The fight was on, my children were my whole life, I had to fight this disease for them even though I was feeling so ill most of the time, sometimes I even had difficulty getting out of bed, dressing my daughter for school and doing her hair was impossible as my hands were so stiff. It was painful to see a three year old child fastening the buttons on my blouse due to my sore fingers, she knew that Mummy couldn't so some things and grew up with having to help me from this young age. One can only imagine the impact this had on my young family and the negative influence it created in many aspects of daily life. Living with a chronic, uncurable condition takes it's toll on your psychological health too, fear, anxiety and uncertainty of the future, frustration at not being able to do things I used to do, and the loss of control over my life were overwhelming. It consumes your whole life and those around you are also affected.
The added worry was the lack of knowledge from the medical profession, it was for this reason that I made a conscious decision to participate in future clinical trials. If they couldn't help me, then by doing this, I could at least help them in their research to help people diagnosed in the future.
If there was any consolation, it was that I had married young and my beloved Dad had given me away at my wedding in 1980. He sadly passed away on July 5th 1982 aged just 51 years old. This was an extremely traumatic time for me, my Dad had delivered me at birth in my childhood home and we had always had a great bond, the loss of him was devastating, I was just eighteen years old at that time.
Also, had I not have had my children when I did, it would not have been possible later on. I believe this was always meant to be.
Scleroderma, or systemic sclerosis, is a chronic autoimmune disease characterized by the hardening of the skin and connective tissues.
Scleroderma can be life-threatening as the skin, joints, tendons, and parts of internal organs can all be affected.
Autoimmune conditions occur when the body’s tissues are attacked by its own immune system.
People with scleroderma have higher amounts of collagen deposits in their body than those who are healthy. This also causes damage to the blood vessels leading to a physical over-reaction to cold or stress (Raynauds's)
Excess collagen is laid down in the internal organs, which in turn results in scarring and reduced normal function of the affected organs.
Symptoms of scleroderma vary for each person, and the effects of the disease can range from very mild to life-threatening depicting on which parts of the body are affected.
While localized scleroderma, the more common form of the disease only affects the skin, systemic scleroderma is very rare and includes hardening of the skin, as well as one or more internal organs.
Mild scleroderma can become more serious if left untreated, however, diagnosis is difficult as the symptoms of scleroderma are similar to those of other autoimmune diseases.
Many people have scleroderma and approximately one third of those people have the systemic form of the disease which is more dangerous and harder to control.
Around 12,000 people in the UK have been diagnosed with scleroderma, with women affected four times as often as men. However, the complex nature of the condition can often lead to misdiagnosis.
While scleroderma is not thought to be directly inherited, some researchers believe that some people have a slight predisposition for the disease as the result of a family history of rheumatic conditions.
Problems from this disease cause Esophageal dysmotility; Sclerodactyly; Telangiectasias, CFS, IBS, Raynaud's.
In my case, I have an overlap of Systemic lupus erythematosus, often abbreviated as SLE or lupus, (or autoimmune connective tissue disease) in which the body’s immune system mistakenly attacks healthy tissue. There are many kinds of lupus. The most common type is systemic lupus erythematosus (SLE), which affects many internal organs in the body. SLE most often harms the heart, joints, skin, lungs, blood vessels, liver, kidneys, and nervous system. The course of the disease is unpredictable, with periods of illness (called flares) alternating with remissions.
The first five years are the most dangerous and it is important for patients to respond to treatment early on. The sooner they can slow down the immune system, the less damage is done to the body overall. In 1981 the statistics showed that people diagnosed had only a 30% chance of exeding five years after diagnosis.
There was also a higher risk of cancer due to a suppressed immune system. More on Paraneoplastic syndrome here.
I did respond well and I had some relief from my painful joints with the high dose of steroids.
The winters of those early years were most difficult with the Raynaud's, I remember coming home from taking the children to school and the intense pain was so bad that it was very difficult just to put a key in the door to get inside to the warmth. The tingling sensation as the blood vesels opened and slowly came back in to my fingers was very painful. These situations can agravate ulcers due to lack of blood circulation and many patients lose digits in the process.
It was around 1986 when I began to develop ulcers, one which has left scarring or (pitting) on my right index finger where I have little feeling even today.
