I Have hEDS. Now What? Part 2
This is the second of two posts on next steps after an hEDS or HSD diagnosis. Part 1 covered the basics that apply to almost everyone: finding someone to coordinate your care, reviewing your medications, physical therapy, everyday habits, pain, fatigue, and mental health. This part covers the conditions that often come along with hEDS, what to tell your providers, and the red flags that point to something beyond hEDS.
The "EDS triad": hEDS, POTS, and MCAS
When hEDS, POTS, and MCAS show up together it's called the "EDS triad," though how the three are connected is not yet fully understood.
For those with dizziness, a racing heart, or fatigue when standing, this could be POTS or another form of dysautonomia. A formal diagnosis is helpful if you can get one, but tilt table testing is hard to find and waitlists are often long. Whether or not it's officially POTS matters less than addressing the symptoms.
Treatment usually starts without medication: more fluids and salt (check with your doctor first, especially if you have high blood pressure, kidney, or heart problems), waist-high compression or an abdominal binder, and exercise that starts lying down or seated, like a recumbent bike, rowing, or swimming, before you work up to upright. Get up slowly, especially in the morning, and drink a glass of water before you get out of bed. Avoid the usual triggers: heat, alcohol, big meals, and standing still for long periods. If you feel faint, cross your legs and squeeze, do calf raises, or squat. Published consensus guidelines for POTS cover all of this, including medication options if lifestyle changes aren't enough, and are worth reviewing with your doctor. If you actually pass out, especially without warning or more than once, ask for a cardiac or neurologic evaluation.
People with flushing, hives, itching, or reactions to foods and medications are often concerned about mast cell activation syndrome (MCAS). More on this below.
MCAS
You react to foods, medications, or your environment, but all your allergy tests are normal. That may be because typical allergy testing rules out one type of allergy, the IgE-mediated kind. It doesn't rule out every kind of reaction, and it doesn't test for mast cell activation.
The main blood test for mast cell activation, tryptase, needs to be drawn within a few hours of a reaction and compared with your own baseline, using published consensus criteria. Blood drawn on a good day often looks normal, which is accurate but not very helpful. Getting blood drawn mid-flare is difficult, so a diagnosis may be harder to come by. Managing the symptoms doesn't have to wait for one.
Side note: if your baseline tryptase is elevated, ask about hereditary alpha-tryptasemia, an inherited trait.
Antihistamines are usually the first thing to try for suspected mast cell symptoms. If symptoms don't improve, there are other medications to explore under the supervision of a physician who has experience with mast cell disorders.
Figuring out what sets off reactions is the other half of managing mast cell symptoms. Common triggers include heat, exercise, friction, stress, alcohol, fragrances, and some medications, but they vary a lot from person to person. Because reactions can show up a day or two later, it helps to keep a simple daily log for a few weeks: what you ate, any medications, activity, heat, and stress, plus any symptoms. When a reaction happens, you can look back over the previous couple of days for a pattern. Bring the log to your appointments. Some diets may be helpful for people with significant mast cell-related symptoms, but working with a registered dietitian is paramount.
Triggers tend to add up, so a day with several small exposures can cause more symptoms than any one of them alone. Some places to start: switch to fragrance-free soaps and cleaning products, and skip air fresheners and scented candles, keeping in mind that products labeled "natural" can still contain essential oils that cause reactions. An air purifier can cut down on airborne triggers at home, and on high pollen days it helps to change your clothes or shower when you get home. Keep humidity down with good airflow or a dehumidifier, and deal with mold quickly.
Sudden temperature changes, like stepping out of a hot shower into a cold room, can set off symptoms too. Warming up the bathroom before a shower, or dressing in layers when going between indoors and outdoors, makes those shifts more gradual. Check ingredient lists on medications as well, since fillers and flavorings can differ between brands. Your pharmacist can help, and your doctor may be able to specify a brand that works for you. As with everything else here, change one thing at a time. Mast Cell Action's self-management toolkit has more practical tips.
Skip the IgG food sensitivity panels. They aren't validated for diagnosing food allergies or intolerances, and they flag foods you probably tolerate just fine.
