Scleroderma patients mostly died from renal failure until the late 1970’s when they started treating the kidneys which kept patients alive long enough for them to die from lung issues caused by Scleroderma then the medical field started treating lungs, heart, esophagus and other symptoms. It can take a few years to get diagnosed with Scleroderma depending on where a patient lives. Scleroderma patients have gastrointestinal tract issues, esophagus swallowing issues, acid reflux and can lead to serious damage. Scleroderma causes PAH (pulmonary arterial hypertension) in the lungs and/or Pulmonary Fibrosis (incurable) the only solution is a lung transplant. Most patients have hiatal hernia also causing heartburn and are advised not to lay flat when sleeping (raise head of bed) because the acid reflux can splay over into the lungs causing more damage and making it harder for the heart to work properly so it is not uncommon for Scleroderma patients to have serious heart issues also. Scleroderma changes a patient’s quality of life as you can imagine just dealing with one of these internal aspects of this disease. No wonder the medical field doesn’t like to take the disease on!
Scleroderma – A Male Perspective
My initial questions and apprehension centered on the circumstances of what was happening to my hands in the summer of 2002. My fingers were turning white, numb and I was developing painful tender open sores on my fingers tips. My job with the United States Postal Service starting in 1998, involved manually handling mail pieces; often thousands of letters during an 8-10 hour work shift, aligning the edges on a vibrating belt for automation machine processing. My hands and fingers were routinely exposed to the jogger belt; but none of this work exposure occurred to me at the time, as a being a cause of the white blanched fingers. The USPS building interior was warm, so coldness and temperature was not an issue, or perceived as causing the numbness. Often the white fingers felt dead and without feeling; though they would eventually return to a bright pink or red color. Accompanying the return of color was usually a deep, throbbing, aching, and excruciating pain that can only be likened to the sensation and pain of shutting a car door on ones hand or foot. An extremely agonizing and painful experience! I first visited a general family practitioner who was unsure of the conditions’causing me problems, he referred a rheumatologist, and after an initial examination and some blood work the words Raynaud Phenomenon, Scleroderma and Systemic Sclerosis entered my vocabulary and my life. Scleroderma? What the hell is scleroderma? And why am I experiencing some first time sexual problems and concerns? This evaluation and assessment of the diagnosis and the ensuing prognosis appeared almost as a virtual death sentence. My prospects toward a potentially long life were clouded by severe doubt and living was fading away. The future looked bleak. As a healthy, viral, sexually active, 48 year old husband and father; why was I experiencing these “new” challenges and what the hell was going on with my hands and these sores on my fingers? The rheumatologist told me scleroderma was a somewhat rare autoimmune arthritic condition, resulting in a hardening of the skin and the development of excess collagen. Okay, now I know a little something about this scleroderma disease or condition; does this have anything to with my sexual problems? What do you mean you don’t know? You’re the doctor, you must know? You’re a medical professional, you are trained to help patients, answer questions and save lives. Again, why don’t you know what is wrong with me and what do you mean you don’t think the 1m has anything to do with scleroderma? What do you mean it is organic’! Here is a prescription for Viagra and see me in a week. A week later we switched to cialis followed by Levitra and even some Revatio. None of this Sildenafil citrate seemed to have any benefits with the ED problem« Raynaud’s were improving with the Sildenafil acting as a vasodilator in the problems with my fingers. During those past several weeks I had learned much more the disease of scleroderma and some of the possible concerns und circumstances to be aware of. First and foremost I learned scleroderma affects women much more frequently than men, perhaps a ratio of 80% females as compared to 20% for males.
Not that I wish to appear unsympathetic or sexist to women’s concerns on health issues but from a male’s point of view – this really sucks! I certainly feel compassion and concern for all those that must endure the challenges of scleroderma but my selfish side was in charge of my feelings back then. Please accept my sincere apologies if this angry ranting offends you.
