Ava Shaye Gumson

AVA SHAYE GUMSON

D/O/B:  3/15/04 (19 years old)

March, 2004 to September, 2005

Ava’s first seizures occurred about a month after she was born.  Up until then, everything was great and “normal”. Nothing seemed wrong.  Then, the seizures started.   Only, at the time we didn’t know they were seizures.  They started in her left arm only, and began with one or two rhythmic jerks.  Over the next few days, they increased to 10-20 jerks at a time, spanning over a minute, occurring many times throughout the day.  After a few weeks searching for the answer, and being advised by our “first” pediatrician that we were simply being nervous first-time parents, our second pediatrician (who still treats Ava) instructed us to go right to the hospital, where we spent a few weeks investigating meningitis and a variety of other rare maladies and Ava was diagnosed with Aicardi Syndrome.  She was placed on Phenobarbital and Keppra to combat the seizures, continuing on both up to about 3 months of age (6/04).

When she was diagnosed, we asked our “first” neurologist what we could do to assist Ava (i.e., therapies, treatments, etc.), and we’ll never forget how he coldly shrugged his shoulders as if to say “nothing” and told us that “this is the way god made her”.  Notice we say he was our “first” neurologist – his complete and utter lack of compassion was shocking, and that’s the last time we ever saw or spoke to him.  We often say his name in vain whenever Ava achieves another milestone.   Who knows how many other families he discouraged by his pessimistic, condescending and unfriendly attitude.

Ava did not have any head control.  At 3 months, she began experiencing Infantile Spasms (more serious seizures which, if not controlled, could cause severe brain damage) and we started a year-long series of treatment with Vigabatrin and Keppra.   In 10/04, our second neurologist just about demanded that we switch Ava’s medicine from Vigabatrin to ACTH because he didn’t notice any substantial improvement in her motor skills or EEG results.  We refused to switch, advising him that we thought Ava had progressed and that we were noticing subtle changes in her abilities and that we had made the decision NOT to go on ACTH based upon our research and the awful side effects Ava would have to endure while on it.  He basically stormed out of the room, and we never treated with him again.  Needless to say, we switched neurologists again, to our third neurologist, whom we completely trust and who actually listens to us, and seems to care about Ava – and, the very next month, as if by divine intervention, Ava’s Infantile Spasms stopped.  Luckily, Ava only had the Spasms for about 5 months – Vigabatrin was a miracle drug for us and seemed to really knock out the spasms.  But, she couldn’t stay on Vigabatrin forever, since prolonged use causes blindness, and we weaned her off it in 6/05.

Since 6/05, Ava has done exceptionally well.  It’s like she is a different child.  She is so alert and the smile that disappeared in the early days of Phenobarbital and Keppra has returned.  We have seen incredible progress over the last few months and know from our own eyes that she is doing better each passing day.  Our belief was confirmed in 8/05 when (and again, we hesitate to write this), Ava’s EEG showed normal brain patterns and background activity for the first time, with no signs of seizures.  She has been seizure free since 11/04.

The 5 months of Infantile Spasms initially made Ava regress as to her motor skills, but her right hand has rebounded well and she is grasping things and self-feeding finger foods.  Her left hand is still not interested in grasping items, so she has not yet been able to hold a cup or other items with two hands.  As of the last month or two, Ava has begun to crawl and is slowly learning to bear weight on her feet, and, with the use of a Stander and Gait Trainer and lots of Aquatherapy in our pool, her legs seem to be getting stronger by the day so that we hope that walking is no longer an unreachable goal.  Swimming is one of her favorite things and, as she’s gotten a bit more mobile in the pool and out, we’ve noticed that she’s shed a few pounds of her baby fat (she’s still a little chunkster, though!)  She eats very well, drinks from straws of all sizes (she even sipped a really thick McDonald’s Chocolate milkshake to our surprise – sipping the shake was hard enough for us because it was so thick – but it was her first taste of chocolate so we guess she was motivated!). She knows how to roll, can prop herself up with both arms and is currently starting to show signs that she wants to sit up and crawl on her own.  She is very alert as to her surroundings and her vision and hearing are excellent – one thing is for sure, if there is a TV in the room, even in a dark, isolated corner behind her, she will find it!  She smiles all day long and her giggle/laugh is absolutely contagious.  We often say that although we wouldn’t wish an Aicardi Syndrome diagnosis on anyone, we wouldn’t change anything because we feel so lucky to have her in our lives.  She has truly taught us more than we have taught her.

We are expecting our second daughter in November, 2005.  We look forward to Ava having a sister to play with and hopefully explore new possibilities.  We know our new arrival will enhance all of our lives and are busy finishing her room and some other home improvements prior to the big day when we bring her home.

October, 2005 to August, 2006

n October 31, 2005, our 2^ND daughter, Lilly Kaye, was born.  Our house just got a whole lot more hectic!   Now there are two infants in the house – guess this is what having twins is like!  During Lilly’s first year, as Lilly has begun to stand, hold her bottle and do so many things that Ava had yet to do, we noticed that Ava was paying increasingly more attention to her sister.  The other day, Ava and Lilly were playing with the same toy, and we tried getting Ava to spin the wheel, which she refused to do.  But then Lilly spun the wheel, and suddenly Ava reached for it and now does it regularly. Who says sibling rivalry is a bad thing?!

Ava’s seizures stopped from November, 2004 through February, 2006 but, sadly have returned.  They occur almost only always while she is sleeping or in the process of waking up, so, luckily, we have not experienced her falling down on the tile or outside while she is awake.  We continue to experiment with different doses of seizure medicines and over-the-counter Melatonin in an effort to regulate her sleep regimen, which, aside from her seizures, is her biggest issue.  She also displays a number of Autistic qualities, like flapping, clapping and shaking her head back and forth repeatedly, and often has uncontrollable meltdowns when we request that she do something she doesn’t want to do.

Our home was greatly damaged by the Hurricanes of 2004-2005 (Adam, Lissa & Ava stayed in our closet and we watched Baby Einstein videos with Ava for hours as the storms raged!) We are in the process of putting a new roof on our house, rebuilding a portion of the house and closing in our garage to make a part of it a gym for Ava (and us!) and hope to have it completed by the end of October, 2006, in time for a Halloween Costume Party to raise money for the Aicardi  Syndrome Foundation.  With all of the problems we had with Ava, and the hurricanes, we have dropped out of the social circles and lost touch with a lot of our friends/colleagues, and we are definitely looking forward to reuniting with everyone again. Most likely we’ll be putting the finishing touches on the house as the guests arrive!

September, 2006 to September, 2007

Ava continues to thrill and amaze us and surpass all of our expectations for her and us as a family.

Ava has been cruising in her crib and propping herself up against furniture, and is able to stand against furniture for long periods of time, especially if she is watching a cartoon or Baby Einstein video.  Still no speech, but she recently began to mimic a few sounds and hum along to the tune of a number of the children’s cartoons she loves (JoJo’s Circus and Little Einsteins are a few of favorites).  Her motor skills have improved such that she’s able to finger feed with her right hand, and we’re hoping that therapy will promote her use of her left hand so that she might use both hands to hold a cup soon.  Although she’ll always be low-toned, she is really, really strong, perhaps due to working a lot in our pool, which she still loves (the other day, we got in and got out immediately because of lightning and she started to cry so hard! So, she is definitely communicating with us and she sure knows what she wants and likes!)  Ava can now touch her nose and/or belly when asked and clap her hands and act out the “itsy bitsy spider” song, and she really loves all kinds of music.  Of all her wonderful characteristics, her greatest attribute is definitely her smile – she seems to be happy all the time and she really lights up the room when she smiles.  We continue to count our blessings as she progresses at her own pace (she’s on “Ava time”!).

Ava completed the State of Florida’s Early Intervention Program when she turned 3 in March, 2007, and attended pre-Kindergarten Summer School for a few weeks with the help of a one-on-one assistant and a private van rather than a bus (to avoid both overheating and the chaos of a crowded bus-ride to school, which we were concerned might promote more seizure activity).  She adjusted to “school” very well and we think she enjoyed interacting with other children in a group setting for the first time.  A few weeks ago, she started “real” pre-K at ARC (formerly the Association of Retarded Citizens) where she is receiving in-class Speech, Vision, Occupational and Physical Therapies.  ARC has a “verbal/mobile” policy, meaning that it will continue to work with a child until he/she becomes verbal and/or mobile – so we’re hoping Ava gets expelled real soon!  We’re ecstatic that she has developed to the point where her participation in school is possible, but still nervous about our little girl being in the real world and hopeful that the other children (and the rest of society) will be accepting of her.

Currently, Ava’s seizure activity consists mostly of myoclonic jerks that occur only during sleep, about 4-5 times a week, although the past few weeks she has, for the first time, had seizures while awake (usually when over-tired or startled).  Aside from the seizures, Ava’s biggest issue continues to be sleeping – she has consistently awakened at 1-2 A.M. and remained awake for the rest of the night, eager to crawl around and play with her toys.  No amount of medicine seems to help.  Our search for sleep (for Ava and for us!) led us to a number of alternative medical techniques, such as Cranial Sacral Massage Therapy, Magnetic Therapy, Chinese Medicine/Homeopathy, Neurological Bio-feedback, Aquatherapy and a Detoxification Program, all of which have had very positive impacts on Ava.  After much soul-searching, we recently decided to wean her off ALL of her seizure medicine (she was most recently on Keppra) as we were concerned that continuing to increase her dosages of medicines to deal with her seizures would subsequently damage her liver and other organs and keep her in a “trance-like” state.  As we have decreased her dosage, we realized that her Eczema, a rash-like skin condition which she has had for 2+ years and for which we have treated with just about every ointment and medicine known to man, has decreased drastically.  Who knows what other issues these medicines are creating in her body?

Our 2^ND daughter, Lilly Kaye, will turn 2 in October, 2007, and she is talking, walking/running around the house and climbing on everything.  Ava has been watching her more lately, and we hope that Lilly’s spunkiness rubs off a bit on Ava and spurs her to try and do more.  We also witnessed the most precious thing the other day, when Lilly obviously recognized that Ava could not use her own cup and, on her own, brought over the sippy cup to Ava and held it to Ava’s mouth in an attempt to help Ava drink from it.  Like another mommy.  Totally a Hallmark moment!

Our 2006 fundraiser on behalf of the Aicardi Syndrome Foundation raised over $18,000.00 and brought us a tremendous amount of joy and satisfaction, and we have decided to make it an annual event.  We hope that any non-Aicardi families who read this will pitch in, even in a small way, to assist the Foundation with its efforts to raise money for research and to assist some of the financially needy Aicardi families with supplies, equipment and travel expenses to and from the bi-annual Convention.

