By Rhonda C.
Incontinentia pigmenti (IP), was a condition that I had never heard of until I was in my early 30s.
I had no idea of how it would shape my future.
Growing up in the beautiful Snowy Mountains of New South Wales, Australia, I spent a lot of time outside and was consistently exposed to the weather.
At a young age, I learned that I could always get warm, but cooling down was always much harder. I seemed to be hot on the inside and out.
The Rash We All Shared
I was born with a rash as were my three sisters, my mother and also my grandmother. The “rash” varied among us. For me, my left leg, hip, buttock and a smattering on my back. My sisters, by comparison, were far worse off with the rash covering both sides of their bodies.
I remember when I was about six, a specialist was visiting our regional area. He was very interested in our rashes and took photos for a medical journal at the time. This was in the early 1960s.
Feeling Different in a Small Town
People are scared or wary of what they don’t understand. I found this to be true for IP.
When our small country town finally got a swimming pool, it was a big deal. Imagine how we felt being told that we were not to use the pool in case whatever was wrong with us was contagious! I will always remember the embarrassment we all felt when it was finally decided that we were probably safe. Never have I felt as scrutinised as I did in my swimming costume that first summer season I attended.
I am not a vain person, but there were many times where I felt embarrassed by my skin and I would always cover as much of myself as possible in social situations.
Life in the seventies were days of freedom, lots of time spent at the beach and having outdoor adventures. I realize now why I felt sick and often exhausted after a day in the sun. Sun protection was a new concept and having a tan was the thing to do.
A Diagnosis, But No Guidance
It wasn’t until I was much older and diagnosed with discoid lupus that the “rash”’ was also given a name, incontinentia pigmenti. However, I was not given any understanding of the disorder itself. Or any information on what I could do to better look after myself.
By the time I reached my mid-thirties, I was having major dental issues. I remember removing my wisdom tooth by hand, with no major after effects. It simply grew out of my jaw! I lost many teeth during this period of my life.
Around this time, my sensitivity to heat and glare dramatically increased. I was having episodes of weakness, nausea and light headedness, that would develop into seizures if not attended to.
A Difficult Journey to Motherhood
At the age of 34, I gave birth to a beautiful and healthy baby girl. She has no symptoms of IP. She was, however, my 13th pregnancy. I usually miscarried at around four months, just pregnant long enough to know and for morning sickness to be a regular happening. I did not know that IP was probably responsible.
Not that knowing would have changed the outcome, but I would not have been looking for a reason why. The emotional roller coaster of surprise, worry and sadness did take its toll. I was planning to have my tubes tied, when I fell pregnant with Grace.
Wondering What Could Have Been
I look back now and wonder, if things might have been different for me if I had been given information about IP?
- I think it would have. I could have been more accepting of my health issues and would have known how best to live my life.
- I would have protected myself more from the hot, sunny days at the beach.
- I would have studied more suitable subjects at university. I have a degree in sustainable land management and later on I became an equine podiatrist. Both are outdoor vocations and hard physical work.
- I would have considered adopting a child.
- I would have begun creative writing earlier.
- I probably would have made many more alternate decisions with the knowledge I now have about IP.
A wise man once said “knowledge is power.”
I have watched over time the effect IP has had on my sisters. There are symptoms in common, but also those that are unique. Hair loss, scalp lesions, split nails, feet issues, cleft palate, and the list seems to go on.
They, too, did not understand IP. I wonder how their lives might have been different if they only knew.
Learning to Adjust and Advocate
I’m in my sixties now and I have learned that I simply have to adjust to my body’s requirements. If I push it, I end up paying for it. My recovery time from episodes of unwellness does take longer as I age. I have also learned that slowing down does have its own advantages.
If I can give one piece of advice as an older woman with IP, it is simply educate yourself as best you can.
There may be no cure, but there is now so much more information about this disorder. Knowledge and acceptance can go a long way to leading a happier, safer and more productive life.
Empowered by Knowledge and Community
These days I find myself explaining what I know of the disease with my health team. I can’t expect them to know about IP.
I read somewhere that there are 28 new cases, (I wonder about the undiagnosed), of IP diagnosed each year, worldwide! That puts it in perspective for me.
I no longer allow for a practitioner to proceed, without acknowledging and knowing at least the basics of IP.
It feels great to be able to express these reflections with a community that understands and supports people with IP. I am grateful for the research that I am able to access. It has made a difference and has given me a sense of acceptance for this body that I have been given.
Rhonda C. is a guest blogger for the National Foundation for Ectodermal Dysplasias. She lives in Australia.
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