All Posts in Category: Ehlers-Danlos Syndrome

Safety First: Ruling Out the “Red Flags”

Click Here to Download this Blog Post – Safety First: Ruling Out the “Red Flags”

By Dr. Nicholas L. DePace, M..D., F.A.C.C – Cardiologist specializing in autonomic dysfunction, Ehlers-Danlos syndrome and POTS.

When you visit a specialist for connective tissue disorders, the first appointment might feel a bit intense. The doctor will ask about your family history, heart health, and specific symptoms.

They aren’t just being nosy. They are performing a critical safety check called a Differential Diagnosis.

The “Big Three” We Must Rule Out

Before a doctor can diagnose you with hEDS or HSD, they must first make sure you don’t have one of the rarer, life-threatening conditions. These conditions affect the aorta (the main artery from the heart) and blood vessels, so missing them is not an option.

Doctors specifically look for signs of:

  1. Marfan Syndrome : Known for affecting the eyes, heart valves, and aorta.
  2. Vascular EDS (vEDS): The type that causes organ rupture.
  3. Loeys-Dietz Syndrome: A condition similar to Marfan’s that affects blood vessels.

The Process of Elimination If the doctor checks your heart and history and rules these “scary” conditions out, they can breathe a sigh of relief. By eliminating the dangerous imposters, what is usually left is a hypermobility disorder like hEDS or HSD.

The Back Door Diagnosis

Interestingly, most patients don’t walk into the clinic saying, “I think my joints are too loose.” They walk in saying, “I feel dizzy, my heart races, and my stomach hurts.”

The Autonomic Connection We often diagnose hEDS backward.

  1. The Complaint: You come in for P&S Dysfunction (dizziness, fainting, digestion issues).
  2. The Investigation: We take a detailed history of your autonomic nervous system.
  3. The Discovery: While examining you, we notice your skin is stretchy and your Beighton Score (flexibility) is high.

We find that the loose joints (hEDS) are actually the cause of the nervous system glitches. In our experience, nearly 100% of hEDS/HSD patients struggle with this combination of loose joints and “busted wiring” (Orthostatic and P&S Dysfunction).

The Good News

This might sound overwhelming, but there is a very bright silver lining.

Unlike Marfan Syndrome or Vascular EDS, Hypermobile EDS (hEDS) has a normal life expectancy. It is a condition that affects your quality of life (pain and fatigue), not the length of your life. With the right management of your nervous system and joints, you can live a full, long life.

Where to Seek Expert Care?

It is important to seek out a clinician with expertise in EDS to make an accurate diagnosis and create a treatment plan. One of the nation’s leading dysautonomia treatment center is Franklin Cardiovascular Associates, under the direction of Nicholas DePace, MD, FACC. They are located in Sicklerville, New Jersey. franklincardiovascular.com, (856) 589-6034


About the Author

Nicholas L. DePace, MD, FACC is a board-certified cardiologist and Medical Director of Franklin Cardiovascular Associates. A graduate of the Mount Sinai School of Medicine, Dr. DePace has decades of clinical, academic, and research experience and has held faculty appointments as a Clinical Professor of Medicine, becoming one of the youngest full professors in Philadelphia at the time of his appointment.

Dr. DePace specializes in the diagnosis and treatment of autonomic nervous system dysfunction (dysautonomia), including POTS, autonomic dysfunction associated with Ehlers-Danlos syndrome (EDS), chronic fatigue, and anxiety-like conditions that are frequently misdiagnosed. He is nationally recognized for his work on parasympathetic and sympathetic (P&S) nervous system imbalance, a core mechanism underlying many complex chronic disorders.

In addition to treating patients from across the United States, Dr. DePace is a prolific clinical researcher and author of multiple nationally distributed medical textbooks published by Springer and W.W. Norton, focusing on autonomic dysfunction, mitochondrial disorders, cardiovascular disease, and mind–body medicine.

👉 View Dr. DePace’s professional profile
👉 View medical books by Dr. DePace

Read More

hEDS vs. HSD: Solving the Genetic Mystery

Click Here to Download this Blog Post – hEDS vs. HSD: Solving the Genetic Mystery

By Dr. Nicholas L. DePace, M..D., F.A.C.C – Cardiologist specializing in autonomic dysfunction, Ehlers-Danlos syndrome and POTS.

If you are navigating the world of joint pain and dizziness, you will likely hear two terms used constantly: hEDS (Hypermobile Ehlers-Danlos Syndrome) and HSD (Hypermobility Spectrum Disorder).

Patients often ask, “What is the difference? Is one worse than the other?”

