Vascular EDS (VEDS) is one, if not the most challenging type of EDS. It is caused by mutations in the COL3A1 gene. (1) Vascular Ehlers-Danlos Syndrome is a rare genetic disorder that affects the body’s connective tissues, primarily impacting blood vessels and internal organs. People with VEDS face unique challenges due to the fragility of their blood vessels, which can lead to life-threatening complications. Incorporating specific activities, maintaining a healthy diet, and engaging in suitable exercise can play a significant role in supporting individuals with VEDS. The prevalence of VEDS has been estimated to be about 1 in 90,000, but this estimate is likely to be low. (2) Explore these various strategies and recommendations to improve the quality of life for those living with VEDS.
While there is no cure for VEDS, several treatment options are available to manage the symptoms, reduce the risk of complications, and improve the quality of life of patients with VEDS. Further research is needed to develop and validate new therapies in VEDS. Find out more at DiSCOVER Celiprolol. From age 20 on, the survival curves for men and women are parallel, and pregnancy did not appear to reduce predicted survival. (3)
Activities for Living with VEDS
- Gentle and Low-Impact Exercises for VEDS Management
Engaging in low-impact exercises, such as swimming, walking, or cycling, can help improve cardiovascular health, muscle strength, and flexibility without putting excessive stress on fragile blood vessels. It is essential to consult with a healthcare professional experienced in VEDS management to determine the most suitable activities.
Here are some other examples of gentle and low-impact exercises that people with VEDS can do:
- Tai Chi
- Water aerobics
It is important to start slowly and gradually increase the intensity of your workouts over time. If you experience any pain or discomfort, stop the exercise and consult with your doctor.
- Avoiding High-Risk Activities When Living with Type 4 EDS
People with VEDS should avoid activities that carry a high risk of injury, such as contact sports, heavy lifting, and activities that involve sudden or jarring movements. Activities with a high risk of falls, such as skiing or skateboarding, should also be avoided due to the potential impact on blood vessels.
- Promoting Joint Stability
Joint instability is a common concern for individuals with VEDS. Engaging in exercises that promote joint stability, such as gentle stretching, yoga, or Pilates, can help strengthen the supporting muscles and reduce the risk of dislocations or subluxations.
- Other VEDS Safety Measures
Environmental safety is crucial for individuals with VEDS. Removing hazards that may increase the risk of falls, such as loose rugs or cluttered spaces, can prevent accidental injuries. Utilizing supportive devices, like handrails or shower chairs, can also enhance safety and mobility. In addition, it is important to make sure that the home is well-lit and that there are clear paths to walk through. Strokes, initially considered relatively uncommon among people with VEDS, represented about 10% of all vascular complications and was often associated with carotid and vertebral artery dissection. (4) Among all the medical problems to which patients with VEDS are exposed, vascular and/or organ rupture are the two most feared and deadly complications. (5) It is also important to have a plan in place in case of an emergency, such as a fire or a medical event.
Diet and Nutrition for People Living with Vascular Ehlers-Danlos Syndrome
Diet plays a significant role in supporting individuals living with Vascular Ehlers-Danlos Syndrome by providing essential nutrients, supporting overall health, and potentially minimizing the risk of complications associated with the condition. Consuming a well-balanced diet rich in nutrients is important for individuals with VEDS. Focus on incorporating a variety of whole foods, including fruits, vegetables, whole grains, lean proteins, and healthy fats. These nutrient-dense foods provide essential vitamins, minerals, antioxidants, and fiber that support overall health, tissue repair, and optimal functioning of the body.
Nutrient-Dense Food for Managing Vascular Ehlers-Danlos Syndrome
- Fruits and vegetables: Fruits and vegetables are packed with vitamins, minerals, and antioxidants. They are also a good source of fiber, which can help with digestion. Adequate fiber intake is important for promoting digestive health and regular bowel movements. Including fiber-rich foods can support gastrointestinal function and prevent constipation, which can be a concern for individuals with VEDS.
- Whole grains: Whole grains are a healthy source of complex carbohydrates, fiber, and vitamins. They can help to keep you feeling full and energized.
- Lean proteins: Lean proteins are an effective source of protein, which is essential for building and repairing tissues. They are also a great source of iron, which is important for carrying oxygen throughout the body.
- Healthy fats: Healthy fats are a potent source of essential Omega-3 fatty acids, which are important for brain health and overall well-being. They can also help to lower cholesterol levels. Omega-3 fatty acids, found in fatty fish, walnuts, flaxseeds, and chia seeds, have anti-inflammatory properties and may contribute to cardiovascular health. Including these sources of omega-3 fatty acids in the diet can potentially support blood vessel health and reduce inflammation.
It is important to eat a variety of nutrient-dense foods to ensure that you are getting all of the nutrients that your body needs.
Staying adequately hydrated is essential to maintain the health of blood vessels. Individuals with VEDS should consume sufficient amounts of water throughout the day. Avoiding excessive caffeine or alcohol intake is also advisable, as these substances can dehydrate the body.
