I am fifty-five years old and live in New Zealand, with my husband.We were commercial greenhouse tomato growers for twenty-two years. In 1996 we took early retirement, and moved to a small piece of land in the country, bordering the sea. We had great plans for the future, but they all came to a grinding halt when I was diagnosed with Diffuse Systemic Scleroderma.
About 31 years ago, when we had only been married a short time, I was diagnosed with Meniere's Disease, which left me deaf in my right ear. I have just recently learned that it is now classified as an autoimmune disease.I have always had trouble also with Interstitial Cystitis, which apparently falls into the same category.
Looking back now it is obvious that I started getting a few symptoms of scleroderma about three years or so before I was actually diagnosed.My mother, and also her father, had always had severe Raynaud's Disease, in their hands and feet, so when I started having this in my thumb and index finger of both my hands during the cold weather, I just thought that it was hereditary.
I also started becoming fatigued for unexplainable reasons, as I previously had nearly unlimited energy.We had about ten years of very stressful events happening in the family, and I just put this as the reason for my ongoing tiredness.
In June 1996, my Raynaud's became a great deal worse, with all my fingers and both thumbs going white completely, instead of just the tips of some, as previously.My toes, and tips of my ears, and the tip of my nose were also affected.But we were having rather a cold winter, so I once again disregarded this.Suddenly I found that my wedding and engagement rings were too tight, so I had them enlarged.I was not putting on weight, so I could not understand the reason for this.
At the end of August 1996, I mowed our lawn, which took nearly two hours.The lawn was long, the weather was hot, and I was very tired.Then my hands suddenly felt as though they were on fire, and were very red.They became so painful that I wore soft gloves for everything.I even put a sheepskin cover on the car steering wheel, as holding that was very difficult.Slowly my hands became stiffer, and I could not make a fist.
Eventually, I went to a doctor.She did blood tests, and found my Antinuclear Antibodies (ANA) to be positive at 1:640. I was not familiar with this but soon found out, and started doing some reading.This test later went to 1:1280, then 1:2560, and then back to 1:1280.
At this stage my feet were becoming sore and I had "pins and needles" in my hands, arms, legs and feet, chest, and face. I went first to a neurologist, who did nerve testing with needles and electric currents, which was very painful.Everything was normal, soI went to a rheumatologist.To cut a long story short, he said I had Undifferentiated Connective Tissue Disease (UCTD), and wanted me to take Plaquenil. I had never heard of UCTD, and I did not want to take Plaquenil after I had read up on it, so I asked to be sent to an immunologist. He was puzzled as to what I had, but did wonder about scleroderma, and tried me on estrogen hormone. In short, I swelled up and felt terrible, so I stopped the hormone.
I then started with another general practitioner, who thought that I might have Chronic Fatigue Syndrome.However as I became worse he also began to wonder about scleroderma.By this time I had also been to a psychologist, as I did wonder about my mind.No particular problems there, and I was feeling very ill and tired.Next came another rheumatologist in May 1997.By this time I was having trouble walking, with sore feet and stiff knees. I had given up driving, and spent most of my time resting.This doctor diagnosed Diffuse Systemic Scleroderma, and my worst fears were realized.He gave me a variety of drugs, including Prednisone.After a week I felt much worse, and he said to double the Prednisone dose.That put me in bed, and I am only starting to get out of it a bit now! I weaned myself off it, and did not go back to him.
By this time my fingers were curling, and my skin was hardening over a large part of my body.I had developed a dreadful itch all over, and very bad pain like severe sunburn just under the skin, also all over me.I was taking painkillers every four hours, twenty-four hours a day, with not much effect.My doctor would not give me any strong ones and I really did not want to keep on living. My husband was at his wit's end, and my eighty-year-old mother had shifted to live just along the road, so that she could help my husband look after me. Her husband, my father, had just died a few months before.I lost nearly seventy pounds in weight, down to ninety-five pounds.I am 5' 7" in height.I started developing bedsores as my bones stuck out. My husband controlled this by making me special foam pads to sit on and put my feet on.
