Category: Scleroderma Systemic Sclerosis

June Is Scleroderma Awareness Month

June Is Scleroderma Awareness Month

Posted on by Marie

June is scleroderma awareness month so I thought I would put together some information about scleroderma for you. First, it is important to note that scleroderma is an umbrella term for a group of diseases. I have been diagnosed with limited cutaneous systemic sclerosis so my blog tends to focus on that. If you would like to add to this blog with similar articles for other varieties of scleroderma or even for other autoimmune diseases or chronic conditions please drop me a line using my contact form. I wish I could pay you but this is a personal blog with no advertising to support it. I am not getting paid for my own work but am happy to do it to assist others who may be going through the frustration of dealing with chronic pain and illness.

I originally found these pictures posted on a Facebook friend’s personal website. They reminded me that I really should do something for Scleroderma Awareness Month.

What is Scleroderma
Calcinosis
Raynauds
Esophageal Dysfunction
Sclerodactyly
Telangiectasias
Muscle and Joint Pain
ILD
PAH
Renal Crisis
Shadow

Symptoms

As I mentioned earlier, I have the limited cutaneous systemic sclerosis variant of scleroderma so that is my focus here. This variant used to be called CREST based on the main symptoms of Calcinosis, Raynaud’s, Esophageal Dysfunction, Sclerodactyly, and Telangiectasia. It was changed to the current name limited cutaneous systemic sclerosis when they realized that this variant was systemic and could affect every body system including your organs, entire digestive system, muscles, joints, and even your blood vessels.

I’ve had a few doctors, including specialists, who think that limited means it is not too bad. I’ve also had doctors who didn’t believe me because I didn’t have any major skin changes. For decades I only had a small sliver of hard shiny skin right below my fingernails, now the tight shiny skin extends to my second knuckle. I also have it on my feet. Limited cutaneous means limited skin involvement. The different between limited and diffuse is that limited does progress slower than diffuse and diffuse has wide spread skin involvement. Both are systemic and both can affect everything in your body.

Digital Ulcers

Calcinosis

Calcinosis are deposits of calcium that start on the inside of your body near the bone. They can be extremely painful as they work their way to push through the surface. They can cause these horrible looking digital ulcers. Typically they seem to be on the fingers but they can happen anywhere. I have them in my feet as seen on x-rays. So far, I’ve been lucky. While it is difficult to walk due to pain, they have not caused ulcers.

Ulcers can get so bad that gangrene and tissue death can set in requiring amputation.

Raynaud's

Raynaud’s Syndrome

Raynaud’s constricts the blood vessels to the extremities. In some patient’s only the fingers and toes turn white but in others they can turn blue or purple with exposure to cold or stress.

This can be very painful and if you can’t get the circulation moving again gangrene can set in. My hands and feet will go white and a dark blue jean blue. I used to get asked if I’d spilled ink.

They will turn red when they start to warm up with pain and tingling.

Esophageal Motility Dysfunction

Esophageal Dysmotility

The esophagus (simply put) is the tube that uses a wave-like motion to take your food from your mouth to your stomach. This picture shows decreased salivary production which causes dry mouth and tooth decay no matter how well you look after your teeth. It can also dry your throat making it even more difficult to swallow. If you have Sjogren’s on top of that swallowing becomes even more difficult.

GERD is very common with scleroderma causing acid reflux and heartburn. I don’t like to eat out any more because I am always afraid that I will choke on food that refuses to go down. It happens quite frequently. I recently had my esophagus dilated and food is going down better for now but only if it doesn’t get stuck in my throat first.

Gastroparesis is another dysmotility disorder. In a nutshell it means that your stomach does not empty well. This can get severe enough that you feel full on just a mouthful or two. Vomiting can occur and if it get severe enough a feeding tube is required.

Then there is dysmotility of the intestines. This can lead to severe bloating, constipation, diarrhea, and even obstructions requiring surgery.

Sclerodactyly

Sclerodactyly is the term used when the skin starts getting tight and shiny. In severe cases the hands can become claw-like and very difficult to use. In Diffuse the entire body can be affected. With limited skin involvement is limited to the hands (up to the elbows) and feet (up to the knees).

