Life with PXE

As my readers know I deal with PXE

When I was diagnosed, in childhood, little was known about PXE. The dermatologist who diagnosed me and my two siblings told us not to believe the literature that said we would die from PXE. At nine years of age I did just that: I didn’t believe the serious nature of what was in my body. At 56 I believe what I read because I know I can die from this. The truth is PXE is brutal and does cause disability and death.

Recently during my weekly chat with my 86-year-old mother (who has better eyesight than three of her four children) I was informed that my younger brother was facing some form of surgery and treatment for his heart. This was and is not good news to hear. It is hard to hear this because I live in a country where PXE is something that doctors know something about. It isn’t that way in the U.S.

I called my siblings and talked to them. I sent some mails with doctors names. My siblings like that I know something about all of this and have access to better treatment for the PXE.

Then Netherlands is a small country. It is roughly the size of the greater San Francisco Bay Area. Because of this it simplifies things medically. There is a central hub in Utrecht at the UMC where treatment for PXE is being directed by a great set of doctors. UMC In the U.S. this type of treatment for PXE isn’t present. The Netherlands as a country decided to put money into the sciences. Medicine is a priority. This makes it possible for my brothers’ doctors to talk with my speak with my doctors who know in more detail about PXE. I’m confident that they’ll learn what they need to know. Maybe something good will come out of all of this.

If you took the time to read the above link on PXE then you learned some crazy and wild stuff. I grew up with complications. I began my life with blindness which turned into low vision. Faces in the Light This just added another layer on to the situation. My ophthalmologist keeps reminding me that with eyes like mine they really don’t know much. The odds of there being anyone else like me are slim. I’m fine with being unique: I just wish they knew more about my uniqueness.

If a person with healthy vision has a problem with their eyes they can tell rapidly there is a problem. I can’t do that. I’ve never had normal vision so how am I supposed to know if my vision has become abnormal? I’ll tell you two stories to illustrate my point.

This is an Amsler Grid It is a tool to check vision.

My older sister had good vision for the first part of her life. She’d test herself with the grid and things were going well. One Sunday while driving with her husband she noticed that the light poles were wavy. Something was wrong. She went to the doctor and found out she’d had a bleed in the retina. She has lost all of her central vision in both eyes.

My sister Beth knew when something had gone wrong. So did my brother. I didn’t know until it was too late.

I didn’t know something had happened until sitting in a hotel room in Nijmegen here in the Netherlands. Things just didn’t look right as I tried to read the text on the page. Print seemed smaller. It had been that way for some time and I was just noticing it at that point because of the intense reading I was doing. That was over a decade ago and the wild ride has been informative.

So yesterday when sitting with the vascular doctor he tells me that he wants to treat me for osteoporosis they just found. I’m fine with this. Then he tells me that one of the things I need to watch carefully is my vision. I only have 12% of my vision left. What vision I do have is central vision. It isn’t normal central vision. I left his office feeling like the only thing that happens there is bad news and that being put into a kind of tail spin is my new normal.

I’m telling you this because I’ve grown accustomed to being different or unique. I’ve made my peace with the fact that when I ask a doctor something the answer might be “we just don’t know.” The upside is that I know these men and women are researching on my behalf so I’m willing to help that research along in any way I can. I don’t view any of this as anything but amedical adventure that will, in time, help others.

After 56 years I’ve made a spiritual kind of peace with my body. I’m not talking about God or anything like that. I’m talking about being happy with who I am. I’m happy that I can have the health care I have. I’m thankful for the universe around me.

I will still get frustrated at times; I’ll still be not OK that some things don’t go smoothly for me: life isn’t smooth. If I have to credit a real starting process for this resolution it would have to be to thank all the good eggs at the Loo Ert It began a process of quiet resolution.

I will add that the Loo doesn’t exist in any form in the U.S.A. I’m glad I had this experience.

Does this mean I’m going to abandon the fight for the disabled? NO!!! What this means is that I’m planning on using a larger voice to plead with the universe for better understanding of not only the physically disabled but also the mentally disabled.

Get on board it’s going to be a great ride of learning that I’ll try to keep fun and informative.

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About Gail Nicolaysen-Shurtleff

I am an American who is living in The Netherlands. I am a Marriage and Family Therapist with a license to practice in California. Music has been a huge part of my life. Singing is something you can find me doing at any time and anywhere. I have to admit that I try to not sing while out in public though. You can also find me in the kitchen cooking up wonderful things. cooking is something that I have just recently (in the last ten years) discovered that I'm talented at doing, and it is really fun. The best way to know me is to read my blog.
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