The answer lies in our DNA, but it is a bit of a mystery story.

The “Missing Gene” of hEDS

Doctors strongly suspect that hEDS is Autosomal Dominant.

  • Translation: If you have it, there is a 50/50 chance you will pass it to your children.

However, unlike the other types of EDS, scientists haven’t found the specific gene yet. We know it runs in families; we know what it looks like, but we can’t point to the exact spot on your DNA map that causes it.

HSD: The “Sibling” Diagnosis

Hypermobility Spectrum Disorder (HSD) is essentially the sibling of hEDS.

You receive this diagnosis when you have the symptoms—the flexible joints, the pain, the fatigue—but you don’t quite meet the strict checklist for hEDS, or your family history is unclear.

The Bottom Line: Whether your chart says hEDS or HSD, the treatment is largely the same because the physical problems are almost identical.

The Rare Cousins: Vascular and Classical EDS

In specialized clinics, hEDS and HSD make up the vast majority of patients. It is very rare to walk in and find a case of Vascular EDS or Classical EDS.

  • These types do have known genetic markers.
  • If a doctor suspects these (due to dangerous symptoms like organ rupture or extremely stretchy skin), they will order genetic testing to confirm it.

The Real Culprit: Sympathetic & Parasympathetic Dysfunction

Whether you are labeled hEDS or HSD, you likely aren’t just dealing with loose joints. You are dealing with a “wiring” problem that causes two main issues:

1.The Gas Pedal: Sympathetic Dysfunction and Orthostatic Symptoms

  • What happens: You stand up, and your body forgets to pump blood upward.
  • The Result: Orthostatic Dysfunction. You feel dizzy, your heart races, and you have chronic fatigue.

2. The Brake Pedal: Parasympathetic Dysfunction

  • What happens: Your body over-reacts to stress with too much “calming” signal.
  • The Result: Syncope (fainting), digestive issues (stomach stops moving), anxiety, and inflammation.
  • Crucially: This “wiring glitch” actually amplifies pain. It turns the volume knob up on your nerves, making your joints hurt more than they should.

The “Fibromyalgia” Trap

This pain amplification leads to one of the most common mistakes in modern medicine.

At least one-third of hEDS/HSD patients have visited a rheumatologist and been diagnosed with Fibromyalgia.

  • The Misconception: Fibromyalgia is often treated as a mystery pain condition.
  • The Reality: For many, that “Fibromyalgia” is actually undiagnosed hEDS/HSD. The loose joints are causing the irritation, and the faulty nervous system (P&S dysfunction) is magnifying that pain signals.

If you have been told you have Fibromyalgia but also have flexible joints and dizziness, it might be time to look at your “wiring” instead of just treating the pain.

Where to Seek Expert Care?

It is important to seek out a clinician with expertise in EDS to make an accurate diagnosis and create a treatment plan. One of the nation’s leading dysautonomia treatment center is Franklin Cardiovascular Associates, under the direction of Nicholas DePace, MD, FACC. They are located in Sicklerville, New Jersey. franklincardiovascular.com, (856) 589-6034


About the Author

Nicholas L. DePace, MD, FACC is a board-certified cardiologist and Medical Director of Franklin Cardiovascular Associates. A graduate of the Mount Sinai School of Medicine, Dr. DePace has decades of clinical, academic, and research experience and has held faculty appointments as a Clinical Professor of Medicine, becoming one of the youngest full professors in Philadelphia at the time of his appointment.

Dr. DePace specializes in the diagnosis and treatment of autonomic nervous system dysfunction (dysautonomia), including POTS, autonomic dysfunction associated with Ehlers-Danlos syndrome (EDS), chronic fatigue, and anxiety-like conditions that are frequently misdiagnosed. He is nationally recognized for his work on parasympathetic and sympathetic (P&S) nervous system imbalance, a core mechanism underlying many complex chronic disorders.

In addition to treating patients from across the United States, Dr. DePace is a prolific clinical researcher and author of multiple nationally distributed medical textbooks published by Springer and W.W. Norton, focusing on autonomic dysfunction, mitochondrial disorders, cardiovascular disease, and mind–body medicine.

👉 View Dr. DePace’s professional profile
👉 View medical books by Dr. DePace

Read More
Young woman with signs of hypermobile Ehlers-Danlos syndrome (hEDS)

The “Tell-Tale” Profile: How to Spot hEDS in Young Women

Click Here to Download this Blog Post – The “Tell-Tale” Profile: How to Spot hEDS in Young Women (Part 20)

By Dr. Nicholas L. DePace, M..D., F.A.C.C – Cardiologist specializing in autonomic dysfunction, Ehlers-Danlos syndrome and POTS.

