Primary lymphedema is a rare, inherited condition caused by problems with the development of lymph vessels in the body. Primary lymphedema occurs most frequently in women, it can be divided into three types.
Milroy’s disease (congenital lymphedema). This is an inherited disorder that begins in infancy and causes the lymph nodes to form abnormally, leading to lymphedema. This is responsible for between 5-10% of cases. It affects multiple limbs and other areas.
Meige’s disease (lymphedema praecox). This hereditary disorder often causes lymphedema in childhood or around puberty sometimes in the 20s. It causes lymph vessels to form without the valves that keep lymph fluid from flowing backwards, making it difficult for your body to properly drain the lymph fluid from the limbs. This accounts for 75-80% of cases and affects usually one leg and often just the foot, calf or ankle.
Late-onset lymphedema (lymphedema tarda). This occurs rarely and usually begins after age 35. This late onset Lymphedema effects about 10% of cases.
World wide there are millions of cases of Lymphedema but often they go untreated or undiagnosed. All types of primary lymphoedema have inadequate lymphatic drainage. The cause is not well understood, it is probably due to genetic faults or from abnormalities that occur in the development of the lymphatic system during gestation.
This is Jessica’s story of living with Primary Lymphedema from a young age and the impact it has on her life. English is not Jessica’s first language so I have edited some but not all her words.
“The year I turned Nineteen left an unforgettable mark on my life. My father died suddenly and I was taken by surprise, I was confronted with the strange path of fate and the unbearable feeling of the irreversible…Years later I would realise that in the same year I would again be confronted by something irreversible…. Primary Lymphedema.
Some months after my father died, my friends tried to cheer me up and we went together on holiday. It was a hot summer, I had some insect bites also before my trip I was working in a restaurant, to earn money for the holiday. During that time I noticed that my feet were swollen….but off course nobody knew what it was and nobody took it seriously.
Later I was diagnosed with primary lymphedema in both legs, at the time I was very frustrated with that diagnosis – although I did not really know the consequences then. I thought it was just horrible to have swollen feet during the summer… When I think of this now, it just sounds stupid… I would gladly to have that situation now!!
Eight years went by and somehow I adapted quite well: in fact most of my friends never noticed. Twice a week I went to the physiotherapist for manual lymph drainage and during the summer I had to wear some kind of compression stockings but only occasionally. The only thing I could not do was wear high heels during summer… so hello flip flops! I spent my years quite care-free: going on long adventure trips, working long days in the restaurant to pay for university and my holidays!
When I finished my degree I started working but on my way home one day I had an accident and I fell on my leg. It started to get swollen but I thought it was temporary and although I had a major infection I did not stay home from work. However as time passed I started to worry, doctors told me it would get better, but that due to my history of primary Lymphedema it would take more time. Six months of intense taping, bandaging, MLD and swimming, did not help it was still the same. I started to panic and visited lots of doctors and all they had for me was bad news, bad news and more bad news. That’s when I found out about the true burden of having primary Lymphedema ….. nobody knows anything about it…and you get the feeling you are the only person in the world with it.
I had lots of testing done, and people were freaking out about it. Visibly I have no big signs (except for my one leg), but the tests showed I have huge problems in both legs… When you are lying there under the scanner praying to get good news once, but the doctors think the scanner is broken because there is nothing to see… then you know that you have troubles.
Some of the things they said are still wandering around in this little head of mine:
“I do not know a lot… but I know that for 100% it will get worse.”
“When you will have kids, it will get much worse, you know.”
“And when you have kids, it is possible that they can have this too, because it is
Ohhhh! Thank you for the information! That makes me feel …. really desperate….!!!
But to stay positive, because I will not give in to this situation. What exactly can I do about it to get it better?
“You can do a lot of things, but it will not get better.” Oh!
“But you can stabilize it… With MLD, wear your compression stockings from when you
get up till you go to bed, swim a lot etc.”
“And there are a lot of things you can avoid, so it will not get worse!!!”
“Be careful with mosquito bites, long trips, alcohol, long standing, heat, warm baths, sunburn, avoid sunbathing, walking barefoot, scratches etc!!!”
Basically do all the boring stuff and stay away from the fun stuff!!!
I thought my life was over, I could never live with this new situation, but a year and a half later I managed to adapt somewhat to this new concept. The mind is strong and flexible and is the most intriguing thing I discovered the past year. What do I miss? What I miss the most is to be able to live care-free as I used too. I never thought I would miss getting out off my bed, seeing the sun and walking barefoot into my garden.
I might find a solution one day – hopefully but things will never be the same just because I know what I know….But then again I am grateful that I got to live 19 years care-free, unlike some children who are born with this…I started to check the internet and I saw the most frightening stories and pictures… I did not know what to do. That is how I found out about the lymph node transfer … and I jumped on the other roller-coaster a search for a solution!!!” Jessica..
Thank you Jessica for sharing your story.. It is not easy to put into words the daily struggle to find a balance and still live a fulfilling life. Jessica has been in touch with Dr Corinne Becker in Paris and may have a Lymph node Transfer in the future, but it is not an easy decision.