By Jennifer VanDerwerken
When I moved back to Upstate New York, I started my first public Instagram account. I wanted to learn more about my new community, network professionally for writing projects, and meet some local mom friends. I can’t recall how we found each other--if Crystal started following me first or if I started following her--but I remember seeing her handle @beatrixblue pop up in my feed and my comments and feeling like I was getting to know a very cool local mom. I loved her shots of her lovely kids, her gorgeous house (seriously though, magazine worthy) and her pithy, irreverent captions which spoke of a wry sense of humor and an utter lack of pretense. I liked her instantly.
I don’t remember what post it was that first clued me in to the fact Crystal was dealing with a situation that no filter could make pretty. Two of her three smiling, beautiful children are terminally ill. Crystal does not shy away from this reality in her social media account, nor does it dominate her every image and story. She is bold, smart, unflinchingly honest, funny, and compassionate.
Crystal is a warrior among us. She is living an experience that is beyond most of our imaginings and she is generous with her knowledge. I believe that Crystal’s story needs telling--big, loud, far-reaching telling. I believe that she has wisdom to share. And I want us all to sit up a little straighter and listen.
Hi Crystal! Tell us a little bit about you and your family.
My name is Crystal Hinman, I am nearing 35 and live in Stillwater, NY with my husband, DeForest, and our three children Adeline (9) Beatrix (7) and Moses (4). DeForest is in finance and I am a stay at home mom. My background is in Early Childhood Special Education and I have spent the last 23 years taking care of children. Just reached 14 consecutive years of diaper changing, which I strongly feel should garner a certificate or something. I drink too much coffee, I love holidays, and I am really into BBC TV. I am a “good enough”-ist and can find something to laugh about even in the darkest of days.
DeForest loves his wife, his kids, and his tractor, in that order. He is incredibly intelligent and a perfectionist. We don't know why we work as a couple but we are glad we do!
Adeline goes to the Stillwater school district and Beatrix and Moses both go to The Prospect Center in Queensbury which is run by The Center for Disability Services. Adeline is a sweet, smart, wonderful girl. She is an old soul and loves the arts. Beatrix is a spitfire, she feels better with us near but loves to run outside in our yard and laugh. Moses is a cuddler, he crawls up in anyone’s lap and rubs their faces and is truly happy to love and be loved.
Can you please tell us about Beatrix’s and Moses’s illness, and how and when they were diagnosed.
It's hard to be brief with this explanation.
Beatrix and Moses were both typical children, they walked and talked at one, they had favorite toys and read books with me. Beatrix loved art, Moses loved the beach. It started with Beatrix so I will begin from there.
When she was 3.5 years old, I had noticed that she was struggling with some motor skills, tripping more, etc. She'd started night wetting again after about six months of being dry. Both of those things could have been phases but I was worried enough that I took her to the pediatrician where we ran some blood work and got a script for a PT/OT evaluation. I wasn't that concerned initially and thought it would work itself out. I was knee deep in diapers and Moses was my toughest sleeper so I didn't have a lot of energy to expend towards worrying.
At 3 years, 8 months Beatrix started having some strange looking episodes in the morning. After a few trips to the pediatrician and the ER and a few brush offs from residents, I finally met our now neurologist Dr. Pugh who said he wasn't sure what was happening but he wanted to see us in office, and in the meantime have my phone handy and if it happened again to film it. I will never forget the day she next had one. I was nursing Moses in a chair and Bea was walking away from me down the hall and suddenly it was like she was drunk, her shoulder kept bumping into the wall and when I called her name she turned around with her head tilted to the back. Then she sort of came to and walked to the fridge and started pointing. I had my camera on for nearly the whole thing. I made sure to say things out-loud that were happening so when I emailed it to Dr Pugh he could hear me. I said she is breathing and drinking, she is following directions but cannot speak. When her voice came back it sounded as if she had had a stroke and the only thing I could understand was the word "mom." I emailed the videos right away and Dr Pugh told us to come into the ER that day and get an EEG.
The results were shocking even to him-- Beatrix had seven seizures in her sleep while hooked up. We got a diagnosis of Epilepsy with complex partial seizures. We were scared but I was extremely hopeful initially that if we could get the seizures under control we could get her skills back. She reacted well to seizure meds and even on a very low dose of the most benign medicine was seizure free. That was September of 2014.
