Tag: Ehlers danlos syndrome

Personalised Exercise Strategies for Managing POTS, Hypermobility, and EDS

Posted on by Sharon Hennessey

Navigating life with Postural Orthostatic Tachycardia Syndrome (POTS), hypermobility, or Ehlers-Danlos Syndrome (EDS) presents unique challenges, especially in the realm of exercise. While exercise is important for overall health and is part of the rehabilitation process, the traditional approach to exercise may not always be suitable for individuals with chronic health conditions. Utilising a personalised approach that acknowledges and adapts to the individual is crucial to long-term success. In this blog, we will delve into how exercise can be personalised to your needs. We will also discuss how to understand and apply the Rate of Perceived Exertion (RPE) Scale to your activities to help find appropriate levels of exercise intensity.

Foundations for a Personalised Approach

Successfully managing POTS, hypermobility, and EDS through exercise incorporates cardiovascular exercise in combination with strength training. You may have heard of or been recommended to complete the Levine or Dallas/CHOP Protocols for POTS rehab; these protocols were adapted from the training methods used in POTS research studies. Based on research the gold standard is to do 3-4 days of cardio and 2 days of strength exercises. They typically are set out in the following phases:

3 phases of POTS exercise rehab

  • Phase 1 (1-3 months): Exercises with low orthostatic challenge such as recumbent cycling, rowing ergometer or swimming
  • Phase 2 (4-5 months): Introduces upright cardiovascular exercises such as stationary biking and graded walking, as your body starts to adapt.
  • Phase 3 (6 months and beyond): Further increases the challenge of upright exercise, including the elliptical machine and treadmill.

Fatigue, pain, and associated symptoms of ME/CFS (chronic fatigue syndrome), can be a barrier to even getting started and meeting the frequencies recommended. Fatigue is also the main reason for participant drop out in research studies.

Using the basis of these research protocols and considering your individual situation is how ‘Not Just Bendy’ can help you come up with a personalised exercise program that you can tolerate.

Making Exercise Work for You

Exercise is not one-size-fits-all, especially for those with chronic health conditions. Each person’s experience with POTS and hypermobility varies, and factors such as current symptoms, energy levels, and coexisting conditions must be considered when developing a program. What works for one person may not work for another.

Many people associate exercise with intense workouts that leave them feeling exhausted and out of breath. A big part of our education with clients is reframing this and understanding that exercise doesn’t have to be hard and exhausting to be beneficial. Even short bouts of light activity can have positive effects.

When energy levels and pain are a barrier to exercise, it’s essential to listen to your body and adjust your exercise routine accordingly. This may mean modifying the type, intensity, and duration of exercise based on how you’re feeling that day. Pacing, avoiding boom and bust cycles and consistency are key. Remember that progress may not always be linear, and it’s okay to experience setbacks. Life changes, increased stress or illness, may necessitate adjustments to your exercise routine. Having an adaptable program ensures that exercise remains a sustainable and enjoyable part of your lifestyle.

Below we will share two stories of client programs based on the above principals and how we at Not Just Bendy are able to tailor POTS rehabs to those with varying severities of POTS and symptomatic hypermobility/EDS.

Margret’s Story:

Margret* is in her mid-40’s and has both H-EDS and POTS. Margret works full-time and is a busy parent. She has been active all her life however always found cardiovascular exercise particularly challenging; her HR would get high very quickly and would feel faint and dizzy after an extended period. In recent years, Margret was diagnosed with POTS and takes a heart lowering medication for management. Currently, she attends a group exercise class 2-times a week to focus on global muscle strengthening and completes a small home program for joint-specific issues but did not find that her POTS and fatigue were improving.

As part of her program updated through Not Just Bendy she has included POTS specific cardiovascular exercises, and has now progressed to an upright, interval-based program. The base program is done on an upright bike with a 5-10mins warm up, followed by 5-rounds of 45sec higher intensity intervals and 2mins active rest, then finishing with a 5-10min warm up. Margret was taught to self-adjusts this program depending on how her body is feeling each day. Due to various external factors such as fatigue and stress she may is not able to tolerate a full 5-rounds of intervals, on those days she will do a shorter session. Margret’s gym can be quite warm and she is not always able to tolerate upright exercises, so she follows the same program on a recumbent bike. The current progression plan for Margret is to work on decreasing her rest periods with her interval training.  Margaret now feels more energised after the gym (instead of drained) and has spoons left to complete her daily tasks easily.

