Morphea Scleroderma and What Women Really Need to Know. It Can Happen to You!
What is Morphea Scleroderma?
Until July 1, 2011, I had absolutely no clue what Morphea Scleroderma was, how it would affect my life, or what it meant to me as a woman and single mother. In the months since July, that has changed, and it changed rapidly. This is my story, and I would like to share it with you in the hopes that my battle with this disease can help other women understand, find a little peace, and possibly even cope with this disease that seems to target mainly women.
Morphea Scleroderma: An Auto-Immune disease affecting mostly women. In the United States there are approximately 300,000 known cases, of which approximately 70 percent are women ages 30-60. Some believe it targets mainly women of African-American or Native-American descent. Others believe that a person with a family history of Auto-Immune Diseases such as Type IDiabetes and Lupus could be more likely to contract this form of Scleroderma.
Alright, so now we know what it is, but does that really tell us the story? I'm afraid not. Hang on to your seats ladies, because here comes the information you'll really want to know. In lay-terms for people like you and I, this auto-immune disease causes our immune system to "short-circuit". This means that for one reason or another, our immune system starts attacking our body. In this case, it starts attacking our soft tissue (skin, joints, ligaments, muscle, tendons). First symptoms may include fatigue, dizziness, and bruise-like lesions on any part of the body from the neck down. Most will run fever regularly, and are most often miss-diagnosed as having some sort of infection initially. Swelling also occurs over almost the entire body, more so on the affected lesion areas.
Once the initial onset has started, pockets of collagen start to form under the skin. These appear white and can be anywhere in the bruise-like lesions which will range in color from dark purple to yellow-ish in nature when they are not in an active phase. When active, the skin will start to harden and become leathery with dry patches and flaking skin. If the feet are affected, the nails could peel, pop off, and the bottoms of the feet become dry, calloused, and flaky as well. As the skin and joints harden and become stiff, they are also swollen and painful. Many patients experience a constant itch with new outbreaks or what the doctors like to call a new active phase. These will be marked by fever, swelling, and red heated spots where the new bruise-like lesions and collagen deposits will form. The itch is terrible, and the thing that I can most closely relate it to is having the most horrible case of Poison Ivy a person could ever have where it occurs.
Okay, if you're still with me and haven't been scared off by this description, it could be that you have experienced some of these symptoms yourself and are curious to know more. Maybe you've already been diagnosed, but are struggling with understanding what this diagnosis will mean for you. If you have some or all of these signs, my best advice to you is DON'T GIVE UP! We know our bodies ladies. We know what feels normal and when something is wrong. This disease can be over-looked as so many different things. From my first signs of the disease that I know of in 2010, to my diagnosis on July 1, 2011,it happened to me. I went through seven different physicians, countless medications, and many a diagnosis. Be persistent. It's your health, and you are the only one who can take charge of it!
What It Looks Like
How Am I Diagnosed?
By now you may have been to your primary care physician several times with an array of medications and/or more than one diagnosis of what is going on with your body. Don't loose heart! Be persistent in your quest to find out about your body. The first step in being diagnosed is to realize that there is something different about your body and listen to what it is telling you. Then, it is up to you to take action. Don't settle or sell yourself short. Pursue answers with your doctor until you are satisfied as best as you possibly can be.
What tests should I insist on having?
1. The first step in finding out if you do have an auto-immune related condition is to have a complete blood work up. Make your doctor pay special attention to your white blood cell count and your Sed Rate. If these are increased, chances are that you have some sort of inflammatory auto-immune process at work. Don't stop there!
2. If your blood work has tested positive for an auto-immune inflammatory process, and you are starting to show signs of the bruise-like lesions, insist on a biopsy. The sooner you have this done, the better. If you are referred to either a Dermatologist or a Rheumatologist, they will ask for this. In fact, it's one of the first questions that they will ask you! This will rule out any other possibilities and tell you whether you in fact have Morphea Scleroderma or Systemic Scleroderma.
