I remember about 4 years (maybe 5 years) ago I was at the Choroideremia Conference in Chicago. It was maybe my second time at one of the conferences. They have them every 2 years. I had recently come back from a 35 day bike trip where my brother-in-law (Al) bicycled across the country to try to drum up awareness, publicity and money for Choroideremia Research. My nephew with CHM was 2 years old at the time of the bike ride. I had video taped the 35 day experience to try to make a documentary out of it. I was interested in gaining insight into the Choroideremia Carrier. Women carry the disease and pass it on to their boys. That has happened in my family. My mom is a carrier and my brother and I have the disease and will go blind from it and my sister is a carrier now.
At the conference theres a point where we all split up and form support groups in different rooms. CHMers, CHM carriers and the love ones. I wanted to sit in on CHM Carriers and try to get some video footage of the discussion. I think it could be helpful for others to hear. I asked everyone if it was ok if I filmed they said it was and about 2 minutes into filming I was asked to stop filming. A woman was over come with emotion and didnt want to be filmed. I stayed for the remainder of the discussion. I thought I would be a fly on the wall and just listen and what ended up happening was everybody asked me questions. Wanted to know my view as a CHMer. Many of these parents were afraid to talk with their children about it. Afraid to ask questions. Afraid to answer questions. Some hadnt even told their children yet about the disease. They lied to their kids about where they were even going that weekend.
I was 27 years old when I was diagnosed with Choroideremia. I was shocked that it was never told to me that I may have something to worry about. Even a vague explanation of maybe there might be something to worry about. Instead my grandparents chose to initially tell their children that my grandfather, who went blind in his 40s, was blinded in a hunting accident. Then as people got older it was explained that my grandfather had a disease but that everyone else was going to be fine. Then as the children starting having their own children the subject was brought up again. Instead this time from my uncle asking what happened to my grandfather, and is there something to worry about. My grandparents confirmed that there was something to be concerned about. That there was a chance it could continue. My uncle tested his kids eyes every 2 years from then on. But he doesnt have the disease and isnt a carrier. I didnt get my eyes tested more than reading the chart in the nurses office in grade school. Not until around 15 years old.
At age 15 i was noticing with more frequency these strange bursts of light in my eyes. They would come even if I was in a pitch black room. I liken them to fire works. They werent painful but they were distracting and strange. Often they would affect my vision for the couple of seconds they were exploding in my view. Still at this point nobody has told me there may be something to worry about with my eyes. I went to an eye doctor to get them checked out. My vision was usually very good. 20/20. No glasses. The eye doctor I went to, took a look and said my eyes were fine and sent me on my way. The flashes never stopped though. On top of that I was still having a hard time seeing in dim lighting, still bumping into things and people when I really should be seeing some of these things fine. I guessed I was just a clumsy oaf. Telling people i'm a 6'0" tall person trapped in a 6'3" tall body.
So when I was diagnosed at 27 with Choroideremia. It ended up clearing up a lot of those questions and situations. But I wasnt told anything for 27 years. And I have to say that I was pained to learn how far and how long the secrecy went. That was always the worst part of it for me. And I told those people in that circle at the CHM Conference. Im not upset at getting the disease, I'm furious at not being told about it. Thats the worst part. The conspiracy of a family to keep information about someone, from that same person is awful. My Grandfather died when i was 15. My grandmother was still alive. We were ordered by my parents to not tell my grandmother because "it would kill her". So telling her could be deemed as murder? A few years later she died blissfully ignorant of what the family was going through behind her back and under her nose. I have to say though that finding out about the cover up and lengths gone through changed the way I viewed my grandparents and my own parents. Im still working (almost 10 years later) on that anger.
I cant encourage enough the idea of letting your kids in on it early and not keeping it a secret. If youre unsure what to do, go talk with a couple of child therapists about it. Ive been in therapy now the last few years and have talked with 4 different therapists about this subject and they all agree, that honesty, openness, communication is paramount to a healthy relationship with each other. If you want kids to be honest with you and open with you, you have to do the same in return. Kids learn by example. In my opinion, these kids that have diseases have every right to know whats going on with them, and to have people that dont have these diseases make the rules is unfair. Im speaking as that kid. The one kept in the dark because others didnt like confrontation or dealing with bad news. If you get to incorporate it in life early enough it'll be the "norm". Not to say there wont be difficulties but at least things can be out in the open.
Theres still a lot of secrecy in my family when it comes to Choroideremia. Still the people who arent going blind from the disease, making the rules. Its what was taught for generations and I would like to see that chain broken. I think you as a parent take great risk in not telling your children. It could affect your relationship permanently for the worse. When they are teenagers and start keeping secrets from the parents, what legs do you have to stand on when the kid turns around and says " you guys lied to me my whole life, but i'm suppose to be honest with you?"
I wont have a child with Choroideremia. At most I'll have a daughter who is a carrier. But I will be honest with her from day one. I will seek advisement if I'm unsure of how to present it. Keeping secrets make it seem shameful. Theres no shame in having a disease you have no control over. Keeping an open and honest environment in the house hold can only breed positivity. Having the kids awareness of whats going on, yes, can and will be devastating an any age. But it wont ruin their lives. They can still lead normal lives and when the vision starts getting affected new choices and decisions are made. Its hard but even without CHM its hard. Having involvement in a solution as a family could strengthen your bond, but secret keeping and lies will not.