October, 2007 to September, 2009

Ava (4) began to walk in December, 2007!   Our joy was impossible to contain – we couldn’t wait for everyone we ever knew to see her two-stepping around (since she’s still left-side weak, she has a tendency to make a lot of left turns, but who cares, she’s walking!)  Although we always thought/hoped she would walk, we are positive that the therapy she received at ARC, and the incredible compassion of her ARC therapists, greatly accelerated that process.  She also began to take a more active interest in feeding herself.  With her newfound mobility, Ava’s whole life has changed – she no longer is content to stay in the playroom for hours at a time, but instead wants to explore.  She enjoys being outside, taking walks, bicycle rides on her 3 wheeler (as long as she wears the harness since she still forgets that she needs to hold onto the handlebars).  She lost a bit of her chunkiness from getting more exercise, and, due to ARC’s verbal/mobile policy, did, in fact, get expelled from ARC.  We say “expelled’ tongue-in-cheek – it was bittersweet – we loved ARC and had hoped she’d be able to complete a 2^ND year there because the therapy she received was so wonderful, but because she was moving around the classroom and pushing all of the doors open, it became a safety concern and we agreed it was time for her to leave and to meet some new challenges.

As Ava was finishing her time at ARC and we were preparing for her transitional IEP and generally having absolutely nothing else to do, we had another baby in April, 2008!   We had labored about whether to have another child and, after much soul-searching, we decided that we should not refrain from doing so simply because of Ava’s issues.   We also thought that she might benefit from another sibling as well as wanting Lilly to be able to experience having an active sibling, too, since, to this point, there had been little meaningful interaction between Ava and Lilly.  Of course, nothing comes easy, so Lissa was on bed-rest for 4 months, gave birth to Zane 6 weeks early with an emergency C-section (during the same week that Adam was moving his law office after being in the same location for 20 years) – you get the picture – total chaos.  We spent 3 weeks in ICU because Zane was a preemie and had developed some very serious respiratory issues and, although it was like reliving the aftermath of Ava’s first few months, we are ecstatic to say that Zane is 16 months old, and appears to be on his way to becoming a middle linebacker – he’s strong, huge and chasing Ava and Lilly around the house!   With 3 kids who are 5 and under, we’ve lost total control of our lives, but our house is a loud and loving and happy one (except when we tell Lilly she can’t have another cookie or chocolate cake).

Ava began to attend pre-K at Eisenhower Elementary in August, 2008, taking a bus with the assistance of a one-on-one aide and experiencing a school environment, albeit in a Special Needs class of about six students.   She still receives Speech, Vision, Occupational and Physical Therapies.  She seems to enjoy it, but we think the bus rides really wipe her out.  Luckily, we were successful in making sure that she did not take naps at school or on the bus rides, so we were never faced with a situation where she had a seizure at school or on the bus.  Her attendance was spotty, as she seemed to pick up every little cold or cough, either from the other students at Eisenhower or from Lilly, who began attending Daycare and brought home all sorts of germs, too.  Nonetheless, her pre-K experience saw her play on the playground, and enjoy music through circle time and start to learn sign language.  She knows a few signs (please, thank you, hello, goodbye) and, although still non-verbal, is definitely communicating with us.  Potty training has been frustrating – sometimes she does well, other times, well, you know!   We kept her home over the summer to spend more time in the pool and, in August, 2009, she transitioned into Kindergarten.   Another Special Needs class, with 5 other students and  5-6 teachers/aides, all of whom are so caring and wonderful.  Can’t believe she’s in Kindergarten!

We had Halloween Costume parties to benefit the Aicardi Syndrome Foundation, in October, 2007 and October, 2008, and now have raised, in three years, over $43,000.00, with our 4^TH Annual event scheduled for October, 2009.

Ava had been completely weaned off Western Medicine from August, 2007 to June, 2009, and her personality was more calm, less volatile.  However, her sleep problems persisted (up 4-5 nights per week from 1-2 A.M. through the rest of the night) forcing us to place her back on Keppra, as well as Chloral Hydrate in July, 2009.  Although we were reluctant to put her back on medicine, it has helped her sleep through the nights and has helped with her seizure activity.   In August and September 2009, our entire family was engulfed in a cloud of health-related issues:  Adam’s Dad had an unexpected quadruple bypass procedure and, while he was recuperating, his mother was diagnosed with a rare form of cancer in her hip (as a result of a defective hip replacement) and both were in the hospital at the same time, in different facilities.  Meanwhile, Ava and Zane both had Swine Flu and our babysitter, Rachel, was hospitalized, too.   What a mess!   As of this writing, we’re thinking of applying for our own soap opera/reality TV show, although nobody would believe it!

Ava still exhibits many Autistic qualities (repetitive motions, limited attention span, likes to clap a lot) and knows the alphabet and lots of songs (she hums!).  To our amazement, she is also capable of some incredibly intellectual feats that defy logic (she can identify states on a map, do math multiplication problems into the thousands, well beyond her supposed capacity, spell words and read full sentences and point to multiple choice answers with surprising accuracy – but only for short spurts of time, and only when she feels like it).  We are very encouraged by the possibilities of these new-found cognitive abilities, but mindful of the balancing act between her potential and present skills.  Still, though, the brain is an amazing organ and we have witnessed some things that cannot be explained other than we are no longer bound by the initial expectations of the Aicardi diagnosis – she has shattered those expectations and we are now along for the ride – go Ava!

One of the happiest days of our lives was Ava’s 5^TH birthday, on March 15, 2009.  For the first time, we invited all of our friends and family over, with all of their kids, about 100 people, to share our joy and help us and Ava celebrate.  We rented a giant bounce-house and had a pool party (those are Ava’s two favorite things) and, for that one day, we didn’t think about germs, seizures, insomnia, medicines, EEGs or anything other than sharing her day with her and the people who had been there for us – it was special for Ava and for us – the perfect day.  It is days like that where the pain, struggles and sacrifice seem minimal – may you all have the pleasure of experiencing that perfect day.

 October, 2009 to September, 2010

The end of 2009 was a very trying time for our family.  Adam’s dad continued to recuperate from quadruple bypass surgery in July and his mother continued to battle cancer with grace and dignity, each from different hospitals.    Adam ping-ponged between hospitals visiting both of them for weeks and weeks, and, in November, after weeks of feeling sluggish (or more sluggish than normal!), was hospitalized for five days with pneumonia and pleurisy (on the floor below his mother).   The phlebotomist was quite confused!  A few days after he was released from the hospital, Adam’s mother lost her battle with cancer on November 20, 2009 at the age of 71.  The holidays were a bit somber, and it took months for Adam to recover from the pneumonia/pleurisy. Despite the sadness, we had a stronger sense of family than ever before, with Adam’s sisters coming in to town regularly and the whole family spending time like never before.   Our kids were excited to spend time with their extended family and, to this day, we are closer than we ever were.

Ava’s development soared during this time, too.   She became much more aware of her surroundings, and seemed to watch Lilly and Zane more than ever and enjoy their company for the first time without being annoyed by them. They loved to run around her while she sat on the floor, and she would pivot left and right and round and round when they went behind her, all the while laughing her infectious laugh.   We met with a Behavioral and Developmental Counselor to help us with Ava’s sleep and autistic mannerisms and, in addition to other advice; she suggested that we look into getting a Service Dog for Ava. Initially, we thought all we needed was another project on our plate, but, the more we thought about it and researched the effect a Service Dog could have on Ava (and us), the more it made sense to us.  We applied in August, 2009 and were told it would take 1½ – 2 years for us to receive a dog trained specifically for Ava’s idiosyncrasies.  It seemed like an eternity, especially since Ava regularly awoke at 1 AM and did not go back to sleep again, meaning that either Lissa or Adam was having an all-nighter at least 3-4 times a week.  The thought of a Service Dog assisting us with Ava’s sleep issue was a beacon of light in an otherwise sleepy existence, but March of 2011 was a long way away. Luckily for us, we learned that our newest family member-to-be, JoJo, a tall and sturdy Golden Retriever, was way ahead of schedule and would be ready in June 2010. Lissa, Adam and Ava spent a week in Tennessee training with JoJo and becoming licensed Dog Handlers through Wilderwood Service Dogs.  We returned home with a sense of accomplishment and hope and, throughout the rest of the summer, continued to train JoJo and promote the bond between him and Ava.

The result of their bond was almost instantaneous.  Ava began mimicking JoJo’s eating and drinking habits and, within a month or two, spoke 5 different words where she had never spoken before.  She no longer uses a high chair at restaurants, but instead sits calmly with JoJo under her feet.  We have stopped using a stroller in places like Home Depot – she now walks confidently with JoJo by her side and her endurance built up where she doesn’t  have a melt-down if she has to walk – she loves it!  Her all-nighters have disappeared, replaced by a few random and limited awakenings per night (2 AM and 5 AM, like clockwork – but we’ll take it!)  Her seizures are much shorter, and under control, and at least a few times JoJo’s licking of her neck or palm seems to pull her out of a trance much quicker than before and, with time, we expect that JoJo will be able to alert us, pre-seizure, so that we’ll be able to prepare for it before it happens.  Even the autistic meltdowns seem to be minimized (on our July, 2010 to the Aicardi Syndrome Foundation Conference in Chicago, while at the airport, Ava plopped down in the middle of the terminal, determined not to walk anymore and, after being informed that JoJo was hungry and needed a treat, she gave it to him and forgot she was mad and just started walking again!)  JoJo has been on an airplane, in cars, and in numerous restaurants and stores, all the while serving as a surrogate parent and/or bodyguard for Ava. As miraculous as some of these developments have been to us we were disappointed to learn that the Palm Beach County School District rejected our request for Ava to bring JoJo to school with her, claiming a bunch of totally bogus reasons such as concern about biting, dander or that her one-on-one aide (who we thought was great) could assist all of her medical needs. We refused to separate Ava and JoJo for 7 hours a day, having been advised that to do so would irreparably affect the bond that we’d spent 3 months attempting to build.  So, when it appeared that the negotiations and legal channels might take months to wade through, we opted to have Ava home-schooled and, as of mid-September, 2010, teachers and therapists have started to come to our home to give her lessons and therapy.  Hopefully, Ava will be able to return to school with JoJo sooner rather than later so that she can see and be with other children and teachers rather than continue a lonely existence at home – she thrives in a school environment and bringing JoJo will only enhance her experiences and be her safe protector.  But, until then, she and JoJo continue to bond at home.

Our other kids, Lilly (almost 5) and Zane (2) play with Ava constantly and are a source of endless entertainment to her, especially in the pool where they jump off the sides and splash and scream and yell.  The pool and her touch screen computer are her favorite things to do.

October, 2010 to September, 2011

On October 23, 2010, we hosted our 5^TH Annual Halloween Costume Party to benefit the Aicardi Syndrome Foundation, this time with a Western theme complete with an Electric Bucking Bronco and Square Dancing.  Thus far, we have raised over $85,000.00 and hope to eclipse $100,000.00 raised at our next event in October, 2011.

