serves to carry oil-based ingredients onto and into the skin, but oil-based ingredients also help lock water into the skin
Ceramides
Ceramides are waxy lipid molecules that are found in skin cell membranes that help prevent moisture loss. They are involved in things like programmed cell death of cells. Ceramides in your moisturizer will help maintain and repair your skin barrier, so that moisture stays sealed in and it helps your body's natural moisture defenses. That is especially important for people who have eczema and psoriasis because studies have found that they have fewer ceramides than people who have normal healthy skin.
Cerave, as the name implies has ceramides in it. It also has Dimethicone, Hyaluronic Acid, Glycerine.
If you follow this link you can get a coupon for it from their website: Cerave Coupon The Itch Relief type also contains Pramoxine Hydrochloride which is an external analgesic, Shea Butter, Tasmannia Lanceolata Fruit Extract,
Curel says it has a proprietary ceramide-rich formula.
Peptides
Peptides are chains of amino acids. Amino acids include the 22 proteinogenic ("protein-building") amino acids, that combine into peptide chains ("polypeptides") which form the building-blocks of proteins. In this case, the peptides and amino acids are involved in building proteins in the skin.
Products that contain short chain amino acids can penetrate the top layer of skin and stimulate the cell production.
Collagen is an important protein in skin. It is involved in the thickness and pliability(stretchiness) of skin. Collagen breaks down for things like environmental factors (sun and stress, chemical exposure) and age. This causes wrinkles. Peptides stimulate the production of collagen thereby reducing wrinkles
Elastin has the ability to stretch and return to its original length—like a spring or rubber band. Elastin is the major component of ligaments (tissues that attach bone to bone) and skin. In people who have connective tissue disease, it is common for collagen and elastin to become injured by inflammation. So if you have MCTD or SLE Lupus or Sjogren's Syndrome, Scleroderma, Rheumatoid Arthritis, or Psoriatic Arthritis your collagen is probably not functioning properly.
If a product has that in it, it's probably something we could use.
Hyaluronic Acid
Hyaluronic Acid works by binding to moisture. It can hold up to 1,000 times its weight in water, making it an excellent natural skin plumper. Hyaluronic acid helps your skin repair and regenerate itself after suffering from dryness, environmental stresses, or irritation. It helps the collagen and elastin in your skin stay moist and helps it to keeps it elasticity. It is also lightweight and isn't oily, which is good if you are acne-prone.
Fatty Acids
Fatty acids come in things like Olive oil, avocado, almond oil, and shea butter which are essential fatty acids that help lock in moisture. Your body needs fatty acids for fuel to do things like produce moisture. But it doesn't produce it on its own, so you have to either ingest it or apply it topically. Foods that have Omega-3 fatty acids in them are things like salmon, mackerel, walnuts, soy, flaxseed, and safflower oil. I take a supplement that combines fish oil, flaxseed oil and borage oil in it.
Glycerin
Glycerin is a simple polyol compound derived from sugar alcohol. It is colorless and odorless. Glycerin and glycols help to retain water in a product, in hair or the top layers of skin by drawing moisture in and then retain it. Glycerin can actually absorb moisture from the air.
Ethylhexyl glycerin
Ethylhexyl glycerin (oct oxy glycerin) is a topical skin care ingredient and deodorizing agent, often indicated as a conditioning ointment in the treatment of eczema. As its name suggests Ethylhexyl glycerin is made using glycerin.
Sodium PCA,
Sodium PCA is used in lotions and hair care products because it is naturally in skin cells and it binds to water in cells and that means it is great for water absorption.
Caprylic/Capric Triglycerides
Caprylic/Capric Triglycerides is a mixed triester derived from coconut oil and glycerin. It comes in the form of an oily liquid and is sometimes mistakenly referred to as fractionated coconut oil. Caprylic mainly works as an emollient, dispersing agent and solvent.)
Piroctone Olamine
Piroctone Olamine is used to treat skin conditions and dandruff. It is an antifungal.
Licochalcone
Locochalcone is a molecule contained in licorice root extract which helps control oil production and is anti-bacterial as well as anti-inflammatory. In short, it helps calm and soothes skin.
Dimethicone
Dimethicone is used as a moisturizer to treat or prevent dry, rough, scaly, itchy skin and minor skin irritations (e.g., diaper rash, skin burns from radiation therapy). It softens and moisturizes the skin and decreases itching and flaking.
Triethanolamine (TEA)
Triethanolamine (TEA)This emulsifier
helps to blend the oil/water mix that makes up moisturizer.
Vitamin A
Vitamin A is usually in the form of retinol or retinoic acid in
moisturizers. It is anti-aging. It works by stimulating collagen
production, which fills in lines and wrinkles
Vitamin C & vitamin E
Vitamin C & vitamin E are
antioxidants and they help repair cellular damage caused by sun,
pollution and free radicals that are created during oxygenation.
Magnesium Aluminum
Magnesium Aluminum Silicate is a naturally occurring mineral derived from refined and purified clay that is used primarily as a thickener in cosmetics and beauty products. It is an off-white powder used in the pharmaceutical manufacturing process as an absorbent; anticaking agent; opacifying agent; slip modifier; and an aqueous viscosity increasing agent. But magnesium is considered as ”the mineral of beauty” in traditional Chinese medicine. Magnesium deficiency results in lower levels of fatty acids on the skin. This results in less elasticity and moisture and that, in turn, causes dryness and inflammation. If you take magnesium to boost the levels in your cells, it helps protect the cells and detoxifies them and encourages healthy skin tissue growth. It helps in DNA replication and repair. It also prevents free radical damage and inflammation.
In a 2007 study cited in the “American Journal of Clinical Nutrition,” Magnesium was discovered to help reduce inflammation caused by an excess amount of E-selectin and C-reactive protein.
When the skin is injured or damaged or when acne-causing bacteria enter the skin, E-selectin is produced. This results in acne inflammation. Magnesium helps reduce E-selectin’s effects and prevents the inflammation.
Magnesium aluminum is sometimes called Kaolin or China Clay.
At the Skin Store website I found this:
“Kaolin (also know as China clay, hectorite or magnesium silicate) is a naturally occurring soft clay. In cosmetics, kaolin helps to cleanse and exfoliate dead skin cells and debris from the surface. It adds absorbency, texture, and bulk to cleansers.
