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 20^th 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