JoJo, Ava’s service dog, has been an incredible addition to our family.  From the first night we got him in June, 2010, his impact on Ava’s ability to sleep has been monumental, somehow calming her as she sleeps so that she sleeps through the entire night (which she never did before). Her restful nights seemed to lead to increased energy and less seizures.  JoJo has enabled us to achieve some semblance of normalcy in our life, accompanying us to restaurants, stores and events, places we previously avoided because of her outbursts or inability to sit still.  He dutifully waits under the table while we’re eating out and patrons always are stunned when we get up to leave since they had no idea he was there.  Plus, he’s really tall (like a mini-horse – on purpose to assist Ava with walking as she grows older). He has become somewhat of a “rockstar” at some of our local hangouts, with waiters bringing over water for him before bringing it to us – but he deserves it!  Fortunately, the School District finally agreed that JoJo could accompany Ava to school beginning in January, 2011, and, after a few weeks of Lissa’s attendance at school to train the staff, he and Ava had a wonderful year together.   Her teachers reported a fabulous increase in her attention span, and her ability to do non-preferred activities, including sitting at a desk and drawing (which she never had the patience to do before).  Of course, JoJo became a rockstar at school too.  But something really cool happened as a result of everyone knowing JoJo – they started waving to Ava and saying hi to her.  Saying thing like “that’s JoJo, Ava’s dog.” So, instead of being ignored or stared at, she has become a bit of a center of attention, tangentially, because of JoJo, which seems to have bolstered her confidence in walking around and in stopping when people say hi (like in a supermarket).

In addition to accompanying Ava wherever she is, he has also been comforting to her while she has seizures, resting his head on her body and permitting her to pet him and, in a few cases, alerting us even before her seizure occurred. In a few instances, both at home and in school, he barked for no apparent reason but, a few moments later, Ava had a seizure.  We’ve probably scolded him a few times for barking when he was really trying to tell us something, so we’ve become a little more diligent about looking at Ava when he does bark (because he’s not a barker).

Despite the progress with JoJo, the months of January, 2011 through June, 2011 saw Ava’s seizures worsen, which we link to her having ingested a ton of chemicals while being sedated for MRIs and CAT scans in dealing with her scoliosis issues.   Nights became interrupted by almost hourly seizures, and she averaged about 10 a day, in varying strength and duration but much worse than the 6 months before. The Drop Seizures (where she’d just fall without any warning) were the worst, coming too quickly for JoJo to sense them beforehand. Having ruled out brain surgery and increasing her medications, we opted to implant a Vagus Nerve Stimulator (VNS) in her chest (similar to a pacemaker, but for seizures) and she had the surgery on June 29, 2011.   Thus far, we have noticed subtle changes in her behavior (playing with different toys, exploring more in the house, seems generally more interested in people rather than always playing by herself) and the seizures have lessened (both in frequency and duration).  We hope that the VNS continues to improve Ava’s quality of life over the summer and that by the fall, she’ll have fully adapted to having a mini-computer in her body and the vibrations that tickle her throat.  2^ND Grade, here we come!

In July, 2011, we drove to North Carolina for a week’s vacation with Lissa’s parents.  Long car rides with three kids and a Service Dog – what a treat!  All in all, our trip was wonderful and it was nice to get away as a family (which we have not really done before).   Ava loved the trip, as did our other two kids, both of whom continue to be doing well.  Lilly (5) tried soccer, basketball and tennis this past year and seems to enjoy sports, although she seems to have a “why can’t we just all get along?” attitude and does not understand why some of the boys in co-ed leagues are so aggressive.  She entered Kindergarten in late August, excited to go to “Ava’s and JoJo’s school” with them.  Zane (3) has had some speech issues, and he has been going to the same school in the mornings for some speech therapy, and then into Day Care again thereafter.  Lissa continues to spend most of her life in the car, shuttling the kids to school, after care, doctors, activities and birthday parties.  After being home most weekends with the kids, Adam welcomed returning to work on Monday like never before!   In addition to the chaos of starting school, the end of August rocked us when Lissa was hospitalized for 8 days when, out of the blue, her speech suddenly became garbled and she lost her ability to walk.  Initially fearing a stroke, we learned after Brain MRIs and Spinal Taps that it was Multiple Sclerosis (MS).   We seem to be cornering the market on incurable disorders.   After a week at home, in bed, she finally started physical therapy and, after using a walker for 2-3 weeks, we are happy to report that she has recently abandoned it, and her speech has fully returned and she is ready to tackle this new diagnosis with the same fervor as every other issue.  Just to make it interesting, while all this was happening, Adam tore his Achilles tendon playing basketball, so, for about a week, he was on crutches and Lissa was on a walker – ZAP – we became our grandparents overnight!   Must’ve looked like quite a pair at the kids’ School Curriculum Night!  Anyway, we’re both on the mend and looking forward to our 6^TH Annual Halloween Costume Party on October 22, 2011 – we debated about cancelling it, but, with the help of our family and friends, are moving forward with the party – maybe we’ll dress up as hospital patients (that’ll be easy for us!)

October, 2011 to September, 2012

Somehow, with a lot of help from our family and friends, we were able to hold our 6^TH Annual Halloween Costume Party and topped $100,000.00 raised for the Aicardi Syndrome Foundation.  Actually, our family and friends jumped in to help us a lot after Lissa’s MS diagnosis, and our lives returned to normal (well, “our” normal) by the end of 2011.  We had some pretty cool things happen with Ava:  as we have previously described, Ava from the time she was 4 could answer written questions (when given 3 answers) with an amazing degree of accuracy.   Since she was 4, we have asked her, on multiple occasions, a variety of questions, including if and when she would speak and at what age.  For 4 years, she always answered “yes” and the age of 8, no matter what other ages we gave her as potential answers.   Up until her 8^TH birthday, her vocabulary consisted of one word, “cat” (pronounced “hat”).   Fast forward to her 8^TH birthday on March 15, 2012, with our whole family sitting around watching her open her cards and presents.  Adam was reading a card to her, and she was following the words as Adam said them and, when Adam said something like “Today you’re 8” out of nowhere and as clear as a bell, Ava said “8”.   The whole family sat with jaws agape, and there was no doubt she not only read the card to say 8 at the right time, but that she was clearly saying 8!   Since that time her language and vocabulary have exploded, and she now says a number of words as well as multiple sounds that she never before uttered.   Not sure why she had to wait til she was 8, but maybe she had a side bet with one of her classmates and couldn’t spill the beans before she actually turned 8!   In any event, that was an exciting day and a great development!  She also continues to enjoy being in the pool as much as possible, and, because she is now tall enough to stand in the shallow end, can balance herself walking back and forth (although she still always walks too far in the deep end without remembering she’s only 3 ½ feet tall and is continually surprised to gulp a mouthful of water!)

Ava finished 2^ND grade saying about 25 words and using her assistive communication device to point to words and pictures.   Her walking with JoJo improved dramatically, as did her comprehension of basic commands (“Let’s go to the car, time for dinner…”) But, her most impressive skill was using her IPad, navigating in and out of a myriad of programs like a champ.  On the down side, she still is not potty-trained and we cannot seem to prevent her from putting her hands in her mouth or mouthing every toy or device she touches.   On most nights, she still can have 5-7 seizures after bedtime and before morning.  But, on the whole, her sleep and seizures are vastly improved since we got JoJo in June, 2010.  We are in the process of scheduling a sleep test to investigate whether there is another reason (other than the seizures) for her interrupted sleep, and hope to rule out sleep apnea.

In March, our family had another serious medical scare with Zane, who, after a few days of an upset stomach and a pediatrician telling us “not to worry about it”, woke up on a Saturday evening with uncontrollable and continuous vomiting.  After a middle-of-the-night ER run, we learned that it was a burst appendix, necessitating emergency surgery when he became septic and another procedure to insert a drain in his stomach when abscesses formed.   Adam and Lissa took turns sleeping in the hospital every night for the two weeks he was there – it was actually a very scary situation, and we are very very lucky that we can laugh about it now – although Zane now remembers it fondly because he got a lot of cool presents.   In early June, Lissa tore her rotator cuff trying to stop Ava from falling and, the same day (misery loves company!) Adam broke his rib playing basketball.   Amidst all of our family’s medical issues from August, 2011 to June, 2012, Lilly somehow managed to complete the entire year of kindergarten with perfect attendance and an award for reading the most books in her class.   We are considering commencing a search for her birth parents as she clearly cannot be our child.  The summer treated us well as we travelled to St. Louis, MO for the Aicardi Syndrome Conference via a rented RV, and we braved rain and close quarters to have a great family trip.  We enjoyed seeing our old friends and meeting the new families at the Conference.  Ava certainly enjoyed seeing all of her Aicardi sisters as well.  We already look forward to the 2014 Conference.

October 2012 to August 2013

In October, 2012, we received nursing assistance for Ava from 7 P.M. to 7 A.M., which as a huge blessing as it permitted us to go to sleep without watching the monitor all night long (when Ava typically has seizures).  While grateful for the additional assistance, we found that many of the night nurses who were sent by the agency to watch Ava’s monitor were overworked, and overtired, and several fell asleep on their shifts and so just did not work out.  Even one day nurse, who showed up at 7 A.M. when we had limited day-time help fell asleep at 9:30 AM in the middle of the playroom, with Ava crawling all around her. Only we would get a day nurse who falls asleep within two hours of starting her shift! We have a video of the nurse, fast asleep in her chair, head bobbing up and down, which is both funny and sad.  Anyway, after some false starts, we finally developed a solid team of 3 nurses which lasted a few months before having to replace one when she moved away.

In December, 2012, Ava had overnight EEGs at Miami Children’s Hospital and Lilly broke her clavicle right before Christmas, 2012, so we really finished the year with a medical bang!  Lissa also discovered after much testing that she had multiple food allergies and intolerances.  In mid-December, she began to shift her diet away from gluten/wheat and 75 intolerant foods, which meant substituting iceberg lettuce for her beloved romaine lettuce – and she immediately noticed a difference in the way she felt after eating and in her overall energy level.  Since Ava had already been on gluten/casein/soy free due to her seizures, 2013 saw our family shift its eating habits drastically, substituting almond milk for regular milk and a variety of other gluten/corn free products for the usual food staples we had used so that Lissa didn’t have to cook multiple meals for each of our family members.  Our son Zane’s frequent ear infections ceased, as did a few other health-related issues for some of our other family members, and so now pizza night happens at our home without ordering take-out, made from corn/gluten free soft tortillas and non-dairy cheese.   Not as good as the real thing, but better than feeling deprived. And way better than eating the good stuff in front of Ava (who has been eating the less-tasty stuff for years).   We’ve noticed a lot of restaurants are starting to offer more gluten-free fare, but it is still easier to fix it at home than try to navigate the unknown ingredients at a restaurant or order from a waitress who can’t believe that one family has so many food issues.