I could no longer walk, due to knee contractures. My hands resembled claws. I had elbow contractures and neck contractures, and could not straighten my arms. My mouth had become small, making it difficult to eat. My skin felt like plastic, stretched and tight.My lungs had some fibrosis.The knuckles on my fingers, and some toes had little ulcers on them, and small bits of Calcinosis. My index fingers and left thumb were shortening.I had no appetite, was continually nauseated, and could not sleep, but the worst thing was the constant itch and pain.I had moisturiser applied all over me, at least twice a day, which did afford a little relief to the itch.I still have this applied every day, after washing, as my skin is rather dry.
At last I found a compassionate doctor, who I still consult, who put me on stronger pain medication. I was on a heavy dose during my worst time, but it was a relief, even though it did not completely help me cope with the pain.The local hospice was a great help at this time.Those were dark years, and I did not want to see anyone except my husband and my mother, and definitely not a rheumatologist. I could not believe that such a thing had happened to me, but many people were praying for me.
Somehow we struggled through, and the pain and itch started to lessen a little.In November 1999 my eyes became very painful and red.I had not been out in a car for over two years and now I had to see an eye specialist.I made it with my husband's help, and received the diagnosis of Iritis, also called uveitis, another autoimmune disease. I was put on steroid drops and eye ointments, and also atropine drops to keep the pupils dilated and stop the swollen iris from sticking to the lens. This cleared up, but I had another attack, in one eye only, in September 2001.
In June 2000, my mother fell and broke her hip, from which she made a good recovery after hip replacement.However, we felt she should not have to look after me, so since then I have had caregivers for six and a half hours per day, seven days per week.Social Security pays for a large part of this.My husband looks after me for the rest of the time.
In July 2000, my doctor found my kidney function was declining, and persuaded me to see another rheumatologist, whom I still go to.He sent me for various specialised tests, and then to see a renal specialist.My kidneys had barely 10% of function left, which had been caused by the scleroderma, and I needed to soon go onto kidney dialysis.After looking at the options, I chose peritoneal dialysis for a number of reasons.I had the catheter placed for the dialysis in December 2000, and commenced dialysis ten days later.As I am not able to do this for myself, my husband was trained on this, and does it all for me.In March 2001 I was given a cycler dialysis machine, which does all the dialysis automatically at night, over the space of ten hours while I sleep.I am hooked up to it at night, and unhooked in the morning.
In April 2001, my haemoglobin dropped alarmingly low, and I was rushed to hospital for a blood transfusion of three units of blood.I also had to stay in for many tests and a gastroscopy.Nothing found, I went home only to have the same thing happen three weeks later.This happened a total of five times. After another Gastroscopy, and a Colonoscopy, as they thought I was bleeding from somewhere, and many other tests, they put me on Erythropoietin hormone injections.When the kidneys fail to function they often do not produce this hormone, which stimulates the bone marrow to produce red blood cells, with the resulting anaemia.About every 6-9 months, depending on the Ferritin level in my blood, I have an iron infusion by IV method.This is necessary to replace iron stores, which are used up in making haemoglobin. These two things have solved my anaemia problem very nicely.
The gastroscopy showed some gastritis, so I was put onto Somac, which controls the stomach acid.This helped also with the heartburn and reflux, which I had developed. However it made my Irritable Bowel Syndrome (IBS) worse, and eventually I stopped taking it.
My blood pressure shot up in August 2001, to 180/110, so I had to take medication for this.This is now well controlled, and in fact is almost too low.
In March 2002, an eye specialist told me that I had some degree of Sjögren's Syndrome, another autoimmune disease.I need lubricating eye drops, and lubricating eye ointment at night, for this, and have a somewhat dry mouth, but hopefully it will not become any worse.
During 2001, I steadily came off the Morphine, and no longer take any.In fact I take very few drugs, except the BP medication, Oxybutynin for bladder spasms, Calcium tablets to bind phosphates, which is a problem in kidney failure, and the erythropoietin injections for anaemia.I have extensive blood tests every month, and these are lately improving all the time.
My improvement over the past year is rather dramatic.When I last saw my rheumatologist he said that my scleroderma seemed to be receding, (he called it "regressing").He said that they do not know what makes it happen, they just know that it happens sometimes!He said that it usually starts at the head and works down, which is just what mine is doing.My scalp used to be immovable, as it was so tight.Now it moves normally, and my hair once straight, is very curly!This may be due to the collagen receding from my scalp, and twisting the hair follicles, giving me curly hair.