There can be other skin involvement as well. For instance my skin in “mottled” on my chest and extremities. When I went to a cancer clinic after having a melanoma removed the doctor told me I had skin damage from spending too much time in the sun. I told him that I hadn’t sat in the sun for decades because it made me sick. He didn’t believe me pointing out the colour of my skin as his “proof.” When going over some old medical files at a later date I discovered that there was a name for the discoloration… Livedo Reticularis and in my case, it had nothing to do with the sun and everything to do with my autoimmune diseases.

Telangiectasia

Telangiectasia can appear pretty much anywhere on your body including your eyes. They are broken blood vessels. They can show as red dots like the photo or as red spider webs. I have them all over.

Muscle & Joint Pain

Wide spread chronic pain is a given with scleroderma and most other autoimmune diseases. A really good day for me would be like how you feel when you are running for Advil to help with the aches and pains you get from having a fever with the flu. I hate the question “How is your pain on a scale of 1 to 10?” because too many doctors don’t get my pain scale. When healthy people say a 10, I would be saying a 3. I am vomiting somewhere between 6 and 7 and am totally incoherent and don’t know where I am at 9.

Organ Damage

Systemic forms of scleroderma can cause organ damage. I have PBC or Primary Biliary Cholangitis which is a liver disease that causes high blood levels of alkaline phosphorous. In PBC the bile ducts in the liver become blocked with fibrosis. It is NOT caused by alcohol. It causes liver pain and can eventually lead to requiring a liver transplant if it is not controlled.

As mentioned earlier, I have ILD or Interstitial Lung Disease which is now well controlled with medication but I am being evaluated for PAH or Pulmonary Arterial Hypertension where the pulmonary artery becomes enlarged and the blood pressure in the artery is high.

Heart damage can also occur. I was diagnosed with a “stiff” heart and told that my heart is strong but doesn’t empty the blood properly. Recently some other changes showed on an echogram so I have to go back to the cardiologist.

These are just a few of the things that can go wrong with your body when you have systemic scleroderma. For additional details be sure to follow the links below. I recommend the Scleroderma Education Project as a good place to start because it is an easier read.

The Scleroderma Education Project

I wanted to make sure that I mentioned the Scleroderma Education Project website since it is filled with excellent information for patients that have scleroderma. I love that it explains the different forms of scleroderma and provides updated information in words that are easy to understand. Ed Harris is the founder of this project, has limited cutaneous systemic sclerosis himself, promotes research, and runs a Facebook support group.

Other Links

Please beware of using Dr. Google. There are so many seemingly legitimate websites that have dated and misleading information out there. On many occasions I have seen posts from patients who are terrified that they don’t have long to live. This may have been true years ago but there are new treatments available and current testing means that dangerous complications are caught much earlier giving a much better prognosis. As an example, I was diagnosed with ILD (Interstitial Lung Disease) a few years ago. It was caught in the early stage and treated. While I will take medication the rest of my life to protect my lungs the medication actually cleared up my lungs to the point that only some scarring shows on my lung CT now while previously they were covered in white spots.

Along with the Scleroderma Education Project some other websites you can use to educate yourself are The National Scleroderma Foundation, John Hopkins Scleroderma Centre, Cleveland Clinic, and some other major hospital websites in the US. I’m still looking for Canadian resources but so far all I’ve really found is Scleroderma Canada.

Viagra ~ A Treatment For Scleroderma?

Viagra ~ A Treatment For Scleroderma?

Posted on by Marie

Lupus, Sjogren’s, Other Autoimmune Diseases, and Gastroparesis

Update

Since I wrote this article I have discovered additional information that Sildenafil/Viagra is also being studied for gastroparesis! I will need to create another article! Could it be that Viagra will be helpful for many different autoimmune disorders and complications?

It has been a while since I have done anything here but this morning I heard a radio show that intrigued me so much I had no choice but to write about it.

Silvia Petrozza “The Autoimmune Slayer”

I spotted the interview with Silvia Petrozza “The Autoimmune Slayer on the Scleroderma Canada Facebook Page.