After Lilly’s clavicle injury in December, we could not shake this sinking feeling that we were a bit haunted, and that perhaps it was our house.  With Lissa’s healthy eating she met a shaman at one of the organic health stores and, in early January, 2013, we had her over to our house to give us an opinion about what was going on, which led her to say some prayers.    If you would have told us a few years ago that we’d ever consider doing something like that, we’d have called YOU crazy but, after the last couple of years living in our shoes, it actually felt “normal” to do it.  And, whatever the heck she said, things were way better the first few months of the year than they had been for years.   With the shaman’s prayers and the additional rest from the nurses, we found ourselves better able to accomplish more projects and we could focus on Ava and our other two kids when we were home instead of having to “get stuff done”.   The period of January, 2013 to March, 2013 was a time of great progress on a variety of home and kids’ projects and, with renewed energy, we felt like we “were back”!  Amazing what a shaman and a little sleep will do ya!

April was our most difficult challenge, to date, with Ava’s seizures.  After a brief illness, Ava began having a series of almost continuous seizures that we could not control with any of her current medications or Diastat, 20-30 per day for a few days, so we found ourselves driving to Miami Children’s Hospital on Saturday, April 6^TH (right in the middle of the NCAA Final Four – so you know it was serious if Adam didn’t even see the Final Four).  At MCH, the neurologists gave her Ativan, but still the seizures continued, and so the Rapid Response Team was called, which is essentially a SWAT team of Medical Superheroes, each with a different function catapulting into the room from who-knows-where within about a minute of the call.

We can talk/joke about it now, but, at the time, it was the most serious situation we had ever encountered, and we might have been too shocked to realize how close we were perilously close to losing our little Ava on two separate occasions.   Finally, the decision was made to move her to ICU and sedate her with Versed (essentially putting her in a medically induced coma so her brain could rest) and for 11 days either Adam or Lissa sat by her bedside for 48 hours at a time, studying the EEG monitor for seizure activity so we could push the button and mark the activity on the EEG.  Luckily, the Versed stemmed the tidal wave of seizures and then after she was weaned off it, the EEG leads were taken out of Ava’s hair after 2 weeks – she kind of looked like Medusa from the early 80’s Harry Hamlin Clash of the Titans movie, but she seemed relieved to have that junk out of her hair.   The EEGs gave the doctors a really long look at her seizure patterns and they started Ava on Vimpat in addition to her current regimen of Onfi and Keppra.   Her happy-go-lucky personality returned as soon as we put in an Elmo video (never saw someone so happy to see an Elmo video!) and she started a pretty difficult regimen of in-hospital Physical Therapy since she could not sit or walk due to the seizures having affected her whole left side.   She was finally able to leave the hospital on April 22^ND, although she still had muscle spasms as side effects from the Versed, left-side muscle weakness/paralysis and issues with reintroducing food to her after 3+ weeks of not eating.   Lilly and Zane were so happy to have Ava (and their Mom and Dad back home) but it was another month or two before Ava regained her left side.   She missed the rest of the school year to recuperate and attend therapies.

May through July, 2013 were filled with multiple therapy and chiropractor appointments as well as swimming lessons as Ava recuperated from the partial paralysis.  She has no quit in her, and would continue to try and stand, toppling over time and time again, ignoring the paralysis.   It was difficult to keep her seated for any length of time, but we do have one funny story about it:  one night, the whole family was watching TV when America’s Funniest Videos came on – and we discovered that Ava really really loves that show! Whenever some out-of-control skier or motorcyclist plowed their face into snow or mud, or some skateboarder landed squarely on his crotch, Ava let out a huge belly laugh and then clapped repeatedly as she gasped for air with delight. We’ve since TIVO’d the whole series and a good friend sent us some old AFV videos as a welcome home present.  Who knew?!   As Ava recuperated and her meds were recalibrated, her seizure pattern decreased substantially, and we noticed a positive change in her attention span and talking.  Although she’s non-verbal, she started to babble a whole lot more and her chatter filled the playroom.   In early June, 2013, she grouped her first set of words together, saying those 3 wonderful little words “I love you” that we all want to hear.  What wonderful words to hear!  And, we got in on video, too!!!

In July and August, 2013 we had a lot of wonderful family time enjoying swimming and summer activities.  Lilly (7) continues to read like a fiend and Zane (5) began riding his bicycle without training wheels.  Both continue to be great with Ava and very helpful to us in assisting with Ava’s daily needs and fun time.

August, 2013 saw us make a concerted effort to potty-train Ava by working with a behavioral therapist who arranged for Ava to sit in the hallway adjacent to her bathroom and place her on the potty every 10 minutes, all day long, hoping that she’d get the feeling of going regularly so that she might attend school without diapers.   We had some successes, and some accidents, and it continues to be a work in progress, but she is getting better and better each day.  When school started in mid-August, all three of our kids attended the same elementary school for the first time, which greatly simplified our lives with pick-ups and drop-offs.  Unfortunately, JoJo did not continue to attend school with Ava as the school became less and less accommodating to his presence and we heard reports that some of the aides were yelling at him and pulling on his leash, which no doubt led to him developing a heart murmur which ultimately ended his ability to be a service dog. Only in our family could our service dog become too ill to service the person who needs it!) As of this writing, we are preparing for our 8^TH Annual Halloween Costume Party, having raised $130,000.00 in the prior 7 years.   This year we have a Carnival theme, so fun games and cotton candy are on the way!

September 2013 to September 2015

Ava started the 4^TH grade oh-so-close to being fully potty-trained and with the hope that every new school year brings.  Unfortunately, she was saddled with yet another ESE new teacher (the 5^TH new teacher in 5 years) and this teacher was by far the worst of the lot.  Completely out of her league, paralyzed by the commotion of an ESE classroom, she was incapable of leading the students or the aides, and utter chaos prevailed in her classroom.   A mini-mutiny occurred, with a few parents switching schools or deciding to home-school.   Before doing anything rash, we decided to have a parent-teacher conference to address our concerns and were pleased that the teacher seemed intent on making some changes. But, months, later, nothing had changed and when we requested that our behavioral analyst have an opportunity to observe her classroom so we could coordinate behavioral modifications at school to be consistent with the ones that had such good results at home over the summer, the teacher refused to permit it, touching off a bevy of emails and meetings with the school that ultimately led to a final meeting with our IEP “team”.   During that meeting, it was revealed that the teacher had been “parking” Ava in a corner so she could deal with some of the more unruly children, which was easy to do since Ava loved to play on the computer or with a particular toy and could do so for hours and hours and be totally content.  When confronted about it, the teacher lied, and lied about a few other actions she said she’d taken.  It was the most shocking experience, and we considered moving Ava out of that school entirely.  Ultimately, we transitioned Ava out of that particular class immediately and attempted to salvage the remainder of her 4^TH grade year. Her final year of elementary school, 5^TH grade, saw yet another new teacher (her 6^TH in 6 years) and she finished her elementary school years in June, 2015 with more of a whimper than a bang.   All in all, our experience with the ESE world has been positive, with many wonderful and caring people going above and beyond their duty.  But, sometimes you find someone who simply should not be in a particular field or who is burned out – and that person can really destroy years of progress.  We are looking forward for Ava to enter her middle school years and to a fresh start with a new school.

Aside from school, we found that the behavioral therapy that had worked so well with potty-training was assisting Ava and us at home with a host of other developments.   Ava became adept at totally feeding herself (albeit a little messy), clearing her plate from the table (with a little hand-over-hand help) and doing a host of post-school day activities designed to provide structure to her. She also continues to engage in therapy to assist her with flexibility and mobility.   She now self-prompts for the potty most of the time, and is much more communicative with food and toy choices than ever before.  She is still a champ with the Ipad and her V-tech toys, and loves her Little Einsteins videos.  She missed JoJo, who we gave to a great family after his heart murmur robbed him (and us) of his ability to be a service dog.  Even without JoJo, she has become a great traveler, adjusting to new weather and time zones better than the rest of the family.  The summer of 2014 we flew to Chicago for the Aicardi Syndrome Foundation Conference and flew to North Carolina to meet Lissa’s parents at a North Carolina cabin and drove home and in the summer of 2015 we went to a dude ranch near Orlando (with swampbuggy and airboat roads) and to the Bahamas on a Disney cruise – and she stayed up late, ate great food and walked more than ever before, all without a whisper of a complaint.

In May and June, 2015, Ava also participated in a TOPS soccer program, a Special Needs soccer program where each child is assigned a “buddy”, a typical high school child who walks the field with the Special Needs child.  So, at any one time, there are like 20-30 people on the field.  Sometimes, the ball might be on one side of the field, with some of the children/buddies chasing it and there might be a whole crowd of other children and buddies milling about on the other side.  It’s all great fun for the children, and their families, who otherwise would never see their kids perform in a sports league.  Ava got so tired of walking that she’d plop down in the middle of the field and lie down on her back, looking into the sky.  Not necessarily the most competitive little tyke, but she really enjoyed it and we loved that she got some exercise while having fun.  And, when we got her home, she slept like a log. It’s a huge effort to get Ava and the rest of the family there for a series of Friday night (6-8 PM) games, but it became a really fun part of our week that everyone really enjoyed.

As to her seizures, UGH! After the 2013 hospitalization where she was in an induced coma trying to stop the seizures, we adjusted her meds again and encountered a relatively stable period of seizures (3-5 per night) from August 2013 to June 2015.   However, staring in June, 2015, she started to have harder and harder grand mal seizures which did not respond to the medication, landing us another stay at Miami Children’s Hospital from July 17^TH to July 25^TH.   As is consistent with our continuing “luck”, when we went to the admitting office their computers were down, and we waited 40 minutes until Ava had a massive seizure which gained us an immediate access to a private room (nice work, Ava!)    When we tell friends that Ava’s seizures have calmed down and they congratulate us, we secretly know that something bad is on its way, so this latest hospitalization was not really a huge surprise.  We have, however, become pretty expert MCH patients, packing a week’s worth of clothes for each of us along with coolers of food, drinks and lots of games and videos for Ava.  It’s just like being at home except we’re in one room, each sleep in a chair bed and shower (occasionally) using sand-paper blankets instead of towels.  Who needs the Ritz?!

