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Joey’s Monster Ball

One of my wife’s very good friends Molly passed along a link to this site, JoeysMonsterBall.com, which was put together by one of her friends Rachael Cross.   Rachael is a young mother whose infant son Joey is terminally ill with Tay-Sachs disease. I have personally never met Rachael or her son Joey, but I was so moved by their story that I wanted to pass it along to the readers of this site.  Before I do – I want to tell you the event they are putting on in Cincinnati:

JOEY’S MONSTER BALL
Sunday, November 2, 2008
5 – 9:30 p.m.

WHERE?
The fabulous 20th Century Theater
3021 Madison Road
Cincinnati, OH 45203
513.731.8000

the20thcenturytheatre.com

WHY? To celebrate the life of Joey Cross, an amazing little boy who desperately needs your help
Suggested donation: $25
Dress up in your favorite costume and come ready to have a ball while making a big difference in the lives of Joey Cross and his family. Get your ghoulish groove on the dance floor with music from a DJ . Strut your stuff in your best costume and you just might take home grand prize in the costume contest. And make sure you’re carrying plenty of cash to bid on all the fabulous silent auction items*, and enjoy the cash bar – all for the love of Joey.There’ll be plenty of devishly delicious appetizers, frightfully fantastic music and so many wonderful ways you can help, it’s scary.Don’t miss it. Invite your friends, family, mailman and anyone else you can think of and make plans now to be there.

I had never heard of Tay-Sachs prior to this.  Rather than try to describe it, I thought I would just pass along “A Letter From Joey’s Mom,” where Rachael chronicles her family’s experience with the disease.

My name is Rachael Cross and this is the story of my son Joey.

Joey was born on May 5, 2007 – the happiest day of my husband Ron and my life. It was a joyous wonderful day that couldn’t have gone any smoother – and it was Cinco de Mayo – a day of celebration and happiness, which felt fitting to us.

My pregnancy started off perfect. I felt good, Joey was growing – we had everything we ever dreamed about. But, at 27 weeks I went into pre-term labor. I ended up being on bedrest for 9 weeks. But in the end, it all worked because our beautiful baby Joey was born healthy – the 9 LONG weeks of bedrest were so worth it.

Then when Joey was about 3 months, I starting having this gut feeling that Joey wasn’t developing normally. I called my friends, Joey’s pediatrician, my physician friend’s, my sisters, saw physical therapists – everyone told me to STOP comparing him to other babies – they all develop at their own pace. Each time a friend would say that, I would do my best to turn off my gut feeling for a few weeks.

At about 8 ½ months I simply couldn’t ignore my gut any longer, I went back to our pediatrician. She finally said we should see a neurologist – to calm my anxiety. When we saw the neurologist, he confirmed that Joey was indeed slow and had low muscle tone. He thought it might be a good idea to get his eyes checked. So, 4 days later we were visiting the pediatric ophthalmologist. When he looked in Joey’s eyes, he looked at me and asked if I was Jewish. I was floored. I only know of one disease specific to Jewish babies, Tay-Sachs, and Joey couldn’t have that as his daddy isn’t Jewish.

2 days later we met with the genetics team at Cincinnati Children Hospital Medical Center and they confirmed my worst fear. Something was wrong with my baby – but it was far worse than I ever imagined. They strongly suspected that Joey had Tay-Sachs Disease. After a series of blood work, they finally confirmed the diagnosis and we learned that our beautiful Joey had Tay-Sachs.

The outlook for a baby with Tay-Sachs is horrible. Tay-Sachs Disease is a progressive neurological genetic disorder that so badly affects the nervous system that life cannot be supported. Babies with Tay-Sachs lack an enzyme, called Hex-A. Without this enzyme, their bodies cannot break down a fatty substance in the brain and it builds up in the nerve cells of the brain. This accumulation causes progressive damage to the cells.

Just like Joey, a baby with Tay-Sachs disease appears normal at birth and seems to develop normally until about 6 months of age. The first signs of Tay-Sachs disease can vary and are evident at different ages in affected children. Initially, development slows, there is a loss of peripheral vision, and the child exhibits an abnormal startle response. By about 2 years of age, most children experience recurrent seizures and diminishing mental function. The infant gradually regresses, losing skills one by one, and is eventually unable to crawl, turn over, sit, or reach out. Other symptoms include increasing loss of coordination, progressive inability to swallow and breathing difficulties. Eventually, the child becomes blind, mentally retarded, paralyzed, and non-responsive to his or her environment. Right now, Joey is doing well. He is now 16 months old. But other than his typical 16 month old size, he can no longer roll over or use his hands or legs. He doesn’t sit, talk, feed himself, or move. And, we don’t believe he can see anymore and he has an extremely sensitive startle response – the smallest noise will scare and surprise him.

But we believe that Joey is still happy. Every once in a while he can still give me those amazing smiles. When he does, it lights up a room. He is the most amazing child I have ever seen. Even strangers comment on his amazing, infectious smile. One of the saddest moments in this horrible nightmare is when I learned he would eventually loose the ability to smile. Joey’s smile is what gets me out of bed every day.

We have taken Joey out of daycare, as the physicians don’t want to stress his immune system by being around so many other children. We now have a nanny at our home. We are also working with Children’s Hospital’s Starshine team (their hospice and palliative care team for terminally ill children).

The future is very scary for all of us. Not only have we had to start coming to terms with the fact that our child will die young, we are also burdened by the financial constraints. In addition to the considerable added expense of a nanny, soon, Joey will need more experienced nursing care. He will need equipment and medications – things that our sad, broken insurance system won’t cover. For us, worrying about money feels so disgusting, when we should be focusing our energy on loving our son.

In addition, I feel that Joey would want us to educate others on this disease. I am Jewish and have always known about this disease. But I was misinformed. I thought both parents had to be Jewish. Clearly, that is not the case. And if a Jewish woman didn’t know the reality, does anyone else really know?

About 1 in 27 Eastern European Jews (Ashkenazi Jews) carry the gene. In addition, 1 in 27 French-Canadians and Cajuns carry the gene and 1 in 50 Irish-Americans carry it, too (Joey’s dad is of Irish decent). And in the general population 1 in 250 carry the gene. Since Tay-Sachs is a recessive gene disease, both parents must carry it in order for their children to get the disease. This is good news for most as even if you are both carriers, you only have a 1 in 4 chance of passing the actual disease to your children. But since it is recessive, it is also VERY scary, too. That means you could be carrying the gene from generation to generation and never know until someone you love gets it. When I told our geneticist that no one in our family has this, he simply said “no one ever has it until someone has it”.

Our lives are forever changed. We have been blessed with our amazing son and feel like we have a mission in this world. The first, to love him every single day of his short life and not focus on the disease or money or any other stressor. The second is to tell the world about Joey so that he can help educate other families that a simple blood test can prevent this disease.

We are asking for your help to carry our mission, to keep Joey’s memory alive and strong, allowing us to educate others – with the ultimate goal of eradicating this horrible infant disease.

Much love,

Rachael Cross

Reading this really puts life in perspective.  I wish the Cross family the best in the days to come.  I can not imagine having to endure something like this.  I admire their courage to speak up and share their experience with the world.  I know that it can’t be easy.  I hope that they are able to enjoy every remaining second they have with their son.   By simply telling their story, they are changing the world.  Please do what you can to help them.  Start by visiting joeysmonsterball.com to make a donation and learn more about how you can help.   Then pass the link along to others you may know.

Joey’s Monster Ball