SCLERODERMA: A Rare Autoimmune Disease
I have Scleroderma. It doesn’t have me!
I typically don’t talk much about my disease. I try not to focus on it or allow it to define me. However, since we recently had our annual fundraiser and with Lori’s prompting, I thought it might be a good time to share more about myself and this rare disease with no known cause and no known cure.
THIS IS THE FACE OF SCLERODERMA
It is important to state that the skin on my face isn’t involved. Sadly, some are faced with that challenge!
FAMOUS PEOPLE WITH SCLERODERMA
You might be aware that Bob Saget’s sister, Gay, was diagnosed with Scleroderma at age 44 and passed away three years later. Bob has dedicated his life since to raising money and trying to educate others regarding Scleroderma. He produced a movie about his sister and Scleroderma titled For Hope.
There was an article in People Magazine about Queen Latifah’s Mother who battled and died from Scleroderma. “Owens, 64, was diagnosed with scleroderma, an incurable autoimmune disease that has caused scar tissue build up (pulmonary fibrosis) in her lungs. Owens also has pulmonary hypertension (blood pressure in the lungs), which impacts her ability to breathe.” Owens passed away this past March.
WHAT IS SCLERODERMA?
Going to try and keep this brief, but it’s not so easy since there are various types and it effects each of us differently.
Scleroderma, or systemic sclerosis, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases.
The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin is one of the most visible manifestations of the disease. The disease has been called “progressive systemic sclerosis.” The disease varies from patient-to-patient.
The symptoms of scleroderma vary greatly for each person, and the effects of scleroderma can range from very mild to life threatening. The seriousness will depend on the parts of the body, which are affected, and the extent to which they are affected. A mild case can become more serious if not properly treated.
It’s estimated that about 300,000 Americans have scleroderma. About one third of those people have the systemic form of scleroderma. Since scleroderma presents with symptoms similar to other autoimmune diseases, diagnosis is difficult. There may be many misdiagnosed or undiagnosed cases.
SYSTEMIC SCLERODERMA LIMITED
(This is my diagnosis.)
Systemic scleroderma (systemic sclerosis) The changes occurring in systemic scleroderma may affect the connective tissue in many parts of the body. Systemic scleroderma can involve the skin, esophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart and other internal organs. It can also affect blood vessels, muscles and joints. The tissues of involved organs become hard and fibrous, causing them to function less efficiently. The term systemic sclerosis indicates that “sclerosis” (hardening) may occur in the internal systems of the body. There are two major recognized patterns that the illness can take – diffuse or limited disease.
Limited scleroderma is sometimes called CREST syndrome. CREST stands for the initial letters of five common features (all of which I have):
- Calcinosis
- Raynaud Phenomenon
- Esophageal dysfunction
- Sclerodactyly
- Telangiectasia
80% of patients with Scleroderma are woman!
MY STORY
October 2013 was when I was officially diagnosed. However, I believe that I had Scleroderma several years before. My symptoms at the time included: thickening of my skin on my hands, which I thought was inflammation. Shooting pains through my hands. At times, my hands would get stuck; and I would not be able to move them. That was the scariest! In my mind, I thought I had arthritis. Both my mom and grandma had arthritis. I mentioned my symptoms to my female doctor at a routine check up. I told her I had an upcoming appointment with my primary doctor. In preparation for that appointment, she ordered blood work. What a smart woman! The results of the blood work were off the charts and prompted an appointment with a rheumatologist. Luckily, I did not go misdiagnosed or undiagnosed as many of my “Sclero Sisters” do. (More about my “Sclero Sisters” later.) Upon meeting with my rheumatologist, I described my symptoms. To say I was unprepared for my diagnosis is an understatement. Of course, I could hardly pronounce Scleroderma and had never heard of it. I was told not to search the internet (especially images) regarding it as it would most likely freak me out. That night I was suppose to meet my husband at the gym to workout. I recall telling him to go without me as I was headed for more testing.
How Scleroderma Has Effected My Body
I see my rheumatologist every six months. Annually, I have an Echocardiogram and Pulmonary Function Test to ensure that my heart and lungs aren’t being effected. My skin is thick from my fingers to my elbows and my feet to my knees. I have minor upper GI issues, which are treated with medication. I’m not a fan of medication and really wanted to find an alternative approach. However, my rheumatologist stressed that if I didn’t treat it with medication I could become susceptible to esophageal cancer. No thank you! My hands, feet and nose are effected by Raynaud’s.
Raynaud’s disease is a rare disorder of the blood vessels, usually in the fingers and toes. It causes the blood vessels to narrow when you are cold or feeling stressed. When this happens, blood can’t get to the surface of the skin and the affected areas turn white and blue. When the blood flow returns, the skin turns red and throbs or tingles. In severe cases, loss of blood flow can cause sores or tissue death, which could result in amputation.