3. If you are having trouble breathing because of the lesions, or start getting short of breath, and/or have chest pain, ask for a pulmonary functioning test and/or a EKG to check your heart. Morphea Scleroderma can affect both your heart and your lungs, whether it is from the disease or from effects of the disease such as the hardening of the skin and the pressure it is causing on your internal organs.
4. If your pulmonary functioning test shows decreased lung capacity, ask for a chest CT. This is to rule out any internal involvement of the disease and to evaluate if it is in fact caused by the hardening skin.
5. Ask for an evaluation of your mobility! If the tightness in your joints and the pressure from the lesions are causing you extreme stiffness and pain, you could be in need of Physical and/or Occupational Therapy.
Other Auto-Immune Diseases
Diabetes Mellitus, Type I
How My Journey with Morphea Scleroderma Began
In the summer of 2010, I had spent the afternoon in the pool with my then five-year-old son. When I came in to shower, I noticed that I had what appeared to be a sunspot on my right breast. The spot was about the size of a quarter, not very noticeable, and didn't cause me any pain, so I didn't give it much thought at the time. I showered, cuddled up with my son, and life went on.
In November of that year, I started having pain in my left side. After showering one day I stood in front of the mirror in my bathroom and noticed a small bruised spot on my left side. No bigger than a golf ball at the time, I figured I'd bumped in to something, or my active son had got me there while playing. So, I dressed, went to bed, and again life went on.
In December I was still having a small ache in my side and the bruise had not gone away. I was also starting to feel tired a lot of the time, which as most of you mom's know isn't too convenient when you're trying to keep up with an active child. This is probably more so for those of us who are single mothers who have our kids 24/7. There just aren't enough hours in the day to be tired. There is always something needing our attention, and we tend to give our own needs a back seat, as any good parent does. It's the holidays, and things are hectic and your little one is over-flowing with holiday cheer and the next thing we know, we're infected with it too.
Things rocked on that way for a while. I put the bruise-like spots in the back of my mind. This, I will tell you ladies, was my first mistake. Make the time! If I can impart nothing more on you than that, make the time to check in to what is going on with your bodies. Don't wait! Time is precious, a lesson which I waited a little too late to learn. I understand that it's hard. You have getting the kids off to school, working a job, taking care of a household, your kids are involved in sports. You have a life in other words.
In February of 2011, I couldn't ignore the fact that something was wrong any longer. The pain had gotten to me. I had to call my mother and get her to take me to the Emergency Room. The golf-ball sized bruise was now covering most of my left side, and I could feel that it was getting hard and rigid. In the Emergency Room I was examined by first the nurses and then the doctor. I was running a fever, and it seemed as though I had been running one off and on for months. I was told that I probably had some sort of inflammation and/or infection, and sent home with my first course of antibiotics. I also had an appointment the next day for a sonogram just to check and make sure that there wasn't something more serious at work. The sonogram showed that I did, in fact, have some inflamed tissue in the fatty layer of my skin. I was told to finish the course of antibiotics and come back for a follow-up visit if things persisted.
I finished my ten day course of antibiotics, and life went back to normal for a little while. The bruising didn't go away, but the pain was better, so I couldn't really complain. Life had me busy again. My son was playing soccer, and I was co-chair of the Relay for Life of my county. Work was as hectic as ever too, but in April I started to notice that my right hand was a little stiff and swollen. My pinkie finger and right ring finger were also stiff and the joints hurt, and my entire hand took on a sort of blue cast. Being a medical biller, this wasn't too convienant. I used my hands to earn my living, so something had to be done. The tiredness reared it's ugly head again too, and I was having trouble focusing sometimes on things. I'd drive down the road and miss my turn because I forgot where I was going. I wasn't sleeping much either. My hands would wake me up at night hurting and swollen. They'd be stiff and aching, and I was afraid.
