Focus On The Fight | Hereditary Lymphedema

Welcome to Focus On The Fight, a series of interviews that will be posted weekly, focusing on a blogger/advocate, their health and their advocacy work.

This week we meet Jordyn, and we will highlight: Hereditary Lymphedema aka Primary Lymphedema.  


Before we dive into the heavy stuff, please tell us all a little about yourself outside of your health! About your family or your hobbies !!

I love to cuddle my service dog, read books, blog, crochet, share my story online, and talk to my friends. I also especially love to garden when it's summertime.

What Chronic Illness (es) have you been diagnosed with? Which one will you focus on today?

Hereditary Lymphedema Erythromelalgia Ehlers Danlos Syndrome Type 3 Chronic Fatigue Postural Syndrome Orthostatic Tachycardia Syndrome Polycystic Ovarian Syndrome Daily Migraines Asthma MTHFR mutation. Today I will be focusing on my Hereditary Lymphedema.

Let’s take just a second to look at Lymphedema before we jump into Jordyn’s Interview.

What is hereditary lymphedema?

According to the National Organization For Rare Diseases Hereditary lymphedema is a genetic developmental disorder that affects the lymphatic system that is characterized by swelling (edema) of certain parts of the body. Hereditary lymphedema is also known as primary lymphedema. The lymph system is a circulatory network of vessels, ducts, and nodes that filter and distribute certain protein-rich fluid (lymph) and blood cells all throughout the body.

Lymphedema occurs when the lymph vessels are unable to adequately drain the lymph fluid from the affected arm or leg. The Mayo Clinic says that Lymphadema can be diagnosed as primary or secondary. Which means it can occur on its own (primary lymphedema), or it can be caused by another disease or condition (secondary lymphedema). Secondary lymphedema is by far the most common and is seen more than primary lymphedema.

What are symptoms of hereditary lymphedema?

  • Swelling of part or all of your arm or leg, including fingers or toes

  • A feeling of heaviness or tightness

  • Restricted range of motion

  • Aching or discomfort

  • Recurring infections

  • Hardening and thickening of the skin (fibrosis)

The swelling that is caused by lymphedema can range from mild, with hardly noticeable changes in the size of the affected arm or leg to extreme changes that will make the limb difficult to use.

What are the causes of Secondary Lymphedema?

  • Any condition or procedure that can damage your lymph nodes or lymph vessels can cause lymphedema. Causes include:

  • Surgery - The removal of or injury to the lymph nodes and the lymph vessels may result in lymphedema. For example, lymph nodes may be removed to check for spread of breast cancer, and lymph nodes may be injured in surgery that involves blood vessels in your limbs.

  • Radiation treatment for cancer -  Radiation can cause scarring and inflammation of your lymph nodes or lymph vessels.

  • Cancer - If cancer cells block lymphatic vessels, lymphedema may result. For instance, a tumor growing near a lymph node or lymph vessel could enlarge enough to block the flow of the lymph fluid.

  • Infection - An infection of the lymph nodes or parasites can restrict the flow of lymph fluid. Infection-related lymphedema is most common in tropical and subtropical regions and is more likely to occur in developing countries.

What are the causes of primary lymphadema?

Primary lymphedema is a rare, inherited condition that is caused by problems with the development of lymph vessels in your body. Specific causes of primary lymphedema include:

  • Milroy's disease (congenital lymphedema). This disorder begins in infancy and causes lymph nodes to form abnormally.

  • Meige's disease (lymphedema praecox). This disorder often causes lymphedema around puberty or during pregnancy, though it can occur later, until age 35.

  • Late-onset lymphedema (lymphedema tarda). This occurs rarely and usually begins after age 35.

How is lymphedema diagnosed?

If you are at a high risk of developing lymphedema or had a cancer surgery that involves your lymph nodes, the doctor may diagnose you based on signs and symptoms. If the cause of your lymphedema isn’t clear your doctor may order further imaging studies so they can get a closer look at your lyonnaise system. Per Mayo Clinic diagmostic tests may include:

  • MRI scan. Using a magnetic field and radio waves, an MRI produces 3-D, high-resolution images.

  • CT scan. This X-ray technique produces detailed, cross-sectional images of your body's structures. CT scans can reveal blockages in the lymphatic system.

  • Doppler ultrasound. This variation of the conventional ultrasound looks at blood flow and pressure by bouncing high-frequency sound waves (ultrasound) off red blood cells. Ultrasound can help find obstructions.

  • Radionuclide imaging of your lymphatic system (lymphoscintigraphy). During this test you're injected with a radioactive dye and then scanned by a machine. The resulting images show the dye moving through your lymph vessels, highlighting blockages.

How is lymphedema treated?