Vitamin C is a water-soluble vitamin that is essential for human health. It is involved in many important bodily functions, including collagen synthesis, wound healing, and immune function. Vitamin C is also a powerful antioxidant, which means it can help protect cells from damage caused by free radicals.
Collagen is a protein that is found in connective tissues throughout the body, including skin, bones, muscles, and blood vessels. It is responsible for providing strength and structure to these tissues. Vitamin C is necessary for the production of collagen, so people with a deficiency in this vitamin may experience problems with their connective tissues.
Individuals with VEDS may benefit from taking vitamin C supplements under medical supervision. Vitamin C can help to reduce the risk of complications associated with this condition, such as blood vessel ruptures and joint problems. It is important to talk to a doctor before taking any supplements, as too much vitamin C can be harmful.
Vitamin C Rich Foods for Living with VEDS
- Citrus fruits, such as oranges, grapefruits, and lemons
- Bell peppers
- Sweet potatoes
- Brussels sprouts
- Collard greens
Foods to Avoid when Managing Vascular Ehlers-Danlos Syndrome
Avoid foods that may affect blood vessel integrity. Some individuals with VEDS may have increased sensitivity to certain foods that can affect blood vessel fragility. Arterial blood with high pressure gains rapid access to the venous system and leads to venous hypertension. (6) These can include foods high in histamine, such as aged cheeses and fermented foods. It is important to consult with a healthcare professional or a registered dietitian to determine if any dietary modifications are necessary based on individual needs.
It is important to remember that each person with VEDS is unique, and dietary needs may vary. Working with a registered dietitian experienced in managing VEDS can provide personalized guidance, taking into account individual preferences, specific dietary restrictions, and any additional medical conditions. Regular communication and collaboration with healthcare professionals, including physicians and dietitians, are crucial for individuals with VEDS. They can monitor and assess nutritional status, address any concerns or changes in symptoms, and provide ongoing support and guidance.
Some individuals with VEDS may have additional allergies or sensitivities to certain foods or medications. It is important to communicate these concerns to healthcare providers and receive appropriate guidance regarding any necessary dietary modifications or medication adjustments. VEDS patients should carry documentation of their genetic diagnosis, such as an emergency letter, MedicAlert®, or VEDS “passport.” (7)
Exercise for VEDS Management
- Physical Therapy for VEDS
Working with a qualified physical therapist experienced in VEDS management can be highly beneficial. They can design a personalized exercise program to improve joint stability, muscle strength, and flexibility while considering the unique needs and limitations of individuals with VEDS.
- Moderate Cardiovascular Exercise
Engaging in moderate-intensity cardiovascular exercises, such as brisk walking, stationary cycling, or using an elliptical machine, can help improve heart health and circulation. It is important to start slowly and gradually increase the intensity, always listening to the body’s signals and avoiding overexertion.
- Strength Training with VEDS
Incorporating gentle strength training exercises into the fitness routine can help build muscle strength,which can help to support the joints and prevent injuries. It can also help to improve balance and coordination, which can help to prevent falls. Strength training can help to improve cardiovascular health, which can help to reduce the risk of other health problems.
It is essential to use proper form, avoid heavy weights or resistance, and focus on controlled movements. Working with a knowledgeable trainer or physical therapist can ensure exercises are tailored to the specific needs of individuals with VEDS.
- Flexibility and Range of Motion Exercises
Stretching exercises can enhance flexibility, improve joint range of motion, and help prevent muscle tightness or stiffness. It is crucial to perform gentle stretching techniques, avoiding excessive or forceful stretching that may strain the joints or tissues.
- Warm-Up and Cool-Down
Prior to any exercise session, individuals with VEDS should engage in a thorough warm-up routine to prepare the body for physical activity. This may include light aerobic exercises, dynamic stretching, and joint mobility exercises. Warming up helps to increase blood flow to the muscles and joints, which can help to prevent injuries. It also helps to prepare the body for more strenuous activity by increasing heart rate and breathing rate. Similarly, incorporating a cool-down phase with gentle stretches and deep breathing can help the body recover and reduce the risk of post-exercise complications. Cooling down helps to gradually bring the body back to its resting state and prevents blood from pooling in the extremities. It also helps to flush out lactic acid, which can build up in the muscles during exercise and cause soreness.
Additional Considerations for Type 4 Ehlers-Danlos Syndrome Management
Regular Monitoring with VEDS Healthcare Support Team
Individuals with VEDS should maintain regular communication with their healthcare team, including physicians, geneticists, and specialists experienced in VEDS management. Regular check-ups, monitoring blood pressure, and other relevant parameters can help identify potential issues and make appropriate adjustments to the activity, diet, and exercise plans. It is important to recognize the signs of such disease, since the right diagnosis, treatment, and prevention of further lesions, may make the difference between life and death. Each patient may have different clinical aspects that require different approaches, so being aware of all sides of this pathology and keeping an open mind as clinicians could make a difference in these cases, regardless of the gravity of their clinical state. (8)
Genetic Counseling for VEDS
Genetic counseling can be beneficial for individuals with VEDS and their families, as it provides education, support, and guidance on genetic inheritance, family planning, and available treatment options. It can also help individuals better understand their condition and make informed decisions regarding their lifestyle choices.