...Because it’s safe nature and has great absorbency and soothing properties, it’s quite the popular ingredient in formulas designed to purify
Kaolin wages war on acne and breakout-prone skin by improving the skin’s balance and decreasing levels of pore-clogging oils, dirt and toxins. General skin inflammation, caused by the usual environmental factors can also be addressed with kaolin cleansers. Kaolin increases circulation to the impacted areas of skin, helping to stimulate healing and to reduce irritation. Using a cleanser that contains kaolin prior to applying makeup can help to control oil production and prevent unwanted shine.”http://www.skinstore.com/blog/skincare/beauty-glossary-kaolin/
Eczema is associated with a magnesium deficiency. If you have a deficiency of magnesium your body will produce histamines. Those histamines will cause you to be itchy and have red blotches or hives on your skin. They are caused by blood vessel swelling that causes fluid to leak into the skin and other tissues.
Taking supplements gives some people diarrhea. But if you can take it, it helps avoid kidney stones and calcification in the joints too. It also good for circulation and high blood pressure. Doses less than 350 mg daily are safe for most adults. When taken in very large amounts, magnesium is POSSIBLY UNSAFE. Large doses might cause too much magnesium to build up in the body, causing serious side effects including an irregular heartbeat, low blood pressure, confusion, slowed breathing, coma, and death. Magnesium strengthens bones; maintains nerve and muscle function; regulates heart rhythm and blood sugar levels, and helps maintain joint cartilage.http://www.arthritis.org/living-with-arthritis/treatments/natural/vitamins-minerals/guide/magnesium.php
I know some people apply magnesium oil directly to their skin. But I don't know about the safety of it.
Vaseline, Intensive Care, Cocoa Radiant, With Pure Cocoa Butter, Non-Greasy Lotion is one of my stand-bys. It has Glycerin, Triethanolamine, Dimethicone, magnesium aluminum, shea butter and cocoa butter in it. And of course vaseline.
Eucerin Calming Cream and the Eucerin Eczema Relief Body Creme is good lotion it has Oatmeal, Ceramides, Caprylic/Capric Triglycerides, Castor oil, Piroctone Olamine, Licochalcone, Ethylhexylglycerin.
"Moist" Is A Disgusting Word - Here's Why
SUNSCREENS
After you have considered all of the other ingredients, don't forget the sunscreen. You need an SPF of at least 30. I happen to think people with Lupus SLE and Sjogren's should use the highest they can get. If it isn't in your moisturizer, you may have to use a separate one. I have some for sensitive skin that I use and that have pretty high SPFs.
I haven't tried it but just by virtue of the fact that it also hydrates this one seems promising.
And since I know the spray on is good, I might try these:
Neutrogena Healthy Defense Daily Moisturizer For Sensitive Skin With Broad Spectrum Spf 30 Sunscreen
A facial moisturizer with an SPF 50 seems like a pretty good idea. I love my Oil of Olay products, but as far as SPF is concerned, I think this is the highest SPF. Olay Complete Daily Defense All Day Moisturizer With Sunscreen SPF30 Sensitive Skin
If you have ever given birth then you are probably familiar with Palmer's Cocoa Butter products for stretch marks. But they make products for your face now and they are excellent. If they can help with stretch marks they have got to be doing something right. If you compare the ingredients to the ones listed above, you can see that they are very good for your skin. And I have some of them and they worked pretty well. I used the serum and day cream in the day and the night cream, obviously at night during a time that my face was super dry.
They have an SPF 15. Some of them say they promote collagen production and they contain antioxidants, a peptide complex, and vitamins A, B1, B2, B3, C and E. It depends on the individual product. Among other ingredients they contain, Hydrogenated Palm Glycerides, Theobroma Cacao (Cocoa)Seed Butter, Dimethicone, Butyrospermum Parkii (Shea) Butter, Oenothera Biennis (Evening Primrose) Flower Extract, Aloe Barbadensis Leaf Juice, Hydrolyzed Milk Protein, Glycerin, Triethanolamine. The night cream contains Natural Cocoa Butter, Retinol and Antioxidant Vitamin E.
They also have a gel oil for the body with an SPF 15 and cocoa butter, vitamin E, shea butter, jojoba oil. Palmer's Formula Moisturizing Gel Oil, Cocoa Butter, SPF 15
Last but not least I just discovered a new moisturizer line that sounds interesting. Garnier SkinActive Moisture Bomb “WATER-LIGHT FORMULAS. ANTIOXIDANT-PACKED HYDRATION.
Including formulas that feature goji berry and pomegranate, our refreshing gel cream, SPF 30 lotion, and all new super hydrating sheet masks blast dryness away, flooding skin with long-lasting hydration for a softer, healthier-looking complexion.”
Lupus, The Skin & Hair - Dr Abby Macbeth
Friday, April 14, 2017
What is Raynaud's (ray-NOHDZ) disease?
What is Raynaud's (ray-NOHDZ) disease?
It is a condition that causes some of the areas of your body, usually but not always the extremities, such as your fingers and toes — to feel numb and cold when they become cold. Stress can also cause symptoms. When you have Raynaud's disease the smaller arteries which supply blood to your skin narrow, decreasing the blood flow to affected areas. This is called vasospasm.
More women than men have Raynaud's disease. It is also called Raynaud's phenomenon or syndrome. People who have it usually just say, “Raynaud's” without the other descriptive words. People who live in colder climates are more likely to have it.
If you have Raynaud's, your treatment is largely dependent upon how severe it is and also any other conditions you may have. It isn't usually life threatening, but it does affect how you live on a day to day basis.
Simple things can bring on an attack, like digging around in your freezer for that roast you know is in there somewhere. It doesn't take long for your hands to turn colors and hurt. And it gets worse when you take your hands out of the freezer and the circulation comes back into them. Other things like going out into the cold or putting your hands into cold water can cause the same symptoms.
Some of the symptoms include:
Cold fingers or toes
Color changes in your skin in response to cold or stress
Numb, prickly feeling or stinging pain upon warming or stress relief
Your fingers or toes get cold and then they turn white. Then they progress to blue and start to feel numb and cold. And then when you seek to warm them up, and the circulation starts to come back into them, they become painful, with throbbing and tingling and they turn red. Some people like me don't have all three color changes. I usually don't turn blue. But that may be because I avoid getting my hands and feet cold. They may never get cold enough to cause the change from white to blue. But I have had them do that years ago. It can take as much as 15 minutes for the blood flow in an affected area to return to normal.
