The Terrible Trifecta – Connecting the Dots of EDS, MCAS and POTs

In the first blog of this series, we looked at hypermobility and the connective tissue disorder Ehlers-Danlos Syndrome (EDS). We learned about the common symptoms of EDS and how this can impact someone’s life. I hope you enjoyed learning more about this condition.

We mentioned in that blog two other conditions that can often go hand in hand with EDS :

• Postural Orthostatic Tachycardia Syndrome (POTs)

• Mast Cell Activation Syndrome (MCAS)

Some people have all 3 conditions, and it has become known as The Terrible Trifecta.

But what are these other conditions?

POTS

Postural Orthostatic Tachycardia Syndrome is a disorder of the autonomic nervous system. It comes under the general umbrella term of a form a dysautonomia. Our autonomic system controls all the things in our body we are not consciously aware of, like blood pressure, heart rate, pupil dilation, movements of the digestive tract and body temperature. POTs can therefore affect the heart rate, blood vessel dilation, blood pressure, blood pooling and body temperature. One of the key features of POTs is orthostatic intolerance.

What’s orthostatic intolerance?

If the blood vessels are not constricting as they should, when we move from sitting to standing, there can be blood pooling in the legs and abdomen. The blood vessel constriction is designed to pump the blood up the legs when we stand up. If this doesn’t happen, there can be a shortage of blood in the heart and brain. Standing up can cause dizziness, light-headedness and even fainting. This can be especially common after eating, as lots of energy is directed to the digestive tract.

To help avoid these dizzy episodes happening, I recommend doing some simple ankle circles or calf pumps before you stand up or get out of bed in the morning. This helps pump the blood up the legs and can prevent dizziness. Try not to stand up too quickly so that you can give the body a chance to adjust to the change in position. Some people also find increasing their water intake and wearing compression garments useful. A medical doctor can also prescribe sodium tablets or other medications if necessary to help control the POTs.

The majority of people with POTs are women aged between 15 and 50. It is not known what causes POTs but it sometimes seems to be triggered by what the nervous system considers to be a trauma of some kind. For some people, trauma can be coming into puberty and having a big hormonal change, for some it is pregnancy or menopause. For others is can start after a viral disease like glandular fever or after a surgery. Everyone will respond differently to events in their life, and this makes the onset of POTs very unique for each individual. The symptoms can range from mild to extremely disabling.

Like EDS and other connective tissue disorders, POTs can be an invisible illness. It can not be seen on the outside but can be a very disabling condition for many people This can make it hard for people to understand what is going on as someone can look well and healthy on the outside. Like EDS, there is still a lack of understanding and awareness about POTs and how it can present itself and disrupt someone’s life. Living with POTs can be extremely challenging both physically and mentally.

I have worked with clients who are unable to be upright for long periods of time, they suffer regular fainting episodes especially with exercise, they can experience brain fog and cognitive dysfunction. Exercise must be carefully approached to avoid increasing dizziness and discomfort.

The good news is there are safe options for exercising with both EDS and POTs and we will be exploring this in more detail in the next blog in this series.

MCAS

Mast Cell Activation Syndrome is a complex condition, but I’ll try to explain it as simply as possible here. It is a condition that causes the mast cells in the body to release an inappropriate amount of certain chemicals into the body. Everyone has mast cells – they are essential blood cells that are part of your immune system. When you get sick, the mast cells activate to help you fight infection and inflammation and return to health.

If you suffer from hay fever for example, mast cells are responsible for this allergic reaction to pollen. I explain that having MCAS is like having hay fever in any part of your body. Because the mast cells are everywhere in the body, pretty much anything can be impacted by a mast cell reaction. But what does happen if those allergy reactions start overworking? In a healthy individual, mast cells release chemicals called mediators. One of these chemicals is histamine. A reaction could be itchy skin, swollen skin, hives, mucus, headaches, wheezing and breathlessness. These reactions help to protect and heal the body and they should be short lived until the reaction passes.

In someone with MCAS however, these reactions can have a very negative impact on the body. They can become part of everyday life and something that must be managed carefully. The ‘normal’ reactions we need to protect us start to go into overdrive and create an excessive response to things that should not normally be considered a ‘threat’.

For example, people with MCAS can react inappropriately to

• Heat

• Cold

• Perfumes

• Foods

• Medications

• Infections

• Stress

• Exercise

In addition to the normal reactions listed above, someone with MCAS may experience all or some of these too:

• Inflammation

• Vomiting

• Chronic pain

• Constipation / Diarrhea

• Palpitations

• Flushing

• Low blood pressure

• Chronic Fatigue

• Sweating

Again, this is another invisible illness – it is not well recognize in the medical world yet and getting a diagnosis and treatment can be challenging. There is no cure, like EDS, so it is a case of managing the symptoms and calming the mast cells down so that they do not continue to overreact.

Medications like mast cell blockers and antihistamines can be prescribed together with lifestyle changes like reducing stress, mindfulness, meditation, pacing, dietary changes and getting adequate rest.

Living with all three conditions

Having had an overview of all three conditions – EDS, POTs and MCAS – can you imagine how it would be to live with these all at the same time? It can be very challenging at times with the need to balance many different aspects of life to avoid flare-ups of the conditions.

Unfortunately, many people do live with all three and that includes me. I was diagnosed with hypermobile EDS in 2018 after developing MCAS due to a very stressful event in my personal life. And although I always suspected I had symptoms of POTs, I was officially diagnosed in 2021 following a Covid-19 infection. As we discussed above, POTs is often triggered or made worse after a viral illness.

As a movement therapist, I was well placed to help myself rehabilitate my body and mind from the chronic pain of EDS and hypermobility. I now work with individuals all across the globe to help them navigate The Terrible Trifecta, or maybe just one aspect of this trio. Everyone is so different, everyone responds uniquely to these conditions, and everyone has a different story to tell.

My role as a movement therapist is to help people find a safe, effective way to move well so that the body and mind can help calm the nervous system – ultimately helping to reduce the impact of these conditions on everyday life.

In our next blog, we will be examining how to find that safe, effective movement therapy program. You can read more about my movement method in my two books – Pilates Without Tears and Hypermobility Without Tears.

My YouTube channel has a variety of movement classes designed for these conditions that you can try at home too.

Jeannie

Movement Therapist and Hypermobility Specialist

www.jeanniedibon.com

www.thezebra.club