Managing My Conditions

Living with multiple chronic illnesses can mean trying to manage lots of different symptoms at once. Some things may actually help more than one condition, whilst others need a little more trial and error to work out what suits you.

Below you can find information about the conditions I live with, the ways they can affect daily life and the strategies that have helped me along the way.

Everyone is different, so these are ideas and experiences to draw from alongside your own care.

EDS and HSD

MCAS

Gastroparesis

What are the Ehlers-Danlos Syndromes (EDS)?

The Ehlers-Danlos Syndromes (EDS) are a group of connective tissue disorders that cause poorly structured or 'weaker' collagen. In EDS there are genetic faults in the way that collagen &/or some collagen-related proteins are made. Collagen is everywhere in our bodies, so symptoms can be very widespread, affecting tissues, organs, bones, ligaments, tendons, lymphatic vessels etc. Depending on the genetic fault, different tissues and organs can be affected, which is why there are several subtypes.

Subtypes of EDS

There are thirteen subtypes of EDS:

Arthrochalasia EDS (aEDS)
Brittle Cornea Syndrome (BCS)
Cardiac-valvular EDS (cvEDS)
Classical EDS (cEDS)
Classical-like EDS (clEDS)
Dermatosparaxis EDS (dEDS)
Hypermobile EDS (hEDS)
Muscularcontractural EDS (mcEDS)
Myopathic EDS (mEDS)
Peridontal EDS (pEDS)
Kyphoscoliotic EDS (kEDS)
Spondylodysplastic EDS (spEDS)
Vascular EDS (vEDS)

What does the Zebra mean in EDS and HSD?

There are a few meanings:

1) Medical students are usually taught this:

“When you hear hoofbeats behind you, don’t expect to see a zebra"

Essentially meaning to always look for the most common diagnosis
However EDS and HSD ARE the unexpected, rarer diagnoses
Therefore “Sometimes when you hear hoofbeats, it really is a zebra”

2) From afar all zebras look the same, BUT if you look closer their stripes aren't identical.

Just like no two people with EDS/HSD will have exactly the same types, symptoms and experiences

3) A group of zebras is called a dazzle.

With this supportive community and shared awareness, Together we dazzle!

The overall prevalence of the Ehlers-Danlos syndromes is between 1 in 3,500 to 1 in 5,000 people.

The hypermobile variant (hEDS) is by far the most common type and often may be missed or misdiagnosed as something else.

Most Ehlers-Danlos Syndromes are rare (e.g. 1 in 40,000 – 200,000), and some are ultra-rare (e.g. less than 1 in a million people).

Ehlers-Danlos Syndromes are inherited and they can be passed down by either dominant or recessive autosomal patterns.

Symptoms of EDS

Symptoms will vary between each subtype of EDS, but also individuals with the same subtype often can experience very different and dissimilar symptoms.

Whilst not an exhaustive list, here are some typical symptoms:

Hyperextensions of several joints
Frequent subluxations and dislocations of joints
Joint and muscle pain
Clicking joints
Variable mobility
Chronic fatigue
Easy bruising
Stretchy skin
Poor wound healing
Digestive issues
Issues with internal organs
Fragile blood vessels
Bladder issues
Curvature of the spine
Dysautonomia / dizziness upon standing

What are Hypermobility Spectrum Disorders? (HSD)

Hypermobility Spectrum Disorders (HSD) are a group of conditions related to joint hypermobility, which is the capability of joints to move beyond the normal range of motion.

Along the continuum fall these four types:

Generalised (joint) HSD (G-HSD)
Peripheral (joint) HSD (P-HSD)
Localised (joint) HSD (L-HSD)
Historical (joint) HSD (H-HSD)

HSD are diagnosed after other possible conditions have been excluded, such as any of the Ehlers-Danlos syndromes (EDS) including hypermobile EDS (hEDS).

The difference between HSD and hEDS is merely by stricter diagnostic criteria, however both can be equal in severity. They require similar management strategies, validation, and care.