As to our efforts on behalf of the Foundation, we had our 8^TH Annual Halloween Party (with a Carnival theme) in October, 2013, bringing the total raised to over $160,000.00.   Following the party, we decided to put our house up for sale and, in June, 2014, after months of endless packing and purging, we moved from our home in Trailwood (Jupiter Farms) after 14 years to a new home “in town”.  It was very difficult to move from neighbors who were wonderful to us and from a home where all three kids had been born and raised, but our new home is simply a better floor plan and location for us and, after we finish with some minor projects, will be a fantastic place to raise the kids.   Cause we ain’t moving again!   Later in the summer of 2014, Adam’s father, Richard, who had a multitude of health issues over the past few years, celebrated his 80^TH birthday on August 23, 2014 with a fantastic family celebration.   We took so many great photos that evening and, as if by divine intervention, Richard died, suddenly, a few days later, almost as if he had accomplished everything he had wanted to and said goodbye to the whole family with a big party.  He was a larger than life personality and there has been a tremendous void in all of our lives.   With the move and Richard’s death, we decided to cancel our 9^TH Annual Halloween Party, although many people donated money to the Foundation in lieu of flowers.  However, as October approaches, we are currently planning our 10^TH annual event, an Oktoberfest, and look forwarding to adding to the $160,000.00+ we have raised thus far.

October, 2015 to September, 2016

Our 10^TH Annual Oktoberfest Halloween Party raised over $25,000.00.  We have raised over $185,000.00 for the Foundation in the 10 years we have been hosting Halloween Costume parties.   Words really cannot express our appreciation to all of the generous people who contribute to the Foundation.

The past year has been an incredible one for Ava. Medical marijuana, which for years had been a distant dream, became a reality in late 2015 when she began taking the strain dubbed Charlotte’s Web and it has substantially reduced her seizures from 5-10 a night to 1-2 (and sometimes none).  The result of the reduced seizures has been an increased awareness and a more active participation in many activities in school, after school and at home.   Ava is more involved in everything she does, from therapy to eating (including choosing her food with a communication device we call “The Talker”) to more participation with our family.   Whereas before she would play in our world for a short time and then go back into “her” world for the vast majority of the day, we are finding that she is more willing to hang out with us (eat dinner with us, watch TV with us, etc.) than ever before.  She is more active with us and her siblings and seems to enjoy our company more.   So, we can connect with her more, too.

Ava entered 7^TH Grade in August, 2016.   Her favorite thing to do, aside from playing with her IPad, is still swimming. She is SO happy when she is in the pool.   Although she cannot swim, she enjoys walking the shallow end.   She still forgets that the pool gets deeper and sometimes shocks herself when the next step brings her into deeper territory and then, BOOM! – she’s under water!   You would think that the shock of being unexpectedly under the water would scare her, but, as soon as she pops up and opens her eyes (which takes a few seconds for her to realize what happened), she gets the biggest smile on her face and she laughs out loud, so we do too.    She’d probably stay in the pool all day if we didn’t drag her out.   One of the really interesting things is that when she is in the pool, she babbles like crazy and mimics us way more than she does at other times, so her best speech therapy sessions come while she is in the pool.

Nursing care has been our biggest problem this past year.  Ava still has mostly nocturnal seizures so we’ve had nursing care from 7 P.M. to 7 A.M. for several years, which permitted us the luxury of going to sleep at night (instead of the all-nighters we used to endure before we obtained nursing care).   However, in November, 2015, one of nurses moved away and so from November, 2015 to August, 2016, we had only one nurse who covered 3-4 shifts a week, leaving us to cover the other 3+ nights a week.  In the interim, our nursing agency sent us a variety of awful candidates who either fell asleep during their shift, couldn’t walk stairs (Ava’s room is upstairs!) or couldn’t lift (Ava is 100 lbs and often must be lifted when she has seizures).   It would have been funny if it happened to someone else.   To make matters worse, Adam was in a car accident in early December, 2015, blindsided by the other driver who turned into him going 30 MPH (no brakes, no horn, totaling Adam’s car) which affected his neck and back. During the ensuing months, it was impossible for him to change Ava or pick her up during and after seizures, so Lissa had to pull more weight during that time, which, of course, was a challenge for her own body.   We are pleased to report that in August we became fully staffed again, and so, at this moment, we are sleeping again.

Our family traveled to Colorado Springs, Colorado in July, 2016 for the Aicardi Syndrome Conference.  It has become our big family trip every other summer and all of our kids now look forward to it since they each now have friends who they reunite with every two years.   There are so many cool things that happen at the Conference that are difficult to explain to someone who does not have an Aicardi child:   learning from other parents who have tried what you are about to try (whether it be a new medicine, a new therapy, a new type of equipment or a renovation to your home to accommodate your daughter’s Special Needs);  mentoring a new family who has no idea what having an Aicardi child will entail;  enjoying an ice cream social with everyone when you know that giving her ice cream now will likely induce a bad seizure later, just this once because it’s so much fun to be surrounded by others in your situation;  watching your daughter “dance” in a sea of people, wheelchairs, braces and machines, without a care in the world, without the horrible stares that typically would follow us;   taking a group photo with 100 other girls, with no one looking at the camera at the same time.   There are also many difficult issues to be tackled at these conferences, with discussions about medical procedures and surgeries, special diets, therapies and death just a few of the heavy topics.   The families that we meet at the Conferences are from all parts of the world and all walks of life, united by the love of their daughters and the common theme of being in a club that no one wanted to be in:  an Aicardi family.   Our entire family is better for attending these conferences, and, when it ends, there is a sadness that we’ll have to wait another 2 years for the next one.    In the interim, we hope and pray for the good health of all of the Aicardi girls and their families.

In September, 2016, our family went to Disney World for 2 days. Ava did EVERYTHING (including roller coasters like Space Mountain, Thunder Mountain, etc.) and she was just so present in the moment.   We used a wheelchair for her because there is SO much walking, but she was such a trooper and seemed to enjoy all of the rides, characters and food.  It is the first time that we felt like she understood where she was, what we were doing.  It was an awesome weekend.

October, 2016 to September, 2017

For much of 2017, the focus for Ava was attempting to train her to use her communication device (“the Talker”) to voice her choices of her preferred activities (ie, what she wanted to eat, which game she wanted to play, etc.) and, when she wanted to, she used it quite well.   There were plenty of times, however, when she would just reach for an item of food or a game without using the Talker at all.  So, it’s still a work in progress.   She did become quite adept at self-prompting for her restroom needs and she rarely, if ever, has an accident any more.

After Ava’s graduation from 7^TH grade in June, 2017, our family relocated temporarily to a rental home nearby while we completed some renovations to our home, including enlarging Ava’s shower so we could get in there with her (her old shower was too small to do that).   Ava was a champ at adjusting to the new temporary home, and loves playing with her Ipad and ABC Town in front of her closet door, which is mirrored.  Her new favorite game is Seek-a-Boo, a picture card matching game which has two of every card.   When she places one card over the other to create a match, she slaps the two card pile like Regis Philbin says “final answer” on “Who Wants To Be A Millionaire”.  Not sure how she learned it, but it is precious and when she does it, we all hoot and holler and clap and so does she, so it feels like she just won a million dollars with all of the commotion.  She plays this game every night before bed, and, sometimes, we get her a bit too riled up with all of the applause, and then she has trouble going to bed.   We were a bit worried about how Ava would adjust to a completely new environment, but she has navigated the rental house perfectly and seems to have had no trouble in figuring out everything.   We should be returning to our “real” house next month, and now know that Ava will adjust quite nicely.

After Ava concluded 7^TH grade in early June, 2017, and we learned that she had been accepted to the Ernie Els Center for Autism, a newly built state-of-the-art school catering exclusively to autistic children which is very close to our home and which we’d been hoping she would attend since she finished 5^TH grade.  Ernie Els is an internationally famous professional golfer who lives in our area and has an autistic son one year older than Ava.  He spearheaded the financing of a special school for autistic children that is the Taj Mahal of schools, in our very own neighborhood.   We were ecstatic about this news, especially since it is very close to our home and will save Lissa hours a week in car-time in dropping off/picking up.   But, we didn’t have much time to bask in the glow of that “victory” because a few days later, starting a few days before our relocation into our rental home, Ava’s seizures went haywire again and we found ourselves in ICU at Miami Children’s Hospital for a 2 week stay from June 23^RD to July 7^TH.  MCH has always been a great place for her, but this stay was abysmal, an absolute comedy of errors: miscommunication of staff, missing or wrong information in charts, meds given untimely or not at all, lack of responsiveness, etc.   Of course, it didn’t help that we were there over the July 4^TH weekend, when it appeared only a skeleton crew was working and the whole hospital was at a standstill.   We were very happy to leave there and return to the rental home, where we had barely had a chance to unpack.   July and August were spent moving in to the rental home and running back and forth to the house to check on the status of the renovations. In September, Hurricane Irma threatened to destroy our community but, luckily, it jogged west a bit and spared us except for some power outages and debris.   Our whole family worked for about a week before the Hurricane and a few weeks afterwards to shutter, un-shutter and declutter our yard at both homes, and it wasn’t until early October that things seemed to be “normal”.  Well, our normal.

In early August, one of our long-time nurses moved away, leaving us with 3-4 nights uncovered, again, meaning that Lissa or Adam take turns sleeping with Ava on those nights so that her seizures can be monitored and handled.   At the time of this writing, we have yet to secure another nurse, so often the weekends (when we do have a nurse) are “go to sleep early nights” to catch up on sleep.   Hopefully, a sane, conscientious Mary Poppins nurse is just around the corner, as it is difficult to continue operating on a few hours of sleep when it is “your” night.   Ava still has about 2 seizures per night, down from the 5-10 she had before Medical Marijuana. We continue to hope to wean her off some of her other seizure medicines because we have noticed a huge difference in her awareness and ability to spend time with us as the amount of the Medical Marijuana has been increased.   As parents of a child with incurable seizures, we continue to be amazed that anyone could oppose the use of Medical Marijuana for any of the number of debilitating illnesses which Medical Marijuana could help, and hope that one day, it will be available to those patients who truly need it.

October, 2017 to September, 2018

Ava had a wonderful 1^ST year at the Learning Center at the Els Center of Excellence.  It is not only a state-of-the art school recently constructed in our backyard which caters to only autistic children but it has warm, welcoming teachers and staff who make it feel like home for us and Ava.   After her first week there, we asked her (by writing down questions and giving her 3 answers to choose from) if she liked her teacher (“yes”!) and her aide (“yes!”) and her new school (“no!”).  “No?!   “Why?!” When we pressed her why she did not like the school, we learned that they did not let her do everything that she wanted (which consists mostly of computer time) and that they made her do non-preferred activities such as using her left hand (which is still very low-toned and weak), writing, and other non-fun stuff, including walking a lot more from room to room instead of sitting all day.  She came home exhausted every day and, for the first time in her life, took naps in the afternoon before dinner. They were working her, which is what she needed!    She has the potential to be a much better and more independent version of herself, and we now have the confidence that she will develop all of the talents she possesses to get there.

