Lymphedema: What Is the Age of Onset for Lymphedema?

Lymphedema, a condition characterized by swelling due to malformation, damage, or obstruction to the lymphatic system, can manifest at various life stages. This guide explores the different types of lymphedema and when it may occur in a person’s lifetime. Throughout this guide, you’ll learn effective treatment and management strategies, providing you with the knowledge needed to understand and manage this condition at any age.

What Is Lymphedema?
What Age Does Lymphedema Occur?
How Do You Treat Lymphedema at Any Age?
From Birth to Elderly: Understanding Lymphedema at Different Ages

What Is Lymphedema?

Lymphedema is a chronic condition characterized by swelling, typically in the arms or legs, due to the accumulation of lymph fluid. This buildup occurs when the lymphatic system, which is responsible for draining excess fluid and waste from tissues, is impaired. Lymphedema can cause discomfort, limited mobility, and changes in the skin, such as thickening or hardening.

Common symptoms of lymphedema include persistent swelling in the affected region, typically occurring in the limbs but can be present in other areas of the body, such as the head, neck, abdomen, chest, and genitals. Patients with lymphedema may experience a feeling of heaviness or tightness, restricted range of motion, and recurring infections. As the condition progresses, the swelling can become more pronounced, and the skin may take on a leathery texture. Additionally, those with lymphedema might experience aching or discomfort in the swollen areas.

The causes of lymphedema are varied, ranging from hereditary factors where the condition is passed down from a parent to secondary causes, such as cancer treatment or an infection.

Understanding these lymphedema risk factors is important for early diagnosis and effective management. Recognizing early symptoms allows for proactive treatment, potentially reducing the severity and progression of the condition. With timely medical advice and intervention, living with lymphedema becomes more manageable, ultimately improving your overall quality of life.

What Age Does Lymphedema Occur?

Lymphedema can occur at any age, from birth to late adulthood. Understanding the difference between primary and secondary lymphedema helps clarify its underlying causes, guide appropriate treatment strategies, and improve your management outcomes.

Primary lymphedema is a result of errors in lymphatic development and can present itself at different stages of life, such as congenital lymphedema at birth, or presenting after the first year.

On the other hand, secondary lymphedema, which is more common, occurs due to injury or trauma to healthy lymphatic systems. This can result from cancer, cancer treatments, infection, chronic venous insufficiency, or blood clots, amongst other causes meaning it can develop at any age. Additionally, there is a strong correlation between lymphedema and obesity, as excess body weight can exacerbate the condition and complicate its management.

Recognizing the symptoms and risk factors across these different lymphedema age groups will ultimately help in timely intervention and treatment.

Primary Lymphedema

In cases of primary lymphedema, there is typically a deficiency or malformation in the lymphatic vessels, impairing the drainage of lymph fluid from tissues. This impairment leads to the characteristic swelling, often noticeable in the arms, legs, or other parts of the body. The exact cause of primary lymphedema can vary, sometimes appearing due to genetic mutations affecting lymphatic development. It may also occur spontaneously without a clear familial history, and at other times primary lymphedema is associated with another syndrome.

Some classification systems of primary lymphedema group age of onset into either congenital lymphedema from birth to age 2, lymphedema praecox from puberty to age 35, or lymphedema tarda after age 35. But, with more recent advancements in research, primary lymphedema can now be categorized into 5 different groups based on a person’s traits, and only two of those categories classify primary lymphedema by age as seen below.

Lymphedema age: <1 year
Congenital onset lymphedema presents at birth. Milroy disease is an example of congenital lymphedema.

Lymphedema age: >1 year
Late onset lymphedema presents after 1 year. An example of this would be lymphedema distichiasis syndrome.

Secondary Lymphedema

Secondary lymphedema may occur at any age and occurs in the absence of anatomical malformation.

Early diagnosis and management are crucial for both primary and secondary lymphedema. While the condition itself is not curable, prompt intervention can help manage symptoms and prevent complications. Treatment usually involves a combination of therapies, including compression garments, manual lymphatic drainage, and skin care practices to maintain skin integrity.¹

How Do You Treat Lymphedema at Any Age?

Lymphedema, regardless of your age group, follows a consistent treatment approach aimed at managing symptoms and preventing progression. Working with a certified therapist is crucial, as they can tailor a personalized lymphedema treatment plan based on your individual needs and current health conditions. This comprehensive approach typically includes:

• Complete decongestive therapy (CDT): This structured treatment combines various techniques to reduce swelling and improve lymphatic function. It involves manual lymphatic drainage (MLD), a gentle massage technique that stimulates lymph flow, and compression therapy to maintain reduced swelling.
• Manual lymphatic drainage (MLD): MLD is a specialized massage technique performed by trained lymphedema therapists to encourage the flow of fluid, helping to reduce swelling and promote fluid drainage. MLD techniques can also be learned to perform by yourself at home. Additionally, for at-home management, pneumatic compression devices can mimic MLD’s rhythmic movements, aiding in maintaining lymphatic flow in the affected area.
• Compression: Wearing compression garments or bandages helps to support swollen tissues, prevent fluid buildup, and maintain the progress achieved through therapy sessions.
• Skin care: Proper skin care, including keeping the skin clean, moisturized, and protected from injury or infections, is crucial to prevent further complications.
• Weight management: Maintaining a healthy weight is a key component of managing lymphedema symptoms, as obesity can further strain the lymphatic system and cause increased swelling.
• Emotional support: Living with lymphedema at any age can have a significant emotional impact. Participating in support groups or seeking therapy can provide valuable emotional support, offering strategies to cope with challenges and maintain a positive outlook.
• Exercise: Regular physical activity and lymphedema exercises, tailored to your individual capabilities, promote circulation and lymphatic drainage, enhancing your overall mobility and reducing swelling.
• Healthy diet and hydration: Adopting a healthy lifestyle, including maintaining a balanced diet, staying hydrated, regular exercise, and avoiding activities that may injure your affected limb(s), supports overall lymphatic health and continues to reduce your swelling.
• Elevation: Elevating the affected limb above the heart allows gravity to encourage the drainage of fluid to reduce swelling.

From Birth to Elderly: Understanding Lymphedema at Different Ages

Understanding lymphedema across different ages reveals its diverse manifestations and challenges, but you’re not alone in managing this condition throughout life’s stages. From the early challenges of congenital lymphedema to the adjustments needed in later years, each phase presents unique hurdles that you can address with proactive care and support.

Managing lymphedema means embracing a personalized approach that suits your needs and lifestyle. It’s about staying informed, seeking early intervention, and adopting effective strategies for your lymphedema self-care at home. Tactile Medical’s Flexitouch Plus and Entre Plus systems are innovative solutions that help you alleviate swelling and maintain lymphatic health on your own terms. These pneumatic compression devices bring the therapy of manual lymphatic drainage straight to the comfort of your own home.

By taking charge of your lymphedema care, you empower yourself to live more comfortably and confidently at any age. Whether you’re just starting to navigate the challenges of lymphedema or seeking new solutions for long-term management, remember that each step you take toward self-care and treatment brings you closer to a better quality of life. Stay informed, stay proactive, and let each day propel you forward in your journey with lymphedema.

References:

1. Van Zanten M, Mansour S, Ostergaard P, et al. Milroy Disease. 2006 Apr 27 [Updated 2021 Feb 18]. In: Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1239/

2. Vemulakonda SHP, Gaur NK, Shaikh OH, Kumbhar US. Lymphoedema praecox in a young woman: a rare disease. BMJ Case Rep. 2021 Sep 17;14(9) https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8451299/