I went to see my primary care physician. Something had to give. I couldn't bear the pain. He examined me and thought that I appeared to have some sort of infection in my hand. He could tell that the joints were swollen and inflamed on my two fingers, and thought that I could possibly have what he called "trigger finger". I received another ten day course of antibiotics and was told to exercise my hand to keep it from getting more stiff. I took the antibiotics, and the pain did seem to be a little less, but my hand was still stiff, I was still running fever almost every day, and I was still very tired, I thought from the lack of sleep. I went back to see my PCP again, and he thought that perhaps the antibiotics weren't strong enough the first time, and that maybe I should be on a course of steroids to help. Another ten day supply was ordered, and I took those.
After I finished my supply of antibiotics and steroids, the big day was upon me. The actual Relay for Life benefit. We were going to relay all night long! I couldn't wait! This event was important to me since I have had so many of my family members pass away from Cancer. I lost my best friend, father, and mentor in 2005 to his battle with Cancer, just five months before my son was born. I had to Relay, so that hopefully one day no other daughter would have to say goodbye to her father because of a horrible disease like Cancer. It was the day after this event, when I went to pick up my son from my mother's house, that I was sitting on her couch. My arm was hurting from my shoulder all the way down to my fingertips. It had been doing that all night, worse as the hours got later. I unfolded my arm, and I saw tiny purple/blue bruises no bigger than the eraser on a pencil dotted all down the underside of my arm. I got SCARED. I'd had enough of my family members die from Cancer to know what this might mean for me. Here it was, what I'd most dreaded, staring me in the face, and I couldn't ignore it anymore.
Monday morning I made an appointment and saw another doctor in the clinic. I laid it all out on the table. My hand, my arm, my fingers, and even my left side. He was perplexed. We did an x-ray of my hand and a CT scan of my side and abdomen, because the bruising had spread to my stomach. By this point I was terrified. I was still in pain, had no idea what was wrong with me, and answers didn't really look like they were coming. I was exhausted, but I didn't have a choice. I had to go on.
The CT of the abdomen showed that I had some blood on the lining of my abdomen wall, which would explain the bruising. I was told that it had to be some sort of infection. My white count was elevated, I was running fever, and had all signs that pointed to an infection. I received another round of antibiotics, another course of steroids, and a follow-up appointment. I finished the antibiotics and steroids, and was in more pain than ever. My right arm was now bruised, knotted, and swollen. I ran fever daily, and had started to bruise, knot, and swell down the back of my right leg. My feet were swelling daily, as were both of my legs, and at times they looked like they were going to burst. The fevers were almost constant, and I had started to itch incessantly. I couldn't take it, I was driven to tears.
Luckily, it was at the same time that my saving grace came to work in the clinic I went to. An Internal Medicine doctor, she was everything that I could have asked for and more. I will never be able to sing her praises enough, or to thank her for what she has done for me. If God has Angels on Earth, then surely she is mine. With one examination she could tell that I definitely had something more than an infection going on with me. She wasn't sure what, but I had finally found someone that wasn't going to give up until we knew. Finally I was given something for the terrible pain that had wracked my body for months, and was ordered a complete work-up of labs. I must have had every single blood test known to man short of DNA and every possible urinalysis they could find, but she was leaving no stone unturned.
At this point, there was no question, I couldn't work. My right leg had a hard lump on it the size of a cereal bowl and it hurt to sit on it. My stomach was also dented, knotted, swollen, leathery, and tight. I was miserable. The doctor ordered me off work, and demanded that I try to get some rest, because at that point I was going two to three days without sleep because of the pain and discomfort. I couldn't stay alone either because I was getting dizzy, my speech was slurred, and my balance was terrible. I had started getting bruises and knots on the bottoms of my feet and toes, and it was making it very difficult to walk.
At this point its the middle of June and my lab work had just come back. I'm still off of work, still in pain, itching, and miserable. Its hard to cut my food, hard to dress myself, and none of my clothes fit the way they used to. My stomach has started to sink in on the left side, and I now have bruises and knotting on the tops of both feet and going down the front of my left leg. The lab work shows that I definitely have some sort of auto-immune issue, but what? There are literally hundreds, if not more, of auto-immune diseases. This was news to me. I had absolutely no clue!