In December I followed up with The Spotted Zebra for a whole child eval and the results were scary. I wasn't expecting the level of need Beatrix had and had to bite my cheek not to cry. PT, 1st percentile, OT 1st percentile, overall cognition 15th percentile, extreme left sided weakness, etc. We had stopped the seizures and I spent my days doing letters and art and hikes, and loving those kids. I knew something else was wrong. We did repeat EEGs and blood work and MRIs and I spoke to the top epileptologist on the East Coast. Bea started lots of therapy and preschool. But she was making zero gains as far as skills go, she was stagnating, some months progressing but then losing the skill by the next therapy session. Every child professional in our lives was saying something must be wrong, something must be wrong, but we had really done all the tests and every result was perfect. Sadly the answer really came on the back of Moses.
When Adeline was 7 and Bea was 5, Moses turned 3. I cannot tell you how wonderful he was for me. His energy and love really got me through all those years with Beatrix. He is a light and quickly became my little buddy while the girls were in their little school programs.
At 3 he was incredibly verbal and inquisitive. He knew his letters and car makes and models and how to get a brownie out of me and how to do karaoke and all the Thomas characters. A week after he turned 3 his Nana and Great Grandparents came to visit and we took them to our house site (we were building) and out to lunch. Moses looked so happy and peaceful and I can still see him snuggled up to his dad at the table.
The next morning was a Monday and Moses came to me first, he was always an early riser. I did my usual and put a Thomas on for him and went back to bed, but about five minutes later he came back in and he didn't look right. I sat bolt upright and told D that something was wrong. Moses looked lethargic and wouldn't talk to me, but then Moses threw up and I just relaxed. Because duh, I bet he has the stomach bug. Even at 3, Moses had already made his opinion know about throwing up, he didn't like it.
So DeForest went to work and since it was summer I just watched Moses closely hoping to keep my couch from getting bombed. About a half an hour after D left, I was sitting next to Moses on the couch and he suddenly stood up, put his hands up palms out, and gasped like he had seen a ghost. I was right there so I picked him up and I could feel that he wasn't breathing, he was turning blue quickly and I called to Adeline to hand me my phone and I called 911. I was breathing too heavy and the operator had to tell me to take a breath and that was all I really needed to hear to reign it back in and start describing the situation. My son is not breathing and he is turning blue, his sister has epilepsy and this looks like a seizure, he is throwing up. Could I finger sweep him the operator wanted to know? His jaw was seized shut so no I couldn't. I rolled him on his side and Adeline got me a cloth and I started wiping his face. We lived about two minutes from the hospital in Menands then and by the time the ambulance got there Moses was breathing again, he was lethargic and unresponsive but color was back and his jaw was open. I followed the ambulance and called DeForest to meet me at Albany Medical Center. We waited in the ER and he continued to have seizures. Dr Pugh was on the floor that day and I felt comforted knowing that he was on our team. We did lots of tests and Moses had lots of seizures for months after that and we thought we might have a possible diagnosis but that too never added up.
By 3.5 Moses was no longer able to speak, there was no spark when I showed him letters, and he no longer had the skills to build a train track or even stack blocks. He had lost almost everything in six months.
By 5, Beatrix had started to lose her mental faculties. She had no control over her moods or her emotions. She had lost so much cognitively that she was easily frightened and startled. They were both no longer potty trained. We ran more tests and repeated tests and Dr Pugh and I agreed we should try Boston again.
You can probably imagine the level of hopelessness and exhaustion that had seeped into my bones by now. I knew in my heart my children were not going to get better but I needed to know why. The Neurologist in Boston recommended we get whole exome genetic sequencing done on all three of the children. Adeline was 8 by then and totally typical and healthy but it was always a concern in the back of my mind. There were many hang ups and mess ups, all on the testing facilities end and there were tearful phone calls where I had to demand action, but finally after months of waiting we got the results.
I want to tell you that once you get results like this you can never unhear them. That once your heart breaks like this there is no repair. It has been a relief to know what we are fighting but an inconceivable amount of pain to know the disease is currently unstoppable. Beatrix and Moses both have Juvenile Tay Sachs Disease. It is a lysosomal storage disorder in which the child is missing an enzyme, Hex A, that the human body needs to break down a lipid. Without the enzyme the lipid builds over time and is toxic. My husband describes it as a bathtub filling up, their drain is only open 15% and while you can hang for awhile with the water slowly draining, eventually it will catch up and you will drown.