Wonderful, great, I have “some women’s” disease – what the hell? I have some chronic female disease; and my rheumatologist doesn’t seem very knowledgeable about this condition and every thing I read and see on the internet scares the hell out of me. And ED too! Yes, as I learned scleroderma in men can cause problems with ED due to blood restrictions to arteries and capillaries along wlth the development of excess collagen. Now I am truly angry and mad and ask silently and aloud “why me”? “Why me?” Scleroderma castrated me! I can handle tightening skin, bent fingers and the gastro esophageal reflux challenges but not the ED. I feel victimized! It is bad enough to have this terrible women’s condition but this “thing” has robbed me of my manliness too! How can this be? As a male, scleroderma stripped me of my dignity and it was difficult to still feel adequate as a man and as a lover! What did I do to cause this? How could I have prevented this? What can I do to make it go away? How can I cure this condition? And how can I regain my manhood? The essence of masculinity and well being had been torn from my life and replaced with this effeminate inequality.
Will friends and family understand that I share this disease with women? Will others stare or laugh? I tried denial but reality always resurfaced. Is this scleroderma thing like getting a feminine diagnosis for breast cancer? I am embarrassed now to be a guy sometimes. I feel hollow and no longer a man. I mourn my lost virility! A guy shouldn’t get this and you sure don’t want to admit to having a female condition, especially some condition that causes erectile dysfunction. This “girlie disease is not some condition a man can proudly display on his sleeve like some well earned war wound or badge of honor; to share with the world of men. I cannot just proclaim “see I survived this terrible thing”. No, give me some manly affliction like prostate cancer that my fellow males equally fear and can relate to. (Some years later I was diagnosed with prostate cancer, which also was difficult to cope with too, but there seemed to be a cure and the ability to rid the cancer from my body.
Surgery can not remove my scleroderma. Scleroderma is not something I am proud to say I have. Quietly, I grieve for my lost quality of life, as I yearn to be a true man again. My male ego was severely wounded; it has taken years to attempt to balance my feelings and emotions. Some day’s scleroderma gets me down but now a days I try to remain upbeat and positive for the future.
Yes, my anger caused me some depression about my life situation and I still become disheartened and withdrawn as the reality of scleroderma ebbs and flows within me. My anger has often turned more to embarrassment, as I have come to accept my women’s disease. It is difficult to be proud or pleased with scleroderma …
At this stage of my life with scleroderma, I hope to maybe be able to support other men who also have been diagnosed and distressed with this terrible disease. Maybe I would Ican share one man’s male perspective, if it helps other’s to better cope.
For all people dealing with the challenges of scleroderma, it can be a difficult path and all of us with this condition have shared many of the same ups and downs of this journey. But for men the heartbreak of this journey and the conditions encountered
along the road can create poignant and profound self doubts that are deep and difficult to grasp in terms of a man suffering with scleroderma. At this point in my journey, I gain strength, courage, empathy and fellowship from local support group members here in Central Oregon. But from a lone male perspective, I endure a quiet desperation as a man succumbing to the absence of true understanding and compassion.
It is truly lonely being a man with scleroderma.
In the summer of 2007, I developed a rash on the left side of the back of my head and called my GP for an appointment but he had left his practise to be a professor at Emory University. My boss suggested another GP who was able to see me within a couple hours on the day I made the appointment. He said I had shingles, darn I was not happy since I had a bad case of chicken pox at age 26 and now this, it passed and nothing to it. He tested me for different stuff and I had pillory bacteria so he put me on medication but also wanted me to go on cholesterol medicine. When I asked for the numbers, he wouldn’t respond and my trust started fading. That winter I felt a horrible pain in my jaw that stayed with me for a long time and began my ride through this murkiness. Eventually my voice changed, my feet and hands became so puffy, I had to stop wearing my wedding rings and other rings. My new GP sent me to an oral surgeon who said nothing was wrong but gave me pain medication and told me to try not to chew or open my mouth for about 6 weeks and if it didn’t help to come back. It didn’t go away but my dentist offered to refer me to another very good oral surgeon and the doctor said my voice changes were due to reflux and the swelling in my hands and feet were caused by my age so therefor I probably had arthritis. He said “you’ll be fine, no issues”. Well, I quit going to him and started asking my gynecologist for referrals. I wasn’t feeling like myself, I always had a sunny disposition but I lost my puppy and was having a hard time dealing with my loss. The doctor offered to