After graduating from 8^TH grade in May, 2017, we were ecstatic to learn that she was accepted to attend High School at a sister school (the Learning Academy, on the same campus), where she will be able to go through age 22.   This is a huge benefit and opportunity for her, since for the first time in her educational history she’ll have some continuity (since she has had a different teacher EVERY YEAR of her schooling).   As of this writing, she is a few months into her 9^TH grade year, in a class with 6 other girls (the first time she’s been in a class without any boys which we think she’s slightly bummed about because she seems to love being with the boys), and we are so thrilled that she is there.

In October, 2017, we hosted our 12^TH Annual Halloween Aicardi Party at Tequesta Country Club, the 2^ND year that we had an event there instead of our home.   As we were putting on our costumes before the party, we saw a weather report predicted a severe rainstorm.   After we got to the event, we learned that it was actually a tornado!   A tornado in Florida?  Hurricanes, yes, but tornados?   We are not in Kansas, Toto!   So, of course a tornado hits the night of our party!  Dampened our attendance a bit, but not the enthusiasm and generosity of our guests, who contributed over $27,000.00.  To date, we have raised over $285,000 for the Foundation.  Not bad for a little Halloween Costume Party!

Lilly and Zane played in a local soccer league in the fall of 2017 and in late 2017/early 2018, Adam coached both of their teams in a local basketball league (he’s coached since 1992, but the last few years he’s been coaching Lilly and Zane which he says is a bit different than coaching “other people’s kids”).   Both kids had a great year of basketball:  Zane’s team lost in overtime in the playoffs and Lilly’s team won the championship with Lilly as the point guard.  Having broken her right wrist a few years ago made her able to dribble better with her left hand than her right hand, so she was difficult to stop when she was dribbling down the court.  Both kids really enjoyed basketball and Adam really enjoyed coaching them too.

In early December, 2017, we moved back into our home after having moved out for 5 months to complete some renovations.    We really needed another month or two before we moved back in due to Hurricane Irma, but our lease was up and we came back to a house still full of dust and debris.   Most of the first few months of 2018 were spent getting organized and moving back in, so we did not do much of anything except try and get back to normal.   The kids were excited to get back to their old rooms and slowly began decorating.   We still have some blank walls that need to be filled, but it is good to be back home.

All 3 of our kids finished school in June, 2018.   Lilly and Zane started the first week of summer at a local basketball camp.  It was attended by a number of Lilly’s school friends, and Lilly was a bit upset that we had decided to send Zane, too, since she thinks he has a tendency to show off in front of, and flirt with, her friends. And, she is right.  But, we sent him anyway!   And, within the first few hours on the first day of camp, Lilly broke her ankle and didn’t/couldn’t attend the rest of camp.  Wouldn’t you know it, Zane played the rest of the week on the same team as Lilly’s friends, much to Lilly’s chagrin.

One of our summer projects was made possible by the local Make-a-Wish Foundation, which assisted us in renovating our pool by raising the deep end so that Ava could walk the entire pool (great therapy) instead of the usual 5 steps and then go under water that she had been doing for years (you’d think she’d figure out that the water was slowly rising over her head, but, no, she’d continue to walk the plank right into the deep end every time if we let her).   We are excited for her as the pool is her FAVORITE activity and now she can really get some good exercise just about every day.

In July, we attended our 8^TH Aicardi Syndrome Foundation Conference (held every 2 years), this time in Minnesota. Our family was really involved: Lissa taught one session on the effect of other children on an Aicardi child and the effect of the Aicardi child on other children, Adam taught one on law-related topics like Estate Planning and Lilly and Zane were on a “sibling” panel answering questions about what it’s like to have a sister with Aicardi Syndrome.  After the Conference, the family spent a few days at the Mall of America (mostly rides and Aquarium, not shopping) and canoeing in one of Minnesota’s many lakes and eating our first batch of Wisconsin cheese curds.   It was a wonderful family trip and we were exhausted when we arrived back home.   After our trip, we said goodbye to our longtime (7 years!) nanny, who had been attending college and decided it was time to take a job in her chosen field.  It was bittersweet as she had become a part of our family and had really helped us through some rocky times, and good times, but we understood that she was moving on to work in her chosen field and we wished her well.   In August, we also said goodbye to Ava’s longtime behavioral analyst, who moved to the west coast of Florida.  Those losses were replaced by getting a new family member on August 18, 2018 when Adam’s sister Jamie married Mike Thomas in a beautiful wedding ceremony on Singer Island.   Ava, Lilly and Zane (along with cousins Devin and Simone) walked down the aisle with Adam’s sister Jodi as an intimate wedding party of family only, and Adam walked Jamie down the aisle and both Adam and Jodi made toasts at the wedding.  It was a wonderful family weekend as we welcomed Mike Thomas into the family (sucker!).

Throughout July and for most of August, we had no nursing care for Ava, so Adam and Lissa resumed taking turns sleeping with Ava each night.  The sleep deprivation brought back memories of Ava’s earlier childhood, when we did this for years and years until our bodies crashed, and we wondered how we slept with Ava every night for so many years without going batty.  After only a few weeks, the sleep deprivation is taking its toll and we hope that someday soon the nursing cavalry will arrive.  Until then, we are going to bed early most nights, so don’t call us after it gets dark!

As we write this, we are gearing up for our 13^TH annual Aicardi Costume Party, celebrating with a Lucky 13 Casino Night. If all goes right, we should cross the $300,000.00 mark for money raised from these parties.   Since some of the Foundation’s longtime fundraising families have bowed out, this party (and those like it) have become even more important because the Foundation will likely not have enough funds to continue research and hold a Conference without more families jumping in to do financing.  So, if you are reading this, we thank you with a full heart and gratitude for your support of Ava, our family and the entire Foundation.

October, 2018 to September, 2019

Ava finished her first year of High School at the Els Center of Excellence, which continues to be an amazing place for her to attend.   She really enjoyed the all-girls’ class and seemed much more communicative about her wants/needs than ever before.    She says words here and there (“yes”, “no”, “oh my”), but not yet with complete consistency, and sometimes her words disappear for a while, no doubt knocked out by those nasty seizures.  She uses her Talker (communication device) with some success.  She loves pounding the buttons to hear certain words (even if sometimes the words are not responsive to the question we asked).   But, she is better at carrying it to/from locations and we hold out hope that she’ll start to use it more as her communication skills improve.

Another personality trait that was dormant for a while, but now is in full force, is stubbornness.    If Ava wants to go to the kitchen pantry, to her room, to the closet for her toys, she is going there no matter how many times you say “no” or try to stand in her way.   She has literally walked THROUGH Lissa trying to get somewhere, and Lissa is no longer strong enough to stop her on strength alone:  Lissa must use psychology, guile and mostly bribery (“Ava, if you come sit nicely over here you can have those veggie sticks/Ipad, etc.”).   We are genuinely happy that Ava has very specific wants and likes, and that she can express them to us in her own way, but sometimes Ava’s relentlessness is difficult to manage (Lissa has already torn both rotator cuffs in holding back Ava or in prior falls). Aside from her acting like a bull in a china shop sometimes, there are a few signs of Ava’s communication skills really progressing, like recently where she was at Adam’s office for a few hours on the weekend while Lissa did things with the other 2 kids and Ava stood up and brought her shoes over to Adam (a sign that she was ready to go) or another time when she picked up the keys to the car and handed them to Adam (an even stronger hint that she was ready to go!)

Ava is most happy in her own room, where she can play with her toys and Ipad to view all of the Sesame Street/Elmo videos on YouTube, over and over again.    She has her favorite parts of each video that she rewinds and watches, each time laughing out loud as if it’s the first time she’s seen it.  It’s hard not to smile when watching her experience such joy in watching her videos.  We have taken so many videos of Ava watching videos that it could be its own show: “Watch Ava Watch Elmo!” She turned 15 in March, 2019, and her greatest joys at this time are Elmo videos, ABC Town, Seek-A-Boo (a matching games with cards of different objects, people, etc.), bowling and swimming.

This year also saw Adam’s sister Jodi battle (successfully!) ovarian cancer via a 6-month chemotherapy routine. In March, 2019, our family and Jodi’s family met in Orlando for a nice family reunion in Disney World as a sort of reward for Jodi completing her chemotherapy.   During a very nice family lunch, Ava grabbed some food off Lissa’s plate when Lissa’s head was turned, choked and proceeded to vomit EVERWHERE.   For those of you who have not experienced the joy of dealing with this in a crowded dining facility in Disney World, Disney has a specific vomit team that comes and cleans the restaurant when this happens.   So, now you know.  Oh well, as the saying goes, “shit vomit happens.”

2019 was also a year of multiple surgeries for Ava: in May, she had her VNS replaced (originally implanted in 2011); in June, she had surgery to release a tethered spinal cord;  and in August she had surgery for scoliosis (inserting two metal rods in her back and a 13 level spinal fusion from levels T2 – L3 for stability and to prevent future deterioration).   She doesn’t know beforehand what’s coming, which probably cuts the anxiety level down to zero from what it would be for a typical child; and, she’s been a champ in all of our stays in the hospital after each surgery.  After the scoliosis surgery in late August, her blood pressure dropped drastically and so we were rushed to ICU to get IVs of dopamine (to raise her blood pressure) but unfortunately she was really sensitive to the dopamine and vomited 5-6 times.  So, she got pretty beat up during that stay, with 4+ IVs and a catheter and multiple other sticks for miscellaneous blood draws. Just to make things more interesting (because an ICU stay is not enough), we had to deal with Category 5 Hurricane Dorian barreling toward Florida on Labor Day weekend (which we had planned to be home for Ava and Adam/Lissa to recuperate over a long weekend).   Thankfully, Adam’s sister Jamie and brother-in-law Mike went to the house in our absence to bring in outside furniture.   Luckily, the storm veered west (after devastating the Bahamas) and we were not affected.  So, we just had the pre-storm stress of wondering about whether we would be released before the storm and the race home to shutter the house when we returned from the hospital – but, after seeing the devastation the storm caused in the Bahamas, our stress seems so inconsequential now.

Because of the back-to-back-to-back surgeries, we kept Ava away from germs to avoid any potential postponement of these surgeries – and so, she was cooped up in her room for much of the time period of May to September – to her utter delight.   In fact, we could barely get her to come out of her room most days, so much so that we dubbed her room “her apartment” since she was vegging in it like a post-college young adult, coming out only to eat (and sometimes eating in her room).   Hopefully, once she recuperates fully and infection is no longer a concern, we can introduce her to the rest of the house and the world again.   But, until then, her apartment remains a pretty big hit for her.

