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Part 1: Albinism vs HPS

This week I was reading my friend Karen’s blog, www.myspace.com-karenshpskorner, and was touched to find that she’d been blogging about me. I was also profoundly struck by the comment left in response to Karen’s entry by another HPS friend, Ana. I e-mailed Ana and asked if I could put her comment here, and she agreed. So, here it is.

“I agree with you wholeheartedly, but when I have expressed some of these feelings in the past, I meet with resistance to my feelings. Recently, in another group, I happened to voice many of those feelings only to be told not to feel that way. I especially detest the comment "I'm proud and happy to have albinism". Even though not every person with albinism necessarily has HPS, I still hear "I'm happy to have HPS"...how could this be? I don't hear anyone saying "I'm happy to have cancer". How could anyone ask me not to feel this way? They are in fact asking me to be in denial then. How can anyone ask me not to feel this way...that I more than likely will not see my daughter become a young woman and graduate from college or maybe even high school, let alone to even think that I will see grandchildren? It is also disturbing to know that some people with albinism would rather ignore it and not go for testing and try to help the cause.”

In just a few short words Ana managed to hit on a huge spectrum of issues that whirl around in the HPS community. Any one of them could be a lengthy blog entry. I’m not sure I’ll be able to get to them all in a single entry.

I’ll start with the whole albinism vs. HPS thing, as some readers might not understand that one. Albinism is a term for a whole collection of genetic mutations that cause a person to have a lack of pigment. HPS is the name given to a subset of these mutations that, in addition to causing albinism, also cause a wide variety of other health problems. The extent and degree of these problems greatly depends on the particular mutation involved. In other words, we HPS’ers are a minority among minorities. The result is that sometimes our needs are at odds with the greater albinism community.

In general, albinism usually involves a vision impairment and sometimes (but not always) involves a reduction in pigment in the skin and hair. Neither of these are problems that affect your overall lifespan. Thus, the general albinism community bristles to hear albinism discussed as a “disease” or a “medical problem.” It would be like describing the skin color of someone who’s an African American as being a “medical problem.” It’s insulting. As for the vision, if you live with a vision impairment you learn to compensate. The amount of vision one has, or doesn’t have, isn’t much of an obstacle in life. The true obstacle is everyone else’s attitudes about it.

But for people with HPS, the color of our skin or the extent of our vision is often the least of our problems. It’s hard not to regard your albinism as a medical problem if it’s affecting your ability to eat, or breathe. Thus, often our message is at odds with the greater message the albinism community sends out.

This is complicated by the fact that HPS is so rare, and the majority of people that have it are completely unaware. The HPS Network is currently diagnosing at least a family a week, and none of them seek out the Network because a medical professional suspects HPS. On the rare occasion that someone learns of HPS from their doctor, we just about fall out of our chairs from shock. More often someone has read about it on the NOAH page, or started to do their own research and happened upon the information by accident. Unless someone with albinism has a confirmed, genetics tested diagnosis of another type of albinism, they can’t be sure they don’t have HPS unless they are tested for it.

There’s nothing wrong with being proud you have albinism. And heck, you can be proud of having HPS if you want to – but because so many people don’t fully understand HPS or its ramifications, often we HPS’ers get blasted by the general albinism community for the way we feel about our albinism. Often even people that have HPS don’t fully understand it, and so they lump themselves into the general albinism category and adopt its lingo and perceptions. When someone comes along in the throws of dealing with the darker side of HPS, we’re often labeled as having a bad attitude, or simply being negative. That’s not fair. For some of us, the complications of our type of albinism are very real and very immediate. And, we are no less albino than anyone else. I suspect that what Ana was dealing with in this other group was either someone in denial, or someone who wasn’t educated about HPS.

Part two coming….




Comments

Anonymous said…
Hey Heather:

As usual...very well-written. Surprisingly enough most of those negative comments were from one of our own HPS list members who IS educated on HPS.

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