Connective Issues Spring 2018



of POTS in individuals with EDS. One term you might see thrown about is dysautonomia. This means that the part of the nervous system that is supposed to be acting automati- cally, without conscious action, is not working appropriately. Our autonomic nervous system has a huge impact on blood circulation, digestion, and mood, so it is not surprising that in EDS, a syndrome with marked dysautonomia, POTS would be prevalent. Treatment of POTS varies depending on the suspected cause. Some of the simpler treatments include compression stockings and exercise to improve blood flow when standing. In some cases, a doctor may recommend increasing the amount of fluid that you drink, as well as increasing your intake of electrolytes and salt. There are also several medica- tions that may be prescribed, including beta blockers, one of the first line treatments for protecting the heart from aortic dissection in Marfan syndrome, but none of these treatments work 100 percent of the time. Because of its range of severity and presentation, POTS therapy must be individualized and monitored closely. The care of a knowledgeable physician is necessary to help manage POTS. Diagnosis and proper treatment of POTS can be difficult because aspects of the condition often mirror other more common conditions such as vertigo, generalized anxiety disorder, irritable bowel syndrome, and being generally out of shape. Having a proper diagnosis of EDS can help as it will raise physician’s suspicion of POTS. This is challenging because EDS doesn’t always present in a textbook manner or respond to treatment as predicted. If you believe you are suffering from POTS, advocating for yourself with your physician is of utmost importance to ensure high quality care. POTS may be a daunting chronic diagnosis, but it can be monitored, treated, and controlled once a patient has the proper tools. Please contact the Foundation’s Help & Resource Center at if you have questions on POTS, or any other medical questions related to EDS, Marfan syndrome, and other related disorders.

By Bob Graybill Reviewed by Dr. Heidi Connolly and Dr. Alan Braverman

Connective tissue disorders go far beyond hyperextending elbows and back pain. In some cases, heart issues related to connective tissue are not limited to life-threatening aortic dissections and valve disorders. Positional Orthostatic Tachycardia Syndrome (POTS) commonly impacts people with Ehlers Danlos syndrome (EDS) Type III (hypermobility syndrome). POTS is a rise in heart rate upon sitting straight or standing from sitting or lying down... combined with the myriad of symptoms that accompany it in EDS patients, a To understand POTS, let’s break down the name of the condition. First, positional: this means that POTS changes based on whether someone is standing, sitting, or lying down. Next, orthostatic: this means “relating to or caused by an upright posture.” Finally, tachycardia means a rapid heart rate. Hence, POTS is a rise in heart rate upon sitting straight or standing from sitting or lying down. This sounds simple, but when combined with the myriad of symptoms that accompany it in EDS patients, a complicated and difficult to diagnose picture can emerge. Common symptoms of POTS include dizziness, lighthead- edness, weakness, blurred vision, and fatigue on standing. Some people also suffer from palpitations, changes in vision, and anxiety. These symptoms are combined with issues in other body systems. Common gastrointestinal symptoms that often go along with POTS may include nausea, cramping, constipation, and diarrhea. Chronic headaches, fainting, and swelling of the legs may also happen. If you think you are suffering from POTS, one of the indications is an increase in your heart rate upon standing using a special table called a tilt table. The exact cause of POTS in EDS has yet to be completely explained; however, its link to Ehlers Danlos is well docu- mented. Contributing factors include issues with the volume of fluid in the body, blood flow, and the nervous system. The nervous system issues are often considered a primary cause complicated and difficult to diagnose picture can emerge.

Bob Graybill is a naturopathic doctor practicing primary care at Vitality NW in the Portland (OR) area. He is passionate about helping individuals with Marfan syndrome and other connective tissue issues because of his experience with his daughter, wife, and extended family’s Marfan diagnoses. Bob is a member of the Foundation’s Marfan Writing Group.


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