Trouble breathing, throat tightness, widespread hives with dizziness, or fainting means emergency care, no matter what past tests showed. If you've had episodes like that, ask your doctor whether you should carry epinephrine.
GI and pelvic floor symptoms
GI symptoms are common in hEDS, but they don't all have the same cause, so they don't all get the same treatment. If suspected, testing for celiac disease may be considered.
Getting uncomfortably full after a normal-sized meal, nausea, and pain or burning in the upper abdomen can all be part of hEDS, especially for people who also have POTS. Smaller, more frequent meals, less high-fat food, staying upright after eating, and a short walk afterward can help. Liquids and softer foods tend to be easier than large solid meals. If symptoms persist, talk to your doctor about next steps.
Constipation with a lot of straining, or a feeling you can't empty fully even with soft stool, may be a pelvic floor problem. Pelvic floor PT that includes biofeedback may help with this, as well as with bladder urgency, leaking, pelvic pain, or painful sex.
Some medications make constipation, nausea, or reflux worse, which is another reason for a medication review. Opioids in particular slow the gut. For pain-heavy gut symptoms, gut-directed hypnotherapy (a guided relaxation approach that targets how the brain processes gut signals) is a studied option.
Food, without the rules
There is no specific diet that improves hEDS symptoms.
It is important to eat enough, and get enough protein, since PT builds muscle and muscle needs fuel. If you have POTS, more fluids and salt are often recommended, but ask your doctor how much. Smaller meals can also help with reflux.
Be careful with restrictive diets. Cutting out food groups without professional guidance can lead to nutrient deficiencies, more fatigue, fear around eating, and disordered eating. Some GI symptoms that get blamed on hEDS can actually come from deficiencies caused by a restricted diet, so the fix can end up making things worse. The goal is the widest diet you can tolerate.
Be just as skeptical of supplements sold as hEDS fixes. If there's a reason to suspect a specific deficiency, get tested for it rather than taking a long list of supplements just in case.
Tell every provider
Anyone who treats you should know you have hEDS.
Many people with hEDS find that local anesthetic works poorly or wears off fast, so tell your dentist before they start. Jaw problems are common too, so ask for breaks during long procedures.
Surgeons and anesthesiologists need to know which of your joints are prone to instability, so they can avoid extreme positioning of your jaw, neck, shoulders, hips, and knees. Talk about wound healing and recovery time too. If you have mast cell symptoms, tell your dentist and surgical team about those as well, since some anesthetics, pain medications, and antibiotics can trigger reactions.
Chiropractors and massage therapists should skip high-velocity thrust adjustments, especially on your neck. Look for someone focused on stabilization and experienced with hypermobile bodies.
Expect more bruising with blood draws and IVs. And if you're pregnant or planning to be, tell your OB early. (I wrote a whole post on that: [link: A Message For My Pregnant hEDS Patients].)
A few traps
Stretching may come naturally, which is exactly why it's tempting, but may also add to instability. Stay within a comfortable range and focus on strengthening, rather than lengthening.
Be skeptical of expensive treatments sold as fixes, because hEDS has no cure.
Patient communities are great for support and practical tips, but symptoms vary a lot, and what helped someone else may not help you. And not every new symptom is because of hEDS. People with hEDS get other conditions too, and new or changing symptoms should be assessed appropriately by your PCP.
Red flags beyond hEDS
Some findings shouldn't be filed under "that's just my hEDS," but should trigger prompt evaluation by your PCP or a specialist.
Certain features point to a different connective tissue condition, such as vascular EDS, Marfan syndrome, or Loeys-Dietz syndrome, which need their own monitoring and can lead to medical emergencies. These features include a personal or family history of arterial, bowel, or uterine rupture, significant aortic enlargement, lens dislocation, or a family history of sudden death or aneurysm. Very thin skin with a clearly visible network of veins, especially on the chest and abdomen, can also be a sign, usually alongside extensive bruising or a history of rupture. If any of this sounds familiar, ask about a genetics evaluation and testing. A new heart murmur calls for an echocardiogram, and known aortic root enlargement needs regular cardiology follow-up.