There are other areas of the body besides the fingers and toes that can be affected by Raynaud's. Your ears, lips, and nose can be affected. Raynaud's can even affect the internal organs. And even more embarrassing areas like your nipples and your rear end. Yes, now you can tell your husband that “turkey butt” is a medical condition and you can't help it.
I purposefully chose photographs that don't look too severe. Not everyone looks like they are ready for amputation when they have an attack of Raynaud's and if you look at the severe cases you might not recognize it in yourself. If you have bad discoloration you can google it and come up with photos.
The exact cause of Raynaud's hasn't been determined yet. Something causes the blood vessels in the hands and feet to overreact to cold temperatures or stress. This overreaction is called vasospam. During vasospasm the arteries that supply blood to your fingers and toes spasm due to cold exposure or stress and narrow which causes a decrease in the blood supply. This eventually causes the arteries to thicken which further limits the blood flow.
Raynaud's phenomena in real time.
There are two classifications of Raynaud's.
PRIMARY RAYNAUD'S
It is also called Raynaud's disease and it's usually isn't caused by some other medical condition. It usually begins earlier in life than Secondary Raynaud's between 15-30 years of age. There seems to be a genetic predisposition to Primary Raynaud's. Approximately 1/3 of patients with Primary Raynaud's have a parent, sibling or child who also has it the disorder.
SECONDARY RAYNAUD'S
It is also called Raynaud's phenomenon and is less common than Primary Raynaud's. and it usually is caused by something else. It is usually more serious. Onset is usually around the age of 40.
There are two main types of the condition.
Things that cause Secondary Raynaud's are Connective tissue diseases like Scleroderma, Rheumatoid Arthritis, Sjogren's syndrome, and Lupus. Scleroderma can cause a hardening or thickening of the blood vessels. Sjogren's, Lupus and RA cause inflammation of the nerves that cause the blood vessels to constrict. Up to 1/3 of Lupus patients have Raynaud's. And 15-30% of Sjogren's patients have Raynaud's. I have both Lupus and Sjogren's so it was probably inevitable that I would have Raynaud's.
Hardening of the arteries also called atherosclerosis and high blood pressure in the lungs called primary pulmonary hypertension also cause Raynaud's. These things can be exacerbated by smoking because it causes the blood vessels to constrict.
If you have Carpel tunnel syndrome causes numbness and pain in your hands due to pressure on the nerves. This can cause your hands to be susceptible to temperature changes and lead to Raynaud's. Operating tools which vibrate can cause you to develop Secondary Raynaud's.
Raynaud's disease is also associated with dysautonomia. With regard to Postural Orthostatic Tachycardia, Neurocardiogenic Syncope and Orthostatic Hypotension, an interview on Dysautonomia International had this to say: “We do not know how many POTS, NCS or OH patients have APS, but Dysautonomia International recently funded a research project designed by Dr. Svetlana Blitshteyn to try to shed some light on the topic of autoimmune markers and autoimmune conditions in patients with POTS. Dysautonomia International will make an announcement when Dr. Blitshetyn’s study results are released.” And in the article, it says that Raynaud's disease is a symptom of Antiphospholipid Syndrome. So you can say that Raynaud's is associated with dysautonomia.What Dysautonomia Patients Should Know About Antiphospholipid Syndrome Postural Orthostatic Tachycardia SyndromeA Dermatologic Perspective and Successful Treatment with Losartan
That's not too surprising. People with dysautonomia and POTS have problems regulating temperature in general and blood flow problems because of inadequate pressure in the blood vessels. And POTS, like Raynaud's disease, is associated with autoimmune diseases.
Causes
Beta blockers, migraine medications, ADD/HD medication, and cold medications can all cause Raynaud's or make it worse because they cause constriction of the blood vessels.
There are also some chemicals that can cause Secondary Raynaud's.
It can become severe enough that you need to seek medical help. You definitely need to see a doctor if you get a sore on one of the areas affected so that you don't end up with an infection and lose an appendage.
Some people have a permanent reduction or blood flow which causes their fingers and toes to become deformed. If a complete blockage of an artery occurs, you can get skin ulcerations and ultimately gangrene, which is why it is important to see a doctor if you get a sore on an affected area to avoid having an amputation.
Treatments
There are things you can do for Raynaud's. Wearing warm clothes, socks and gloves in cold weather are essential. Ear muffs and masks and scarves to keep your nose warm are also good ideas. Some people wear the socks and gloves when they are sleeping. And the gloves come in handy when you have to get into the freezer, but I personally never remember to do it. Avoid getting cold in the first place. Smoking is a no-no. Avoid stress because it can bring on an attack. And exercise increases circulation.
If you get cold, go inside and warm up. Two things that you should do are almost instinctive for people to do. Wiggling your fingers and toes and rubbing them to get them warm. You can also put your hands in your armpits and swing your arms to increase blood flow. You can run warm water over them too. Just don't get the water too hot.
Medications
And there are medications that help.
Calcium Channel Blockers relax the small blood vessels which help avoid the vasospasms. Common drugs in this class are Procardia, Norvasc, and Verapamil and Nifedipine.
Alpha blockers like prazosin and Cardura work by counteracting noradrenaline also called norepinephrine. This hormone causes blood vessels to constrict.
Vasodilators Nitroglycerin works as a vasodilator and comes in a cream and will facilitate the healing of ulcers. Losartan, which is a high blood pressure medication, and Viagra, as well as antidepressants like fluoxetine, can help treat Raynaud's.
Fish oil has fatty acids in that are supposed to make you less susceptible to cold. Studies have shown that it didn't help people who have Secondary Raynaud's. You have to be careful when taking high doses of fish oil because it acts as a blood thing and increases your risk of bleeding, particularly if you take blood thinners, like as warfarin (Coumadin), clopidogrel (Plavix), or aspirin. Discuss it with your doctor.
Gingko is also supposed to be helpful by opening up the blood vessels. One study showed that Raynaud's patients had less pain if they took 160 mg of ginkgo per day. DO NOT take ginkgo if you have a history of seizures. Ginkgo can also increase risk of bleeding, especially if you take blood thinners. Talk to your doctor.
Evening primrose oil also contains a type of fatty acid which keeps your body from making chemicals that narrow blood vessels. Studies have shown that taking Evening primrose oil will lessen the severity of attacks as well as the frequency. EPO can make seizures worse in people who already have them and they shouldn't take it. And just like fish oil, there is a risk of bleeding with it.
