Click Here to Download this Blog Post – The One We Can’t Miss: Understanding Vascular EDS

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

In the world of Ehlers-Danlos Syndrome, there is one specific type that doctors are always on high alert for. It is rare, but it is the one diagnosis we absolutely must make early.

It is called Vascular EDS (vEDS).

Why is this one different?

As we discussed in the last post, the common type (hEDS) can be painful and exhausting, but it is benign—meaning you have a normal life expectancy.

Vascular EDS is different. Because it affects the strength of your major blood vessels and organs, it can be life-threatening. The average life expectancy for untreated patients is often in the 40s. This is why getting a diagnosis before an emergency happens is the most important goal of any EDS specialist.

The “Silent” Disguise

Here is the tricky part: Vascular EDS often doesn’t look like EDS.

If you have the common type (hEDS), you probably have super-stretchy skin and very loose elbows and knees.

• vEDS patients usually do NOT have stretchy skin.
• vEDS patients usually do NOT have loose large joints (though their fingers or toes might be flexible).

Because they don’t look like the “textbook” flexible patient, they are harder to spot during a physical exam. This requires doctors (and patients) to be detectives.

The Red Flags: It’s All in the History

Since the physical signs are subtle, the clues almost always come from your medical history or your family tree.

You should alert your doctor immediately if you or a family member have a history of:

1. Aneurysms or Dissections: This is when a blood vessel bulges or tears. If this happened to a relative at a young age, it is a major warning sign.
2. Organ Rupture : A history of intestines or bowels tearing (perforation) without a clear cause.
3. Surgical Complications: If tissue tore unexpectedly or failed to heal during surgery, especially during childbirth or gynecological procedures.
4. Severe Bleeding: We aren’t just talking about easy bruising (which is common in all types). We mean significant, hard-to-stop bleeding.

The Takeaway

If you have hypermobility and pain but none of the red flags above, the odds are overwhelmingly in your favor that you have the benign type (hEDS).

However, if your family history includes sudden vascular events or organ trouble, do not ignore it. Genetic testing can confirm vEDS, allowing you to take preventative steps that can save your 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

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

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

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.

2. 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.

3. 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

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