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When searching for avm survivors I came across an amazing young lady that had an avm in her foot which led to amputation - her smiles & braveness inspire me. Below is her story
Zahava was born first, she made me a mom, and came into the world with the mentality that she’d be first for everything. She was the first granddaughter on both sides of our family and she has continued to show the world that she will be uniquely first for anything she can and center stage for as long as Gd continues to allow her spark to shine in this world. To that extent, long before any crazy diagnosis her personality inspires, leads and provides light to others where ever she goes.
I distinctively remember her first-year monthly-check-ups; head-to-toe checks and those lengthy questionnaires about the baby’s development. Everything she did was “advanced” and “above average”. I didn’t question a thing- except for the strange red birth mark on her right toe. Every appointment I asked the pediatrician if it was normal and was reassured it was “just a birth mark” a “port wine stain” they called it. I continued to ask each year, at each appointment, something just didn’t feel right about it, but I was always reassured that it was “just a birthmark.”
And it was “normal” until Zahava was 7 years old and going in for a routine tonsillectomy. Moments before the surgery, nurses came to take her back to the OR, Zahava was climbing into the hospital bed and stubbed her right foot on the wheel of the hospital bed. She was crying so loud everyone on the floor could hear her and NOTHING could console her. The nurses kept promising toys and rewards and pain meds but nothing could calm her down and no one could figure out how stubbing her toe could make her have THAT much pain. Eventually they were able to get her calm enough to take her into the OR and after about an hour the ENT came out to say the surgery had gone as expected but that we really should get her foot xrayed because she probably broke some toes.
Since Zahava was on pain meds for the next 2 weeks while home recovering from the tonsillectomy, we forgot about the broken toes. Finally the day when she was ready to go back to school, she was getting her shoes on and started screaming out to me “mommy my shoes won’t fit on, my foot is too big” that’s when I remembered the broken toes. It was then that we went for the X-rays. What the X-rays revealed however was not broken toes but some mass growing in her foot and the radiologist was convinced we needed a dermatologist because of her birthmark “port wine stain” on the exact toe she had stubbed. A few weeks later when we finally got an appointment with the dermatologist we were told that we were at the wrong type of doctor and needed to go to an oncologist because the mass was most likely cancer.
At that point I began panicking thinking of all the horrible things that could go wrong with my child. But thank gd when we finally got to the oncologist a month later, he took one look at the X-rays and said very confidently that it was not cancer. He then referred us to an Interventional Radiologist at the children’s hospital who finally made the diagnosis of AVM. Since AVMs are rare to begin with but showing up in a foot are even more rare it was a hard diagnosis to confirm. So it made sense that we had been pushed around to several doctors.
During these few month’s Zahavas foot had swollen so badly that she could no longer wear shoes. She had been given a walking g boot by the ER and could barely get comfortable enough to put weight on that foot with the boot on. She was trying to be a kid but slowly her childhood was being taken from her.
When we finally met with the IR doctor at the children’s hospital he explained what exactly an AVM was and after an initial angiogram discovered that the mass in Zahavas foot was actually a cluster of 5 AVMs throughout her foot and toes. The plan was to embolize the 3 larger ones but there were 2 that were 2 small to touch. We’d have to wait until Zahava grew more to operate on those. After embolizing the 3 she seemed to be doing ok (despite still not being able to wear shoes or walk normally) until one day a few months later when she started complaining of pain again. We went back in for some more scans. It seems that although the blood flow was stopped to the 3 large AVMs her the blood redirected itself and created new flow into NEW AVMs. She now has 10 AVMs instead of the initial 5. The plan was to go back in and embolize the large ones again.
This process of embolizing the new growth of AVMs occurred almost every 6 weeks for 3 years. During that time it was COVID and Zahava had been in 3 different schools because her anxiety was making it difficult for her to perform academically. Finally the IR doctor presented us with a new trial drug for kids with her type of AVM. It was an immunosuppressant drug, Sirolimus and she’d now need to be monitored by an oncologist. Zahavas blood pressure and lipid panel went crazy from being on the sirolimus but those seemed like small prices to pay to manage the AVMs and not have surgeries every 6 weeks. Zahava was on the sirolimus for a year with no surgeries and we truly thought it was our “miracle drug”. She was able to wear crocs (not any other shoes) and she could sort of limp around as opposed to before in the walking boot.
About a year into taking the sirolimus the skin on her foot started breaking down and becoming ischemic and ulcerated. So ischemic that she needed wound care daily, and again couldn’t walk on her foot and eventually needed
a knee scooter to get around. She would hobble around the house on the side of her foot but definitely couldn’t wear shoes and definitely wasn’t able to live a normal 10 year old life.
It was then that our team of doctors at children’s hospital in Chicago suggested we go to CHOP in Philadelphia because they have a specialized vascular anomaly program that focuses all their time on kids with AVMs. The oncologist who heads the team, Dr Denise Adams has been the head of o colony at Cincinnati, Boston and Philadelphia and has privileges at even more hospitals. As they call her, she’s the “mother of all oncologists”. She was in the middle of putting together a plan for Zahava moving forward and had intentions of presenting it to us at our next appointment. Then one Friday afternoon Zahava walked into my parents house in New Jersey (we were visiting them so we could go to our appointment at CHOP) and my father noticed some blood coming out of her sock, she looked down and proceeded to pull her sock off. Upon pulling her sock off blood began squirting out faster than anyone could control. 911 was immediately called and zahava was airlifted from my parents home to CHOP - an hour and half flight away. By the time we got there she had a tourniquet on and they informed me they might need to amputate if the bleeding didn’t stop when they removed it.
Thank Gd the bleeding did stop but she was on permanent bed rest and now a grade 4 AVM patient. It was apparent that she was going to have more artery bleeds and something drastic needed to happen in her treatment plan. It was then that Dr Adams presented us with our options, a little sooner than she had anticipated. She told us that the sirolimus had actually done more harm than good, that our doctors in chicago didn’t truly know how to treat AVMs - they did their best but that’s not their specialty, and what looked on the surface like a “cure” was actually making the AVMs grow bigger. She told us that there is a new trial drug, selumetnib a targeted therapy (basically radiation in pill form). She said that the AVM world has seen great results when given selumetinib at the onset of new AVMs anywhere in the body, but that it wouldn’t be wouldn’t be able to cure or heal the old ones. Basically the only thing to do at this point was to amputate Zahavas leg and THEN have her take the selumetnib for about 6-8 months post the amputation to try to “retrain” her brain to not create new AVMs like it had done in the past.
Now Zahava is 12 years old. She had her amputation just 5months ago in January 2023. She was diagnosed with AVMs in April of 2018. And she started this new drug selumetnib in April of 2023. We are hoping her future is bright but it has been a long miserable journey and we count our blessings everyday that although her journey was bumpy at least her AVMs we’re in her foot and not her brain. We truly empathize with all the other warriors and survivors on this site and in the world.