On her 3 month birthday, Keira was diagnosed with a rare disease called Aicardi syndrome. It affects roughly 1500 people worldwide, almost exclusively girls. It is terminal, however there is a range of disability with some having milder symptoms. Aicardi syndrome has several hallmarks, one of them being a very severe, treatment resistant form of epilepsy.
Around 2 months old, we started to notice Keira was doing some things that didn’t seem normal. But with every question came assurances from medical providers that she was a typical, beautiful, healthy baby girl, and everything we noted was a part of the normal growth and development process.
But it kept happening, and worsening. We kept pressing and visiting doctors, and researching. Based on our own research, we were fairly certain that Keira was having infantile spasms and we shared this information, but our concerns continued to be discounted.
One evening, Keira became inconsolable following what we now know was a “cluster” of infantile spasms, and we made the decision to take her an hour and a half away from home, to the Emergency Department at Oishei Children’s Hospital in Buffalo. She was admitted to the hospital and they immediately began testing. At that time she was hooked to an EEG to monitor her brain activity for suspected seizures.
That first hospital stay was 10 days and she was discharged home, ultimately, with the devastating diagnosis of Aicardi syndrome.
Despite aggressive, and sometimes incredibly expensive treatments and medications, Keira still typically has 100’s of seizures a day. The longest seizure cluster she has had without a break was 2 hours and 40 minutes. Being the tiny super hero she is, with the help of her amazing support team, the seizure activity was stopped. Ultimately that seizure came at a huge price both physically and mentally. One thing she lost was her ability to smile, and she had the most beautiful little smile.
We honestly can’t say enough about Oishei Children’s Hospital, Oishei outpatient neurology/UB neurology and the Niagara Street special needs clinic. Keira’s care is provided by physicians that are literally some of the best in their field. They treat her as they would their own child and they have saved her life, literally countless times.
Keira has 32 active medications, provided through 4 different pharmacies. She has 16 medical providers and therapists and three durable medical equipment vendors. To date, Keira has 19 different diagnoses that she is treated for. She has scheduled therapy 6x/wk and multiple doctor visits a week.
We are fortunate that we have received many grants and donations for things not covered by her insurance. But, we also have many out of pocket expenses.
Keira has two other siblings, whom she loves dearly, three dogs and a cat. Both of her parents attempt to work full time, but often unpaid time has to be taken.
Keira is the strongest person I know. She is so brave and she faces life head on with the most beautiful smile that happily returned this fall. Every single day we have with her is borrowed time, and a gift. We thank everyone for their support and the opportunity to raise awareness about rare diseases and epilepsy.
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