Every one of their skills will be erased over time. In the end they will be in a vegetative state unable to talk or walk or maybe even see us. They will likely die of a respiratory complication like pneumonia. Tay Sachs is always fatal, there is no treatment, and no therapies to lengthen life. Life expectancy can be hard to predict with Tay Sachs, every child is different. But we do know that 10-15 seems to be a common age range. Moses, although younger, is much more affected than Beatrix and lost skills faster so we know that the children may not die in order. Thankfully Adeline is healthy and will never get Juvenile Tay Sachs. This is a disease that takes 2 parents having a mutation to pass and even then our offspring only had a 25% chance of inheriting both copies. Moses and Beatrix did, Adeline only inherited mine. It is extremely rare and hard to diagnose and on average takes 3 years from onset of symptoms which is exactly where we were at.
I want to add that I know this is all devastating and we did struggle for years with answers, but we also lived a life that whole time. We went to the beach, we took the kids to Disney, we had Easter egg hunts, and I have folded a million pounds of laundry. I still hate cooking dinner and cleaning up the guinea pig cage. We built a house and planted a garden, and even though life is hard we are and have always been actively living it even if the children are actively dying. Like many families with medically fragile or even terminal members, we keep on, we love, we laugh a lot, and we cry too.
I want to add that I know this is all devastating and we did struggle for years with answers, but we also lived a life that whole time. We went to the beach, we took the kids to Disney, we had Easter egg hunts, and I have folded a million pounds of laundry. I still hate cooking dinner and cleaning up the guinea pig cage. We built a house and planted a garden, and even though life is hard we are and have always bee
What are the most supportive words and gestures that friends and family can offer you and your children?
I try to be very open to all the love languages people might use to show us support. Our families and friends have rallied around us for years. They have watched our kids, cooked us meals, cleaned our houses, delivered pets to our home, delivered pastries to the ER, donated money and time and above all held us in their hearts the whole way.
Especially other moms man, they show up. They showed up so hard every time we called. The kids' therapists have cried with me on the phone and their dentist has mapped out plans to keep their teeth healthy even in the end when they won't be able to chew. So as far as gestures I try to be very aware that people show love lots of ways. Words wise I think we get our best support when people acknowledge our reality. I am not bothered by people saying "woah, that must be hard!". Yes, it is. I am also not bothered by questions about the kids, but do love it when some of those questions are also about what they like and what their favorite food is instead of just their diagnosis. If you need to cry I am okay with that, and if you are the type of person who doesn't usually emote in public well welcome to my world, sister! The rare disease community has been especially amazing. These are groups of parents who have been through similar things and might have already lost their child and they welcome you with open arms to their small but fierce community.
What are the least supportive/helpful words and gestures?
I can usually find the good in what people say even if it comes out kinda wonky, but I am bothered when people brush off what I am saying like it can't be true. I know its a defense mechanism--they hear this terrible news and so they want to say it can't be. Or they imply if you have enough hope, a cure will be there.
Hope is not a cure and I am not hopeless, I am just hopeful for different things. I am hopeful that the kids will die in their home with us next to them, that Adeline will survive this experience of helping guide her siblings to the end, that our daffodils will grow, that I will appreciate every smile my children have before the last one. My hope is still there it's just shifted. A lot of people have a hard time thinking about death and the process of dying and that is okay, I just do not have that option.
Also if you make me a decaf you are out of my life! Ha!
How do you cope with day-to-day life in the face of this struggle? What big and small things get you through the hardest days?
I have always had an even temperament that is geared well for emergencies. I laugh a lot! I love instagram and silly memes and chatting with just about anyone. I think coping for us right now is focusing on the living part, any way we can experience life with our family and friends and soak up memories. I try my hardest not to live in anticipatory grief and it is challenging. Especially when holidays arrive and I think about future holidays with less children. I start thinking about how it's likely that neither of Adeline's siblings will see her graduate from high school..and see how fast that was? I am in anticipatory grief. I have to then dial that back, think of something we have to do today (I have to cook dinner again?!). I think of Adeline and Beatrix laughing with each other on the swingset, and bring it back to now. It is hard work and I love those moments that I can forget even if it is just for a little while. Also our life is busy! It is hard to sit down and think about anything too long when I have diapers to change and lunches to pack and Adeline has a field trip. I am still running a household over here.
Add in phone calls to all of our medical staff and volunteering at the littles' school two times a week and I am swamped. In the end I rely on lots of mental support and little positive things to get through the day just like any busy parent. Occasionally I will get a big thing like a spa day but mostly I just want Adeline to grind my espresso beans for me or for Bea to have a good school day.