Charlotte’s Story:

On the other flipside we have Charlotte* who is in her late 20’s with generalised-joint hypermobility, ME/CFS and POTS. Charlotte has been unwell the past few years with pain and fatigue being major limiters to her day-to-day function. As such she was often bedbound and deconditioned when she first attended Not Just Bendy.

Her initial program and sessions involved education on symptom management and pacing. All her rehab exercises are down laying down focusing on improving joint stability and isometric strength exercises for the leg muscles. She has been working on slowly increasing her upright tolerance by increasing sitting time and take a walk around the house when able. Six months in, we have now been able to introduce gentle seated stationary pedal exercises working starting once a week for 5mins at a constant pace, low intensity, and building towards increasing her exercise duration. Charlotte has much improved tolerance for short walks around her home and has ventured to the local coffee shop with a friend in her wheelchair which has been a goal for a few years.

The RPE Scale Explained

The RPE scale ranges from 1 to 10. It provides a subjective rating of how intense an exercise feels. The scoring is completely individualised to each person and is variable over time. It is a simple measure that does not depend on other metrics such as speed, weight, or heart rate (which is not always reliable for those on heart rate-lowering medication for POTS). We can use this simple measure to adjust exercise intensities accordingly to your needs.

RPE scale

The RPE Scale:

  • 1-2: These levels indicate very light activity. You should be able to converse easily without any breathlessness.
  • 3-4: Activities falling into this category are considered moderate. You might notice your breathing rate increase, but you’re not out of breath.
  • 5-6: Here, you’re entering a zone that’s challenging yet still manageable. Conversation becomes harder, signalling increasing cardiovascular effort.
  • 7-10: These levels represent high to maximum effort. Speaking is very difficult due to the intensity of the exercise, indicating that you’re pushing the limits of your capacity. Activities in these zones are not intended to be sustained for long periods.

Applying RPE in Your Exercise Program

As mentioned earlier RPE is personalised and variable over time. It allows for day-to-day adjustments based on how you feel, ensuring that you’re exercising within an appropriate and beneficial intensity range for each given day.

  • Starting Low: Begin with exercises that are low on the RPE scale, particularly if you’re new to exercising. This might include activities like your rehab program, mobility exercises, gentle swimming or recumbent cycling.
  • Monitoring Progress: As you become more comfortable, gradually increase the intensity of workouts such gentle walking, upright cycling and progressed strength exercises to higher RPE ranges. As you become more aware of your body and how it responds to exercise, you can also use RPE to help keep your day-to-day activities within a range that does not cause further fatigue and pain flare ups-post.
  • Adjusting for Symptoms: On days where symptoms are more pronounced your RPE may not always be at ‘0’ when resting, on those days you may only have the capacity to increase you RPE a little during exercise a little or are better to rest instead.

Exercising with POTS – Tips for Success

6 tips for exercising with POTS

1. Avoid Exercising in the Heat – Exercising in hot conditions can worsen POTS symptoms.

2. Minimize Postural Changes – Plan workouts to limit the need for frequent changes in posture, reducing the strain on your cardiovascular system.

3. Hydration and Electrolytes – Consuming fluids and electrolytes 20-30 minutes before working out helps expand blood volume, potentially reducing exercise-induced symptoms. Keep hydrated throughout your session to maintain blood volume.

4. Medication Timing – Timing exercise with the peak effectiveness of your medication can improve exercise tolerance and reduce symptoms whilst exercising.

5. Food Timing – Avoid eating 2 hours before exercising to minimize gastrointestinal discomfort and reduce blood flow diversion to the intestines for digestion.

6. Pace Yourself – It’s crucial to progress at your own pace. Active recovery and rest are just as important as progression, ensuring that every step forward is sustainable.

Exercise, when appropriately tailored is a powerful tool for those living with POTS, hypermobility, and EDS. Your body’s responses, your progression pace, and your collaboration with your practitioner are the foundations to a successful program. Each person will progress through each ‘Phase’ at their own pace. By utilising a personalised approach to exercise, “Not Just Bendy Hypermobility Services” is committed to supporting you every step of the way in your journey.

