Living Well with LDS
Around the time of diagnosis, individuals with Loeys-Dietz syndrome (LDS) may receive imaging, treatment for noticeable symptoms, and emergency medical care.
However, factors that impact quality of life (pain, mental health, and exercise) are often overlooked and leave people and families with more questions than answers. It is best to address these concerns from the start with a multidisciplinary team of healthcare professionals.
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Pain Management and Loeys-Dietz Syndrome
While pain may not go away completely, proper pain management can help to decrease pain levels and improve quality of life.
Proper pain management is based on:
- Identifying the source of pain;
- Building a multidisciplinary medical team;
- Implementing a combination of pain-reduction strategies including patient education, physical therapy, psychotherapy, and pharmacological therapy.
Chronic pain is best managed by a multidisciplinary team of professionals who understand and/or are willing to actively learn about chronic pain and connective tissue disorders. The team may include a primary care provider, chronic pain specialist, psychiatrist, psychologist, physical therapist, and occupational therapist.
An occupational therapist is a health professional who helps people perform daily tasks at work, school, home, and leisure activities. A physical therapist is a health professional who helps individuals improve movement and reduce pain through patient education, physical care, and exercise recommendations. Physical and occupational therapists can help people protect their joints and exercise correctly.
How can you protect your joints? With proper posture, an ergonomic work or school set-up, taking a break from repetitive mechanical activities (like typing, chopping food, and vacuuming), and external bracing. For people with hypermobility, joint protection and stability are especially important.
Over-the-counter or prescription medication can be used to relieve pain. It is generally recommended to use medication along with other pain-reduction strategies.
Cannabis can be a safe and effective way to manage pain. There are several ways to use cannabis, such as smoking, vaporization, or ingestion.
To learn more about cannabis for pain management:
- Dr. Jordan Tishler, a cannabis specialist, presents a webinar on medical marijuana and pain management.
- It is recommended to consult with a medical professional about using cannabis. They can learn about an individual’s pain management needs and explain the dosage, timing, and pros and cons of different administration methods (such as smoking, vaporization, and ingestion).
There are many reports of Loeys-Dietz syndrome (LDS) and chronic pain (pain lasting at least three or four months).
A 2020 study of people with LDS subtypes 1-4 revealed that 79% had chronic musculoskeletal pain. Pain can impact physical and mental health, sleep quality and energy levels, and ability to participate in work, school and leisure activities.
People with LDS may have pain in various areas of the body, including:
- Neck pain;
- Spinal or back pain;
- Rib and breathing pain;
- Abdominal pain;
- Arm pain;
- Ankle and knee pain.
People with LDS may experience different types of pain, such as:
- Mechanical pain: pain from stress on joints and muscles. This pain may be caused by hypermobility, joint instability, or improper posture.
- Neuropathic pain: pain related to the nervous system.
- Inflammatory pain: pain related to inflammation and tissue damage. This pain may occur after surgery, injury, diet, or daily repetitive movements (like walking and chopping food) that can cause overuse injuries.
LDS patients have reported that pain intensity and flare-up frequency increase as the temperature drops. Colder weather can worsen joint and muscle pain, pain related to inflammatory and autoimmune disorders, and conditions like depression and anxiety that may coexist with chronic pain.
Tips to limit pain in colder weather:
- Stay warm: turn up the heat in your home, dress in warm layers, use an electric blanket or heating pad, take warm baths or showers, and drink warm beverages like coffee, tea, or hot chocolate.
- Stay active: gentle, low-intensity movement is good for the body and the mind.
- Stay hydrated: drink enough water and avoid alcohol.
- Sleep well: try to get sufficient, good-quality sleep.
- Eat well: focus on eating a variety of healthy foods, and try to avoid foods that may increase inflammation like red meat, fried foods, processed starches, and sugar.
- Get enough sunlight: natural light may help alleviate feelings of depression or anxiety (which may coexist with chronic pain), and enjoying the sunshine outside can also help you to stay active.
- Avoid tobacco: smokers may be more vulnerable to developing and feeling chronic pain.
- Enjoy yourself: social activities and hobbies can be a good distraction and mood-booster.
- Speak to a professional: pain is complex and the tips and tricks listed above may not cause a substantial pain-reduction on their own. Medical and mental health professionals are in the best position to help you find the source of your pain and build a care plan that works for you.
Staying Fit with Loeys-Dietz Syndrome
Individuals with Loeys-Dietz syndrome (LDS) are encouraged to stay fit within their limits. Movement is important for general mental and physical health and can lower heart rate and blood pressure over time. Proper exercise can help the body to become stronger and more stable, decrease the risk of injury and pain, increase the release of endorphins (substances in the body that can improve mood and decrease pain), and improve cardiovascular health by reducing blood pressure and heart rate.
What kind of exercise is safe for people with Loeys-Dietz syndrome?
- Speak to a professional. Individuals should discuss their physical activity plans with a medical professional. A professional can determine what type of movement is safe based on each individual’s physical features, such as cervical spine instability, heart valve function, and size of blood vessels like the aorta. A professional can also propose an exercise routine that is tailored to each individual’s body, needs, and goals.
- Be ready to start slow. It is recommended to start slowly. Over time, exercise frequency and time spent exercising may increase.
- Think long-term. The physical benefits of exercise may take time to appear. For some people experiencing pain, it may take months to stop feeling worse and a year or more to start feeling better.
- Get regular but gentle exercise.
- Perform light and moderate aerobic exercises in moderation. These activities include swimming, walking, gentle hiking, jogging, and cycling.
- A moderate level of activity means being able to have a conversation without needing to take breaths in the middle of short sentences
- Exercise any muscle group to the point of exhaustion. This includes both static (isometric) and dynamic (aerobic/anaerobic) activities.
- Maintain an elevated blood pressure or heart rate. Individuals with LDS should not maintain a heart rate greater than 140 beats per minute.
- Perform abrupt isometric exercises that strain muscles, such as sit-ups, pull-ups, rope climbing, resistance training, or heavy weight training.
- Participate in gymnastics or tumbling as they may contribute to cervical spine instability.
- Perform contact or high-intensity sports such as football, basketball, handball, rowing or any activity with a high risk of a sharp blow to the head or chest.
- Participate in activities with rapid acceleration or deceleration, such as running sprints, as they may increase the risk of blood vessel dissection.
- Use movements that hyperextend joints or cause joint injury or pain.
Mental Health and Loeys-Dietz Syndrome
Living with Loeys-Dietz syndrome (LDS) can be difficult and affect an individual’s mental health. It is normal for an LDS patient as well as their friends and family to experience feelings of overwhelm, anxiety, depression, anger, uncertainty, fear, isolation, PTSD, and more during diagnosis or at any stage of the LDS journey.
Loeys-Dietz syndrome can cause acute, high-stress, and life-threatening health events (hospitalization, surgery, etc.) as well as chronic, long-term struggles (balancing work and school with LDS, chronic pain, family dynamics, etc.). Both acute and chronic components can take an emotional toll. It is crucial that people and families with LDS find the resources and support they need.
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