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Amara’s Story
Site created on April 24, 2021
Amara’s journey with a rare disease and a cancer diagnosis began on March 23, 2021, and has been full of emotional curve balls. On that Tuesday evening, we noticed Amara’s left leg having a bit of tremor, while she was on the diaper changing table and while she was standing in the bathtub. On Wednesday we saw it happen again a handful of times throughout the day, but thought nothing of it. She was still dancing and active, as always. On Thursday, March 25, her leg shook so much as she tried to move from standing to a squat, that she lost her balance. Our pediatrician was away that day but his colleague told us we should probably head to ER to get some neurological exams. We spent all evening and the early hours of her 18th month birthday (March 26) in the Emergency Room at UCLA Santa Monica. She got a painful IV (that required 5 people to hold her down while she screamed), some blood work, a CT scan, a pelvic x-ray and a consult with the neurologist. Tests came back negative, we were sent home, and told to return if things got worse.
Things got worse. The tremors increased significantly and moved to her left arm by Saturday. That night, we were admitted to UCLA Santa Monica hospital. She got an EEG that evening, and co-slept (very uncomfortably) on me on the hospital chair. She spent the morning fasting, and was sedated for an MRI and a lumbar puncture the next day. She got several blood draws, and urine tests. We spent a week at UCLA Santa Monica, during which time every single test came back negative and she started to lose her ability to walk. She was diagnosed with Acute Cerebellar Ataxia (ACA), possibly due to an unknown viral infection (as all viral panels came back negative). While our nights were difficult as she would wake up screaming in pain, she was a real hoot at the hospital – playing with all the nurses, trying hard to walk despite not being able to, and bearing it all in stride. We were discharged after 7 days on April 2, and told it may get worse but that it should clear up on its own. Our follow-up appointment with the neurologist was scheduled for 2 weeks later – on April 16. We were given no additional guidance on what worse actually means, and when we should be concerned.
That night, we noticed weird eye movements, which we later came to know were opsocolonus. We reached back out to UCLA Santa Monica and were told it doesn’t change her diagnosis of ACA. Our days at home were really tough. She had extremely disturbed sleep. One of us would have to rock her constantly to try to calm her to sleep. She seemed to have jerky body movements while she slept that woke her up, and her eye movements got worse. She lost her voice, and her speech slowed. She was drooling a ton, and now unable to hold herself up while seated. We kept holding on to any decrease in her tremors as a sign of her improvement. But in all honesty, she was getting worse. On Tuesday, April 6 we got her an ultrasound of the abdomen to check for a tumor, but it came back negative. On Wednesday we decided if she doesn’t sleep a wink that evening, we would head back to the hospital. By that point we had spent countless hours on google, researching what could be wrong with our baby. We stumbled upon opsoclonus myoclonus syndrome (OMS) and prayed that wasn’t her diagnosis. But we feared it was…and it meant the consequences were grave. On Thursday April 8, we went to the Emergency Room at UCLA Ronald Reagan in Westwood to be admitted to the Mattel Children’s hospital and the next day she was diagnosed with OMS.
Opsoclonus Myoclonus Syndrome, also known as Opsoclonus Myoclonus Ataxia Syndrome or dancing eyes dancing feet, is an extremely rare disease – it only affects 1 in 10 million children. It most commonly shows up at 18 months, and tends to occur slightly more in girls than boys. In more than 50% of cases, the cause is a neuroblastoma cancerous tumor. In the rest, the cause is a viral infection or the cause is unknown. Amara was never ill – she never showed any signs of an infection. OMS is an autoimmune response in which misguided antibodies attack the cerebellum in the brain, the part of the brain responsible for motor function. The trigger event is a tumor or infection. There is no cure, but there is treatment to suppress the immune system, and hope the body learns to course correct. This means long-term and regular immune therapy combined with physical, occupational and speech therapy.
On Friday April 9, Amara got sedated yet again for a second MRI, this time of her chest and abdomen. The test also came back negative for a tumor, but her OMS symptoms kept getting worse. She lost the ability to hold herself up while seated, and lost her neck control. They started her on IVIg (intravenous immunoglobulin) to help give her body donor antibodies to replace her misguided ones. She was on IVIg for 4 nights. These were 4 long nights – her inability to self-soothe meant one of us was holding and rocking her through the night, and holding down her body tightly as she suffered through a myoclonus attack. We then had 3 days of pulse steroids, followed by a course of IV Rituximab, to kill off her plasma B cells (which make antibodies). More blood draws and urine tests were ordered to continue to check for infection, auto-immune processes and cancer. All came back negative.