Referral to Our Exercise Physiologist and Physiotherapists for POTS management

If you’re looking for personalized support and guidance in managing POTS, hypermobility, or EDS through exercise, our in-house exercise physiologist, Avery, is here to help. Avery specialises in creating tailored exercise programs that cater to your unique needs and health conditions. Additionally, we have a team of skilled physiotherapists who are experienced in working with individuals with hypermobility and EDS. To learn more about our services or to schedule a consultation, please visit our website and explore our team of professionals. Let us support you on your hypermobility journey.

Exercise Physiologist of NJB - Avery Wu

References:

Mast Cell Activation Syndrome (MCAS) and its Relationship to Hypermobility

Posted on by Sharon Hennessey

There is a significant overlap in the conditions and the symptoms of MCAS, POTS and symptomatic hypermobility. Learn more in this informative blog.

From an early age, I never really coped with band aids and would always end up with a rash.  By mid 20s, I knew using strapping tape on myself was not a solution due to the harsh skin reactions (a problem, as I was burning out as a physio working 55 hours a week and I needed external joint support via tape to get through). Gardening also started to become problematic, as my skin would have a similar reaction if Australian Native Plants touched me.

I knew I was odd and reacted in strange ways to medications – lots of side effects for some and no effects for others.  I started to learn to only try new medications on a weekend and at half the recommended dose to give my body time to recover.  I, of course, battled my hypermobility and POTS without a lot of understanding until my children were born in my early 30s. Then, I began to realise a lot of my management strategies (rest, electrolytes, pacing, salt, strength exercises) finally made sense.

But Mast Cell Activation Disorder (MCAS) caught up with me when I was in my early 40s and suffered with idiopathic urticaria (unexplained hives all over my body).  Everyone told me I was stressed or allergic to something, but I seemed to be allergic to the world!  Anything I touched or anywhere I had clothing – socks, shoes, underwear, and pants left me with raised welts for days. I now know this is called dermatographia. Dermatographia, also known as skin writing, is a condition in which the skin becomes raised and inflamed when it is stroked or scratched lightly. This condition is believed to be caused by an over-reaction of the mast cells in the skin.

My first bout of urticaria lasted about 3 months and settled with antihistamines. Eventually, I forgot all about it as it was just another “one of those things.”   However, the second bout started 9 months later and lasted for nearly 18 months.  Imagine having 400 mozzie (mosquito) bites all over your body, extremely itchy all the time.  If I scratched them, I ended up bruised.  It wasn’t until I took a truck load of medication and injections (see the medications list below as I had them all) AND took mast cell stabilisers, that I am proud to say no hives for nearly two years! This also helped my gut to function better which was a major improvement of my quality of life.

Now, I am still allergic to tape and plants – but at least I know what to do!  It is quite possible that MCAS has also been behind many of my other issues such as fatigue, stress, and pain but this is hard to know, as I have been managing my symptoms conservatively my whole life.

Also, before I start explaining MCAS, it is important to note that Postural Orthostatic Tachycardia syndrome (POTS), hypermobile-Ehlers Danlos syndrome and MCAS are frequently connected conditions, as often clients have all three conditions with similar symptoms therefore difficult to distinguish the underlying cause.

MCAS is very hard to explain.  And forgive me if I do not make it completely clear.  Even as an experienced scientist it has taken a long time for me to have a rudimentary understanding of it all.

Please understand that this information is intended for educational purposes only and is not intended as medical advice. Everyone is encouraged to obtain independent medical advice from their own medical team about their management.

There is a significant overlap in the conditions and the symptoms of MCAS, POTS and symptomatic hypermobility. Kohn & Chang (2020)

Mast cells are a type of specialized white blood cells that play a vital role in the body’s immune system. They are found in large numbers in the skin, lungs, nerves, vessels, gut, and other organs. Inside the mast cells are hundreds of different mediators which are usually released in a controlled way when the body needs them.  Some of these mediators are important in controlling inflammation, stimulating healing, and protecting the body from infection. They are also involved in the production of histamine, which is a key factor in the body’s allergic response, substances that can cause inflammation, allergic reactions, and other responses in the body. These substances can also be involved in the development of autoimmune diseases, allergies, and even certain cancers.

Mast cells are also involved in the production of various hormones, including serotonin and cortisol. Serotonin is a neurotransmitter that is involved in mood regulation, while cortisol is a hormone that helps the body to regulate stress levels. Mast cells may also be involved in the regulation of blood pressure and the production of certain immune cells. When mast cells are over-activated, it can lead to an exaggerated response in the body and a wide range of symptoms, and through this link to hormones they can affect the heart rate/blood pressure and a cascade of other effect of the sympathetic nervous system including POTS (Postural Orthostatic Tachycardia Syndrome).  