Listen to the interview with Sauga 960 AM

Christine Elliott Ontario’s Minister Of Health

What I found so intriguing about Silvia’s interview with Sauga is the fact that she has been advocating the use of sildenafil/Viagra as a treatment for certain aspects of scleroderma. Silvia wants to see coverage for “the little blue pill” by the Ontario Drug Benefits Program (ODB). She says that one or two pills can keep scleroderma patients out of the hospital for treatments that are high risk and take at least a couple of days of 15-minute monitoring. Sounds like big savings to me. If it works perhaps more people could be treated because so many doctors won’t prescribe these risky treatments.

Silvia has been working with Christine Elliott to get coverage for nearly 3 years now. There is concern that the June provincial election will mean having to start over with the new minister of health. I’m hoping that the new minister will be empathetic after reading whatever files Christine Elliot has on this.

This article talks about a few of the symptoms of systemic sclerosis or systemic scleroderma and how the little blue pill may be able to treat some of them.

Types Of Scleroderma

To start, it is important to know that there are several different types of scleroderma that confound the issue for many doctors and that patient symptoms vary greatly.
(See symptoms of systemic sclerosis)

The Scleroderma Information Project is one of the better websites I have found to obtain a wealth of good solid information about scleroderma. The man behind it all is Ed Harris who has the limited form of scleroderma himself and is a researcher in the US. The chart showing the different types of scleroderma is from this site.

Based on the radio interview, I believe that Silvia has Diffuse Scleroderma or Diffuse systemic sclerosis which means that along with all the internal involvement she has skin involvement as well. Scleroderma is an umbrella term for the various types and the names have all been updated which can lead to even more confusion.

I have limited cutaneous systemic sclerosis. Don’t let the word limited fool you. Notice that the word cutaneous comes after limited so it means limited skin involvement. My skin is only hard and shiny on my fingers, palms, and feet. My mouth is getting smaller, and I don’t have many wrinkles around my eyes for my age. These are the only outward signs I have but on the inside, I am a mess. I also have multiple organ involvement which comes with its own challenges.

Why am I putting this out there? To try and bring some awareness of what people with scleroderma are facing because far too often we look healthy. I don’t believe I look all that healthy anymore though. I think my disease has been ignored to the point that I can no longer compensate.

People have heard of lupus and doctors understand lupus better. That does not mean that the medical system is good to those with lupus because it is not. Scleroderma is rare and doctors often don’t think we are sick because most of us do not look ill unless there is a lot of skin involvment. To confuse the issue I many doctors equate how sick you are to the degree of skin of skin involvment. I have very little skin involvement and wish I had a dollar for every doctor who told me that I either don’t have it or I’m not that sick. It has only been the last few years that I’ve really shown any sign of skin involvment and it is very little in spite of having scleroderma for decades. I do have a lot of internal issues including heart, lungs, veins, and my entire GI system, not to mention problems with my eyes.

I have not been given a formal diagnosis of Overlap yet but more and more I believe that is what I have rather than limited. I asked my rheumatologist if she would test me for lupus because I’ve had what looks to be the lupus butterfly rash for quite some time. I was originally diagnosed with rosacea but none of the rosacea creams worked but the cream I was prescribed for my recent diagnosis of psoriasis which is also prescribed for lupus did. She looked at my chart and said “Oh, you do have the lupus antibodies so we don’t need to test.” When I asked why I wasn’t informed about it she didn’t have an answer for me. When I asked if I had psoriatic arthritis I was told that it was entirely possible but it would be too difficult to get a formal diagnosis because the symptoms overlap so much with scleroderma and Sjogren’s (yes, I have that too). From what I’ve read about psoriatic arthritis it does fill in the gap for a few of my symptoms that are not listed for my other autoimmune diseases.

Can The Little Blue Pill Actually Work For Scleroderma?

According to Scleroderma News, the answer could be YES! It has already been approved in the US and Europe for Pulmonary Arterial Hypertension or PAH

Sildenafil Approved For PAH

Sildenafil, the main ingredient in Viagra, has already been approved to treat PAH, a complication of scleroderma, by the US Food and Drug Administration (FDA) in June 2005 and by the European Medicines Agency in October 2005. Studies showed that Sildenafil significantly reduced blood pressure in the lungs thereby reducing the effects of PAH.