There is a form of vitamin B3(niacin) called Inositol hexaniacinate. It may reduce the number of Raynaud's attacks. But it requires high doses and needs to be monitored by a doctor.
Taking Magnesium supplements opens the blood vessels, but there haven't been any studies to show if it works. Some people get diarrhea from taking it, so take it with a meal to help avoid this problem. It can also interfere with some medications like high blood pressure medication and antibiotics, so your doctor needs to be aware if you are taking it.
Biofeedback to lessen the effects of stress on your body may help lessen the frequency of attacks. Accupuncture is another treatment option that helps by improving blood flow to the affected areas. There is also a specific kind of biofeedback called Thermal Biofeedback that studies have shown works.
Surgery and injections
When lifestyle changes and medication don't help enough, sometimes things like surgery and injections of chemicals are the next line of treatment.
There are nerves in your feet and hands that control vasoconstriction and dilation. There is a surgery called a sympathectomy, in which these nerves around your blood vessels in your hands or feet are cut to stop the over-constriction. For some people it is helpful.
Botox injections can also block the sympathetic nerves and block the over-constricion.
For more information visit this link for a guide to Raynaud's
Raynaud’s Guide: The Cold Facts on Raynaud’s
These are links to my sources:
http://www.mayoclinic.org/diseases-conditions/raynauds-disease/basics/definition/con-20022916
http://www.umm.edu/health/medical/altmed/condition/raynauds-phenomenon
It is a condition that causes some of the areas of your body, usually but not always the extremities, such as your fingers and toes — to feel numb and cold when they become cold. Stress can also cause symptoms. When you have Raynaud's disease the smaller arteries which supply blood to your skin narrow, decreasing the blood flow to affected areas. This is called vasospasm.
More women than men have Raynaud's disease. It is also called Raynaud's phenomenon or syndrome. People who have it usually just say, “Raynaud's” without the other descriptive words. People who live in colder climates are more likely to have it.
If you have Raynaud's, your treatment is largely dependent upon how severe it is and also any other conditions you may have. It isn't usually life threatening, but it does affect how you live on a day to day basis.
Simple things can bring on an attack, like digging around in your freezer for that roast you know is in there somewhere. It doesn't take long for your hands to turn colors and hurt. And it gets worse when you take your hands out of the freezer and the circulation comes back into them. Other things like going out into the cold or putting your hands into cold water can cause the same symptoms.
Some of the symptoms include:
Cold fingers or toes
Color changes in your skin in response to cold or stress
Numb, prickly feeling or stinging pain upon warming or stress relief
Your fingers or toes get cold and then they turn white. Then they progress to blue and start to feel numb and cold. And then when you seek to warm them up, and the circulation starts to come back into them, they become painful, with throbbing and tingling and they turn red. Some people like me don't have all three color changes. I usually don't turn blue. But that may be because I avoid getting my hands and feet cold. They may never get cold enough to cause the change from white to blue. But I have had them do that years ago. It can take as much as 15 minutes for the blood flow in an affected area to return to normal.
There are other areas of the body besides the fingers and toes that can be affected by Raynaud's. Your ears, lips, and nose can be affected. Raynaud's can even affect the internal organs. And even more embarrassing areas like your nipples and your rear end. Yes, now you can tell your husband that “turkey butt” is a medical condition and you can't help it.
I purposefully chose photographs that don't look too severe. Not everyone looks like they are ready for amputation when they have an attack of Raynaud's and if you look at the severe cases you might not recognize it in yourself. If you have bad discoloration you can google it and come up with photos.
The exact cause of Raynaud's hasn't been determined yet. Something causes the blood vessels in the hands and feet to overreact to cold temperatures or stress. This overreaction is called vasospam. During vasospasm the arteries that supply blood to your fingers and toes spasm due to cold exposure or stress and narrow which causes a decrease in the blood supply. This eventually causes the arteries to thicken which further limits the blood flow.
Raynaud's phenomena in real time.
There are two classifications of Raynaud's.
PRIMARY RAYNAUD'S
It is also called Raynaud's disease and it's usually isn't caused by some other medical condition. It usually begins earlier in life than Secondary Raynaud's between 15-30 years of age. There seems to be a genetic predisposition to Primary Raynaud's. Approximately 1/3 of patients with Primary Raynaud's have a parent, sibling or child who also has it the disorder.
SECONDARY RAYNAUD'S
It is also called Raynaud's phenomenon and is less common than Primary Raynaud's. and it usually is caused by something else. It is usually more serious. Onset is usually around the age of 40.
There are two main types of the condition.
Things that cause Secondary Raynaud's are Connective tissue diseases like Scleroderma, Rheumatoid Arthritis, Sjogren's syndrome, and Lupus. Scleroderma can cause a hardening or thickening of the blood vessels. Sjogren's, Lupus and RA cause inflammation of the nerves that cause the blood vessels to constrict. Up to 1/3 of Lupus patients have Raynaud's. And 15-30% of Sjogren's patients have Raynaud's. I have both Lupus and Sjogren's so it was probably inevitable that I would have Raynaud's.
Hardening of the arteries also called atherosclerosis and high blood pressure in the lungs called primary pulmonary hypertension also cause Raynaud's. These things can be exacerbated by smoking because it causes the blood vessels to constrict.
If you have Carpel tunnel syndrome causes numbness and pain in your hands due to pressure on the nerves. This can cause your hands to be susceptible to temperature changes and lead to Raynaud's. Operating tools which vibrate can cause you to develop Secondary Raynaud's.
Raynaud's disease is also associated with dysautonomia. With regard to Postural Orthostatic Tachycardia, Neurocardiogenic Syncope and Orthostatic Hypotension, an interview on Dysautonomia International had this to say: “We do not know how many POTS, NCS or OH patients have APS, but Dysautonomia International recently funded a research project designed by Dr. Svetlana Blitshteyn to try to shed some light on the topic of autoimmune markers and autoimmune conditions in patients with POTS. Dysautonomia International will make an announcement when Dr. Blitshetyn’s study results are released.” And in the article, it says that Raynaud's disease is a symptom of Antiphospholipid Syndrome. So you can say that Raynaud's is associated with dysautonomia.What Dysautonomia Patients Should Know About Antiphospholipid Syndrome Postural Orthostatic Tachycardia SyndromeA Dermatologic Perspective and Successful Treatment with Losartan
That's not too surprising. People with dysautonomia and POTS have problems regulating temperature in general and blood flow problems because of inadequate pressure in the blood vessels. And POTS, like Raynaud's disease, is associated with autoimmune diseases.