Mast cells can also play a role in tissue repair, as they can produce growth factors that can help to promote the healing of damaged tissue. Mast cells can be involved in the repair of blood vessels, and they can also play a role in wound healing. Finally, mast cells can be involved in the production of enzymes that can help to regulate the body’s metabolism.

Mast cells can be activated to release mediators by a variety of triggers, including allergens, infections, and certain medications. When activated, mast cells can lead to symptoms such as skin rashes, itching, hives, runny nose, wheezing, and more. In some cases, over-activation of mast cells can lead to mast cell activation diseases/syndrome, which can cause a variety of symptoms.

Someone may suspect they have mast cell activation diseases (MCAS) if they experience a combination of any of the common symptoms (see infographic below). It is thought that between 17-20% (Afrin, 2023) of the world’s population has some sort of Mast Cell Activation problem, as it is thought to underlie asthma, dementia, irritable bowel syndrome, Hypermobile-Ehlers Danlos Syndrome, fibromyalgia, skin rashes, chronic fatigue syndrome, long covid as well as many other common conditions.  Unfortunately, the acceptance of MCAS in the medical community is still in its infancy and no education is offered for doctors in their training courses.

A mutation in the Kit gene can lead to the inappropriate production and release of subsets of mast cell mediators.  These mediators release into the blood stream and activate a series of cascades that present as inflammation, allergy and in some cases abnormalities of growth.

Symptoms in MCAS may include skin rash or flushing, itching or hives, runny nose or congestion, abdominal cramping or vomiting, diarrhea or constipation, dizziness or fainting, shortness of breath or wheezing, facial swelling, joint pain or swelling, headaches, palpitations or irregular heart rate, excessive fatigue, anxiety or panic attacks, cognitive impairment or memory loss, weight loss or gain, night sweats, fever, hair loss, and unexplained bruising or bleeding.

If you experience any of these symptoms, it is important to speak with a healthcare professional to discuss further testing and treatment options.

Due to the lack of very clear blood tests to identify MCAS, it remains a condition that is poorly understood or accepted by a lot of the medical profession.

Diagnosis of MCAS remains difficult and is often a clinical diagnosis based on symptoms which depends a great deal on who is conducting the assessment.  Definitive diagnosis can be obtained through microscopic analysis of gastrointestinal tissue taken during an endoscopy.  If you have had an endoscopy in the past, it is worth checking as some collected cells are stored for 5-10 years, therefore testing can be completed on cells that were collected years before.  Cells collected during endoscopy can be tested with the CD117 stain to look for excessive mast cells.  This can be organised by a medical practitioner.

Other tests are becoming available in Australia now.  The problem with blood testing to this point is that it has not been collected, stored, and analysed in an environment that has kept the mediators from breaking down prior to the test being completed ie they need to be collected and transported cold.  Elevated heparin can be demonstrated in approx 80% of MCAS clients – but it has a half-life of 1 minute unless refrigerated immediately! This means that the heparin will break down in the sample if left at room temperature for more than one minute. This has made the collection and analysis of heparin in Australian very tricky.

The most effective treatment for mast cell activation syndrome (MCAS) is to manage the underlying cause or environmental irritant.  This takes some detective work to figure out if there are any environmental stressors/irritants. 

Keeping a diary to track symptoms, foods consumed and products used, can be useful in ascertaining your environmental irritants.

Once a potential allergen has been identified, trying to decrease the exposure to this is essential.  Consulting a dietician to trial a low FODMAP or low histamine diet is essential if you are concerned about food reactions. This website can be helpful to find foods that adhere to certain allergies and diet types https://www.whatthebleepcanieat.com. But it highly recommended that you consult a qualified and experienced dietician before you start on any dietary restrictions.

Medications can be used to treat mast cell activation syndrome (MCAS). Antihistamines can help reduce symptoms, but other medications may be helpful.

Over the counter anti-histamines such as Telfast, Clarytine and Zyrtec can be trialled with the assistance of your pharmacist/GP and each works slightly differently so Dr. Afrin ( a medical specialist with an interest in this field) suggests you try each one at a time for two weeks and note which one has the best effect.  With medical assistance often a dosage of twice per day can be trialled. These are the first line as such and are the H1 blockers.