If you are a young woman (or parent of one) who seems to be “falling apart” without a clear reason, you fit the most common profile for Hypermobile Ehlers-Danlos Syndrome (hEDS).

While hEDS can affect anyone, clinical experts have noticed a very specific pattern, especially in young females. Recognizing this pattern is often the key to getting a diagnosis after years of confusion.

The Hallmark Sign: It’s Not Just the Joints

You might think the main sign of a joint disorder would be joint pain. While pain is definitely part of it, the #1 hallmark that doctors look for is actually Orthostatic Dysfunction.

What does that mean? Simply put: Your body doesn’t know how to handle gravity.

When a typical person stands up, their body automatically adjusts to keep blood flowing to the brain. In hEDS patients, this system fails. This is often a form of Dysautonomia (a glitch in the autonomic nervous system).

Common hEDS Symptoms in Young Women

If you have hEDS or HSD, you likely have a mix of these three major categories:

  1. The “Standing Up” Problems (The Hallmark)
  • POTS (Postural Orthostatic Tachycardia Syndrome): Your heart races wildly just because you stood up.
  • Dizziness: You feel lightheaded or see “stars” when moving upright.
  • Temperature Issues: Your internal thermostat is broken. You are sweating when others are cold, or freezing when others are hot.
  1. The Physical Structure
  • Hypermobility: You are “double-jointed” or just very flexible.
  • Easy Bruising: You find bruises on your legs and arms and have no idea where they came from.
  1. The “Crash”
  • Chronic Fatigue : This isn’t just being sleepy. It is a bone-deep exhaustion that sleep doesn’t fix.
  • Chronic Pain: A nagging ache in your muscles and joints that never really goes away.

The Odds Are in Your Favor (Sort of)

The world of Ehlers-Danlos Syndrome can be scary because there are 13 different types, including some that are dangerous to your blood vessels (Vascular EDS) or skin (Classical EDS).

However, here is the reassuring news: 98% of the patients seen in these specialized clinics have the Hypermobile Type (hEDS) or HSD.

While hEDS can make life difficult and painful, it does not carry the same life-threatening risks as the rarer types. It is by far the most common connective tissue disorder, even though many general doctors rarely diagnose it.

 

Where to Seek Expert Care?

It is important to seek out a clinician with expertise in EDS to make an accurate diagnosis and create a treatment plan. One of the nation’s leading dysautonomia treatment center is Franklin Cardiovascular Associates, under the direction of Nicholas DePace, MD, FACC. They are located in Sicklerville, New Jersey. franklincardiovascular.com, (856) 589-6034


About the Author

Nicholas L. DePace, MD, FACC is a board-certified cardiologist and Medical Director of Franklin Cardiovascular Associates. A graduate of the Mount Sinai School of Medicine, Dr. DePace has decades of clinical, academic, and research experience and has held faculty appointments as a Clinical Professor of Medicine, becoming one of the youngest full professors in Philadelphia at the time of his appointment.

Dr. DePace specializes in the diagnosis and treatment of autonomic nervous system dysfunction (dysautonomia), including POTS, autonomic dysfunction associated with Ehlers-Danlos syndrome (EDS), chronic fatigue, and anxiety-like conditions that are frequently misdiagnosed. He is nationally recognized for his work on parasympathetic and sympathetic (P&S) nervous system imbalance, a core mechanism underlying many complex chronic disorders.

In addition to treating patients from across the United States, Dr. DePace is a prolific clinical researcher and author of multiple nationally distributed medical textbooks published by Springer and W.W. Norton, focusing on autonomic dysfunction, mitochondrial disorders, cardiovascular disease, and mind–body medicine.

👉 View Dr. DePace’s professional profile
👉 View medical books by Dr. DePace

Read More

Why Does Standing Up Feel So Hard? The “Gravity Battle” in hEDS and HSD

Click Here to Download this Blog Post – Why Does Standing Up Feel So Hard? The “Gravity Battle” in hEDS and HSD
(Part 19)

By Dr. Nicholas L. DePace, M..D., F.A.C.C – Cardiologist specializing in autonomic dysfunction, Ehlers-Danlos syndrome and POTS.

If you live with Hypermobile Ehlers-Danlos Syndrome (hEDS) or Hypermobility Spectrum Disorder (HSD), you probably know the feeling: you stand up to start your day, and suddenly your heart is racing, your head feels foggy, and you’re hit with a wave of exhaustion.