I learned to wrap up and create more layers than an onion when the very cold weather arrived each winter. This is when I realised I had been born in the wrong country as hot climate countries have fewer reported cases of Raynaud's, Caribean countries being the least affected with no reported cases. I was always much better when on holiday in Europe where the sun was shining, then there was the added benefit of the much needed vitamin D, which is necessary to enable the body to absorb essential nutrients.
Malabsorbtion plays a role in how I am affected by these autoimmune disorders, my inability to absorb calcium, magnesium and potasium means that I have to take these as supplements, without which the body is unable to function; especially the heart.
"Secondary Raynaud's disease is the same as primary Raynaud's disease, but occurs in individuals with a predisposing factor, usually a form of collagen vascular disease. What is typically identified as primary Raynaud's is later identified as secondary once a predisposing disease is diagnosed. This occurs in approximately 30% of patients. As a result, the secondary type is often more complicated and severe, and is more likely to worsen.
There are three distinct phases to an episode of Raynaud's disease. When first exposed to cold, small arteries respond with intense contractions (vasoconstriction). The affected fingers or toes (in rare instances, the tip of the nose or tongue) become pale and white because they are deprived of blood and, thus, oxygen. In response, capillaries and veins expand (dilate). Because these vessels are carrying deoxygenated blood, the affected area then becomes blue in color. The area often feels cold and tingly or numb. After the area begins to warm up, the arteries dilate. Blood flow is significantly increased. This changes the color of the area to a bright red. During this phase, persons often describe the affected area as feeling warm and throbbing painfully.
Raynaud's disease may initially affect only the tips of fingers or toes. As the disease progresses, it may eventually involve all of one or two digits. Ultimately, all the fingers or toes may be affected. About one person in ten, will experience a complication called sclerodactyly. In sclerodactyly, the skin over the involved digits becomes tight, white, thick, smooth and shiny. In approximately 1% of cases of Raynaud's disease, deep sores (ulcers) may develop in the skin. In rare cases of frequent, repetitive bouts of severe ischemia (decreased supply of oxygenated blood to tissues or organs), tissue loss, or gangrene may result and amputation may be required."
Many people are able to find relief by simply adjusting their lifestyles which is what I have done for many years.. Affected individuals need to stay warm, and keep their hands and feet well covered in cold weather. Layered clothing, scarves, heavy coats, heavy socks, and mittens under gloves are suggested because gloves alone allow heat to escape.
It was't until a few years later that I developed Raynaud's mildly, in both my feet and my face, the more attacks that occur, the more scar tissue is produced in those affected regions. When an attack occurs on the face, it can make speech difficult as the whole area is painful and numb.
Because episodes of Raynaud's disease have also been associated with stress and emotional upset, the condition may be improved by learning to manage stress, which was difficult for me with a family of small children; a home and working commitments.
During these early years I also took part in trials at the Clinical Science building in Salford and was offered (but refused) Vasodilators which are injected in to the blood stream to help improve circulation. My theory was to avoid the attacks altogether by not exposing myself to extreme cold and fluctuating temperatures.
Life was difficult but I managed well and aged 25, I became the star patient for my excellent response to treatment. Ok, so now I had exceeded the first five years, I had another fight on my hands to reach the age of thirty!
In 1986 and determined to live life as normal as possible, I applied for a post at a local hospital and was examined by a consultant Doctor Tubbs, who could tell immediately what I was suffering from even before I spoke a word.
I knew from my visits to the Scleroderma Clinic in Manchester that all patients have a similar recognisable look which is not noticed by people without the knowledge of symptoms.
I was amazed when following my examination, he told me that he was prepared to give me a chance and that he was interested in my case, it being so rare, and wished to follow my journey. At that time, very few Doctors would come in to contact with a sufferer and there were many occasions where this consultant would detract me from my duties to present me to his latest unsuspecting registrar who had to guess what was wrong with me as I stood very embarrased before him. Not many would get the correct diagnosis.
Now I was happily working within the NHS who I was indepted to for saving my life, my secret was safe between Doctor Tubbs and myself.
It was a stuggle in those early years, but with drugs to stabalise the condition and pain management consultations, I was able to have some normality back in to my life . I was determined to make something of myself now I had been given this chance and following several management courses at Cauldon College, I gained promotion in my new workplace.
Life was looking up!