Once the best H1 blocker for you has been found, then Dr. Afrin suggested, to stay on the H1 blocker and try the H2 blockers (eg Famodine/nizatidine etc) one at a time and see what works best for you.

A third line of management, “Mast cell stabilizers” can help to prevent mast cell over-activation, while leukotriene receptor antagonists (eg singular/montelukas) can help reduce inflammation caused by leukotrienes. Compounded medication including Ketotifen or sodium cromoglycate can also be helpful for some people.  It is important to speak with a healthcare professional to determine the best medication for your individual needs.

The final line of defence is a monoclonal antibody injection knows as omalizumab (or Zolair) but these are only covered by medicare for severe cases of hives and asthma. It is essential to discuss any of these managements with you GP or specialist and some article references are included at the end if you need to provide educational material to your health professionals.

Depending on the individual, management also involves lifestyle modifications, dietary change (avoiding processed food and low histamine diet), improving sleep, psychological support, movement/exercise, and a sense of belonging (support groups and family/friend support).

Other treatments may include supportive therapies such as physiotherapy, acupuncture, and psychotherapy.

Physiotherapy can be an effective treatment for mast cell activation syndrome (MCAS). Physiotherapy can help to reduce inflammation, improve range of motion, and manage pain. When combined with other treatments, such as medications and lifestyle modifications, physiotherapy can help to improve overall wellbeing and quality of life. Physiotherapy can also help to maintain and improve muscle strength, flexibility, balance, coordination, and endurance. Finally, physiotherapy can help to reduce fatigue and stress, which can help to manage MCAS symptoms.

It is important to note that everyone’s treatment plan will be unique, depending on their specific needs. It is important to consult with a healthcare professional to determine the best treatment plan for your own needs. Additionally, it is important to stay informed about the latest research and treatments for MCAS to ensure you are receiving the best possible care.

This over-activation leads to a wide range of symptoms, including dizziness, fatigue, palpitations, headaches, and more. Hypermobile Ehlers-Danlos Syndrome is also related to mast cell activation diseases because those with the condition often experience an increase in mast cell activity, which can lead to a variety of symptoms. Living with these conditions can be managed with lifestyle modifications, medications, and other treatments by health professionals interested in these areas.

References:

Afrin LB, Ackerley MB, Bluestein LS, Brewer JH, Brook JB, Buchanan AD, Cuni JR, Davey WP, Dempsey TT, Dorff SR, Dubravec MS, Guggenheim AG, Hindman KJ, Hoffman B, Kaufman DL, Kratzer SJ, Lee TM, Marantz MS, Maxwell AJ, McCann KK, McKee DL, Menk Otto L, Pace LA, Perkins DD, Radovsky L, Raleigh MS, Rapaport SA, Reinhold EJ, Renneker ML, Robinson WA, Roland AM, Rosenbloom ES, Rowe PC, Ruhoy IS, Saperstein DS, Schlosser DA, Schofield JR, Settle JE, Weinstock LB, Wengenroth M, Westaway M, Xi SC, Molderings GJ. Diagnosis of mast cell activation syndrome: a global “consensus-2“. Diagnosis (Berl). 2020 Apr 22;8(2):137-152. doi: 10.1515/dx-2020-0005. PMID: 32324159.

Afrin, L.  Presentations at the Mast Cell Activation Disease Conference: Current Concepts.  Brisbane, Australia (March, 2023)

Kohn A, Chang C. The Relationship Between Hypermobile Ehlers-Danlos Syndrome (hEDS), Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS). Clin Rev Allergy Immunol. 2020 Jun;58(3):273-297. doi: 10.1007/s12016-019-08755-8. PMID: 31267471.

Seneviratne, SL, Maitland, A, Afrin, L. 2017. Mast cell disorders in Ehlers–Danlos syndrome. Am J Med Genet Part C Semin Med Genet 175C: 226– 236.

Weinstock LB, Pace LA, Rezaie A, Afrin LB, Molderings GJ. Mast Cell Activation Syndrome: A Primer for the Gastroenterologist. Dig Dis Sci. 2021 Apr;66(4):965-982. doi: 10.1007/s10620-020-06264-9. Epub 2020 Apr 23. PMID: 32328892.