I'm ordered to continue to stay off of work until further notice and get as much rest as I can. The search begins for a specialist for me to see, someone who deals with auto-immune issues and will know what the next step is for me. Most of the doctors that we contact have a year long waiting list to even get an appointment. You see, I have found out that the ratio of patients with Morphea Scleroderma to doctors is about 300 to 1. My doctor is a candidate for saint hood by this time, because she has found a doctor in Dallas that is willing to see me a week later. She tells him that if I have to wait until next summer to see him, I might not make it. Sobering words for a 32-year-old single mom to hear.
I go to the physician in Dallas, and he gives me a very thorough examination. I will say, that is the only examination that I received while in his care. After the examination and consultation, and reviewing my medical record up to this point, he agrees that there is definitely some sort of auto-immune issue, but isn't exactly sure what it is. He decides that I should see a dermatologist that works in the same facility and have a biopsy of the affected tissue to be sure. With luck, and since I live three hours away, he can see me that afternoon before close of business if I hurry. Now ladies, if you want a comical picture imagine this. Here I am "hurrying" to this man's office which is one very long walk away to another building. I have on a t-shirt, shorts, and flip-flops because at this point I can't stand for much to be touching my skin and my feet are so swollen that my normally size eight foot is now in a size ten flip-flop!
In the dermatologist's office I win the lottery. You guessed it, not one biopsy but two are performed! Here they do what is called a plug biopsy or punch biopsy. Yes, that really is as pleasant as it sounds. For the second time that day, I'm asked to take my clothes off and get in to one of those nice crinkly paper gowns that do absolutely nothing for warmth, and very little for modesty. I'm poked around on until the nice little old doctor, who is as sweet as they come finds two spots that aren't absolutely too hard to biopsy. He takes what looks like a pen with a metal cylinder on the end of it and "punches" it in to my skin after it is deadened and twists it while pulling, effectively removing a "plug" of skin. This is done first to my left side. The wound is cauterized with a laser and dressed with gauze and tape. Next is the back of my right thigh, and the process is repeated. I'm given two vials of a creamy white lotion, and told that the wounds need to be cleaned with alcohol and dressed every three hours, with the lotion applied to it so we can avoid infection. After three days, I should leave the bandages off for a few hours every day to let air get to the wounds.
This procedure was performed on Friday and the tissue was expressed out of the office that same day straight to a lab for processing. At this point I endure the long, uncomfortable ride home in a haze of pain. I feel every bump on the over 200 mile trip, and eventually fall in to a light sleep not far from the edge of the town we live in. I'm scurried away to my mother's house, not being able to handle the stairs at my own, and settle in to wait for the next chapter to begin, and it does. The dermatologist calls me himself at 1:00 PM on July 1, 2011 and says two words that have forever changed my life. Morphea Scleroderma.
The next chapter of my life had begun. A chapter with this thing I didn't know or understand. I was afraid, confused, and in all honesty naïve about what the new normal for my life would become. In the three months since my diagnosis I have learned a lot. By now stretch of the imagination do I know everything about this disease or the long-term effects it will have on my life. I do know that I will never be the same. Life will never be the same as it once was for me, but maybe that is my destiny.
If you or someone you know has been affected by Morphea Scleroderma and would like to talk, I would be more than happy to. I hope that you have found something helpful in my introduction to me and my disease. Since my initial diagnosis there have been many more twists and turns, of which I hope to share with you all here. Please know that you aren't alone out there in this world. May God bless you on your journey.
The journey to healing begins with friendship and love.
- Heidi Jacobe, MD - Faculty Profile - UT Southwestern
Dr. Heidi Jacobe is the leading Morphea Scleroderma physician in North-Central Texas. She is currently involved in building a DNA Repository for the study of the disease.
- Scleroderma Foundation - Home Page
The Scleroderma Foundation is a national nonprofit health organization dedicated to a three-fold mission of Support, Education, and Research to help fight this challenging autoimmune disease.
- MorpheaMom on Facebook
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- MorpheaMom at Inspire.Com
Inspire connects patients, families, friends, caregivers and health professionals for health and wellness support. Connect with others who know what you're going through.