Prognosis of Ehlers-Danlos Syndromes (EDS) and HSD

Diagnosis of Ehlers-Danlos Syndromes are based on the 2017 diagnostic criteria which look at a patient's symptoms and align them to the according major or minor criteria to fit a certain subtype.

For all EDS types apart from hEDS, testing exists to identify the responsible variant for the gene affected in each subtype. hEDS is therefore diagnosed as a 'clinical diagnosis' based on meeting pre-requisites
in the criteria. Whilst there is no cure for Ehlers-Danlos Syndromes, there are management strategies which can improve quality of life.

Next steps if you suspect them

Diagnosis of EDS and HSD is typically through a rheumatologist, however other specialists in fields where symptoms overlap may have knowledge and awareness and be able to diagnose or refer
over to rheumatology.

You may suspect EDS/HSD and need to advocate for a referral from your primary care provider or General Practitioner (GP) for further investigations. You can find this GP toolkit here.

For many more resources and support:
The Ehlers-Danlos Society

Ehlers-Danlos Support UK

Misconceptions about Ehlers-Danlos Syndromes (EDS) DEBUNKED

There are several misconceptions about Ehlers Danlos Syndromes and Hypermobility Spectrum Disorders, so across these image graphics you can learn more and educate yourself further.

How can EDS or HSD affect daily life?

Symptoms can vary and be very unpredictable. EDS and HSD can be disabling and create a huge myriad of issues with multiple triggers. Below you can find examples (whilst not an exhaustive list).

Cervical Instability

Triggers:

turning neck side-to-side
bending forwards
jumping
going up stairs or up hill

Shoulder, Wrist, Hand & Finger Instability

Triggers:

lifting anything
opening heavy doors
pressing buttons / typing
side sleeping

Pelvic Instability

Triggers:

sitting down
twisting
crossing legs
any slight movement

Digestive Issues

Triggers:

many different foods
co-morbidities include food allergies or sensitivities, MCAS, IBS and gastroparesis

Chronic Fatigue

Triggers:

any physical activity
specific foods

Dysautonomia (autonomic dysfunction)

Triggers:

standing up from sitting
changes in temperature
eating
exercise

Managing Ehlers-Danlos Syndromes (EDS) and Hypermobility Spectrum Disorders (HSD)

Using several strategies together or ‘creating a flare up toolkit’

This takes trial and error and may be developed and added to over time
Each person will respond differently to any strategy or therapy so this combination will be unique
to each person
A guide to create your own is on this page

Physiotherapy and Exercise

This is to enhance your positional sensing (proprioception)
- Physio, shoe inserts/orthotics, compressive clothing
Focusing on strengthening of muscles around the joints
- Gentle daily physiotherapy, repetitive strengthening movements, regular movement, low impact exercise e.g. walking / swimming, hydrotherapy, balneotherapy etc
Physiotherapy is a often a crucial management strategy for EDS and HSD.
- Whilst lack of strength can increase the prevalence of subluxations/dislocations, de-conditioning can also cause further issues
You should find a specialist physio who’s experienced in managing EDS. They’ll evaluate your posture, range of movement, strength, gait and mobility, stability and proprioception - this will be unique for each person. Based on this they’ll recommend suitable exercises for you including helping to gain core strength, improving proprioception and strengthen those muscles around the joints. This is often low impact exercise like swimming or hydrotherapy, and gentle walking. They may suggest basic strengthening movements that can even be done lying down. Regular gentle movement may also be recommended which can include simply getting up to do a certain activity every so often and you may set timers or reminders to avoid you staying in that one position for too long. If stretching is advised, it should be very gentle with a good awareness of joint range of motion to avoid joint injury.
Remember everyone is very different in what helps them so your exercise should be always tailored by a qualified individual who's specialised in your condition.