Causes
Beta blockers, migraine medications, ADD/HD medication, and cold medications can all cause Raynaud's or make it worse because they cause constriction of the blood vessels.
There are also some chemicals that can cause Secondary Raynaud's.
It can become severe enough that you need to seek medical help. You definitely need to see a doctor if you get a sore on one of the areas affected so that you don't end up with an infection and lose an appendage.
Some people have a permanent reduction or blood flow which causes their fingers and toes to become deformed. If a complete blockage of an artery occurs, you can get skin ulcerations and ultimately gangrene, which is why it is important to see a doctor if you get a sore on an affected area to avoid having an amputation.
Treatments
There are things you can do for Raynaud's. Wearing warm clothes, socks and gloves in cold weather are essential. Ear muffs and masks and scarves to keep your nose warm are also good ideas. Some people wear the socks and gloves when they are sleeping. And the gloves come in handy when you have to get into the freezer, but I personally never remember to do it. Avoid getting cold in the first place. Smoking is a no-no. Avoid stress because it can bring on an attack. And exercise increases circulation.
If you get cold, go inside and warm up. Two things that you should do are almost instinctive for people to do. Wiggling your fingers and toes and rubbing them to get them warm. You can also put your hands in your armpits and swing your arms to increase blood flow. You can run warm water over them too. Just don't get the water too hot.
Medications
And there are medications that help.
Calcium Channel Blockers relax the small blood vessels which help avoid the vasospasms. Common drugs in this class are Procardia, Norvasc, and Verapamil and Nifedipine.
Alpha blockers like prazosin and Cardura work by counteracting noradrenaline also called norepinephrine. This hormone causes blood vessels to constrict.
Vasodilators Nitroglycerin works as a vasodilator and comes in a cream and will facilitate the healing of ulcers. Losartan, which is a high blood pressure medication, and Viagra, as well as antidepressants like fluoxetine, can help treat Raynaud's.
Fish oil has fatty acids in that are supposed to make you less susceptible to cold. Studies have shown that it didn't help people who have Secondary Raynaud's. You have to be careful when taking high doses of fish oil because it acts as a blood thing and increases your risk of bleeding, particularly if you take blood thinners, like as warfarin (Coumadin), clopidogrel (Plavix), or aspirin. Discuss it with your doctor.
Gingko is also supposed to be helpful by opening up the blood vessels. One study showed that Raynaud's patients had less pain if they took 160 mg of ginkgo per day. DO NOT take ginkgo if you have a history of seizures. Ginkgo can also increase risk of bleeding, especially if you take blood thinners. Talk to your doctor.
Evening primrose oil also contains a type of fatty acid which keeps your body from making chemicals that narrow blood vessels. Studies have shown that taking Evening primrose oil will lessen the severity of attacks as well as the frequency. EPO can make seizures worse in people who already have them and they shouldn't take it. And just like fish oil, there is a risk of bleeding with it.
There is a form of vitamin B3(niacin) called Inositol hexaniacinate. It may reduce the number of Raynaud's attacks. But it requires high doses and needs to be monitored by a doctor.
Taking Magnesium supplements opens the blood vessels, but there haven't been any studies to show if it works. Some people get diarrhea from taking it, so take it with a meal to help avoid this problem. It can also interfere with some medications like high blood pressure medication and antibiotics, so your doctor needs to be aware if you are taking it.
Biofeedback to lessen the effects of stress on your body may help lessen the frequency of attacks. Accupuncture is another treatment option that helps by improving blood flow to the affected areas. There is also a specific kind of biofeedback called Thermal Biofeedback that studies have shown works.
Surgery and injections
When lifestyle changes and medication don't help enough, sometimes things like surgery and injections of chemicals are the next line of treatment.
There are nerves in your feet and hands that control vasoconstriction and dilation. There is a surgery called a sympathectomy, in which these nerves around your blood vessels in your hands or feet are cut to stop the over-constriction. For some people it is helpful.
Botox injections can also block the sympathetic nerves and block the over-constricion.
For more information visit this link for a guide to Raynaud's
Raynaud’s Guide: The Cold Facts on Raynaud’s
These are links to my sources:
http://www.mayoclinic.org/diseases-conditions/raynauds-disease/basics/definition/con-20022916
http://www.umm.edu/health/medical/altmed/condition/raynauds-phenomenon
Raynaud's Awareness
Pathophysiology Raynaud's Disease
Friday, September 16, 2016
Causes of POTS/Postural Orthostatic Tachycardia Syndrome
Part of the process of being diagnosed with POTS is wondering what caused it and if you did something to cause it and questioning in general.
The list of things that can go wrong in the body and cause POTS is quite extensive. In addition, there are disorders with symptoms like POTS. The secondary disorders are usually treatable and will in turn, treat your POTS symptoms. I would ideally like to make a separate post for each thing. But it will be more efficient to just make links to information within this post.
Adrenal disorders like Addison's disease is one of those conditions that has POTS-like symptoms, meaning that it mimics POTS. If you have an auto-immune disease, you can have an autoimmune type of Addison's disease. And to complicate that further, autoimmune thyroid disease can put you at risk for the autoimmune form of Addison's disease. Other things associated with it are hypoparathyroidism, hypopituitarism, pernicious anemia, testicular failure in men, diabetes type I, and vitiligo.
One of the problems with adrenal glands can be caused by a form of tumor called a Pheochromocytoma. This causes the adrenal glands to be overactive. This can lead to high blood pressure and cause symptoms such as
- Headaches
- Sweating
- Pounding of the heart
- Being shaky
- Being extremely pale
The hormones produced by the adrenal glands belong to a category called Catecholamines, which are released into the bloodstream in response to physical or emotional stress. The ones specific to the adrenal glands are epinephrine and norepinephrine, also called respectively adrenal and noradrenaline. The other catecholamine is dopamine. But it isn't produced by the adrenal glands. Dopamine is produced in the brain. It is a neurohormone that is released by the hypothalamus. Its action is as a hormone that is an inhibitor or prolactin release from the anterior lobe of the pituitary
Pheocychromatoma and paragangliomas cause an overproduction of these hormones. Blood and urine tests to measure the amounts can help detect a pheochromocytoma.
The urine test is a 24-hour urine collection. The blood test for catecholamines is sometimes done when a person has unexplained hypertension or sudden paroxysmal hypertension.
People with Anemia can have symptoms like POTS and if they also have a folic acid deficiency, it will make their anemia and POTS symptoms worse.
