The EDS Cluster refers to the three diseases of Ehlers-Danlos syndrome (EDS), postural orthostatic tachycardia syndrome (POTS), and mast cell activation syndrome (MCAS), which are commonly associated in patients. For those living with EDS, POTS, and MCAS, life can often be challenging. These conditions can cause severe physical pain, dizziness, fatigue, difficulty concentrating, and other symptoms. But by understanding the severity of these conditions, as well as the treatments and coping mechanisms that can help manage them, life can become a bit more manageable. This blog offers information on what EDS, POTS and MCAS are, the severity of their symptoms, common treatments for the conditions, techniques for daily life management and tips on how to cope with them. Read on to understand these conditions better and gain the tools you need to manage your life with these chronic illnesses!
What is the Ehlers-Danlos Syndrome (EDS)?
Ehlers-Danlos Syndrome (EDS) is a disorder of the connective tissues that affects many parts of the body. It is most commonly associated with other conditions, such as Postural Orthostatic Tachycardia Syndrome (POTS) and Mast Cell Activation Syndrome (MCAS).
EDS is a genetic, “rare” disease that affects the connective tissue of the body. There are 13 types of EDS, with Hypermobile EDS (hEDS) being the most common.
There is connective tissue in every organ system of the body, meaning the EDS can affect all areas of a person’s body – from the joints to the gut, to the vascular system, to the neurological system, and everything in between. Every person’s EDS is unique, even within the same types.
I have hEDS, which is the only type of EDS that doesn’t have a defined genetic marker at this time (it exists, but scientists haven’t found it YET). As mentioned before, this is the most common type of EDS, affecting about 90% of patients with EDS. It is believed that 1 in 3,100-5,000 people have hEDS.
With hEDS, we are said to have faulty collagen, as collagen is the fundamental part of connective tissue. Connective tissue’s strength comes from the amount and quality of collagen in it. With this deficiency, all areas of the body are affected.
EDS can cause a range of symptoms, from joint pain, muscle weakness, fatigue, skin fragility, digestive problems, headaches and more. Diagnosis of EDS typically involves genetic testing or evaluation by a specialist in musculoskeletal disorders to determine if it is indeed EDS or another condition which has similar symptoms but requires different management methods for optimal outcomes.
What is Postural Orthostatic Tachycardia Syndrome (POTS)?
POTS is a disorder that affects the nervous system and circulation. It is most common in people who are genetically predisposed to it, and is most commonly associated with other conditions such as EDS (Ehlers-Danlos syndrome) or MCAS (mast cell activation syndrome). Symptoms of POTS can include fatigue, dizziness, low blood pressure, and difficulty concentrating. If left untreated, POTS can lead to more serious disorders such as heart failure or even death. If you or someone you know is experiencing any of the symptoms of POTS, it is important to seek medical attention.
How POTS manifests for me is that I get lightheaded, dizzy, or fully pass out when I go from a sitting to standing position. POTS symptoms are usually associated with a change from laying down to sitting, and/or from sitting to standing. What happens is that our blood vessels do not constrict properly (due to our faulty collagen), and blood rushes from our head to our legs when we change positions.
What is Mast Cell Activation Syndrome (MCAS)?
Mast Cell Activation Syndrome (MCAS) is a chronic, underlying condition that can be associated with Ehlers-Danlos Syndrome (EDS) or Postural Orthostatic Tachycardia Syndrome (POTS). It’s caused by an overproduction of mast cells, which can lead to various symptoms such as fatigue, headache, hives and difficulty breathing. Treatment for MCAS usually involves medications to reduce the production of histamine in the body, and lifestyle modifications including stress reduction techniques and dietary changes. It’s important to work closely with your healthcare team when managing any type of EDS or POTS related syndrome like MCAS in order to get the most effective treatment possible.
My MCAS is quite severe – it is actually how I started my journey to being diagnosed with the cluster (see my diagnosis story here). I get hives just from looking at something funny. Ok, not really, but it does feel like it. The interesting thing about MCAS is that you can develop a new allergy at any time. That means you could be out to dinner eating your favorite meal, and then you can go into anaphylactic shock from said favorite meal (it happened to me). That is why carrying your Epi-pen is so important.
Understanding the Severity of Symptoms in EDS, POTS, and MCAS
Ehlers-Danlos syndrome (EDS), postural orthostatic tachycardia syndrome (POTS), and mast cell activation syndrome (MCAS) are related disorders that share common symptoms. Understanding the severity of symptoms in each disorder is essential to creating an individualized treatment plan. Symptoms can vary depending on the severity of the disorder, but may include chronic pain, fatigue, dizziness, fainting spells, digestive issues, and more. Treatment for EDS, POTS, and MCAS depends on understanding the individual’s specific combination of symptoms and creating an effective plan. By working together with a healthcare professional, you can get the most comprehensive care for you or your loved one.
Treatments for EDS, POTS, and MCAS
Chronic illnesses like EDS, POTS, and MCAS are often referred to as “inter-related chronic illnesses.” This is because they share common features and can be treated with a variety of lifestyle modifications and medications. Some of the most common treatments for EDS, POTS, and MCAS include avoiding triggers such as processed foods and stress, getting enough sleep and exercise, drinking plenty of water or herbal tea to stay hydrated, and taking medications such as antihistamines or antidepressants depending on individual needs. In addition to lifestyle changes, other non-medication options, like massage therapy and acupuncture, may help relieve pain associated with these conditions.
Managing Daily Life With These Conditions
Living with Ehlers-Danlos Syndrome (EDS), Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Disorder (MCAS) can be a difficult experience. It is important to practice self-care by eating a healthy balanced diet, getting plenty of rest, and staying hydrated throughout the day. Exercise also helps to manage symptoms as it can help increase energy levels and reduce pain and fatigue associated with EDS/POTS/MCAS. In addition to self-care, support groups are available for those who suffer from these conditions. Similar to this blog, they offer an outlet to share experiences, tips, and advice on how best to cope with living life while managing these chronic illnesses daily. I’ve had a lot of luck with Facebook groups!
Tips on how to cope with these conditions
Here are some tips on how to cope with EDS, POTS and MCAS. First and foremost, it is important to speak to your doctor and find out what treatment options are available. Make sure to get enough rest, eat the right foods, exercise regularly and maintain a healthy lifestyle. In addition to this, learn about strategies like mindfulness, which can help manage symptoms related to EDS, POTS & MCAS. If you’re looking for more mental health support, talk therapy with mental health professionals may be beneficial for managing the stress associated with these conditions.
Why are people with EDS called zebras?
In medical schools all over the world, baby doctors are taught the phrase, “If you hear hoofbeats, think horses, not zebras.” In this way doctors are instilled with the understanding that they should always look for the most common cause of a person’s symptoms, and never look to the ultra-rare diseases. However, like our motto here at Living with the EDS Cluster (when you hear hoofbeats, sometimes it is a zebra) says, there are those rare diseases out there. It is also thought that people with EDS are referred to as “zebras” because everyone’s EDS is different, like how no two zebras have the same stripes.
Conclusion
It is evident that Ehlers-Danlos Syndrome (EDS), Postural Orthostatic Tachycardia syndrome (POTS), and Mast Cell Activation syndrome (MCAS) are all inter-related conditions with common symptoms and treatments. Although these chronic illnesses can be difficult to manage, there are ways to cope and lead a normal life. With the right support, education, and lifestyle changes, anyone suffering from EDS, POTS, or MCAS can find hope and healing. If you’d like to learn more about living with these conditions, check out some of our other posts!
Here are some resources I used to write this article:
