Understanding Primary Lymphedema

People born with lymphedema have primary lymphedema, also known as congenital or idiopathic lymphedema. It may be caused by malformation at birth or other dysfunctions of lymph nodes and collectors, but the reason is not always known. Researchers continue to learn more about primary lymphedema and its cause. There are three common types of primary lymphedema: hypoplasia, hyperplasia and aplasia.

Hypoplasia accounts for the majority of primary lymphedema cases. This means the lymph collectors are too small or too few to carry out the lymph fluid produced by the body. The lymph system might be working but could be half the size of normal lymphatics, so it has to work harder just to keep up with normal loads. This leads to lymphatic collector failure because of fatigue. Also, as an infant grows, their limited lymphatic system (that is already over stressed) can’t keep up.

Hyperplasia is less common and results from lymph collectors that are too large. The valves in the lymph system do not shut properly and allow lymph fluid to flow backwards. This back flow can cause more fluid buildup and expand the collector, making the problem worse as time goes on. Eventually, as a child grows and the lymph system continues to weaken and swell in size, what little fluid the lymph system carried out of the skin will slowly lose its effectiveness.

Aplasia or “no growth of lymphatics” is the least common diagnosis and is simply a lack of growth of lymph collectors.

Symptoms of primary lymphedema vary. Swelling can occur in entire extremities, a section of the extremity or multiple locations. The face and organs can be affected by the lack of functioning lymphatics. If you see this swelling in your child, consult with your pediatrician and ask for a referral to a lymphedema therapist. A lymphedema therapist is a highly trained medical professional (usually from physical therapy, occupational therapy, massage therapy or nursing) who can help reduce swelling and manage lymphedema. It is important to remember to tell your medical professionals to talk to each other, so they can formulate the best plan for your success and reduction.

Treatment for primary lymphedema is the similar to other lymphedema treatments, which includes Complete Decongestive Therapy (CDT), but it will be customized to your (or your child’s) needs. CDT includes Manual Lymph Drainage (MLD), compression, exercises and skin care to reduce your swelling. Using all four parts for your therapy will give you the best results and training to manage your swelling for the long term.

Step 1: MLD is the mild stimulation of the skin (similar to massaging the skin) and specific to you. Your therapist should have specialized training to do it properly with effective results. They will manually (and gently) move the fluid from your swollen area to healthy nodes that will filter it before it’s reabsorbed into the blood stream.

Step 2: Compression starts with bandaging, using a short-stretch compression wrap or bandage, that will help reduce the size of your limb. This compression helps prevent back filling of edema/swelling into your affected area. People often ask why they have to wear compression garments for so long. This is because fluid is moving around your body 24/7, and some fluid is left behind when you are not compressed. Lymphedema wrapping is not like other wrapping, it is specific to your swelling and it is graduated (meaning firmer at the lower end and decreasing firmness as you get closer to the heart). Eventually, you’ll wear medical compression garments instead of wrapping, which are easier to put on and take off. These garments also offer more consistency in maintaining your reduced limb size.

Occasionally, people think the compression garments are supposed to reduce their swelling, but this is not true. Garments are made to maintain your limb size, not make it smaller. Learn more about the benefits of medical compression therapy. It’s important to understand that your treatment is based on your unique swelling. If you have minimal swelling in your limb, you will likely wear mild compression. Your therapist and certified fitter will determine the appropriate size, compression range, style and material with the necessary degree of containment for your medical compression garment. Today’s compression garments are available in a variety of fashion colors and print choices. Spend some time finding the right garment for you.

Step 3: Each person has different needs when it comes to exercise. It is important to stay active in your compression to move the fluid and blood out of your limb. Your therapist may suggest repetitive exercises to increase the joint muscle pump and range of motion. They also may suggest different shoes to help increase fluid and blood flow out of your legs as you walk. Your therapist also will work with you to become independent with your lymphedema self-care. That means you will need range of motion to wrap your extremity and put on or take off your compression garments. Each exercise will meet your needs but also help you reach the goals you set during your initial evaluation.

Step 4: Skin Care is essential since lymphedema is limited only to the skin in almost all cases. That makes taking care of you skin extremely important, as your skin can fill up like a sponge, get stretched out and become fragile and thin on the outer layers. The larger your skin gets, the more likely you are to contract fungal or bacterial infections due to the excess fluid and the body’s limited ability to fight infections in the affected skin area – this can lead to wounds. Cleaning your skin and knowing signs and symptoms of infections are very important, as is keeping your skin soft with lotions. Note that Juzo garments are not affected by any lotions you use.

If you find after two weeks you are not seeing results, sit down with your therapist and discuss your progress or lack thereof. It may be possible there are other issues that are making you swell or slowing your progress. Either way, make sure you are talking with your pediatrician, physician and therapist to determine what is the best course of action and make sure they are talking to each other about your diagnosis, symptoms and treatment.