My dentist is amazed in the difference to my mouth, which was once so tight, and now is much softer.My skin is softening all the time, and I actually have wrinkles on my face again!The tight skin had previously ironed out all wrinkles.I have very little pain and no itch. My contractures are slowly becoming a bit less.I still can't walk, and my hands are difficult to use, (I type with my thumbs and one index finger), but there is some improvement.
My physiotherapist has noticed a difference in various measurements of my hands, and the muscles in the palms are growing again, and giving me hollows in my palms, which were once flat.My arms are nearly straight again, and best of all my brain is no longer a "grey fog".
My lungs have improved, and I have increased my weight by about 25 pounds.My Raynaud's has also improved. My renal specialist tells me that if I continue to improve like this, and if I can get out of my wheelchair, they would definitely look at doing a kidney transplant.At present that would possibly harm me.
I am looking forward to the future now, but still have to take one day at a time.I do not know how far my improvement will go, but it is all very welcome.I am only in my present improving position with the constant support and help of my husband, my mother, my caregivers and other good caring people; and most of all with the help of God.Fellow sufferers of scleroderma, take heart, there is hope out there, and light at the end of the tunnel!
In June I found, after tests, that my kidney function had some improvement! Then just last month my therapist observed that my legs were at least 15 degrees straighter than a few months ago! This is just happening of it's own accord. My therapist is mainly concentrating on my hands, (which are also improving). The contractures are improving a little, and they are very warm. I rarely have Raynaud's problems now with them.
As mentioned in my main story, my rheumatologist said that improvement usually starts from the head, and works down. The hands are usually the last to come right. This seems to be happening with me, and I must say that I am very hopeful, but I try not to expect too much, as this disease is so unpredictable. However, any improvement is great!
I have just recently purchased an exercycle with a proper chair on it, as a regular seat would be uncomfortable for me. I am just pedaling a short while each day, as I do not want to overdo it and put myself back. I certainly could not have done this even a few months ago. If I am going to walk again I need to improve the muscle tone in my legs, which the exercycle will do, and it will also help stretch my legs some more.
My renal specialist was delighted with my progress, and thought that I only had about another fifteen degrees to go with my legs, at which time they would be straight. He was generally pleased with my overall improvement.
At present I am having a little trouble with my blood pressure being too high. However, it started climbing when my erythropoietin injections for my anemia were changed to a different brand. They possibly do not agree with me, so I am off all this for a month, and then we will see. The blood pressure is already dropping after only about ten days off the drug. I may have to go back to my original brand, which I was very settled on.
I am steadily getting more wrinkles on my face as my skin softens. Who would have thought that I would welcome this! My dentist and renal specialist both remarked on the softening of my face, and my dentist especially, on my mouth, which used to be very tight.
Much of this has happened since I volunteered to help on this scleroderma site. I am sure that once more feeling motivated plays a large part in making one feel positive, which in turn can help the body heal. I am sure too, that my husband has played a large part in my improvement. With his constant help, and with him looking after all my dialysis requirements, which is no mean feat, I am in a much better position than I would be otherwise.
Also to the many friends, and my dear mother praying for me, I give thanks, and above all to God.
As I said in my main story, there is definitely hope out there!
Sherrill Knaggs passed away today. We will always cherish her memory and her great legacy. See our Sclero Forums: In Loving Memory of Sherrill Knaggs.
Story submitted 05-31-02.
Story posted 6-19-02.
Story update submitted 9-29-02.
Story posted 10-1-02.
Sherrill was the ISN News Guide
and an ISN Story Artist.
Read her ISN Profile!
This story is featured in the book Voices of Scleroderma Volume 2
Chronic Fatigue Syndrome
Diffuse Systemic Scleroderma
Irritable Bowel Syndrome (IBS)
ISN: Sherrill Knaggs
Microstomia (small mouth)
Renal (kidney) Involvement
Sclerodactyly (hands like claws)
Sherrill Knaggs: ISN Profile
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