Specific Medications or Devices

Oral medications for pain (with awareness of long term effects and use in conjunction with other strategies)
Acute medications and preventative medications for specific symptoms or co-morbidities
Topical pain relief creams, balms, gels or patches
Heat or cold therapy
Mobility aids
Specific therapies or devices for certain symptoms

Specific joint supports, splints, tape or braces

Used as advised by a medical professional as prolonged use can weaken muscles and possibly further exacerbate symptoms

Use of cushions, pillows, supportive sofas and mattresses

Sofas that offer comfort and support for problem joints; chaise sofas, electric recliners, adaptable backrest sofas, ergonomic office chairs or makeshift support options
Coccyx cushions, lumbar supports, adjustable pillows wedge cushions, bolsters, U or V pillows, full body pillows, knee pillow, bean bag pillows etc.
- A range of these can help to both prevent and alleviate pain and symptoms any physical activity

Nutrition Changes and Supplements

These will be unique to each person and their co-morbidities
May include avoiding trigger foods
- keep a food diary to establish these
Use of medications for alleviating symptoms with gastro flare ups or allergens
Vitamins or supplements that target mineral deficiencies or inflammation etc.

Having accommodations around the home to pre-empt a symptom

Support and help from others
Specific gadgets or other useful tools or aids
Changing locations of items, adapting rooms and layouts or even moving homes
- Support or evaluation from an occupational therapist could help with these implementations

Pacing of necessary weekly tasks or activities

Routine changes: prioritising only essential weekly tasks, spreading those tasks across the week into bitesize chunks
This may include workplace adjustments like working from home, flexible working, less hours working or stopping working to manage health
Balancing regular rest breaks with regular gentle movement wherever possible
- A guide to implement pacing in more detail can be found on this page

Looking after Mental Health

Implementing mental health strategies that look at the cognitive perception of pain;
- Cognitive Behavioural Therapy (CBT) is frequently recommended by practitioners for pain management
- Distraction techniques
- Regulating the Autonomic Nervous System; Breathwork, Meditation, Mindfulness, Sophrology, EFT, other alternative therapies or strategies
Other types of therapy to manage co-morbidities such as anxiety and depression etc.

Support Networks in Place

Ensuring your friends and family are aware of how to best support you
Connecting with other groups of people with similar conditions/experiences
Access to medical care teams for any changes in symptoms or co-morbidities

Learning to Advocate

Preparing for medical appointments and tracking symptoms over time. You can find a guide here to help with preparation including a free template.
Educating others about your conditions - you may like to share this page with them for a general understanding of living with chronic illness
Knowing when to seek further medical care
Confidence to ask for help from others (especially in public) when needed [use of cards, badges or bracelets where required] - you can find templates to create your own here

Managing Mast Cell Activation Syndrome or Disorders (MCAS)

Mast Cell Activation Syndrome (MCAS) is a type of Mast Cell Activation Disorder where the chemicals called mast cell mediators are released too frequently or abundantly, and/or in response to triggers that are not typically considered to be harmful, for example; foods or chemicals in the environment.

This can lead to which causes a wide range of symptoms that affect multiple parts of the body. MCAS forms part of a spectrum of mast cell disorders.

People with MCAS may also have other mast cell disorders such as mastocytosis or hereditary alpha tryptasemia syndrome (HATS). Triggers and symptoms of MCAS vary greatly between individuals." (Mast Cell Action Website)

Below you can find some examples of possible triggers.

Possible Triggers for MCAS Symptoms

Scents and smells:

Perfume and beauty products
Laundry detergents on peoples’ clothes
Scented cleaning products
Cooking smoke or BBQs
Tobacco or vape
Petrol or diesel fumes
Scented soaps and body washes
Synthetic materials e.g. car smells or furniture etc
Air fresheners
Candles
Paint

Skin contact:

Vibration
Fabrics on our skin
Insect bites
Animal fur or dander

Airborne:

Mould
Infections
Animal fur or dander
Pollen
Dust
Scents and smells
Changes in climate
Changes in barometric pressure
Changes in temperature

Bodily activity:

Changes in body temperature
Exercise
Stress
Anxiety
Hormonal changes

Ingested:

Medications
Foods (known trigger &/or random)
Liquids, even water

What are MCAS Symptoms?