If you see me with my hands in my armpits or sitting on my hands, there’s a good chance that I’m trying to warm them up from a Raynaud’s attack. I have gloves in all our cars along with hand warmers especially in the winter. Grocery shopping in the frozen section is really a challenge no matter the season. Typically, my husband puts the cold items in our cart. Raynaud’s is painful. I try to avoid having an attack at all costs. Once I get cold especially bone cold, it can be very difficult to warm up.
If you ever see me rubbing/massaging my hands, it’s because I have either shooting pain or pain in my joints. The sharp shooting pains in both my feet and hands can shock me and take my breathe away. The pain seems to be worse in the evening. Sometimes my hands/fingers feel tight and it’s difficult to bend my fingers. My hands don’t always work very good and I drop things…a lot! This is a source of frustration for me.
My fingers are very thick causing me to no longer be able to wear many of the beautiful rings my husband has gifted me over the years. It might seem shallow but it’s been heart breaking for me. Especially the fact that my original wedding ring doesn’t fit. Often I’m told to take them and get them enlarged. Since I don’t know how thick my skin will get, I don’t really know what size I should enlarge them to and am concerned they still wouldn’t fit. I did finally decide to take my wedding rings in to get them enlarged. The jeweler told me that my finger was three times the size it once was and that the diamonds would be compromised if he enlarged my rings. I couldn’t control my tears. Redesigning my rings or taking them apart was not an option in my mind. I put my rings away for safe keeping. My hands and wrists are much bigger than they once were causing me to get rid of many bracelets. My feet are thicker causing some of my shoes to be too tight. I once had narrow feet.
“SCLERO SISTERS”
My mom and her friends rallied around and wanted to fundraise, support me, etc. I was so touched by their concern and support. Through internet searches we discovered The Scleroderma Foundation. There also happened to be a local support group. My mom and several of her friends attended a support group meeting on my behalf and to investigate the participants and report back to me. They were honest and open when they attended the meeting. I didn’t want to subject myself to “Debbie Downers” and “Poor Me” mentalities. They reported that the woman in the group were wonderful, welcoming, supportive, positive and informative. I went to the next meeting and met my “Sclero Sisters”! It is a small group of women that is amazing! It also feels really great to be able to talk with others who completely and totally understand! Besides those of us pictured here (we plan the fundraiser) there are others in our group.
We are Scleroderma Warriors…Unbreakable!
FUNDRAISING
For the last three years, I have assisted in the planning of our annual fundraiser called Reason to Rock. The funds raised go towards research and patient information days, which I have directly benefited from attending. The event is held at the Northwest Medicine Field where the Kane County Cougar’s baseball team plays. The event includes a walk around the baseball diamond, car show, raffle items, food and drinks, information booth, inflatables and face paining for the kids, a DJ and my husband’s band, Singblade, has played the past three years. Here are pics from this year’s event:
I always get my nails and toes painted teal since it’s the Scleroderma Awareness color.
Each of us put together a team. My team is called “Julie’s Jewels”; and we wear sashes and tiaras…not surprised I’m sure!
SET UP: NEIGHBORS COLE, DEANNA & JACOB
RAFFLE WORKERS – DEANNA & LORI
SUPPORTERS: TIFFANY (STEP-DAUGHTER) & VIOLET (GRANDDAUGHTER ~ MY FAVORITE GIRLIE!)
VIOLET & OZZIE (KANE COUNTY COUGAR’S MASCOT)
SUPPORTER: MARY (TIFFANY’S MIL)
FACEPAINTERS – KARIN & CAROL
SLINGBLADE – DAVE, MIKE, DARIN, PAUL & LEN
FTF
Drove home with tears in my eyes! Tears of gratitude and appreciation for my friends and family who supported me both through donations and giving of their time to volunteer at the fundraiser! Love to all of you 
Because of everyone’s generosity I was the top individual and team fundraiser!
CAUSE AND CURE
As previously stated, there is no known cause and no known cure. This is the greatest source of frustration for me. It’s a wait to see if body systems/organs fail. If they fail or become involved, you see a specialist and treat the symptoms. To my knowledge there is not much we can do proactively to keep the disease from progressing. Therefore, I try to maintain a healthy lifestyle with the hopes that it will benefit me overall.
BE KIND
You might not always know that someone has a disease based on how they look…
Thanks for taking the time to read this post! Hopefully, you now know more about Scleroderma and how it might affect those with this diagnosis.
I HAVE SCLERODERMA. IT DOESN’T HAVE ME!
Prayers and/or donations are always appreciated! Thanks in advance!
TO MAKE A DONATION
FTF: How did you get involved in modeling for the Fashion Show?