put me on hormones but decided to refer me to a new doctor who was not taking new patients but his receptionist and I share the same zodiac sign and squeezed me in for an appointment. This doctor said you are so healthy and everything looked great but I still don’t feel well. He decided to do an EKG after I informed him that every so often, I felt chest pains and my heart beat funny. After the test the doctor said he didn’t like the test because two of the lines were a bit funky and it kind of denoted that I might of had a heart attack, probably not but just in case, he wanted me to have a stress test. So I made the appointment to have a stress test but I think from then on my life was never the same. The stress test showed no signs of heart attacks but in the middle of it I had a Raynaud’s attack (although up until then I didn’t know it’s name), always thought no big deal, it will go away and it’ll be fine. My hands turned black and Chuck (my husband) and the technician both freaked out a bit but I tried to assure them that give it a minute and my hands would return to normal. At my next doctor’s appointment we discussed hormone treatment again because we both still thought my voice changes and swelling hands and feet was a hormonal issue. I had been researching my problems but silly me I wasn’t thinking it was as serious as it actually was so the doctor referred me to a vascular surgeon (who turned out to be a jerk but I thank the jerk because of his lack of care, I ended up being taken care of). He tested my fingers with little blood pressure cuffs to see how they responded to heat and cold. The tech doing the test was so scared watching my fingers that she wanted to stop the testing but I told her to continue because I didn’t want to come back another day. She asked me if I had Lupus, I had heard of it but didn’t really know what it entailed. Went back for the results but guess the surgeon was too busy to see little old me, he sent his assistant to tell me they couldn’t do anything for me but if my fingers ever stayed black for a day or so, then come back and he would help, also take a baby aspirin everyday in case I had loose little clots running about my circulatory system. Went back to my other GP told him what they said and he tried to assure me that the jerk was very good and he still trusted his judgement but would be willing to send me to another specialist that might be able to help me. Went to my first Rheumatologist on June 25, 2010 with Chuck and was diagnosed one hour after hearing my symptoms and issues and answering his questions. He stood by his desk, propped his left foot on his desk’s chair, put his right hand on his waist and with his left hand pointed upward and said “I believe you have Scleroderma” the Crest version. Chuck and I replied, we weren’t familiar with this disease so he gave us a couple of sites on the internet for researching Scleroderma. Then he told us, there is no cure and it would advance until it would kill me. The story continues to grow, since that day this Rheumatologist has been fired, when he told me he had no time to discuss my many issues, that I needed to pick 2 or 3 issues and make sure they were the important ones. Since, I still haven’t attended medical school to choose which issues are important or not, we decided to find us a Rheumatologist that can help us a bit through this labyrinth. I think the best part of this is that I’ve gotten to meet a bunch of the coolest people, I don’t know if it’s the attitude of “what do I have to lose?” that makes us more open to other Sclerodermias or what, but its been a better ride because of y’all. Written by Carolina Bonelli in GA
11 years ago woke up and felt as though I’d been hit in the nose. I had 2 root Canal’s done and thought it might have something to do with that so I went to see the dentist, it might be Trigeminal Neuralgia but that was ruled out later. I had numbness on both sides of my face . it wasn’t Bells Palsy he sent me to an ENT DR. They found nothing off, saw a Neurologist, she’s the one that noticed my Raynaud’s . She did an EMG. I had got down to 129 lbs and they put me in the hospital . 9 days there before they came back with a diagnosis of MCTD. i had no insurance so they kicked me out. After that the Sclero hit me. That was in 2008 and That’s the year I flied for SSI which took 19 months to get. It’s rare for men to get this, one in 10,000. I have 7 autoimmune diseases and take drugs to control it. Medicaid pays for most of the drugs, I’m blessed with family and friends or I wouldn’t have made it , I have friends that are worse than me.
Michael Nesbitt’s story
My encounter with Scleroderma
In December 2005 the pain in my feet while lying down in the night started. Gradually it became so worse that I used to cry due to pain every night. I had to tighten my feet with the ropes to stop the pain. Then I decided to go a GP, who diagnosed me with high Uric Acid in the blood and started the treatment. He advised me to stop taking high protein diets, like pulses and dairy products. Neither this nor the medicines helped me at all. The pain continued to make me cry every night. After 18 months of no improvement in the pain, my GP advised me to go to a rheumatologist, which I ignored for another 18 months and stopped all the treatment. I started taking pain killers very often, whenever the pain was unbearable.
