Mast Cell Activation Syndrome (MCAS), Postural Orthostatic Tachycardia (PoTS), and Ehlers-Danlos Syndrome (EDS)
Written By: Hannah Chapeskie
Dietetic Intern, Acadia University
Mast Cell Activation Syndrome (MCAS), Postural Orthostatic Tachycardia (PoTS), and Ehlers-Danlos Syndrome (EDS) are three conditions that commonly occur together and affect women more often than men. These rare conditions can go mis- or undiagnosed as their symptoms can overlap with other conditions and many physicians are unaware of their existence. Keep reading to learn about these conditions.
Have you been diagnosed with one of these conditions and are seeking dietary guidance? Book with one of our dietitians today!
Mast Cell Activation Syndrome (MCAS)
Mast Cell Activation Syndrome (or MCAS) is a rare condition that involves repeated episodes of allergy symptoms or anaphylaxis, often without a known trigger. Mast cells are cells that are a part of our immune systems and are responsible for allergy symptoms. When a person encounters their allergy trigger(s), these cells release chemicals (called mediators) that cause inflammation and allergy symptoms. In MCAS, a person’s mast cells produce these chemicals in excess or in response to unharmful triggers such as foods, emotions, pain, fatigue, heat, smells, hormonal changes, and even the sun. A person with MCAS can have multiple triggers that change over time making it very difficult to identify exactly what is causing their symptoms.
The symptoms of MCAS can be different for every person. Symptoms can range from mild to anaphylaxis and can change over time. MCAS symptoms can affect many parts of the body including:
It is unknown what causes MCAS, but it is thought to be genetic. 74% of patients have at least one first degree relative who also suffers from this condition.
Your doctor will review your medical history, complete a physical exam, and a urine and/or blood test. You may also be asked to keep a log of your reactions including potential triggers to help narrow down the cause or causes.
In order to be diagnosed with MCAS, you must meet the following criteria:
There is currently no cure for MCAS, but symptoms can be managed with lifestyle changes and medication. If you are able to identify triggers, try your best to avoid them and seek guidance from a dietitian if it requires altering your diet. It is also recommended that people with MCAS avoid using NSAIDS like aspirin and ibuprofen as they can trigger a reaction. You may be prescribed medication such as antihistamines, mast cell stabilizers, anti-leukotrienes, or corticosteroids to help treat symptoms and supress reactions.
Have you been diagnosed with MCAS and are seeking dietary guidance? Book with one of our dietitians today!
Postural Orthostatic Tachycardia Syndrome
Postural Orthostatic Tachycardia (or PoTS) is a rare condition that involves feeling dizzy or fainting when going from a seated, or reclined position to standing, and sometimes when standing for long periods of time. When a healthy person stands, their blood vessels constrict, and heart rate slightly increases to allow blood to continue to get to their heart and brain. In PoTS, the nervous system does not properly adjust to this change in body position resulting in an extreme increase in heart rate, a restriction of blood flow to the brain, and an increase in the chemical norepinephrine in the blood which is responsible for our fight or flight response. PoTS affects about 1% of the population and 90% of those diagnosed are female.
The symptoms of PoTS can be different for every person and can range from mild to debilitating. Symptoms tend to be worse in the morning and can persist for 2-5 months or lifelong. In PoTS, symptoms are triggered when one goes from laying or sitting to standing, and/or during periods of prolonged standing. Symptoms can include:
The cause of PoTS is unknown, but it is thought to be genetic. Some abnormalities that may cause PoTS include impaired nerve function, prolonged bedrest that leads to loss of muscle tone and heart strength, as well as an elevated fight or flight response. Symptoms seem to begin after life events such as puberty, pregnancy, major surgeries, excessive blood loss, viral illnesses, and/or menstrual periods.
Your doctor will review your medical history and complete a physical assessment. They will also do routine blood work and may use an ECG to rule out heart conditions. If your doctor suspects that you may have PoTS, you will likely undergo the Active Stand Test which is where your heart rate and blood pressure are measured when you are lying down, and then 2, 5, and 10 minutes after you stand up.
In order to be diagnosed with PoTS, you must meet the following criteria:
There is no cure for PoTS, but diet and lifestyle changes, as well as medication can help manage symptoms. You may be prescribed a high sodium diet of 10g of salt/day, or sodium tablets if it is safe in your situation. You will also be encouraged to drink 2-3L of fluid/day and to avoid large meals of refined carbs as this can trigger symptoms.
Patients with PoTS often experience symptoms while exercising, leading them to avoid exercise which can make PoTS symptoms worse. Individuals with PoTS should progress to 30mins of exercise, every other day. Participating in exercises that do not require standing such as swimming, rowing, recumbent biking, and/or Pilates until symptoms improve can be beneficial.
Wearing class 2 compression tights to help with symptoms and avoiding triggers such as heat and prolonged standing help with symptom management. Learning and practicing maneuvers to prevent fainting such as crossing the legs and clenching the fists and abdominal muscles can be helpful when it is not possible to avoid prolonged standing.
Medication is used as a last resort in PoTS as most patients can find relief in diet and lifestyle changes. Medication that increases blood pressure, increases blood volume, and/or slows heart rate can be prescribed if necessary.
Have you been prescribed a high sodium diet for PoTS symptoms? Our dietitians can help you with this dietary change!
Elhers-Danlos Syndrome (or EDS) is a group of disorders that affect the connective tissues in the body which includes bones, cartilage, ligaments, tendons, and skin. In EDS, the structure or production of collagen, the main protein that makes up our connective tissues, is abnormal due to defective genes. EDS affects 1/5,000 people and occurs more commonly in women. There are a total of 13 different types of EDS with the most common being Classic EDS and Hypermobile EDS.
In all classes of EDS, sufferers experience pain and fatigue. However, symptoms can vary between each type of EDS. In the two most common types, symptoms can include:
All classes of EDS are genetic meaning the condition is inherited.
Your doctor will review your medical and family history. During a physical exam, your doctor will assess for hypermobility in your joints and will note the texture of your skin, how easily it scars, and how much it stretches. If available, you can complete genetic testing using a blood sample to determine exactly which of your genes is abnormal, allowing your doctor to identify which type of EDS you have (genetic testing is not completed for Hypermobile EDS as we do not know which gene is affected in this condition).
To be diagnosed with Hypermobile EDS, you must:
There is currently no cure for EDS. Those with EDS greatly benefit from receiving regular physical therapy to strengthen muscles and ligaments to prevent dislocations and pain. You may be advised to avoid contact sports and lifting weights and to try wearing assistive devices that eliminate pressure on the joints. When outside, individuals with EDS should always wear sunscreen as their skin can be very delicate and requires protection. In some cases, a patient may require surgery to repair damaged or dislocated joints and may be prescribed pain medication if experiencing regular, significant pain.
These three conditions, though rare, are becoming more common. They most often impact women and can occur together as a triad. If you suspect that you may have Mast Cell Activation Syndrome (MCAS), Postural Orthostatic Tachycardia (PoTS), Ehlers-Danlos Syndrome (EDS), or a combination of these conditions, book an appointment with your physician for guidance.
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