Type 4 EDS Support Groups
Living with VEDS can be challenging both physically and emotionally. It is important to seek support from healthcare professionals, support groups, or counseling services to help individuals and their families cope with the psychological aspects of the condition. Developing a strong support network and sharing experiences with others facing similar challenges can provide invaluable emotional support.
Healthy Lifestyle Habits for Vascular Ehlers-Danlos Syndrome
Living a healthy lifestyle is crucial for individuals diagnosed with Vascular Ehlers-Danlos Syndrome (VEDS) to optimize their overall well-being and manage the unique challenges associated with the condition. In addition to activities, diet, and exercise, there are several other healthy lifestyle habits that individuals with VEDS can adopt:
Vascular Ehlers-Danlos Syndrome Medication Management
Some individuals with VEDS may require specific medications to manage symptoms or complications associated with the condition. It is important to strictly adhere to prescribed medications, follow dosage instructions, and communicate any concerns or side effects to healthcare providers. Medication management can help control blood pressure, reduce the risk of complications, and improve overall health.
Stress Management While Living with Type 4 Ehlers-Danlos Syndrome
Chronic stress can impact overall health and potentially worsen symptoms associated with VEDS. Implementing stress management techniques, such as mindfulness meditation, deep breathing exercises, yoga, or engaging in hobbies and activities that promote relaxation, can help reduce stress levels and improve overall well-being.
Type 4 EDS Sleep Hygiene
Adequate sleep is vital for overall health and well-being. Establishing a consistent sleep routine, creating a comfortable sleep environment, and practicing good sleep hygiene habits can support better sleep quality. Sufficient restorative sleep can contribute to improved immune function, mental well-being, and better management of chronic conditions like VEDS.
Injury Prevention in VEDS
Taking proactive measures to prevent injuries is crucial for individuals with VEDS due to the fragility of their blood vessels. This includes maintaining a safe living environment, removing hazards, and utilizing assistive devices such as handrails and non-slip mats. Wearing protective gear, such as helmets or joint braces, during activities that carry a potential risk of injury can also minimize the chances of accidents.
Genetic Counseling and Family Planning When Living with Type 4 Ehlers-Danlos Syndrome
Genetic counseling plays a crucial role in helping individuals with VEDS and their families understand the inheritance patterns, genetic testing options, and family planning considerations. Genetic counselors can provide information and support in making informed decisions about family planning, including preconception counseling and prenatal testing.
By adopting these healthy lifestyle habits, individuals with VEDS can proactively manage their condition, improve their quality of life, and reduce the risk of complications associated with VEDS. It is important to work closely with healthcare professionals experienced in VEDS management to tailor these lifestyle habits to individual needs and ensure optimal care.
What to Avoid when You Have VEDS
Avoidance of Smoking and Substance Abuse: Smoking and substance abuse can have detrimental effects on cardiovascular health and overall well-being. Individuals with VEDS should avoid smoking and limit or avoid the use of alcohol and recreational drugs, as they can exacerbate the risk of blood vessel complications and other health issues.
Engaging in appropriate activities, following a well-balanced diet, and incorporating suitable exercise can significantly support individuals with Vascular Ehlers-Danlos Syndrome (VEDS). It is essential for individuals with VEDS to work closely with healthcare professionals experienced in managing the condition to tailor activity plans, dietary recommendations, and exercise programs to their unique needs and limitations. By adopting a holistic approach and making informed lifestyle choices, individuals with VEDS can enhance their overall well-being, improve joint stability, maintain cardiovascular health, and reduce the risk of complications associated with this rare genetic disorder.
Type 4 Ehlers-Danlos Syndrome Treatment Study
Join the advancement of VEDS treatment methods. The DiSCOVER Trial is a decentralized study evaluating Celiprolol on VEDS-related events in individuals with confirmed diagnosis of Vascular Ehlers-Danlos Syndrome.
Individuals must have a confirmed diagnosis of VEDS based on genetic testing and meet specific clinical criteria, be at least 15 years old, and not currently pregnant to be eligible for the Discover Celiprolol trial.
As a beta-blocker medication, celiprolol works by alleviating the stress on blood vessels and other organs, which reduces the risk of rupture. Celiprolol is an investigational drug and therefore its safety and efficacy have not been established. There is no guarantee that celiprolol will receive health authority approval or become commercially available for Vascular Ehlers-Danlos Syndrome.
If eligible, participation is remote so you can continue living as you normally do. A registered nurse visits your home, and periodically study visits will be conducted online. All instructions will be provided, and the investigational drug will be shipped directly to you.
Learn more and find out if you are eligible for the VEDS clinical trial.
Have more questions? Check out the FAQ for some answers.
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