These can vary hugely between individuals. They can affect a whole range of body systems. MCAS is a condition that can fluctuate in severity over the course of someone's life and symptoms can vary over phases or come and go. It may be well managed at points, but less well managed at other points. Some triggers may be clearly identified but it can also be totally random or without a seemingly evident trigger. This PDF by Mast Cell Action charity outlines the symptoms, you can find them below too.

Neurologic (brain and nerves)

Headache
Brain fog (memory and concentration problems )
Numbness, pain, or tingling skin Anxiety
Behavioural issues, rages

Nasal-ocular (nose and eyes)

Nose congestion
Eye watering and itching

Respiratory (lungs and breathing)

Sore throat

Hoarseness

Wheezing

Shortness of breath

Throat swelling

Cardiovascular

Chest pain
Low blood pressure
Fast heart rate
Fainting or light-headedness

Musculoskeletal

Joint and muscle pain

Osteoporosis (brittle bones)

Loss of bone mass

Gastrointestinal

Bloating

Stomach cramps or pain Reflux
Feeling or being sick Diarrhoea
Constipation
Dumping syndrome
Food allergies or intolerance

Genital and urinary

Genital pain or swelling
Pain when urinating
Vaginal pain, discharge or itching

Bladder urgency or loss of control

Dermatologic (skin)

Itching with or without a rash

Flushing/redness
Hives or wheals
Swelling

General

Extreme tiredness
Anaphylaxis, which can be life threatening

The Histamine Bucket Theory to Explain how MCAS may work

You can imagine your body with a histamine bucket that’s empty, but exposure to all things in the environment can gradually fill up the bucket to your personal histamine levels.

You may completely tolerate these things on some days, as your histamine bucket doesn’t fill up to the limit as quickly.

On other days it may overflow beyond the limits that your body can process the exposure to triggers and you’ll likely have more reactions and symptoms; to anything and everything.

A combination of managing triggers, reducing exposure to known triggers, and taking medication could all help to manage the level of your bucket.

Diagnosing MCAS

This can often be tricky as many healthcare systems still are not acknowledging the condition because only a handful of years ago the diagnostic criteria was established, and only around 15 years ago was the condition 'coined'. In the UK there are currently no NHS or NICE guidelines for the condition, which makes diagnosis difficult. Not all practitioners recognise or are aware of the condition. In addition, the methods for diagnosis aren't always accurate. There are thousands of mast cell mediators, yet we can only test ten and most healthcare systems only test one, so it's not surprising how challenging it actually is to diagnose 'objectively' and most diagnoses may be due to a positive response to medications and fitting the medical symptomatic profile.

If you're in the UK, you can find more information at Mast Cell Action. If you need support, the best thing to do is to contact the charity directly with info on your case and they'll be able to guide you with next steps. It's also important for them to gather data showing the need to governmental bodies, so if you need help please reach out to them directly here.

If you're in the US, you can find more information at TMS for a Cure.

Managing Mast Cell Activation Syndrome

It's important to note that all information shared is for informational and not prescriptive purposes, and you should always consult a doctor to guide you further with this.

Medications and Supplements

Mast Cell Stabilisers

Ketotifen
Sodium Cromoglycate

Mediator Blockers

H1 antihistamines
H2 antihistamines
Anti-leukotrines
Anti-Prostaglandins

Corticosteroids

Prednisolone

Vitamins and Supplements

Vitamin C
Vitamin D
Probiotics
Magnesium
Quercetin
Luteolin
Sodium Butyrate
Liposomal L-Glutathione
Digestive Enzymes

Antibody Therapies

Omalizumab (Xolair)

Emergency Relief

Adrenaline Auto Injector e.g. Jext Pen or Epi-Pen
Albuterol reliever for asthma

Other Strategies to Limit MCAS Triggers and Reactions

FOOD

Following a low histamine diet.
- This may reduce your bucket but you may also have triggers that aren't low histamine, so avoiding all trigger foods where possible too.
- Keeping a food diary to establish triggers and symptoms from certain foods.
- Look into other triggers beyond histamine like oxalates, salicylates etc.
- Working with a dietitian or nutritionist to ensure that any restrictive diets aren't detrimental to your health.