It turns out, your body is fighting a literal battle with gravity. For the vast majority of patients with these conditions, the “glue” that holds the body together (connective tissue) is stretchy, but the “wiring” that controls the body (the nervous system) is also glitching.

 

The Two Main “Wiring” Glitches

Your Autonomic Nervous System has two main branches: the Sympathetic (the gas pedal) and the Parasympathetic (the brake pedal). In hEDS/HSD, these often malfunction in two specific ways:

  1. Sympathetic Withdrawal (The Failed Gas Pedal): Usually, when you stand up, your sympathetic nerves tell your blood vessels to tighten up, pushing blood upward to your brain. In many patients, this signal fails. The “gas pedal” cuts out, blood pools in your legs, and your brain is left “starving” for blood flow.
  2. Parasympathetic Excess (The Stuck Brake): Sometimes, when your body is under stress, the “brake pedal” slams down too hard. This can cause sudden drops in heart rate or energy when you actually need a boost.

How “Poor Blood Flow” Mimics Other Illnesses?

Because your blood isn’t being pumped efficiently to the top half of your body (a problem called poor perfusion), your organs start to send out distress signals. These signals are often mistaken for other serious diseases:

  • Heart & Lungs: You might feel palpitations, chest pain, or shortness of breath. Often, the heart is perfectly healthy—it’s just struggling because it doesn’t have enough blood to pump!

 

  • Brain & Mood: When the brain doesn’t get enough blood, it can cause brain fog, fatigue, and fainting. It can even trigger or worsen anxiety, depression, ADHD, or OCD. Your brain is essentially “revving” itself to stay awake and alert despite the low fuel.
  • Digestion: These same nerves control how food moves through your gut. When the wiring glitches, you get stomach pain, bloating, or bathroom issues.

The Difference Between “Stretchy” and “Dangerous”

While hEDS and HSD are the most common types, there is a much rarer form called Vascular EDS (vEDS). It is vital to know the difference:

  • hEDS/HSD: Usually involves “stretchy” skin, loose joints that pop out (subluxations), easy bruising, and chronic pain. While it makes life very difficult, it is not typically life-threatening.
  • Vascular EDS: This involves the blood vessels and organs themselves being fragile. Red flags include a family history of aneurysms, ruptured blood vessels, or intestinal tears—especially in people under 40.

The Path Forward: Testing the Wiring

The good news is that your organs are usually healthy! The problem is the P&S (Parasympathetic and Sympathetic) nerves that tell them what to do.

Specialized testing can help your doctor see exactly how your “wiring” is misfiring. Once you understand your specific needs, you can start a treatment plan—like salt, fluids, compression gear, or medication—to help your body win the battle against gravity.

 

Where to Seek Expert Care?

It is important to seek out a clinician with expertise in EDS to make an accurate diagnosis and create a treatment plan. One of the nation’s leading autonomic dysfunction centers is Franklin Cardiovascular Associates, under the direction of Nicholas DePace, MD, FACC. They are located in Sicklerville, New Jersey. franklincardiovascular.com, (856) 589-6034


About the Author

Nicholas L. DePace, MD, FACC is a board-certified cardiologist and Medical Director of Franklin Cardiovascular Associates. A graduate of the Mount Sinai School of Medicine, Dr. DePace has decades of clinical, academic, and research experience and has held faculty appointments as a Clinical Professor of Medicine, becoming one of the youngest full professors in Philadelphia at the time of his appointment.

Dr. DePace specializes in the diagnosis and treatment of autonomic nervous system dysfunction (dysautonomia), including POTS, autonomic dysfunction associated with Ehlers-Danlos syndrome (EDS), chronic fatigue, and anxiety-like conditions that are frequently misdiagnosed. He is nationally recognized for his work on parasympathetic and sympathetic (P&S) nervous system imbalance, a core mechanism underlying many complex chronic disorders.

In addition to treating patients from across the United States, Dr. DePace is a prolific clinical researcher and author of multiple nationally distributed medical textbooks published by Springer and W.W. Norton, focusing on autonomic dysfunction, mitochondrial disorders, cardiovascular disease, and mind–body medicine.

👉 View Dr. DePace’s professional profile
👉 View medical books by Dr. DePace

Read More

Could Poor Perfusion in EDS Be the Hidden Cause of 20 Different Symptoms?

Click Here to Download this Blog Post – The Great Masquerader: Why One Glitch Can Cause 20 Different Symptoms
(Part 18)

By Dr. Nicholas L. DePace, M..D., F.A.C.C – Cardiologist specializing in autonomic dysfunction, Ehlers-Danlos syndrome and POTS.

