A couple of weeks ago I went in for a routine blood draw at my local Kaiser clinic. I gave the phlebotomist my ID card and told her my name and birthdate: for some reason they like to verify these things, right? Anyway, what happened this time was… she exclaimed that didn’t look my age. (Well, yeah. That may be one of the bigger red flags for scleroderma… no wrinkles.) “It’s just my disease doing that,” I assured her. “I really am that old.”
This lady wanted to know what I had done to get rid of the wrinkles. She wanted this magic wrinkle remover too! I carefully told her about scleroderma. “I never heard of that!” she said. Still thinking that I was a lucky person, she drew my blood and I got out of there. I would be happy to have every single wrinkle that I have earned over the years, but no… I have scleroderma. The systemic form with limited skin involvement. Lucky me.
That’s why there is this thing called World Scleroderma Day. This is a complex autoimmune disease with illusive symptoms (brain fog… trouble swallowing…GERD…swollen fingers…trouble breathing sometimes…fingers that suddenly lose circulation and turn white or blue…) that make the disease difficult to diagnose without specialized testing and evaluation by a scleroderma specialist.
Systemic sclerosis (the form of scleroderma that I have) is thought to start with some event (still unclear) that triggers the formation of antibodies that target specific molecules the nucleus of cells. These antibodies lead to damage in blood vessels and their linings; the damaged blood vessels release chemical signals that trigger a cascade of events that impact other cells of the body. Some cells are related to inflammation. Other cells are tipped over into forming uncontrolled scar tissue (fibrosis). Here is the problem: the tissue engaged in fibrosis is all of the connective tissue. What is connective tissue, you ask? Think of all the ways a person is held together. Tissue that makes your skin stretchy. Tissue that holds your muscle fibers together. Tissue that makes up your tendons, ligaments, and the structure in your joints. Tissue that holds your intestines, heart, kidney, and lungs together. The cartilage in your ribs. All inflamed, under attack, and forming thicker and thicker layers of scar tissue. Some of this damage causes calcium deposition. Unobservable from the outside, the patient is slowly hardening inside. The most obvious symptoms is the skin thickening and then pulling tight over time.
Scleroderma, and especially systemic sclerosis, has the highest fatality rate of all rheumatic diseases. Did I mention that there is no cure? There are treatments for complications that develop like pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD), but they, like the immunosuppressant drugs that many of us take, mostly stabilize and slow progression. (Dear Kaiser lady… that’s why I was getting a blood draw. The drugs that I take are risky, and my doctors need to make sure my kidneys and liver are tolerating the meds okay.)
So, what is the purpose of World Scleroderma Day? It’s to highlight this condition that impacts more people than it should even though it is rare. It’s to raise awareness of the symptoms and hopefully aid in other people getting an appropriate diagnosis. Maybe shining a spotlight on scleroderma will help in getting funding for research, treatments, and even a cure.
Here is where you can learn more about scleroderma, it’s symptoms, and treatment.
- Forms of scleroderma
- Symptoms of scleroderma
- More information from the Cleveland Clinic
- More Information from Yale
PS How was my blood work? Yay, doing okay!! Kidney function is up into normal ranges again!!! How old am I? I was born in 1951. 🙂
Yarn, Books & Roses 
I’m pretty sure this is my third reading of your announcement of WSD, Marilyn: we’ve been communicating for a fair while, then. 🙂
(I was born in 1943, so put that in your pipe and smoke it !) [grin]
And, most interestingly, back in those “early” days, you were dead against showing yourself to your followers: nary a glimpse of you did we ever get. Shots of you doing anything – such as carrying Hannah – were cropped at the shoulders !! Now, delightfully, you have abandoned that and we all know your face – a good, intelligent one … but a suffering one.
Oh, how I wish for a magic wand !
I’ve been camera shy all of my life, but I decided to go for it this time because… I was talking about fewer wrinkles at my age than I should. I used to have crow’s feet, and they are now gone. Crazy! Also, you can’t see it, but half of my eyebrows are gone because the skin s too thick…
And yet, I am a lucky, lucky person. I have responded well to the medical interventions, and I tolerate my drugs well. Not every scleroderma patient can say that.
And we’re tickled pink that you can say that, me darlin old thing: but that you omit the extremely salient fact of your intelligence and teaching qualifications enabling you to study your disease/s and understand what’s going down. I can’t think but that must be quite marvellously helpful. Of course, it’s not so great when you’re in a bad patch, and might know how long it’s likely to last …
But overall, having a really functioning brain simply has to be A Good Thing. I refuse to think otherwise. 🙂
It always helps to have good computing capacity, but at the end of the day, I think that emotional intelligence is the deal breaker when dealing with a rolling series of body blows like this crazy disease (not to mention all of the friends that it invites onto the Little Teal School Bus!)
Emotional intelligence … yes ! Makes absolute sense, Marilyn. Even more challenging. 😐
I can appreciate the issues of “invisible” diseases. You do look at least 20 years younger but agree would trade the winkles to not have the disease. FYI – I was so focused on the lack of wrinkles, I didn’t notice the oxygen line until I read your caption.
When I started wearing oxygen, I had to make a mental adjustment because… no longer invisible! Hey, if you have to use a cane and wear oxygen, rock the look!! That helps me feel young too, but I still would trade all of this for the wrinkles…
Hi, Lady! Nice to make your (photo) acquaintance.
Complex disease indeed. Kudos to modern medicine for figuring out all those disparate things belonged in the same bucket. I’m so glad you found the doctors you have now. Now, to find a cure …
A sunflower. What a perfect symbol. Always turning its face to the sun. There’s a lesson in that for all of us.
Way to go on the lab results. Keep up the good work!
It really is a crazy complex disease. The problem in my lungs is… the inner lining of my blood vessels is growing, and that increases the blood pressure as the opening narrows, and in some cases the lung tissue has died because the blood vessel was completely blocked. I went to a conference session on PAH and the presenter said the cells are showing some of the hallmarks of cancer. The drug that I take directly targets those cells and so far I am doing really, really well. I just got my latest echocardiogram report, and pressures are normal!! Yay, modern medicine.
Yay, indeed!!
Of, forgot to add… the sunflower symbol is about always turning towards the sun. It is a metaphor for turning towards strength and resilience. I love the sunflower symbol much more than butterflies!
thanks for explaining this, I knew a little but not much about this disease. I’m glad that you found a doctor that understands this and is treating you for it, rather than trying to have to figure it out
I have a great, integrated team of 4 doctors who work together to create my treatment plan. I feel so lucky them!
you are certainly lucky for that
My mom has this. God bless. 🙏
Oh, I am so sorry to hear about your mom. Hugs to both of you!
Thank you so much for sharing the information, and hopefully more awareness will lead to progress in fighting and one day curing this disease!