One fat leg and one thin leg: Parkes-Weber syndrome
Isa-Bella Leclair is a likeable young Canadian woman. She is studying Civil Engineering at the University of Ottawa, plays the guitar and piano, practices yoga, loves spending time with her friends and – like many in her age-group – keeps an Instagram account. This is where Isa-Bella publically shows how she differs from most 21-year-olds. Her right leg is almost twice as thick as the left, because Isa-Bella has been living with Parkes Weber Syndrome since birth. This causes abnormalities in the blood vessels, which is why lymphatic fluid accumulates in the individual limbs. As a result, Isa-Bella Leclair developed lymphoedema. In the medi healthcare interview, she reports on how she masters everyday life with the disease, what role compression garments play and why everybody should enjoy life passionately and confidently.
Ms Leclair, you suffer from Parkes Weber Syndrome (PWS). When was the diagnosis made?
“I was diagnosed with PWS when I visited the Boston Children’s Hospital in 2003. Before that, my diagnosis had been Klippel-Trénaunay Syndrome (KTS), a very similar disease.”
What are the symptoms of the disease and when did you recognise the first signs?
“The doctors diagnosed me with KTS at birth. The symptoms that led them to that conclusion were hypertrophy of my lower right limb (feet, leg and hip) and a port-wine stain that covered the same area. As I grew up, the doctors noticed that I was not developing the other symptoms associated with KTS, such as pain in the affected limb. Then other symptoms started showing such as heart problems and primary lymphoedema. In 2003, at the age of seven, I visited Boston Children’s Hospital and was diagnosed with PWS.”
Is the Parkes Weber Syndrome curable?
“No, there is no cure for PWS, only ways to manage it.”
How was the disease treated in the past? Was the therapy successful?
“I have never had treatment for PWS specifically, but more for its symptoms. I have been followed closely by orthopaedic specialists and cardiologists since I was very young. In Boston in 2003, I was supposed to get an embolisation (surgical technique that blocks the blood supply to a specific area of the body), but the risks ended up being too high compared to the rewards. I have also been treated for lymphoedema with compression stockings and lymphoedema massage therapy.”
© J.Vaillancourt Corp. Valco
How did you hear about medi and our compression stockings?
“I have been wearing compression stockings since I was eight years old. At first, I was with a different company than medi, but the stockings were ultimately not effective enough for the level of lymphoedema I had. Then, one day, during an intensive session of lymphoedema massages, my physiotherapist recommended medi. I have been wearing medi compression stockings since 2015.”
What is your experience with the mediven flat knit garments?
“The mediven flat knit garments are really great! They are comfortable and never slip down around the knee or ankle. The toe cap is also amazing and has improved the condition of my toes significantly.”
Do you wear compression garments in bright colors?
„The compression garment that I wear the most is the skin coloured one. I live in Canada and it is cold most of the year, so I am always wearing long pants that mostly cover the compression garment. I also wear the black ones a lot under dresses. I generally wear the bright coloured one during summer with shorts and dresses.”
Where do you get information about the issue lymphedema?
“When I have a specific question about lymphoedema, I usually ask my physiotherapist, who is certified in lymphoedema treatment and massage. I sometimes research the web for more general questions.”
Do you get support from your family and friends?
“My family and friends have always been extremely supportive! They do not see me differently, because I have PWS or lymphoedema, and they are also always there during appointments or when I get sick.”
Do you have any special hobbies?
“I have a lot of hobbies! I adore the arts and thus everything that involves drawing, writing, music, guitar playing, piano playing, etc. I also love to read and watch movies and TV shows. I love to cook and try new food, I follow a vegan lifestyle. I also love to spend time with my friends and my boyfriend.”
What kind of sport can you do with the syndrome?
„I can get involved in any sport that does not require too much effort from my heart. My favourite sports are snowboarding, biking and yoga, but I also love badminton, hiking and softball.“
Do you have any contact with other lymphatic patients?
„“I have got into contact with a couple of patients and some have also reached out to me. We have talked a little about our experiences with lymphoedema. I always love to give support to people with lymphoedema and sometimes get some good advice myself in return. I also enjoy being able to connect to others.”
© J.Vaillancourt Corp. Valco
You are very open-minded and published your story of Instagram. Why did you do that?
“The picture of myself in a bikini on Instagram was just a picture that I posted like any other person would post a picture of themselves in a bikini. I was sharing memories of my trip in Florida. I had not really intended any other use for it at that time. But when it got picked up by a media outlet that wanted to cover my story, I was happy to do so. It is a great picture, because it shows how I have been my whole life; confident in my body, even if it is different. At the time, I hoped that people affected with PWS, lymphoedema or any body image issue could find comfort in my story and that it could maybe spark some confidence in themselves.”
Is there anything you would like to say to people who are not very self-assured?
“Mostly I want to tell them that they are deserving. They deserve to be loved and appreciated by others, but most importantly by themselves. It is not something that can change overnight when you have low self-confidence, but like anything in life it can improve slowly and surely. Find something you love about yourself, not what you do not. And those parts you do not like, try to look at them from a different perspective. One of my favourite authors, Matt Haig, once said: Ugliness is just a failure of seeing.”
Ms Leclair, many thanks for the interview.
Parkes-Weber-Syndrome (PWS)
PWS is a complex congenital abnormality of blood vessels (angiodysplasia). It is caused by an unnatural, direct connection between a vein and an artery (arteriovenous fistula). This results in one-sided gigantism of the lower limb (hypertrophy of the bones and soft tissues). A port-wine stain (naevus flammeus) often covers the affected area. Some patients also develop lymphoedema in the affected leg that accentuates the asymmetry of the two lower limbs.
Klippel-Trénaunay-Syndrome (KTS)
Although the transition from PWS to KTS is smooth, the cause of the latter form of blood vessel abnormalities is not an unnatural connection between a vein and an artery. The exact cause has still not been elucidated. The external appearance of Klippel-Trénaunay Syndrome is similar: gigantism of the affected limb, formation of a naevus flammeus and the onset of varicose veins (varices).
Important note: It is essential to distinguish between the two syndromes, because the treatment is different. While surgical intervention may also be useful in PWS (e.g. embolisation), KTS is mainly treated conservatively.
The story of Isa-Bella and Valco
Lynn Vaillancourt (J. Vaillancourt Corp. Valco, Canada) first saw the picture of Isa- Bella in a bikini with the caption ‘Born this way’ in 2015. “Many patients with lymphoedema are sometimes discouraged and often tend to hide their limbs. So I was impressed by Isa-Bella’s positive message and found her to be ideal as the image for lymphoedema in Canada”, explains Lynn Vaillancourt. The result is an inspiring cooperation with photos and a short video. Valco also invited Isa-Bella to be a speaker at the 2017 Canadian Lymphedema Conference in Montréal where she gave an empowering presentation to patients from all over Canada.
Sources
All images are property of J.Vaillancourt Corp. Valco and are not available for download.