Common Symptoms
- Swelling in arms, legs, or other body parts
- Heaviness or tightness in affected limbs
- Restricted range of motion
- Recurring infections (cellulitis)
- Skin changes including hardening or thickening
- Aching or discomfort in affected areas
Understanding Primary Lymphedema
Primary lymphedema is a relatively rare condition that occurs when the lymphatic system doesn’t develop properly. Unlike secondary lymphedema, which results from damage to a previously normal lymphatic system, primary lymphedema is caused by inherent abnormalities in the lymphatic vessels or nodes.
Types of Primary Lymphedema
Congenital Lymphedema (Milroy Disease)
Present at birth or appearing within the first two years of life. This hereditary form typically affects the legs and feet and is caused by mutations in genes responsible for lymphatic development.
Lymphedema Praecox (Meige Disease)
The most common form of primary lymphedema, typically appearing during puberty or young adulthood (before age 35). It most often affects the legs and is more common in females.
Lymphedema Tarda
Develops after age 35 and is the least common form of primary lymphedema. The cause is often unclear, though there may be a genetic component that causes the lymphatic system to fail over time.
What Causes Primary Lymphedema?
Primary lymphedema results from abnormalities in the lymphatic system, including:
- Missing or malformed lymph vessels: The lymphatic channels may not develop completely
- Fewer lymph nodes: Reduced number of lymph nodes to process lymph fluid
- Damaged valves: Valves within lymph vessels may not function properly
- Genetic mutations: Several genes have been identified that affect lymphatic development
Diagnosis
Diagnosing primary lymphedema typically involves:
- Detailed medical history and family history
- Physical examination
- Ruling out secondary causes
- Imaging studies (lymphoscintigraphy, MRI, or other tests as needed)
- Genetic testing in some cases
Living with Primary Lymphedema
While primary lymphedema is a lifelong condition, effective management can help you:
- Control swelling and prevent progression
- Reduce the risk of infections
- Maintain function and mobility
- Participate in work, activities, and life
Our Treatment Approach
At LORC, we understand that primary lymphedema requires lifelong management. Our approach includes:
- Comprehensive Evaluation: Thorough assessment of your condition and its impact on your life
- Complete Decongestive Therapy: Evidence-based treatment to reduce swelling
- Self-Management Training: Teaching you techniques to manage your condition independently
- Compression Fitting: Ensuring you have properly fitted garments for long-term maintenance
- Ongoing Support: Regular follow-up and adjustments to your treatment plan as needed
Family Considerations
If you have primary lymphedema, family members may also be at risk, particularly for hereditary forms. We can provide:
- Information about the genetic aspects of your condition
- Guidance for at-risk family members
- Referrals for genetic counseling when appropriate
How We Can Help
Our certified lymphedema therapists provide comprehensive treatment using Complete Decongestive Therapy (CDT), including manual lymphatic drainage, compression therapy, therapeutic exercise, and skin care education. We work with you to develop a personalized management plan that helps control swelling and improves your quality of life.