Anemia patients sometimes exhibit the symptoms of POTS. Some patients may have a folic acid deficiency, which is contributing to their anemia and POTS symptoms.
Angiotensin II
Some people with POTS have an increased level of Angiotensin II. The renin-angiotensin-aldosterone system (RAAS) helps regulate blood volume by regulating sodium and water retention. Renin and aldosterone can be reduced in POTS patients. But this study found that Angiotensin II was increased. http://www.ncbi.nlm.nih.gov/pubmed/16262605. Angiotensin II is a hormone and it causes blood vessels to constrict.
There may be a gene called the angiotensin II type one receptor gene involved in POTS.
Autoimmune Diseases
Besides anemia and Addison's disease and thyroid disorders, there are several other autoimmune diseases that can cause POTS. See: Lupus, Sjogren's, Guillain-Barre. Sarcoidosis, Crohn's Disease
There is an antibody to neuronal nicotinic acetylcholine receptors of autonomic ganglia that has been discovered. Some POTS patients have higher levels of this antibody. Some patients that also had anhidrosis, constipation, urinary dysfunction, sicca syndrome and pupillary dysfunction had even higher levels. And those that had the highest levels of the antibody, had more severe autonomic dysfunction. And levels of the antibody lower when patients symptoms get better. This indicates cause and effect. Meaning that the antibody can cause POTS and POTS can cause an increase in the level of the antibodies. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2671239/
Cardiac Problems
Usually, doctors try to rule out cardiac disease before any diagnosis of POTS.
Problems with the electrical conduction of the heart such as atrioventricular conduction and ventricular repolarization can cause POTS in some patients. Sinus node abnormalities and abnormal P-waves can also cause it. Some patients undergo a procedure called ablation to treat these type of problems. But studies show that the long-term benefits were not good. And it really isn't recommended as a treatment.
In addition, if a patient has been misdiagnosed with sinus tachycardia, it will make their POTS worse to go through ablation. Heart rate-dependent electrocardiogram abnormalities in patients with postural tachycardia syndrome.
Spinal Problems
Cervical stenosis is when there is an area of the spinal canal that is too narrow, and it compresses the spinal cord and nerve roots. Some patients have had craniovertebral decompression which resulted in a decrease or cessation of their POTS symptoms. But other patients did not benefit from it.
Chiari malformation is when the cerebellar tonsils protrude down into the spinal cord. And it can cause the flow of cerebral spinal fluid to be restricted. Some patients have a procedure to correct the Chiari malformation. But not all of them get better afterward. Some doctors think that Chiari malformation causes all POTS, and some do not. Doctors from the NIH and The Chiari Institue say there is a connection between as well as a connection to EDS/Ehlers-Danlos syndrome. There are lots of links here on Chiari malformation and POTS: http://www.dinet.org/index.php/information-resources/pots-place/pots-useful-links
Syringomyelia is a medical condition caused by a cyst growing in the spinal cord. This sometimes causes POTS. Some people also have Chiari malformation. Many POTS patients also report a worsening of symptoms upon straining. One possible explanation of POTS in syringomyelia patients is partial sympathetic denervation of the legs.
Chemicals
Exposure to some chemicals might cause POTS in some patients. Here is a table of some of the possible chemicals: Chemicals and 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).
There can be many symptoms and problems that are associated with the classical and hypermobile types of EDS. Mitral valve prolapse, gastric emptying or motility issues, dilation or rupture of the aorta, hiatal hernia, premature rupture of the membranes during pregnancy, poor wound healing, bruising, joint dislocation, etc.http://ihpotblogspot.blogspot.com/2016/09/joint-hypermobility-syndrome-and-eds.html
Joint Hypermobility Syndrome is a similar disorder. You can read more on them here:
Electrical injury
Electrical injury and being struck by lightning has reportedly occurred prior to the development of POTS in some cases. Lightning strike and autonomic failure -coincidence or causally related?
Liver Disease
A type of liver disease called compensated cirrhosis is when the liver is damaged but is able to compensate for it. This condition causes hypovolemia and vasodilation, POTS and pooling of blood in the lower extremities. Autonomic dysfunction in chronic liver disease
Mast-cell activation disorders
Some people with POTS or other orthostatic intolerance have flushing, palpitations, shortness of breath, chest pain, headache, lightheadedness, hypotension, or hypertension, and syncope
may play a role in the development of POTS in some individuals. Some patients with orthostatic intolerance suffer from episodes of flushing, palpitations, shortness of breath, chest discomfort, headache, lightheadedness, hypotension or hypertension and occasionally syncope, and it is sometimes brought on by an increase in activity. They may also have fatigue, sleepiness, increased urination, and sometimes diarrhea after they have an attack. There are tests for an increase in urinary methylhistamine, which is a marker of mast cell activation, that can be done to find MCAS.
You can read more about MCAS here: A Tale of Two Syndromes – POTS and MCAS
Neuropathy
Some people with neuropathy have POTS. It may be caused by autonomic neuropathy in the cardiovascular system. There may also be sympathetic denervation in the legs. The Neuropathic Postural Tachycardia Syndrome
Nitric Oxide deficit
Nitric Oxide (NO) controls blood vessel size with through changes in blood flow and blood vessels during inflammation and blood vessel leakiness. If you have a deficit of nitric oxide, you may develop POTS. Nitric oxide and regulation of heart rate in patients with postural tachycardia syndrome and healthy subjects
Researchers have found that NO levels can be increased by blocking the most important receptor for angiotensin-II. This may lead to treatments in the future in select groups of POTS patients.
Norepinephrine transporter deficiency
Norepinephrine transporter deficiency causes POTS is some people. These patients have an irregularity in how norepinephrine is used in the body. Under normal conditions, the body recycles norepinephrine. For some people, the protein that causes norepinephrine to be recycled doesn't work properly. And excess norepinephrine is spilled over. They then have depleted levels of norepinephrine if their neurons continue to be stimulated. They go from having excessive amounts of norepinephrine to having no norepinephrine, at which point they crash.Orthostatic Intolerance and Tachycardia Associated with Norepinephrine-Transporter Deficiency
Some patients have hypermethylation of the norepinephrine transporter (NET) gene promoter. When this happens, the gene for the protein that transports norepinephrine (NET) is turned off because its promoter is turned off.
