By: Dana Wolinsky

When my son was four years old, he was given his first soccer jersey, specifically, a red Manchester United jersey bearing the name Chicharito on the back. He had no idea who Chicharito was or that soccer was played outside the four corners of the AYSO field. So his father pulled up a YouTube video to show my son a clip of Chicharito in action, and just like that, he fell in love with all things soccer. From that point forward, we spent hours watching YouTube clips of Lionel Messi’s top 100 goals, debating whether Messi or Christiano Ronaldo was the best player in the world, and, as any mother would do, I invariably let his arguments in favor of Messi prevail. We spent even more hours playing one-on-one, him pretending to be his favorite team – Manchester United – and assigning to me whatever team was the worst in the league. And, as any mother would do, I invariably let him win.

Fast forward three years, and we were still rooting for the mighty Red Devils. We were still playing “football” (now that we were true fans, we felt obliged to switch terminology) non-stop, only now my son was running circles around me fair and square. He had now graduated from AYSO to competitive soccer. And, as any mother would do, I spent hours schlepping him to and from practices, games, and camps. During one camp session in July 2015, while my son was playing a friendly scrimmage, another boy stepped on his foot in the exact wrong spot. That was the beginning of it all. The pain began, and it never ceased. His foot swelled up, and it never shrunk.  

We spent the next year jumping from doctor to doctor, trying to determine what was wrong. As with many patients of rare diseases, that year was filled with countless doctors’ appointments, absences from school, misdiagnoses, and poking and prodding of my son as if he were the poor earthworm in a high school biology classroom. With each x-ray, MRI, and surgical biopsy, my son hopefully asked, will this be the last one; did they fix my foot? As the year progressed, his foot became more swollen. He felt excruciating pain every time it came into contact with anything, every time he ran, and as time went on, even when he walked. Eventually, he began to walk with a limp. Yet he continued to play the sport he loved so much with a smile, even though simply putting on his cleats caused him to shriek out in pain. And, as any mother would do, I continued to cheer him on, holding back tears as I watched him limp across the soccer field.

As it turns out, you learn a lot about your child when adversity strikes. My husband and I learned that our son is strong. He is brave. He is selfless. And he is unwaveringly optimistic. With the doctors’ green light, through unspeakable pain, he never missed a game, despite our pleas for him to rest. He wants nothing more than to play with his friends and to be a good teammate. And his attitude never suffered.

With perseverance, we inched closer and closer to a diagnosis. Finally, in the summer of 2016, after talking to countless orthopedists, interventional radiologists, oncologists, hematologists, dermatologists, pathologists, and other specialists, we were provided with our answer, first by the wonderful Birthmarks and Vascular Anomalies team at Children’s Hospital of Wisconsin, and then confirmed by the amazing Vascular Anomalies team at Boston Children’s Hospital: my son has a fibro adipose vascular anomaly (FAVA), a disorder so rare that when our friends Googled it to learn more, instead of finding a Wikipedia definition, they were directed to websites discussing the legume. Fibro adipose vascular anomalies were first recognized only in 2014 by, among others, members of Boston Children’s team. So, as any mother would do, we hopped on a plane to Boston to determine our options. We were told news that no parents want to hear: there is no cure and no slam dunk treatments. After weighing the pros and cons, we picked the least invasive option with the lowest risk of complications and hoped for the best.

My son has now been taking the medication, Sirolimus, for his FAVA for the past three months. As we have come to expect, he bravely endures the side effects, including painful mouth ulcers, his new cholesterol-lowering diet (who knew he would find bison burgers to be a satisfactory substitute for In-N-Out?), and monthly trips to the lab for blood work.

While the medicine has managed his pain immensely, my son does not bear the burden of knowing what I know – that his pain may resume when weaned off the medication; that, even if it doesn’t, it is most likely to surface again when he goes through puberty; that he may need to ingest this medication and endure its side effects again in the future; that the swelling will increase as he gets older; that, short of major medical advancements, the pain may increase, to the point where we may eventually need to resort to crutches, a wheelchair, or worst case scenario, amputation.

As any mother would do, I will continue to be by his side as we deal with his FAVA. As he continues to dream of playing professional soccer one day for Manchester United, I will continue to hold him as he cries from the pain. I will continue to distract him with humorous stories as he is poked with needles. I will continue to lift his spirits when he is self-conscious about the appearance of his enlarged foot. I will continue to stay on top of research as experts explore the uncharted waters of this disorder. I will continue to fight against the insurance companies’ anticipated chants of “deny, deny, deny!” I will continue be his greatest advocate until hopefully, one day, a cure is found. And my heroic little Red Devil will continue to inspire me.  

 

 

2 thoughts on “When FAVA Isn’t Just a Bean: Living with a Fibro Adipose Vascular Anomaly”

  1. Brianna Jennett-McNeill says:

    Thank you for your story. I too was diagnosed with FAVA 2 years ago, I feel his pain. Unfortunately in Canada I do not have any specialists, so I’m left playing the waiting game.

  2. Margaret F says:

    What a beautiful story! I know the pain and swelling all too well. I have been diagnosed with FAVA and lived with it my whole life. I am 20 years old. Sirolimus truly has given me my quality of life back because I am not in constant pain. I’ve been on it for almost 3 years. The Vascular Anomalies Center at Boston Children’s is truly amazing. I am glad your son doesn’t let this disease get to him and he still does what he loves!

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