I had another problem. Besides acute pain, fingers in my hands used to turn blue, white and red in cold weather. I could not touch cold water as it caused sharp pain in my hands. In September 2008, after much suffering for 3 years I finally decided to meet the rheumatologist in the same hospital where my GP used to practice. After hearing my symptoms he straight away diagnosed me with scleroderma and Reynaud’s phenomena. To be doubly sure he suggested a few blood tests which came positive and my treatment started.
After a year or so I started having dry cough while lying down, which was diagnosed as Interstitial Lung Disease (ILD). I had to consult a pulmonologist (in consultation with my rheumatologist) who suggested PFT to be done annually and to take 2 puffs of inhaler twice a day. My voice was becoming hoarse and I had to clear my throat time to time. I went to the ENT specialist who diagnosed it as Laryngopharyngeal Reflux Disease (LPRD) and the medicines for acid reflux also started. I want to add here that I have had open heart surgery for Mitral Valve Replacement (MVR) done in April 2001. Also I was diagnosed with Bipolar Mood Disorder in August 2012.
It has been now 4.5 years, since my treatment started. I am in contact with 5 specialist doctors. I have to take regular medicines for Scleroderma, ILD, LPRD, heart and bipolar. Regular tests (monthly, quarterly, annually) are done to keep an eye on the improvement of the conditions and side effects of the medicines.
Scleroderma seems to have been in control. Hopefully, the progression of the disease has stopped. The medicines are minimum (I insist every time I go to the doctor to keep the medicines on the minimum, never the less, I have to take 12 tablets daily plus 3 tea spoon of syrup and 2 puffs of inhaler twice a day). I am a fighter. Though the opponent is much stronger than me and the weapons I have, are not good enough to kill him, I will never leave the battle. I have the full support of my family, friends and a team of doctors in the fight against this dreadful disease. I am sure that some day we will find a cure.
Written by a female Scleroderma Warrior
In 2002 both my daughters got pregnant,, worse things can happen I know, then one got anti natal depression, I know that normally happens after the birth not while you are pregnant ,, the other one discovered there was something wrong with the baby, I went to every appointment with them both,, I was worn out by the time the babies arrived,, but it wasn’t over, my daughter got over her depression when the baby arrived, the other one well her problems were just starting,, we knew the baby had problems, we had looked into what she had, we had one our research, but the baby wasn’t feeding properly so my girl took her to the hospital, I was there with her sister in labor, I went to see her on the children’s ward, were the doctors discovered a lump on the babies head, it was there when she was born, but maternity hadn’t noted it down, and they wouldn’t say they had seen it, so my poor daughter was accused of child abuse, social services stepped in, they took our new baby away from us, put her in care, we were devastated,, they put her in care, then my sister was allowed to look after her,, my sister grew attached to her, all hell let loose, she was trying her hardest to keep our baby,, my daughter was rebelling against her and the authorities, she ended up having to live in a mother and baby unit miles away from us, but she had her baby, it took 12 months a whole year to get it sorted out, we won on evidence we gave to them, it just took them forever to get it sorted properly, she sued them for it,,, but once everything was sorted out I started to notice changes, pains too and even strange lumps,, my hands were changing color = Raynaud’s, lumps on my elbow = calcium, pain in my shoulder also = calcium, by the time I got to the right doctor (Rheumatologist) it was 2006 and he diagnosed me straight away, yay I thought now I know what I have, not realizing I had I a life threatening disease, but I truly believe my Scleroderma was brought on by stress, it was dormant for years then bam, one problem after another and Scleroderma reared it’s ugly head, I had noticed other things in the past, like calcium deposits in an awkward place, at the time I didn’t know what they were and to embarrassed to mention it to the Doctor but you live and learn, so 7 yrs on it has gradually gotten worse, I really do consider myself better off than others, I am lucky well luckier than some.
Liz Ledbetter from the U.K.