This is a condition is which the left renal vein is congested because it is being compressed by the aorta and the superior mesenteric artery. It may cause a disruption in the renin-angiotensin system. And it also may disrupt the sympathetic adrenal system and overproduction of catecholamines.
Thyroid disease
Thyroid disease can sometimes cause symptoms that are similar to those of POTS.
Tumors
When the body tries to get rid of a tumor by producing antibodies to attack it, sometimes they also attack part of the nervous system. This is called Paraneoplastic Syndrome. This is a rare condition. If the autonomic nervous system is attacked, then the result can be POTS or dysautonomia. Some people get better after the tumor is removed. Some people have to have intravenous immunoglobulin or other immune modulating treatments to try and reduce the harmful antibody levels.
Physical Trauma, Surgery, and Pregnancy
Trauma such as surgery, pregnancy, bariatric surgery, and Traumatic Brain Injury, car accidents, etc. have been shown to precipitate POTS
Viruses
It is estimated that 50% of patients with POTS have a recent history of some sort of virus when they become ill. It has been associated with Epstein-Barr virus. It is believed that viruses may affect the autonomic nervous system directly or that they may cause an autoimmune response that results in POTS.Postural Orthostatic Tachycardia Syndrome (POTS): A Diagnostic Dilemma
Vitamin Deficiencies
People with digestive problems, that are common in people with dysautonomia, frequently have B12 deficiency.
Don't forget to check out the videos at the bottom of the blog. You have to scroll down to the bottom. There is a good one on norepinephrine transporters and one on the sympathetic nervous system.
Wednesday, September 14, 2016
Joint Hypermobility Syndrome and EDS And The Association with POTS
What is Joint Hypermobility?
Hypermobility is when your joints are more flexible than normal and move beyond the normal range of motion. It is often referred to as being double jointed. When this accompanied by muscle or joint pain but without any systemic disease, it is referred to as hypermobility syndrome. But when it has a more widespread effect on the body it usually involves conditions or syndromes like Marfan or Ehlers-Danlos syndrome.
Girls are more hypermobile than boys are. It tends to run in families, but the exact cause isn't known. It is believed that the genes involved in the production of collagen, which is an important protein for joints, tendons, and ligaments, are involved. Syndromes like Ehlers-Danlos and Marfan are inherited disorders. People with Down's Syndrome are frequently hypermobile. Other conditions associated with hypermobility are SLE/Lupus, fibromyalgia, and chronic fatigue syndrome. And I will eventually get to POTS
Some children do not have any symptoms. But others have joint and muscle pain in the afternoon or evening after they have been active. The knees, elbows, calf and thigh muscles are commonly affected. And the pain gets better after rest.
Children with hypermobility are prone to sprains and soft tissue injury, as well as dislocated joints. They also have something called impaired joint position sense and back pain along with flat feet.
This can cause chronic pain. Their skin may also be loose, and they may have an unusual amount of bruising.
The cramping and deep muscle ache commonly referred to as growing pains are more prevalent in children with hypermobility.
This excessive flexibility lessens as the children get older and the symptoms get better. But for some people, this doesn't happen and they remain prone to dislocations for their whole life.
SOME SIGNS OF HYPERMOBILITY
- Can you touch the floor with the palms of your hands flat while the knees are unbent?
- Can your elbows go beyond straight?
- Can you move your thumb to touch your forearm?
- Can your little fingers be moved so that they are perpendicular to the upper arm?
Treatments for hypermobility has to be individualized. And it is dependent upon the severity of the symptoms. Some children do not require treatment.
Exercise is important. Maintaining good posture when standing and sitting is important. Standing with the knees bent slightly and avoiding over-extending joints is very important. Patients need to wear good shoes that have good arch supports do to flat feet. Physical therapy is necessary in some cases to strengthen the muscles and joints and prevent injuries.
Medicine for pain management usually is limited to acetaminophen an NSAIDS. For severe cases, pain management doctors may be necessary. http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Hypermobility-Juvenile
For more information; http://hypermobility.org/help-advice/
I'm pretty sure I have hypermobility and it seems to run in the family. Because I can do several of these things in the photos. However, mine is probably associated with Lupus, which I will get to later.
Joint hypermobility - have you heard of it?
EDS/ Ehlers-Danlos Syndrome
In general Ehlers-Danlos Syndrome or EDS differs from other types of hypermobility in that there is skin involvement due to collagen problems.
Ehlers-Danlos Syndrome or EDS is a group of heritable connective tissue disorders. It is believed to alter the way collagen works in the body, and can affect multiple systems in the body. Their are certain physical characteristics to each type. Most of the types have known genes that cause them.
Statistically, 1 in 2,500 to 1 in 5,000 people have Ehlers-Danlos Syndrome. It effects both male and females as well as all races and ethnicities.
Common to all types of EDS are hypermobile joints, skin involvement, like soft, stretchy, saggy or think skin, bruising easily, easy wounding and wounds that heal poorly and leave scars.
But each type still has unique features. Usually people don't have more than one kind of EDS.
People with EDS have hypermobility of joints. Their joints frequently dislocate and sublux. Their joints hurt. And they can hyperextend. They usually have osteoarthritis early in life.
Their skin can be described as velvety soft. It is very stretchy, and it is fragile, meaning that it tears and bruises easily, and the bruising can be severe. Injuries leave severe scars, and wounds heal poorly and slowly.
There are also miscellaneous and less common symptoms, depending on type.
Hypermobility type sometimes has chronic, early onset, musculoskeletal pain. Vascular Type sometimes has arterial/intestinal/uterine fragility or rupture; Kyphoscoliosis Type sometimes has scoliosis at birth and scleral fragility; Arthrochalasia Type sometimes has poor muscle tone; and they all can have mitral valve prolapse; and gum disease.
The different types of Ehlers-Danlos Syndrome is differentiated by whatever problem it causes with making or using collagen. Collagen is the protein that the body used to make tissue strong and to make it elastic. Collagen is the most abundant protein in your body and different types are found in the skin, muscles, tendons and ligaments, as well as the blood vessels, organs, gums, and eyes. In normal collagen tissues do not stretch beyond the limit, and will return to normal. Collagen is all over in the body. EDS is a problem with this basic building material in the body. The structure of the collagen is defective. Because of this tissue can be pulled beyond what is the normal limits and this results in damage.
Because collagen is in so many areas of the body EDS is a systemic problem.