Increases in blood sugar can trigger histamine release, so eating low sugar or refined sugar free foods where possible
Looking at all labels carefully and advocating when eating out about dietary restrictions. You can create your own Allergy Cards and Foods That You Can Eat cards with my free templates on this page.

BEAUTY, HYGIENE AND HOUSEHOLD

Using unscented beauty and hygiene products
- Asking people you live with to do the same
Patch testing everything before use
- This may include getting sample sizes of products or finding brands that offer refunds if the products don’t work for you
Swapping to more natural alternatives and unscented versions of household cleaning products
Opting for dried flowers rather than fresh ones for home decor if pollen is an issue

SOCIALISING

Ensure you have all regular medications alongside abortive medications like asthma inhalers, EPIPEN and antihistamines when going out in public.
Asking people to kindly avoid spraying perfume or fragrance before you meet up with them or they come to your home.
Avoiding giving hugs to greet people. Even if they're not wearing perfume, their laundry detergent, shampoo or beauty products may have scents that could trigger you.
- Before they approach you explain "I'm not doing hugs - air hug / fist bump / hand shake" if they ask why explain you have allergies and sensitive to scents. This may take some getting used to but I've found it's highly worth it.
Keeping away from places with tobacco smoke, vapes, wood burners, BBQs or simply staying out of the kitchen when food is being charred.
Showering (including hair wash) as soon as you're home from any outside location.
If people have pets that trigger reactions, even dander on their clothes can cause reactions so asking them to wear fresh out the wash clothes their pet hasn’t been on could help.
When you have guests over or are staying overnight elsewhere, using an air purifier with a HEPA filter to prevent reactions from additional triggers (like their beauty and hygiene products and laundry detergent etc). You can even bring a small and portable air purifier (that looks a little like a lantern) with you for travel.
Opting for socialising outside in open spaces if you are able to; at home or an another outdoor space.
Wearing a mask with an adequate filter to reduce exposure to airborne allergens, VOC's and fragrances.
Full rest and home isolation before and after exposure to triggers.
- For me I aim to do this for many days to weeks depending on how my 'histamine bucket' is.

CLOTHING AND FABRICS

Opting for super soft bed linen to avoid reactions from rough fabrics. Always wearing clothes that are breathable cotton or soft fabrics that you tolerate. If you want to wear a knitted jumper in winter, wear a soft cotton basic top underneath to avoid rashes from fabrics.

Useful Resources

I'm constantly learning about MCAS and things I can do to manage debilitating symptoms and what can be done to change the system to enable better support in the future for others with MCAS. I'll share resources I've found helpful along the way for when researching on your journey with MCAS.

Mast Cell Action

Resources and support if you're in the UK

TMS for a Cure

Resources and support if you're in the US

Dr Tania Dempsey and Dr Lawrence Afrin

Dr Tania Dempsey and Dr Larry Afrin are two of the world leaders in MCAS research and expertise who are based in the US and share a clinic the AIM centre.
Q&A with Dr Tania Dempsey and Katya here

Ehlers Danlos Society

Resources on their website about MCAS

American Academy of Asthma and Immunology

Resources about MCAS

PoTS UK

Resources on their website about associated conditions to PoTS (MCAS)

What is Gastroparesis?

It's also known as delayed gastric emptying and is where the stomach is partially or fully paralysed. This means that food moves slowly through the stomach or completely stops moving.

These are the signs and symptoms of gastroparesis:

Vomiting
Nausea
Abdominal bloating
Abdominal pain and discomfort
Feeling full very quickly when eating
Vomiting undigested food eaten a few hours earlier
Acid reflux / Heartburn
Changes in blood sugar levels
Lack of appetite
Weight loss and malnutrition

There is no cure but management strategies exist, and everyone will have different experiences.

Diagnosis is often through the following hospital tests: This is how it’s often diagnosed

Gastric emptying study (scintigraphy)
Wireless capsule test
Barium x-ray
Breath tests
Upper gastrointestinal (GI) endoscopy
Ultrasound

It can fluctuate from mild to severe.