To rule out a tumor, the oncologists performed one last test on Thursday April 15th – an Mibg scan with a radioactive isotope tracer. This scan showed a tumor near Amara’s liver. She would need surgery to resect the tumor, as well as bone marrow biopsies and lymph node sampling to check for metastatic cancer. Over the weekend we met with the surgical team, who informed us that Amara’s tumor was in a very tricky spot – tucked behind the liver, and next to several major blood vessels. They would need a liver transplant specialist to assist, and our consent for a blood transfusion if needed. The surgery would take 4 hours. They would also perform a bone marrow biopsy at the same time.
On Monday April 19th, Amara underwent surgery to remove the tumor. They found a second tumor hidden behind the primary mass. Thankfully, they didn’t need to mobilize the liver and Amara didn’t need ICU-level care post-op. Since the surgery, we have learned that her cancer has spread to two of her lymph nodes but it’s been a waiting game to find out the results of the rest of the tumor pathology as well as additional tests being conducted by Children’s Oncology Group. We still don’t know what her cancer treatment protocol will look like and whether or not she will need chemo.
On May 2, Amara received a second round of Rituximab and will now be getting IVIg and pulse steroids at least once a month to manage her OMS symptoms. Her myoclonus jerks cause her severe sleep disturbances and it’s been hard to console her through them, let alone get a good stretch of sleep. Her speech is still mostly incomprehensible and she is still unable to sit or walk.
This has been a harrowing journey for all of us. Our sweet, active, vibrant and boisterous toddler has suddenly become a rare statistic and has gone through so much pain, discomfort and fear already. Still, she carries on with a smile when she can. We are letting her strength and courage guide us.
Thank you for joining us on this journey as Amara battles (and beats!) not only childhood cancer but also the very rare Opsoclonus Myoclonus Syndrome and regains her ability to sit, walk, talk, dance and say no again.
Last month, Amara had her 12th treatment in the hospital. We have been on this journey for 15 months now. This recent treatment was 10 weeks from the previous one. When we started last year, we were going into the hospital for treatments every 4 weeks. This year, seeing Amara's progress, we have been able to slowly space out treatments. A larger gap between treatments has been so good for our quality of life, even though it does mean more anxiety, dread and discomfort during each treatment as we lose some of our muscle memory (and trauma) and start to lead normal lives. The photos below are from a sunny moment between infusions during which Amara wanted to get away from the hospital floor and head outside to practice her jumping, as she has been learning in physical therapy. She is still lagging behind on that skill for her age, as she is on running and climbing.
This treatment was one of the worst we've had since the early days, for many reasons. Being older and stronger, Amara has more awareness of her pain, anxiety about what is to come, and strength to resist, which made every intervention more painful and traumatic. She was more lethargic during the treatment, and was wobbly with slurred speech for a week after. We're not sure if this is because her body isn't used to treatments anymore, doesn't need as much treatment, or something worse. Our specialist thinks her next treatment, 12 weeks later at the end of August, could be her last. Then it will be a wait and see game. Wait and see if she relapses. Wait and see if an infection triggers the OMS. Or, what we are hoping and praying for, wait and see her move smoothly into full remission.🤞
On the oncology side, we have been so lucky, blessed, grateful that there has been no recurrence of her cancer. After some big scares upon seeing abnormal counts, and a series of additional diagnostics, second opinions etc., our worry was lifted. We can now space out MRIs to every 4 months instead of 3. Some schools of thought suggest we can go even longer without screening. Others are more conservative for the first 5 years. We are waiting to see what the consensus is among our oncologists.
Meanwhile, Amara continues to get physical therapy, occupational therapy and behavioral therapy once a week. Mom and dad are finally starting to catch up on our long-ignored health appointments and screenings as well.
Sadly, the OMS journey is not as smooth for many. I've heard from other parents about kids who never see progress despite the same or more aggressive treatment protocols for years, or progress does happen but takes much much longer, with many relapses. For others, 6 months of treatment is all it takes. No matter where we land on that spectrum, OMS will always be our story, and we will continue to fight for more awareness, research and a cure.
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