There is no cure for lymphedema si treatment focuses on reducing swelling and controlling the pain. Per Mayo Clinic treatment options include:

  • Exercises. Light exercises in which you move your affected limb may encourage lymph fluid drainage and help prepare you for everyday tasks, such as carrying groceries. Exercises shouldn't be strenuous or tire you but should focus on gentle contraction of the muscles in your arm or leg. A certified lymphedema therapist can teach you exercises that may help.

  • Wrapping your arm or leg. Bandaging your entire limb encourages lymph fluid to flow back toward the trunk of your body. The bandage should be tightest around your fingers or toes and loosen as it moves up your arm or leg. A lymphedema therapist can show you how to wrap your limb:

  • Massage. A special massage technique called manual lymph drainage may encourage the flow of lymph fluid out of your arm or leg. And various massage treatments may benefit people with active cancer. Be sure to work with someone specially trained in these techniques. Massage isn't for everyone. Avoid massage if you have a skin infection, blood clots or active disease in the involved lymph drainage areas

  • Pneumatic compression. A sleeve worn over your affected arm or leg connects to a pump that intermittently inflates the sleeve, putting pressure on your limb and moving lymph fluid away from your fingers or toes.  Compression garments. Long sleeves or stockings made to compress your arm or leg encourage the flow of the lymph fluid out of your affected limb. Wear a compression garment when exercising the affected limb.

  • Complete decongestive therapy (CDT). This approach involves combining therapies with lifestyle changes. Generally, CDT isn't recommended for people who have high blood pressure, diabetes, paralysis, heart failure, blood clots or acute infections

Please share Briefly what led you to the decision to become a blogger who focuses on chronic illness and patient advocate (if that Applies to you).

I have been sick for a long time. Over two years ago, I was at the worst that I have ever been. We were still looking for answers and I had become bed bound at this point. I had a lot of time to think and questioned myself, "What if I never find out what is going on with me?" I was not only interested in the situation, I did not want to do it.

Diagnosing a Chronic Illness can sadly be a VERY long process! For you, how long did it take for an official diagnosis? And did you have to act your own advocate to get a doctor to take what was going on in your life seriously and look for a diagnosis?

It took eight 1/2 years from the time that I was experiencing symptoms, until the time I was diagnosed last year. I had to advocate for every single treatment that I have gotten. Especially since lymphedema causes swelling, it can be misconstrued as being overweight. Doctors would label me fat, tell me to exercise, despite my other diagnosed conditions, and would not look into it.

Chronic illness is just that, chronic. Please share with us how your illness (es) impacts your daily life!

My chronic illnesses impact my daily life by making me mostly homebound. I have a hard time standing and walking for over 5-10 minutes. I get dizzy and will pass out if I sit up too fast. My wardrobe includes several sizes of clothing because of the lymphedema and how swollen I am that day.

Being chronically ill can take a toll on all kinds of relationships. Have you noticed this in your life? If so how has your health impacted the relationships in your life?

The only people in my life to understand and believe my chronic illnesses are my mom, grandma, grandpa, two friends, and an online friend. Day to day, the only person that is physically here is my mom. I have had to advocate for myself to be able to understand and unfortunately many of my friends and family did not.

Living with chronic illness can be life changing. Have you found that your life has changed significantly over the years of living with your condition? If so, in what ways has it changed?

My life has changed significantly since I became ill at 14 years old. For the first several years, I was still able to be relatively active and participate in things with a local church youth group. Now at 21, I do not leave the house unless it's a doctors appointment or the occasional outing with the friend.

All diseases have different kinds of flare ups, and every one will exhibit different signs of flare ups. For you, what signs do you know to watch when a flare is coming your way?

The signs that I know to watch for swelling, pressure in the abdomen, fire like pain, hardness in the abdomen, and the need to change into a larger clothing size. (for hereditary lymphedema)

What do you do to treat your flares? Or to make your bad days better?

On flare days, I like to lay flat to make my stomach feel like there is less pressure on it. I play my favorite music, watch youtube, and hang out in my room. A lot of the times blogging during a flare helps me as well. I have found that it is lessens the emotional side that comes with a flare.

Being diagnosed with a chronic illness can be very overwhelming. If you could give one piece of advice to someone who is going through the diagnosis process, or is newly diagnosed, what would it be?

My piece of advice to someone going through this journey is to trust yourself. Doctors are going to misdiagnose you, say hurtful things, and make you question yourself. Trust that you know what you are going through and keep going, eventually there will be a doctor who understands.

Living with a chronic condition is hard, it just is! Especially because for the most part there are no outward symptoms that can be seen by others. If you could share one thing with the public about living with a chronic illness what would you want them to know?

I want the public to know that just because I look okay or am feeling happy, it does not mean that I am not dealing with something at the moment. I am always dealing with something, whether physically or mentally. I just do not always talk about it, because then that's all I would talk about.

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With Love,

Amber