Ehlers-Danlos Syndrome is divided into six different types, and several different mutations that don't fit into those categories. There are different signs and symptoms for each type. And a child who has EDS will always have the same type as their parent has. A parent who has Classical Type Ehlers-Danlos Syndrome will not have a child with Vascular Type Ehlers-Danlos Syndrome. The types are:
- Hypermobility Type
- Classical Type
- Vascular Type
- Kyphoscoliosis Type
- Arthrochalasia Type
- Dermatosparaxis Type
- Other Types
You can read more about the types here: http://ehlers-danlos.com/eds-types/
The diagnosis of Ehlers-Danlos Syndrome is based on specific criteria depending on the type. Diagnosis is made based on the family history and clinical observations. Genetic testing can be done for Ehlers-Danlos Syndrome, except in the most common type, Hypermobility Type.
Different EDS types have different prognosises. Those who have Vascular Type EDS have shortened life expectancy because of the possibility of organ and blood vessel ruptures. The other types of EDS do not have a shorter life expectancy. The severity of the condition varies from person to person even within families. Each case is unique to the patient. There is no cure for Ehlers-Danlos Syndrome, but the symptoms can be treated.
http://ehlers-danlos.com/what-is-eds/
https://www.facebook.com/ehlers.danlos/posts Ehlers-Danlos Society
Other Types of Hypermobility
Marfan Syndrome
OsteogenesisImperfecta
Sticklers Syndrome
Pseudoxanthoma Elasticum
I am not going to cover all of them, but I am going to go over Marfan Syndrome and the association of Lupus with hypermobility. And then POTS.
MARFAN SYNDROME
Marfan syndrome is also genetic. It affects the connective tissue by in a different way than EDS.
There are other proteins that make up connective tissue besides collagen. In Marfan Syndrome the problem is with a protein called Fibrillin. There is not enough of it. And there is a problem with how it interacts with Transforming Growth Factor – Beta. This causes the tissues to be weak and fragile.
Because connective tissue holds all of the bodies cells, organs and tissue together, it is involved in how the body grows and develops. With Marfan Syndrome, the aorta, bones and eyes are involved.
With Marfan Syndrome there can be aortic enlargement, which can be life threatening. The lungs, skin and nervous system can also be affected.
Around 1 in 5,000 people have Marfan syndrome. This includes men and women of all races and ethnic groups. Around 75% of people with Marfan syndrome inherit it, meaning it is a genetic mutation. Some people with Marfan have no other family members who have it and this is called a spontaneous mutation. People who have Marfan Syndrome have a 50% chance of passing it on to their children.
Even though people with Marfan Syndrome are born with it, signs and symptoms do not always show up immediately. Sometimes complications like aortic enlargement do not show up until later in life. Marfan affects heart and blood vessels, as well as bones and joints, and it is usually a progressive condition.
Early diagnosis is key in MFS. It is important to have good medical care if you have Marfan Syndrome/ MFS. Patients are at risk for life threatening complications.
People with MFS can have aortic tears as well as bulging aorta called an aortic aneurysm. There is also sometimes mitral valve prolapse and congestive heart failure.
With the lungs, besides lung collapse, patients can have asthma, emphysema, and sleep apnea.
Some of the signs of MFS are obvious, although each patient will have different combinations of them:
- Long arms, legs and fingers
- Tall and thin body type
- Curved spine
- Chest sinks in or sticks out
- Flexible joints
- Flat feet
- Crowded teeth
- Stretch marks on the skin that are not related to weight gain or loss
Some of the other signs are sudden lung collapse and eye problems that include severe nearsightedness, and sometimes farsightedness called hyperopia, detached retina, dislocated lens, early glaucoma and early cataracts. There is also a condition called strabismus, where the eyes don't line up and look the same direction at the same time, similar to lazy eye.
The most obvious sign of Marfan Syndrome is how the people are built they are unusually tall, with unusually long arms, legs and fingers. Abraham Lincoln may have had Marfan Syndrome. Abraham Lincoln and Marfan Syndrome He had disproportionately long limbs and fingers.
Marfan's Syndrome - CRASH! Medical Review Series
Marfan Syndrome Diagnosis and Treatment-Mayo Clinic
Lupus and Hypermobility
"Thirty nine (48%) patients with SLE and 42 (15%) of the control group were hypermobile. A logistic regression model was developed. The odds ratio of the association between laxity and SLE after adjustment for age and sex was 2.31 in the group younger than 49 years, and 17.99 in the group aged 49 years or older. Neither the clinical and analytical profile nor the use of corticosteroids was related to joint laxity.
Conclusion: Patients with SLE showed more hypermobility than controls. Hypermobility was more profound in older patients with SLE (≥49 years). Joint laxity was not associated with any clinical or analytical pattern. Treatment with corticosteroids was not related to joint laxity." Annals of Rheumatic Disease The Eular Journal, Association of systemic lupus erythematosus and hypermobility
Eighty percent of patients with Ehlers-Danlos Syndrome hav POTS. But not all POTS patients have EDS. Those patients who do have EDS usually have Type III EDS, the hypermobility type.
Lupus and EDS and MFS all cause POTS at least in part because they have connective tissue abnormalities which allow excessive amounts of blood to pool in these patients' lower limbs when they stand up. In the case of Lupus, there can also be all of the neurological autonomic system problems.Autoimmune disorders,such as Guillain-Barre (Singh, Jaiswal, Misra & Srivastava, 1987) and lupus are suspected of causing POTS symptoms in some individuals.
"Researchers have discovered an antibody to neuronal nicotinic acetylcholine receptors of autonomic ganglia (Vernino, Low, Fealey, Stewart, Farrugia & Lennon, 2000). Some people with POTS have an antibody titer test that is positive to this antibody. Patients with orthostatic intolerance, anhidrosis, constipation, urinary dysfunction, sicca syndrome and pupillary dysfunction had higher antibody titers than subjects that did not (Gibbons & Freeman, 2009). Patients with the highest levels of these ganglionic-receptor-binding antibodies have the most severe autonomic dysfunction. Physicians have discovered that antibody levels lower as some patients improve, which suggests a cause and effect relationship. Patients interested in being tested for the ganglionic antibody should have their physician contact:"
Mayo Medical Laboratories
1-800-533-1710
mml@mayo.edu
"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)." http://www.dinet.org/index.php/information-resources/pots-place/pots-causes
Don't forget to check out the POTS videos at the bottom of the blog. You have to scroll down to the bottom to find them.
Connecting the Dots Between EDS and POTS
PoTS and Hypermobility Syndrome - Blair Grubb, MD
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