If you have Ehlers-Danlos Syndrome (EDS) or Hypermobility Spectrum Disorder (HSD), your medical chart might look like an alphabet soup of diagnoses.

You might be seeing different specialists for:

It can feel like your body is falling apart in ten different ways at once. But what if these aren’t ten separate diseases? What if they are just ten different symptoms of one underlying problem?

Understanding P&S Dysfunction in Ehlers-Danlos Syndrome

As we have discussed, the core issue in EDS/HSD is often a malfunction in the Parasympathetic and Sympathetic (P&S) nervous systems. When this “wiring” is faulty, it creates a domino effect that impacts almost every system in your body.

The biggest consequence of this glitch is Poor Perfusion.

  • Translation: Your blood isn’t getting pumped efficiently to the top half of your body.

When your nerves don’t tighten your blood vessels correctly, gravity wins. Blood pools in your legs, leaving your brain, heart, and upper muscles starving for oxygen.

What Is Coat-Hanger Pain?

One of the most common complaints in EDS patients is a deep, aching pain that runs across the tops of the shoulders and up the back of the neck.

Doctors often call this “Coat-Hanger Pain” because the pain follows the shape of a clothes hanger.

  • The Mistake: Many people think this is just “stress” or “bad posture.”
  • The Reality: This is actually a sign of poor blood flow. Because your upper body isn’t getting enough blood, the muscles in your neck and shoulders essentially stiffen and cramp. It is your muscles crying out for oxygen.

The Brain’s “Panic Button” (Adrenalin Storms)

When your brain realizes it isn’t getting enough blood (poor cerebral perfusion), it goes into survival mode. It thinks you are in danger, so it slams the alarm button.

This triggers an “Adrenalin Storm.”

  • Physically: You might feel sudden chest pressure, sweating, or a racing heart.
  • Mentally: You feel a surge of Anxiety or a Panic Attack.

This isn’t necessarily “psychological” anxiety. It is a physiological attempt by your body to force more blood to the brain.

The Connection Between Brain Fog, ADHD, and Cognitive Function

This lack of blood flow can even explain cognitive struggles.

  • Brain Fog: The brain is running on low battery.
  • ADHD/OCD: Some researchers believe that the hyperactivity seen in conditions like ADHD (or even high-functioning Autism) might be the brain’s way of keeping itself stimulated to maintain blood flow. It is “revving the engine” to keep from stalling.

A New Way to Treat: Why Treating the Nervous System Comes First?

This perspective changes everything about treatment. Instead of taking one pill for anxiety, one for pain, and one for digestion, the goal should be to treat the P&S Dysfunction first.

We have seen that when we fix the blood flow issues and calm the nervous system:

  1. The “Coat-Hanger” pain fades because muscles get oxygen.
  2. The “Anxiety” drops because the brain stops hitting the panic button.
  3. The “Brain Fog” lifts.

The Golden Rule: Treat the wiring first. If symptoms still persist after the blood flow is fixed, then (and only then) do we treat the specific organs.

Finding Specialized EDS Care

It is important to seek out a clinician with expertise in EDS to make an accurate diagnosis and create a treatment plan. One of the nation’s leading autonomic dysfunction centers is Franklin Cardiovascular Associates, under the direction of Nicholas DePace, MD, FACC. They are located in Sicklerville, New Jersey. franklincardiovascular.com, (856) 589-6034


About the Author

Nicholas L. DePace, MD, FACC is a board-certified cardiologist and Medical Director of Franklin Cardiovascular Associates. A graduate of the Mount Sinai School of Medicine, Dr. DePace has decades of clinical, academic, and research experience and has held faculty appointments as a Clinical Professor of Medicine, becoming one of the youngest full professors in Philadelphia at the time of his appointment.

Dr. DePace specializes in the diagnosis and treatment of autonomic nervous system dysfunction (dysautonomia), including POTS, autonomic dysfunction associated with Ehlers-Danlos syndrome (EDS), chronic fatigue, and anxiety-like conditions that are frequently misdiagnosed. He is nationally recognized for his work on parasympathetic and sympathetic (P&S) nervous system imbalance, a core mechanism underlying many complex chronic disorders.

In addition to treating patients from across the United States, Dr. DePace is a prolific clinical researcher and author of multiple nationally distributed medical textbooks published by Springer and W.W. Norton, focusing on autonomic dysfunction, mitochondrial disorders, cardiovascular disease, and mind–body medicine.

👉 View Dr. DePace’s professional profile
👉 View medical books by Dr. DePace

Read More