Our other 2 kids, Lilly and Zane, had good endings to their 2018-2019 school years:   Lilly got straight A’s in 7^TH grade, started on her middle school basketball team (although she broke her wrist in the first game and had to wear a brace for much of the year, which limited her ability to dribble and shoot) and played JTAA (rec) basketball and soccer in addition to beginning a lucrative babysitting career.   She has discovered the joy of spending her earnings at the mall and on-line shopping and recently used her earnings to purchase a new cellphone (so she could take even more pictures of nothing as every kid these days does).   Lilly began her last year of middle school (8^TH grade) in August, 2019, and is excited to “rule the school” as well oversee her brother Zane (who started 6^TH grade there).   Zane finished 5^TH grade with a flurry of activity, including wining the school’s art contest for the best yearbook cover, building a Lego city in our playroom and playing JTAA (rec) basketball and soccer as well as a spring basketball league in which he was named an All-Star and a summer basketball league in which he competed and had the high score in his first 3 Point Basketball contest.   He began Middle School in August, 2019, nervous and excited to take the bus with Lilly 2 hours later than he had to leave for Elementary School.

As to the Aicardi Syndrome Foundation, Lissa and Adam continue to be amazed at the generosity of donors.  After last year’s 13^TH Annual Halloween Costume Party fundraiser, we reached a grand total of $385,000 raised (in part through the incredible donation of $50,000 by a wonderfully altruistic family). We were also thrilled that so many other Aicardi families took the challenge to begin fundraising for the Foundation since some of our long-time families had bowed out.  We have had friends who have had birthdays, wedding anniversaries and deaths and asked “their” friends to write checks to the Foundation rather than them as a gift/gesture, so we are learning that there are many ways people can contribute to a cause like the Aicardi Foundation. As we write this summary, we are planning for our 14^TH Annual Halloween Costume Party on October 26, 2019, looking forward to and proudly surpassing the $400,000 mark in funds raised for the Foundation.

October, 2019 to September, 2021

Ava is such a champ!   A 13 level spinal fusion cannot keep her from playing with her I-pad!   Or eating.   If she even hears a wrapper opening within 500 yards of our house, her bionic ears notify her and her head swivels around almost 360 degrees. Do not crinkle any paper near her or you will be attacked!   No doubt, Ava loves her food and the end of 2019 and the beginning of 2020 saw her gain some weight due to her sedentary post-surgery malaise.  As 2020 started, we fully expected that her PT and OT therapy would help her work off the extra pounds she gained, but a little thing called Covid-19 suddenly halted all of her in-person therapies by March, 2020.   With the world stopped, Ava’s therapies did, too, and the weight loss we had hoped for was put on hold as we navigated how to deal with the worldwide pandemic. Because Ava is so immune-compromised, our family became THE MOST conservative Covid family of all time.   We did not go anywhere, and all 3 kids went virtual for school and all extracurricular activities were halted.    Lilly’s 8^Th grade and Zane’s 6^TH grade years in 2020 ended with a whimper with no in-person attendance at school, no sports playoffs or banquets and no end-of-year dances/mini-proms.

The summer of 2020 was spent hibernating at our home.   The first few months actually were quite enjoyable, with our family going back in time with family game nights and movies since all after-school/work activities ceased.   Life was much more simple, Lissa cooked each night and we all felt healthier.  Adam lost 30 pounds by avoiding the “eating cheeseburgers on the way to a game” lifestyle that had been the norm before Covid.    We fixed up the house, worked on our business, and invited immediate family over to the backyard for distance get-togethers with separate tables for each guest’s food/drinks.   Ava enjoyed the whole family being home more often and we did more with the whole family together than ever before.   It was wonderful.

Although the togetherness of the Covid hibernation continued throughout the rest of 2020, the continuity of our nursing care for Ava did not.   A few of our nurses who were doing training at nursing homes or other facilities were exposed to Covid patients and we had to part ways for the safety of our family.    In one case, a wonderful nurse contacted us after coming into contact with a Covid patient, crying, telling us that our own agency had instructed her not to tell us because it knew that we would not have her back into our home for a while, which meant that the agency would lose shifts.  We couldn’t believe that the agency supposedly responsible for the care of our daughter would take the position that the nurse’s Covid-contact was not something that we should know, and, over the rest of 2020, we lost a number of nurses who either worked at other locations or who moved away.

Throughout 2020, Lilly became a fashionista;  it started simply enough when she sold some of her old clothes from her closet on-line.  Then she began buying clothes on-line and selling them, everything from Champion sweatshirts to skirts, shirts and miscellaneous other clothes.  She has on-line events and packages and ships items and now our upstairs loft has become a mini-garment district with postage scales, cameras and miscellaneous other equipment.  She has thousands of followers. It’s been a great project for her and is a good lesson in sales, economics and business.   Both she and Zane did well in their 2020-2021 school year and both are actively planning the 2021 year as to classes, sports and, for Lilly:  driving soon.

The end of 2020 and the start of 2021 was most definitely the worst 3-month span of our family’s life.   Ava’s seizures started to go crazy in December 2020, and we found ourselves giving her rescue meds (the kind you’re supposed to give once in a while in an extreme emergency) several times a day.   Her seizures were absolutely the worst we had scene, one after the other, and there seemed to be no way to break them.    On New Years’ Eve of 2020, they came so hard that  we thought that her eyes would catapult out of her head, and we actually thought “Is this the one?” which is the worst thought to ever pop in a parents’ head.  We debated driving down to Miami on the worst night to drive all year, in the middle of Covid.   We opted to take her a day or so later, and she was admitted into ICU at Miami Children’s Hospital for 4 weeks as they attempted to break her pattern by inducing a coma.   Lissa stayed in Miami with Ava while Adam stayed at home with Lilly and Zane.    At the end of the month, Lissa and Ava were released, stayed in a hotel for 10 days to quarantine (yes, we were that conservative about Covid) and returned home for a few days.  Unfortunately,  Ava’s seizures returned, even more severe, this time ending in her being trauma-hawked (by emergency helicopter) back down to Miami for another month in another induced coma.

The hospital stay was the most traumatic experience of all of Ava’s hospital stays.  It was touch and go and so stressful for Lissa that her MS flared up and, at one point, she could not walk while in the hospital.  And, of course, she was Ava’s caregiver while there because the aide they assigned to Ava’s room at night (so Lissa could sleep) fell asleep and Lissa told the hospital administrator never to send that aide back to the room (although the aide was one of the only aides available). Where did all of the nurses and aides go during Covid?

In March, 2021, Ava returned home and we had to watch her like a hawk, never leaving her side.    Because Ava was gone for the January and February of 2021, some of the nurses on her case took other jobs and by the end of March, 2021, we were down to ONE night nurse 3x a week, and ONE day nurse 3x a week (after having been staffed with 7 night and 7 day shifts), meaning that Adam and Lissa sleep with Ava for the other 4 nights a week and Lissa is totally alone with Ava during the days for 4 days a week.    The lack of night nursing means that we often are up for much of the night because every time Ava rolls over we have to check to make sure she is not seizing.   Do you know how many times Ava turns over every night?!   A LOT!   The sleep deprivation is slowly robbing us of energy and mojo, yet despite our efforts, it has been impossible to find additional nursing assistance.   In June of 2021, we contacted a new nursing agency since our current agency had failed to find any additional nurses and the new agency told us that they had a great candidate for us and sent a 70 year old nurse who was a head shorter than Ava and who had back issues (so she could not lift Ava) to our home for an interview. It was a very quick “no”.  And, our lone night nurse left our case to go back to days on another case, so since late July we have been night-nurse-less, with Adam and Lissa taking turns sleeping with Ava every night, with the one NOT on duty trying to go to sleep early the nights the other is the night-nurse.   Not much of an existence for us, but we continue to pray that we find another “Mary Poppins” soon.

All of our kids finished school in mid-June, 2021 and, after a very brief summer break, started up again in early August, 2021.   We say proudly that Lilly and Zane have been a huge help to Lissa in caring for Ava:   Lilly takes Ava to the bathroom and does more than any 15 year old should have to do as a younger sister, but does so without complaint; Zane is wonderful with Ava, and kisses her goodnight every night, often waiting for her for 15-20 minutes while Lissa goes through the nightly routine of bathroom, brushing teeth, medicines, etc.   It was never our intent for Lilly and Zane to be so active in Ava’s care, often sitting with Ava while Lissa cooks, or showers, or cooks in the shower, or whatever, but they have become an integral part of her care out of necessity inasmuch as our nursing agency cannot staff our case.    As much progress as Ava has made, we still cannot leave her alone without fear of seizure or another behavior (hand-wringing, biting/scratching herself, etc.) and so often Lilly and Zane are incredibly helpful in that regard.  We greatly appreciate their willingness and understanding to help and they are wise beyond their years because of our family dynamic.

We took a few local mini-trips in the summer of 2021, including to Mount Dora, Naples, Tampa and Singer Island with immediate and extended family.  Each trip was super enjoyable and we were able to feel slightly “normal” getting out and exploring the world during Covid times.  We look forward to making more fun family memories again as soon as it is safe (for us) to do so.

October 2021 to September 2022

Ava was Zane’s and Lilly’s biggest fan at their sporting events, attending Zane’s flag football games (he won the championship in November, 2021), and Lilly’s high school varsity basketball games in January 2022 (where she was the team’s leading scorer and 2^ND leading rebounder as a sophomore, which was amazing since she had not played much basketball because skipped her whole freshman year due to Covid) and Zane’s middle school basketball in March 2022 and volleyball games in May 2022.  Zane especially enjoyed volleyball because all of his friends played on the team.  At times it was difficult to tell which one was him since he and his friends all have the same awful Shaggy hippee haircut.   If Zane puts his face in front of Ava and his hair flops over his eyes, Ava will brush his air back and laugh.  And then we laugh.   At Zane.  For his stupid haircut.

In addition to sporting events, Ava joined us for a day trip to Miami to see the Van Gogh Exhibit in January 2022 and often came to dinner with us (which went fantastic as long as her Ipad was a fully charged and the restaurant had good internet so she could access YouTube).   We celebrated Ava’s 18^TH birthday at the local bowling alley in March, 2022 (she still loves bowling and loves watches her ball roll SLOWLY down the lane).  This was a special special day for our family, recognizing that when she was diagnosed a few weeks after she was born we were told that she had a 2 year life expectancy.  18 years of memories of laughter, anxiety, worry and fear during seizures and hospitalizations and more laughter came flooding over our minds during her birthday week.   So proud of her.   So grateful too.

Ava also continues to love the pool, and spent many a summer day sitting on the pool step watching the water ripple and the tree leaves wave.  As to her electronics, she has been hooked on Elmo and Abby’s Valentine’s Day song (unbelievably happy and catchy but quite annoying after 1,000 listens), Usher and Jason Derulo on Sesame Street and the Simple Songs musical videos.    She often will be watching one of those videos on one iPad, another video on a laptop, playing 2 Alphabet Town games and the matching Seek-A-Boo cards, all at the same time. It’s like watching a really good one-man band because she is doing and watching a lot of things at the same time.