Fractures from minor injuries, or frequent fractures, can point to genetic conditions that affect bone strength.
New or worsening neurological symptoms need a neurology evaluation: numbness, weakness, loss of coordination, bladder or bowel changes, headaches that keep coming back or get worse when you're upright, symptoms that change with your neck position, or trouble swallowing or breathing. Chiari malformation, spinal fluid leaks, craniocervical instability, and tethered cord have all been reported with hEDS, and would require imaging for diagnosis. Progressive muscle weakness can point to a neuromuscular condition, some of which also cause joint hypermobility.
Hot, swollen joints with long morning stiffness suggest inflammatory arthritis. Bleeding beyond easy bruising, like frequent nosebleeds, bleeding gums, or heavy periods that cause anemia, should be formally assessed by hematology.
Where to start
You don't have to do all of this at once. Pick the one thing that affects your daily life most, make one small change with your care team, and build from there. That's a perfectly respectable plan.
This post is educational, not medical advice. Every case of hEDS is different. Talk to your doctors before changing your treatment, medications, diet, or exercise.
Learn more or get evaluated
New to this? Start with this guide to the types of Ehlers-Danlos syndromes or hEDS vs HSD post.
Questions? Feel free to reach out.
Education and resources
The Ehlers-Danlos Society: Information about hEDS, HSD, diagnostic criteria, and symptom management.
The Hypermobility Syndromes Association: Practical guidance for living with symptomatic joint hypermobility.
Norris Lab: Research updates on the biology and genetics of EDS and related hypermobility disorders.
Dysautonomia International: Education and support resources for POTS and other autonomic disorders.
The Mast Cell Disease Society: Evidence-based education about mast cell disorders and diagnostic evaluation.
Mast Cell Action MCAS Self-Management Toolkit: Practical tips for identifying and managing everyday mast cell triggers.
ClinicalTrials.gov: Searchable registry of studies involving EDS, HSD, POTS, and related symptoms.
References
Aziz, Qasim, et al. "AGA Clinical Practice Update on GI Manifestations and Autonomic or Immune Dysfunction in Hypermobile Ehlers-Danlos Syndrome: Expert Review." Clinical Gastroenterology and Hepatology, vol. 23, no. 8, 2025, pp. 1291-1302. doi:10.1016/j.cgh.2025.02.015.
Chung, Tae Hwan, and Satish R. Raj. "Postural Orthostatic Tachycardia Syndrome (POTS): A Review." JAMA, 2026. doi:10.1001/jama.2026.14809.
Hakim, Alan. "Hypermobile Ehlers-Danlos Syndrome." GeneReviews, University of Washington, Seattle, updated 2024.
Malfait, Fransiska, et al. "The 2017 International Classification of the Ehlers-Danlos Syndromes." American Journal of Medical Genetics Part C, vol. 175, no. 1, 2017, pp. 8-26.
Malfait, Fransiska, et al. "The Ehlers-Danlos Syndromes." Nature Reviews Disease Primers, vol. 6, 2020, article 64.
Raj, Satish R., et al. "Canadian Cardiovascular Society Position Statement on Postural Orthostatic Tachycardia Syndrome (POTS) and Related Disorders of Chronic Orthostatic Intolerance." Canadian Journal of Cardiology, vol. 36, no. 3, 2020, pp. 357-372.
Rashed, Eman R., et al. "Cardiovascular Manifestations of Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders." Vascular Medicine, vol. 27, no. 3, 2022, pp. 283-289.
Weiler, Catherine R., et al. "AAAAI Mast Cell Disorders Committee Work Group Report: Mast Cell Activation Syndrome (MCAS) Diagnosis and Management." Journal of Allergy and Clinical Immunology, vol. 144, no. 4, 2019, pp. 883-896.
Yew, Kenneth S., Kara A. Kamps-Schmitt, and Robyn Borge. "Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders." American Family Physician, vol. 103, no. 8, 2021, pp. 481-492.