Raynaud’s Relief?

For those not familiar, Raynauds is disease where the blood vessels spasm in response to cold, stress, or emotional upset. This happens mainly to fingers and toes. For me, my entire hands and feet are affected. For others, it can also happen to the knees, nipples, and nose.

When this happens the affected body parts can turn white or even blue! This is very painful both when it happens and when blood flow is restored and turns the hands red. Raynaud’s can also cause painful sores that are difficult to heal.

This video is from the Raynaud’s page on the John Hopkins website. It shows what happens during a Raynaud’s attack.

Clinical trials show that Sildenafil appears to decrease the number of Raynaud’s attacks that scleroderma patients get but does not seem to have any effect on the severity.

Of interest, Raynaud’s can happen on its own or with many different connective tissues or autoimmune diseases so could be a treatment for these patients who have Raynauds also. Raynaud’s can also be caused by smoking, chemical exposure, injury or trauma, repetative actions such as typing, using vibrating tools such as a jack hammer, and as a side effect of certain medications.

Sildenafil may be an effective treatment for Raynaud’s patients who are resistant to the current treatment of blood pressure medications or those of us whose blood pressure is too low to take blood pressure meds.

Digital Ulcers

As you can see, digital ulcers are nasty looking sores that can be caused by Raynaud’s and the resulting lack of blood flow. These sores are often infected. However, I read a post by someone in one of my support groups about how she was made to feel like a fool for going to the ER for help with one of these painful sores that was not infected.

The doctor laughed at her for coming to the ER for a small sore on her finger. Unfortunately, many doctors don’t understand what we go through and we are often treated this way for just about everything we go to the ER for. Most of us just stop going. In 2015, I had a tiny blister on my baby finger that took me to the ER. I’d had a very severe bout of Raynaud’s several days prior caused by an extreme emotional upset at work (harrassment by supervisors) and my baby finger stayed blue for well over a week and would not pink up. It is still sensitive to this day! I was lucky that my appointment with my rheumatologist happened while I was dealing with this issue. She prescribed a nitroglycerin cream to try and get the blood flowing again. It hurt like the dickens every time I applied it but the concern was that it could turn to gangrene and require amputation if the blood didn’t start pooling again.

I think it was only because I had the prescription with me that the ER doctor believed my story and took a closer look at my finger. She declared that it was infected and that she would lance it. She was amazed at the amount of pus came out because it only looked like a blister on the surface. I was given a course of antibiotics.

I consider myself to be lucky that my sore was just an infection because a lot of doctors don’t get that these digital ulcers start on the inside at the bone and work their way out! They are quite nasty and extremely painful especially when infected. They can even lead to amputation if gangrene sets in which does happen.

Clinical Trials For Digital Ulcers

The result of a clinical trial in France showed that sildenafil reduced the number of digital ulcers present compared to a placebo. Unfortunately, the trial did not prove it’s primary focus that sildenafil improved the healing of digital ulcers because there was a high number of healing in the placebo group as well. This may have been due to patients in the trial taking the placebo that were also taking calcium channel blockers were greater than the number taking blood pressure meds in the sildenafil group.

However, the sildenafil group did show a higher number of healing ulcers than the placebo group. It was just not determined significant.

Conclusion

In my opinion as a scleroderma patient, I’m not a researcher or medical person of any description, I think there may be something to the little blue pill. Sildenafil is marketed by Pfizer as Revatio and is the main ingredient in Viagra.

I for one am keeping my fingers crossed that Silvia Petrozza is successful in getting this treatment covered by the Ontario Drug Benefit Program. It seems rather absurd that it is not covered already when the alternative could be several days in the hospital on a regular basis for risky treatments requiring monitoring every 15 minutes.