What we plan with thoughts and actions for our future so we can enjoy retirement/older age, right? Life can change drastically when a rare, incurable disease such as Systemic Scleroderma attacks our bodies without warning! My life started changing in Fall 07 in my hands, progressing fast to include weak/painful muscles, swelling, loss of range of motion and depression until 3/30/09 diagnosed with Systemic Diffuse Scleroderma which no one has ever heard of until diagnosed and adding insult to injury, neither have most doctors, leaving patients to fend for themselves on how to find treatment for symptoms because Scleroderma cannot be treated as yet. Most Scleroderma patients look normal in appearance but the damage is extensive on the inside of our bodies, stage 4 kidney disease, hiatal hernia, pulmonary fibrosis, aneurysm in heart, GI issues, skin changes and Raynaud’s in hands & feet are just a few of my Scleroderma challenges. I am one of the more functionable patients and grateful I have doctors who care and support from Sweetheart, family and friends. Some patients don’t have either doctors nor support and rely on facebook Scleroderma chat groups for advice, love and support from people who relate and understand what they are experiencing. My life has changed dramatically from 5 1/2 yrs ago and I am grateful for my Scleroderma journey because the more I reach out to others the more rewarding my life has become. I am a patient advocate helping to educate and promote public and medical awareness of the desperate need to recognise Scleroderma as the life threatening disease it is. Scleroderma was first documented 260 yrs. ago, now we want awareness to help alleviate the horrible suffering this disease causes physically, mentally and emotionally. Please help. http://www.sclerodermaangel.org
The long wait
Ann Havelock spent about a year and a half meeting with physicians about her symptoms before she was given a diagnosis.
It took more than a year — and nearly half a dozen doctors — to figure out why Ann Havelock’s hands hurt.
Her pain began in fall 2007, when the then 67-year-old was helping her husband put a storm door on their Crooked River Ranch home. It wasn’t cold out, she said, but her hands felt frigid. She brushed off the episode.
Soon, however, her hands started to ache so badly she couldn’t ignore the pain. As a hair stylist in Bend, she worked all day, then came home and wrapped her hands in a hot pad. They hurt constantly.
“It got to the point where I hated to go anywhere, except go to work,” Havelock said. “I didn’t want to go to church. It was just miserable driving, doing anything other than just crawling into myself and trying to retreat into sleep to get rid of the pain.”
Havelock first went to see her primary care doctor. When he couldn’t figure out what was wrong, he referred her to another doctor, then another and another and another and another. Each time, leaving without answers, Havelock became more frustrated. The typically upbeat woman fell into depression. She cried, she said, nearly every morning.
Though her disease is uncommon, her ordeal is not. The diagnosis of disease is not an exact science and, especially when the disease is rare, it often takes several physicians to figure out what’s wrong. People will live with pain or other symptoms for years, sometimes a decade or more, before they know what’s wrong.
Even the best doctors can find diagnoses difficult. Symptoms of many diseases are vague or common to many illnesses. Many disorders do not have a test that can give sure answers. And, a patient may have something so rare that a doctor has not seen it since medical school, if at all.
There are no estimates of how often a patient leaves a doctor’s office without a diagnosis, but both physicians and other experts say it happens often.
“Frequently, it’s not like there’s this clear entity” that can be easily diagnosed, said Dr. Michael Feldman, a nephrologist at Bend Memorial Clinic who saw Havelock. “It’s very typical that you have this multi-system complaint and there have been a lot of negative tests and a lot of frustration along the way.”
The problem is particularly acute with rare disorders because, by definition, most doctors have less experience with them. In addition, physicians’ training often teaches them to look for the likeliest explanation, said Stefanie Putkowski, a registered nurse at the National Organization for Rare Disorders.
“Medical students are taught when they hear hoofbeats, think horses, not zebras. And, from our perspective, sometimes they need to think zebras.”
Doctor to doctor
Havelock first saw her primary care physician, Dr. Alan Hilles, for her hand pain in March 2008.
Havelock’s medical records from that visit suggest Hilles thought she had fractured or sprained her wrist; he ordered an X-ray of her wrist and over-the-counter pain medications.
The medications didn’t help, Havelock said. She was back less than a month later and again a few weeks after that without a firm diagnosis. Hilles referred her to Feldman, according to medical records, for the pain in her hands and, because she had previously been a kidney donor, for possible complications of having just one kidney.
Feldman suspected complex regional pain syndrome, a chronic pain condition with an unknown cause. “She had this funny pain syndrome in her hands,” he said. “It fit nicely” with the pain syndrome diagnosis. He put her on an antidepressant that is also prescribed for pain. Havelock said that did not work. “After two months, I was so depressed and I was swollen so bad,” she said. “I weaned myself off the antidepressant, and I didn’t go back to him.” Someone suggested to her she try a neurologist. She did, in Bend in July 2008. Her patient history notes a series of seemingly unrelated symptoms: burning sensation in her hands, a wrist injury with no evidence of fracture, possible complications of living with one kidney and perhaps even an allergy to ibuprofen. Her medical records suggest nothing stood out besides the pain.