People with milder gastroparesis, may experience waves or flareups of symptoms that can usually be managed with dietary intervention and/or medications. You can find more info below on this.

For people with more severe gastroparesis, more invasive methods can be used:

Surgery (gastroenterostomy/gastrojejunostomy)
Botox injections
Electrical stimulation

If getting adequate nutrition becomes an issue, tube feeding can be trialled

Enteral nutrition - through your stomach or intestine
Parenteral nutrition - directly into the bloodstream

In 2021, I received test results showing delayed gastric emptying or also known as gastroparesis. Diagnosis in my case was through a gastric emptying study where I ate radioactive porridge and had images taken of my stomach over 4 hours.

I reached out to this community for strategies to manage. The responses and support were AMAZING.

Everyone's different and not all of these will suit each person, but this guidance has been SO helpful to me that I wanted to create this resource for others to refer to. It’s not medical advice, and was simply strategies shared by others in the community with what works for them.

This is what was recommended to do:

Aim for 4-6 small frequent meals per day
Chew food well
Sit up straight during meals
Sit or walk (for 1-2 hours) after eating rather than lying down
Eat a lower fibre diet to reduce work of digestion
Try to swap foods for Gastroparesis-friendly options rather than cutting out fully
Sip fluid throughout the day rather than drink large quantities in one go
Try nutrient rich liquids or puréed foods (particularly during a flare)
Opt for meats cooked in a pressure cooker / slow cooked that are broken down more and can be easier to digest
Listen to your stomach no matter what you crave

This is was recommended to avoid:

Avoid eating 2-3 large meals per day
Avoid high fat foods. Fat-containing liquids may be tolerated
Avoid hard to chew foods (nuts, seeds, corn etc.)
Avoid fizzy drinks including carbonated water and alcohol
Avoid raw foods, especially with skins and salads
Avoid high fibre solid foods. May be tolerated in liquid form

The Step Ladder Strategy:

This is from one patient’s experience that is the management strategy they use to cope with their gastroparesis and has worked for them

Aiming to stay on each step till your tummy seems happy for 24-48 hours
If you move up a step and it doesn’t go well, back down one step immediately
If symptoms are severe, go immediately back to step 1

Step 1. Clear Liquids

Beef broth

Chicken broth

Vegetable broth

Step 2. Liquids

High calorie liquids / Meal replacement shakes

- Aymes Smoothie (vegan) not refined sugar free

- YFood (vegan) contains Splenda sweetener

- Neocate jr formula (or similar)

- Drink with Duocal supplement (or similar)

- Predigested drinks e.g. Kate Farms (or similar)

Fresh smoothies

Fresh juices

Pureed soups

Step 3. Soft foods

Chicken noodle soup

Applesauce

Yogurt

Baby food

Blended up meals

Step 4. Solid Foods

Gastroparesis-friendly foods

Foods that are low in fat and fibre

SUMMARY

These strategies may work for me, but may not work for you and is not medical advice. I’m sharing the concepts so that you can create your OWN step ladder strategy to use as a management strategy with the safe steps and food types that you’ve established work for YOU. Find templates to create your own here.

Nutritional Resources

The following nutritional resources were shared with me from other patients with gastroparesis as nutritional resources they found useful in their own management.

Living Well with Gastroparesis

https://livingwithgastroparesis.com/

From Crystal Saltrelli

Diet Tips for Gastroparesis

https://www.fairview.org/patient-education/522875

Dietary and Nutritional Recommendations for Patients with Gastroparesis

Link

From registered dietician Emily J

Gastroparesis Nutritional Info

Link

From registered dietician Emily J

Nutritionist Recommendation

https://www.instagram.com/ashleysauvehealth/

These strategies for gastroparesis are the generous support + help that the community have shared with me to help me begin to navigate my new diagnoses.

With regards to everything on this page, please do remember that everyone is unique so what works for some may not work for others. It's a general guide of strategies that could help, but may not necessarily.

If you have anything you'd like me to add to this page that's helped you, please reach out using the contact form below

Get in touch