How do you navigate your family’s situation with your older daughter, who does not have Juvenile Tay Sachs?
Adeline is very intelligent and has seen the progression of the disease from the beginning. She remembers when they could all read together and sing songs, and so she has always been aware with us. She got dragged to more appointments than she cares to remember before full day school started.
We have always been honest with her, but let her pick the pacing of when she learns things and that has worked well. When we told her we had a diagnosis she was scared and sad and very angry and relieved to know she was okay. I keep the conversations open and flowing and she eventually asked about life expectancy and what they would look like at the end.
She has a wonderful counselor, and teacher, and her lifelong best friend is also aware of what is happening and had been there right from the start. Adeline knows she has people to talk to. We make sure she has time in our day and our life for her activities and for time with just the three of us after the littles go to bed. She is attending a weekend camp in may for children who have a family member with a serious or terminal illness and will see that she isn't alone even if she is rare.
How do you nurture yourself?
I have some alone time everyday in the car after school drives that I usually cry and lament the universe during. The kids were just home for ten days on break and I could feel a build up and was glad for Monday when I could just weep at home for an hour. There is a sadness you carry with you everywhere, its is like a weight, and I need to relieve that pressure. It's a good thing for me. I have gotten better at putting myself first on the list just as I have grown in my parenting. When we start out we kind of fall off the list (like Oprah says!) and newborns take over everything, but as the kids have gotten older I have made my needs known. I will drink hot coffee, I will sleep in occasionally, I will leave for an entire girls' weekend. I am also a little better at reading when I am at my breaking point and ordering a pizza or just buying an espresso instead of making it. This is where my non-perfectionism is really paying off because at the end of everyday I usually end up thinking I did a pretty dang good job and it is pretty nurturing to love yourself unabashedly.
How do you nurture your marriage?
DeForest and I are lucky enough to be an easy fit. We met in Kindergarten and started dating at 18 and we are still really madly in love. We have put in the work to communicate well and recognize our efforts and support each other, because we are really two very different people but in the end we balance each other pretty naturally. We have always had a very physical relationship and even now when you would think sadness would tamp everything out spark wise, I am still really digging sex. That is always good for our marriage! Ha! I know D well enough to know when he has taken one more bite of food than he should have and he is too full, so I also know that when he spends time building a swing set or making the land flat so the kids won't trip that he is working through his grief even if he isn't saying it. He knows those things about me too. We have spent 16 years observing each other and finding ways to love one another. We both really like making the other happy. Every once in awhile we check in with each others level of sadness and add support pieces when needed but mostly we communicate through action and try to live the life we have right now. We go on dates and spend every evening wrapped up together, even if we are watching different things--because real life! We are both good at talking instead of fighting and that is just a miracle that was bestowed upon us and we are grateful for it. DeForest will always be first on my list. Of course I love the kids but I chose DeForest and I will keep choosing our marriage.
What would you want to say to other families raising medically fragile or terminally ill children?
I love you. I love your children. I am so sorry we are on this path together but I am glad to have the knowledge and love that you have shared with me. We are a community of warriors fighting for the best possible life for our children and our families. That is so hard but has been made so much easier for me because we have one another. So just remember you have me too. Even if our paths are parallel and never intersect, I will hold your hand from my side.
Is there anything you’d want to say to parents who do not face these kinds of struggles?
I want you to love your life, yourself, your family. Love its imperfections and its challenges. Live your life right now and don't wait for later to start doing the things you love and taking care of yourself. I don't think it is possible that we all live everyday like it is our last, but let me share with you that a very precious clarity came to me when we got the diagnosis, about what to use my energy towards and what to walk away from.
I am here to participate and enjoy and mourn my children's lives. I am here to witness for them and so are you for your children. Parents don't look away, even when we want to.
Readers, I know that if you made it to the end of this article, you are now as in awe of Crystal and her family as I am. And you might be wondering if there’s anything you can do to show them some support. The pool at the Charles R. Wood Aquatic Therapy Center at Prospect Center, a division of the Center for Disability Services, which the Hinman family uses, was rendered inoperable in January of 2018. The restoration has an estimated cost of over $100,000.00. They are currently fundraising to get the pool back up and running. You can donate here: https://app.mobilecause.com/vf/pool
And you can follow Crystal on instagram at @beatrixblue.