That, doctors said, can make a diagnosis difficult.
“Medicine is pattern recognition,” said Feldman. Doctors look for groups of symptoms that suggest a disorder, then test to confirm or exclude specific diagnoses. Where there’s no pattern, the diagnosis comes slower.
For the patient, that can be agonizing. “It’s very, very stressful,” said Putkowski. “People in that time period (before a diagnosis) often can’t work because they are so ill. It impacts people in every way possible.”
Havelock was growing frustrated. “By that time, I feel like I’m getting the runaround because here’s three doctors so far and I’m not getting anywhere.” Medical records indicate that she see a rheumatologist, who would be able make a firm diagnosis of scleroderma.
Havelock took a break from the physician merry-go-round that fall, and her medical records indicate she didn’t see Hilles again until December. A few weeks later, at a follow-up visit, Hilles noted that she was “distraught” over not getting a diagnosis. He referred her to another doctor, an endocrinologist.
That visit did not result in a diagnosis or meaningful new findings.
“Every blood test came back normal,” Havelock said. “But at the same time, I knew it wasn’t all in my head. ~ I may have had one symptom of this disease, two or three symptoms of that disease, but not enough symptoms of any one disease to fit.”
In March, she made another trip to another physician’s office. This time she saw Tianna Welch, a physician assistant who specializes in rheumatology.
As soon as Welch saw her, she said, she knew what was wrong. “People with scleroderma just kind of have a certain appearance,” she said. “I suspected it pretty much right off the bat.”
On March 30 of this year, more than a year and a half after the beginning of her symptoms, Havelock finally had her diagnosis. She had scleroderma, a disease characterized by a hardening and tightening of the skin.
That tightening of the skin, which had grown worse and more visible since she first started seeking a diagnosis, was key to the diagnosis, said Welch. “You kind of lose the elasticity. It becomes harder,” she said. “When you pinch (normal) skin it molds and it moves around. This one, it doesn’t want to do that as much. It’s tight.”
Scleroderma is a rare disease in which the body produces too much collagen, the protein that gives skin structure and strength. As it did with Havelock, it often causes pain, swelling, tightening and even an exaggerated response to cold temperatures – known as Raynaud’s phenomenon – that Havelock first experienced when installing the storm door.
There are two primary types of scleroderma: localized scleroderma, which just affects the skin, and systemic scleroderma, which can involve blood vessels and internal organs, including the lungs or kidneys. Havelock has the second type, often considered more serious, though individual cases vary widely.
There are about 300,000 people in the United States with scleroderma; about one-third of them have systemic scleroderma.
Havelock did not feel relief at having been given her diagnosis. “It hit me because the pain that I was going through and the swelling that I was going through for almost two years now, and I finally get an answer and it’s not a good one. It’s a bad one.”
She misread an information sheet she was given, thinking it indicated there was a good chance she would die soon. She crumpled, she said. “I just lost it.”
Living with scleroderma
Scleroderma can be particularly difficult to diagnose, said Dr. Greg Borstad, a rheumatologist at BMC who recently began seeing Havelock. The disease is rare, he said, and many of the symptoms are similar to other, more common disorders. A year or two to diagnosis “is almost average, unfortunately.”
There is no treatment for scleroderma. The only thing that can be done is alleviating symptoms. “You’re sort of left treating whatever the complication is,” said Borstad.
For Havelock, that means several medications to control pain, swelling and blood pressure.
Havelock is stable now. But just after being diagnosed, she had an episode with her kidney, a complication of scleroderma, that put her in the hospital for several nights. She was heading toward kidney failure, said Feldman, who treated her during that episode, but she pulled out of it.
These days, she tries to look at the bright side. The tightening of her skin has taken away the fat on the underside of her arms. “I can wear a tank top without my upper arms waving,” she said, smiling.
Havelock also started a support group, which meets once a month at BMC, for patients with scleroderma. “I will do everything I can to bring awareness to the center for this disease,” she said.