My Medical Issues

My Medical Issues

Posted on by Marie

Okay, here goes. This is a quick overview of the medical issues I have had to learn to live with. In future posts I will provide more details about these issues and provide you with links to legitimate resources about each one. As mentioned in my first post, my goal is to help as many people as I can based on my personal experience with these diseases and my years of research.

Limited Cutaneous Systemic Sclerosis (LcSSc)
AKA Scleroderma CREST

This was the first autoimmune disease I was diagnosed with back in the 80s. It took over a decade to get a diagnosis and I was often told it was in my head. I was even sent to a psychiatrist. Lucky for me, the psychiatrist was able to discern that there was nothing wrong with my mental health and that I needed to keep pushing my doctor to figure out what was wrong.

Originally, I was told all I had was Raynaud’s and that the only thing it caused was hands and feet that would turn blue with the cold. This was discovered while waiting for a doctor to do a physical exam. I turned blue in his cold office so he was able to see it happening. I was then referred to the rheumatologist that told me that Raynaud’s was nothing. I know now that Raynaud’s can cause a lot of issues if it is not treated properly. With Raynaud’s the blood vessels spasm with cold or stress and can cut off the circulation to the point that gangrene can set in. Raynaud’s can be a big deal even if that is all you have.

Limited cutaneous systemic sclerosis is a variety of scleroderma that previously known as CREST. Unfortunately, the only thing limited about it is the amount of skin involvement. It also does not progress as quickly as Diffuse scleroderma but can still be deadly if/when it starts to attack the internal organs.

CREST

CREST is an acronym that stands for the most common symptoms of this variety of scleroderma. It stands for: Calcinosis, Raynaud’s, Esophageal Dysfunction, Sclerodactyly, and Telangiectasias.

While the above lists the common symptoms of this subset of scleroderma it does not tell the entire story and so they changed the name from CREST to limited. As mentioned above, the only thing limited is the amount of skin involvement. This disease can affect all internal organs and systems and cause widespread chronic pain.

At some point, I plan to add a lot more information about scleroderma but for now, here is a link to some of what the Mayo Clinic has to say about it. Be sure to scroll down to the complications. Not to scare you but it is important to know how devastating this disease can get. The key to a good prognosis is early detection of the complications. For example, when I was first diagnosed with Interstitial Lung Disease (ILD) I was immediately put on Prednisone and azathioprine (Imuran). At the time of discovery, my lung CT scans showed that the bottom lobes of both lungs were collapsed and my lungs were covered in white spots. With time and treatment, my lower lobes are no longer collapsed and the white spots have pretty much gone. My respirologist discharged me on the condition that my rheumatologist schedule regular pulmonary function tests and keeps an eye on things to ensure that it doesn’t start deteriorating again.

Sjogren’s

Sjogren's

Sjogren’s, is another systemic autoimmune disease. Most patients experience severe dry eyes and mouth which are the 2 most common symptoms. However, Sjogren’s is systemic and often causes fatigue and joint pain.

Sjogren’s can also attack and cause dysfunction of the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and even the central nervous system. Sjogren’s also increases the risk of non-Hodgkin lymphoma.

Primary Biliary Cholangitis (PBC)

Primary Biliary Cholangitis PBC

Primary biliary cholangitis is a liver disease where the immune system attacks the bile ducts and damages them. PBC as it is often called, is a complication of both scleroderma and Sjogren’s. When I was diagnosed back around 2004 it was still called Primary Biliary Cirrhosis. With treatment, it is rare for PBC to progress to the Cirrhosis phase so they changed the name to make it more accurate. Please note that this disease has nothing to do with alcohol abuse. It is a complication of both scleroderma and Sjogren’s.

Interstitial Lung Disease (ILD)

Interstitial Lung Diseases (ILD)

Interstitial lung disease is a yet another complication of both Sjogren’s and scleroderma.

ILD is a fibrosing disorder of the lungs that causes scarring and can lead to respiratory failure if not treated.

I got lucky with this one. My interstitial lung disease was found early and I started treatment with both Plaquinil and Imuran. At my last appointment with my respirologist he declared me stable and discharged me back to my rheumatologist. When it was first found the bottom lobes of both lungs were collapsed and both lungs were covered in white spots on the lung scan. It took time but each lung scan showed improvement. My lungs are no longer collapsed and there is only sparse white spots on the lung scan. Unfortunately, there is still scarring that won’t likely go away. It is recommended by the respirologist that I continue with yearly pulmonary function tests and echocardiograms.