After a horrific seizure year in 2021, we placed Ava on a modified Atkins/Ketogenic diet (high fat, very little carbs, lean protein) and, thankfully, Ava’s seizures for most of 2022 were much less violent than in 2021.  Ava seemed to sleep much better and for longer stretches without seizures.   She seemed to “talk” more, with more coos and sighs and variations in her intonation.   She also lost 15 pounds which was a huge benefit to her and something that had not been able to accomplish until the new diet.   Ava now eats every 3 hours while awake, and it is uncanny how, without being able to tell time, she knows exactly when the 3 hours has elapsed and heads to the food or when it is time to go sleep (she knows every night!)

In other family news, Lilly (16) obtained her Driver’s License in July, 2022 (after 5 postponements due to things like lightning and the local Courthouse being flooded); maybe the multiple postponements was God sending us a message that she was not ready, but we did not heed the signs from above.    She now drives to/from school, to the delight of Zane, who would otherwise be taking the bus 30 minutes earlier and now has a willing chauffeur as opposed to a reluctant parent too tired to pick him up or drop him off anywhere after 8 PM.   Lissa’s mother Jean fell in June 2022 and broke her neck and, after a very uncomfortable few months in a neck brace during the hottest summer on record, she was permitted to remove it in early September, just in time to be able to start rehab to revive the muscles which have atrophied before her big 80^TH birthday on October 26, 2022.   Only in our health-challenged family can a broken neck not even make it into the top news of the year. Adam and Lissa had a very busy 2022 with each having a little more the plate than either could manage, or manage well, with Adam’s office expanding its staff to handle the increased volume of business and Lissa overseeing certain office and home repair/improvement projects as well as managing Ava’s special diet and Lilly/Zane’s menu choices due to their increased desire to have food delivered.   Adam always jokes that no matter what time he comes home from work, or whenever else, Lissa always seemed to be in the kitchen putting the finishing touch on someone’s breakfast, lunch, dinner or snack.

October 2022 to September 2023

October was an incredibly busy month for our family. Our 17^TH Annual Fundraiser took in over $40,000+, bringing the total raised since 2005 to over $535,000. Lilly turned 17 and started  working at Blaze Pizza to pay for her car. Lissa’s mother, Jean, turned 80 on October 26, 2023 and we had a wonderful family dinner to celebrate.  The next day, Adam and Lissa took their first child-less trip in years to travel to Marfa, Texas to attend the wedding of the daughter of Adam’s college roommate.  You don’t just “pass by” Marfa; you fly to San Antonio and THEN drive 3 hours to the middle of nowhere and arrive at a small, artsy, bohemian slice of art galleries and great food.  We have joked it was our first and last visit there (only because of how long it took to get there) but it was an awesome long weekend with great food, drinks and friends.  Since college, Adam has kept in touch with 6 other Duke friends for 30+ years by having a variety of “guys” weekend trips to play golf, basketball, tennis, ping-pong and pickle ball and when they are together, they are like the teenagers they were when they first met.   And the stories!  They laugh ALL THE TIME!  As time has passed, the 7 guys have also mingled in a variety of couples’ weekends, starting with their own weddings and now evolving to their childrens’ weddings and, unfortunately, funerals of their parents.  It is an incredible lifetime bond amongst these families that now extends to the wives and children and, despite the years and distance, seems to have strengthened over time.   Leaving Ava for 5 days was a herculean undertaking for Lissa, cooking all of Ava’s special meals and preparing all of her different medicines for the village of caregivers it took to cover the 100+ hours we were gone.  It was a ton of work but well worth it, and came with the increased understanding that the effort to travel independently is something that needs to be made so that the marital relationship exists beyond parenting the kids.

We rented a cool log cabin in Astor, Florida for a long Thanksgiving weekend and to celebrate Jean’s 80^TH birthday;   enjoyed some good home cooking, leftovers and playing family games and watching college football.  Ava was a champ traveling and we had a great family trip.

As we rang in 2023, our family became entrenched in the end of Lilly’s and Zane’s basketball seasons and in the spring, Lilly’s flag football and Zane’s volleyball games. Ava continued to attend many of the games.  She loves the squeaking of the sneakers and the referee’s whistle, and the clapping.  She is an excellent clapper!   On March 12, 2023, Ava had a day time seizure.  She hadn’t had one in over a year and when they come – we know we are heading back to Miami and into an induced coma. Unfortunately, we were right:  the seizures would not stop, and continued more and more frequently.  She became so exhausted that she did not have the energy to eat, or drink, and over a period of a week or two, we found ourselves preparing for a trip to the hospital.  On the morning of March 23, 2023, we filed a Petition for Guardianship (which we had held off on doing but decided we better do before heading to the hospital) and drove to Miami.  Despite having been in constant contact with our neurology over the prior 2-3 weeks and making 2-3 calls on our way to Miami to coordinate a quick admission to the ICU, we were kept waiting in the waiting room while Ava seized uncontrollably.   When we subsequently complained to our neurologist about the delayed admission, we were told that Neurology and ICU operated under two different protocols, and once we stepped inside the hospital, Neurology no longer had any authority.   Therefore, ER required us to try certain medicines that we knew did not work for Ava, and it was  constant battle trying to speed up the process so that Ava could be admitted without having to jump through a number of preliminary hoops.   When we finally arrived at ICU, Ava was again placed in an induced coma to give her brain a rest from all of the non-stop seizures.   We actually had her Guardianship Hearing, via Zoom, while still in the hospital, introducing Ava to the Judge via our laptop computer (while Ava was still in the coma).   We stayed in her room for 3 weeks, tried to keep up with work and had nightly Zoom calls with Lilly and Zane, who stayed alone in our house for the first time ever.  Not that they were happy that we were gone, or why we were gone, but they enjoyed some new freedom and a sense of responsibility to do things on their own. Thankfully, after almost 3 weeks, we successfully weaned Ava off the additional emergency meds and we were finally released on April 9^TH. While we were ecstatic about leaving the hospital, and we were thrilled to return home, Ava was severely affected by the extended hospital stay and the after-effects of being in a coma.  She was unable to put any weight on her feet, had to be carried to and from the bed to a chair or to the bathroom, and slumped to one side because her core was so weakened that she could not sit upright.  Her smile disappeared.   She was incredibly lethargic, unable to eat/drink due to being so weak that she could not close her lips and swallow.  She fell asleep while eating, while on the toilet, anywhere she was.   She was just beat up.  She was on so much medicine that there was little difference in her demeanor between sleep and awake stakes.  In all of her 19 years, we’d chosen to toe the line between giving her a good quality of life without over-medicating her vs. giving her enough medicine to battle the seizures which required continuous medication and re-calibration of medicine when she gained weight or when the seizures morphed.  Now it seemed like we had crossed over to giving her so much medication that she turned into a zombie.

Since her bedroom was upstairs, and she could not navigate the stairs and needed constant supervision, we decided to move into our master bedroom (which was downstairs), and she has been living in our bedroom since that time. From April through June, we had numerous therapists (PT/OT) come to the house to try and build up strength in her legs and stamina.   Even the shortest walk made her so tired.  As July approached, we questioned whether Ava could withstand a long-planned trip to Denver, Colorado to attend the Aicardi Syndrome Conference.  However, like the champ she has always been, Ava rallied and we made the trip to the Conference in early July.   We still had to lift her in and out of the car and bed, but she was able to walk (with assistance).   Lissa worked ahead to ship an oxygen machine and rent a wheelchair and the Gumson family loaded a car with so much luggage that it looked like 20 people were traveling.  The two hour time change wreaked havoc with Ava’s medicine and feeding times, but she was a great traveler.

It is difficult to put into words how important the Conference is to our family.   It permits us an opportunity to communicate with the only other people in the world who understand what it is like to raise a child with Aicardi Syndrome. People who understand seizures, and incontinence during seizures, and medicines, and wheelchairs, and a host of things that only people “in the club” understand.  Adam and Lissa eat and drink with the parents, Lilly and Zane socialize with Aicardi siblings, and Ava socializes with her special friend Sierra who lives in Washington (about as far away from us in Florida that is possible).  There is nothing better than watching Ava and Sierra sit next to each other, each with their own Ipad, and then watch as each tries to watch and take the other’s Ipad while still watching her own.  Sierra is the closest thing to having a friend that Ava has ever had, and watching them sit next to each other is precious.   We must’ve snapped 100+ pictures and videos in the 3-4 days of the Conference, each one better than the next.

It had been 5 years since the last Conference so everyone’s kids looked entirely different/older.   We were incredibly proud of Lilly and Zane, who taught a session to younger siblings and sat on a sibling panel and took questions from the audience inquiring about what it was like to have a sister with Aicardi Syndrome or asking to relay funny stories about their sisters. Adam taught a few sessions about Estate Planning, Guardianship, Fundraising and also hosted a Q&A for siblings (including Lilly and Zane) where the siblings could discuss different scenarios (including what would happen to their sisters if their parents predeceased them).   Heavy, serious stuff, but a number of the participants came up to Adam in the day or two after the session to thank him for leading the session and providing an opportunity for discourse on some serious topics.  It was an incredibly rewarding and enlightening experience for Adam, and one of the highlights of the trip for him.

Adam’s sister Jamie attended the Conference for the very first time and was a huge help by watching Ava at certain times so Adam/Lissa could spend time with the other parents when Ava went to sleep (she is still on a very restricted medicine schedule and the nighttime dose knocks her out, almost immediately).   Despite having showed her pictures/videos of prior Conferences and trying to explain what happens at the Conference, being there was a totally eye-opening and draining experience for Jamie. The Saturday night slide show, showing Aicardi girls (including those who have passed) with their families was particularly emotional. Perhaps the most profound comment of the weekend was when Jamie said, “Now I understand.” And then she added, “YOU all need this” and the “YOU” referred to all of the Aicardi parents. No truer words were ever spoken.

As to other family issues, Lissa’s mother Jean fell again, in May, shattering her hip and undergoing emergency surgery while in Jacksonville, Florida, several hours from her home.    She had to do 2+ weeks of therapy before Lissa and her dad, John, drove to Jacksonville to pick her up and bring her home.  From May through August, Jean suffered and learned in late August that the emergency surgery was unsuccessful and that she still needs to undergo a total hip replacement sometime in the next few months.   The Golden Years!

School started for Lilly and Zane in late August and Ava is planning on returning to school after Labor Day in early September.  Lilly entered her Senior year and is currently working on her college applications while Zane is entering his Sophomore year.  With Lilly’s work schedule and her/Zane’s many extracurricular activities, it is becoming more and more rare that our entire family is together and we know that in the blink of an eye, at the end of this school year Lilly will be going off to college and our family dynamic will change dramatically.  How did this happen so quickly?!

Thank you for your interest in our story and taking a peek inside our world, where we try to live by the motto on a sign in Ava’s room: “One Day At A Time”.

Adam & Lissa Gumson