Heart Issues

So far tests show I have a stiff heart which, from what I’ve read, means fibrosis. I’ve also been diagnosed with diastolic dysfunction, and some mild pericardial effusion.

My interventional cardiologist explained that my heart is very strong and can squeeze well but has trouble relaxing so it doesn’t fill up with blood very well. Another report I saw indicates that I have some right-sided pressure which could mean PAH or pulmonary arterial hypertension but no one is saying anything about that yet. Once again, these are all complications of both scleroderma and Sjogren’s.

I’ve also had issues with palpitations and some serious spikes in blood pressure. I don’t have a lot of confidence in my new family doctor at this point as I feel he doesn’t understand my autoimmune diseases. It scares me when he told me no more tests because “they just complicate things.” As I mentioned above, early diagnosis of complications is very important as to the outcome of those complications. I did manage to get him to do a 24-hour ambulatory blood pressure test BUT he had his secretary contact me to cancel my appointment saying that my blood pressure was well within the normal range. I already knew that BUT the spikes in blood pressure are dangerously high. My home care nurse is very concerned in particular with my diastolic blood pressure.

Mental Health

Due to harassment and bullying at work in 2014, I also developed PTSD, anxiety, panic disorder, and major depressive disorder. It was during and after this incident that my autoimmune diseases decided to take a nosedive even though they had been more than manageable prior to this. So, yes Virginia, stress does play a very important role in the prognosis of autoimmune diseases.

Fibromyalgia

Fibromyalgia

More recently, I was informed by the scleroderma specialist at Mount Sinai in Toronto that I had complex pain syndrome and fibromyalgia. I had been told decades ago that I had fibromyalgia but then told no, I had CREST scleroderma. Guess I have it all!

Fibromyalgia is not just aches and pains. It is thought to be caused by damaged nerves.

I’m not sure about the fibromyalgia diagnosis since it was a diagnosis from years ago before I was diagnosed with autoimmune diseases. I was later told I didn’t have it and my current rheumatologist never mentions it so who knows for sure.

Interstitial Cystitis

Interstitial Cystitis

For over a year I would get one urinary tract infection after another. At least that’s what the doctors thought. I did get a kidney infection that put me in the hospital but most were not UTIs.

Doctors were not waiting for the results of the culture growths when I would complain of UTI symptoms and would just start me on antibiotics right away. Eventually, I was referred to a urologist.

It was the urologist that determined that, for the most part, the culture did not grow and yet I always had symptoms and blood and leukocytes in my urine. Tests confirmed that I had interstitial cystitis.

The urologist’s theory was that my Sjogren’s was causing my urethra to be dry and inflamed and that was causing the blood and leukocytes. He recommended Vagifem cream to try and add some moisture. I started using the Vagifem at the first signs of symptoms and it helped to a degree. It was not until my internal medicine doctor prescribed lactated Ringer’s IV fluids every 3 weeks that I stopped having symptoms.

The IV treatment has helped with all dehydration-type symptoms including headaches and cramps but if I have to go longer than 3 weeks I start to get pretty sick again.

Melanoma

Melanoma

Melanoma is the most insidious of the skin cancers. It doesn’t look like much but it will spiderweb underneath the skin where you can’t see it and can be fatal if not caught.

This picture is similar to what mine looked like.

I was lucky enough to have an observant family doctor who referred me to a dermatologist right away. The dermatologist thought the mole looked suspicious as the edges were not well defined and ordered a biopsy that came back positive. When the melanoma was removed I received the good news that they got it all and the margins were clear.

While my melanoma has been cured, I thought it important to put down here since it is possible that I was at a higher risk due to my autoimmune diseases.

Living Document

This page will be a living document and will likely have updates as I get organized and start adding more and more information. I think this page will be a sort of site map with a separate page complete with links to each disease and the complications of each disease. Please come back and check again.