Scleroderma Awareness

~~~My Blood Diseases~~~

I wanted to write a page about my blood diseases. First, I will tell you my story, then I will give you some information, that hopefully, can help someone out.

~~~My Story And Personal Thoughts~~~

In August 1997 I began having problems with my hands. It started out one day when I couldn't get my rings to fit on my fingers. At that time I didn't really think anything of it, but then I continued to have this same problem. I also noticed that my hands were swelling every day, especially after working for a few hours at the pencil factory. At night I would wake up with my arm, from the shoulder down, tingling, like a thousand little pin pricks.

This really started to bother me, so I decided to see the company Dr. I was told that, basically, there was nothing wrong with me and put on a steroid. This helped the swelling, but when the medication ran out, I had the same symptoms as before.

Confused, I went to see a family Dr. and he sent me to a specialist for Neurology. There I was diagnosed with a severe case of Carpal Tunnel after many painful tests. Things with my hands began to get better while I was off of work for a while and taking medication for that.

Then, I began to notice the ends of my finger tips turning blue at times. This is when I was suspect of Raynaud's Phenomenon. So back to the Dr. I went, this time to a Rheumatologist. On my first visit I was diagnosed with Scleroderma and Raynaud's Phenomenon. Blood test confirmed this. I went through several other tests like, a Pulmonary test to check my lungs and a heart test to see what kind of Scleroderma I had. I have what is called Localized, which only affects my hands and forearms.

I feel very fortunate, after two years, that I have been spared the pain and heart ache of having a much worse and fatal kind. My heart goes out to those who suffer from any disease. I think most of us will always question "why me?". It is only natural human emotion to feel anger, guilt, fear, hurt, rejection and depression. I went into a spell of depression for nine months, because I felt less than a person, but then I realized something that kick-started me right out of it: those who give up and give in, don't live. I wanted to live, not only for myself, but for my family. Also realizing that the Lord was in control helped me to come out of it. That's not to say that I don't still have feelings like the ones above once in a while, because I do. But I do have so much still to be thankful for!

Scleroderma is a rare disease (14 people to 1 million) and no one really knows "how" you get it. There are several studies going on to try to determine this. Genetics, emotional stability, environment, diet and several other things play a huge role in your life prior to and after being diagnosed with this disease. If you suspect that you may have one of these diseases, don't hesitate in asking your Doctor about it. Sometimes, since Scleroderma is a rare disease and the information on it is limited, it is hard to diagnose. If you have had history of Arthritis that runs in your family, it might be worth looking into if you have run into any of the following problems. I have wonderful Doctors that were able to get a diagnosis on it the first time they saw the symptoms coming on.

It is also important to have family members to understand this and what is going on with you. I have a wonderful husband who has been there for me, sometimes he has even had to do little things for me like tie my shoes. Support is of the utmost importance and having someone there to talk to. I have come to terms with many aspects of this disease by just talking to family members who really wanted to listen and be there for me. Don't be afraid to reach out, many times I have found that family and friends really do want to help in any way they can.

UPDATE!
August 2003

It has now been 5 1/2 years since I was diagnosed and a lot of things have changed in my life and to me physically. I had a baby in March 2001 and found out right after that I had another blood disorder called Factor V Leiden. You can read about it here: What Is Factor V Leiden?. This 'Syndrome' has cause a few problems for me since before we knew about it.

For a pregnant woman, this can cause sudden fetal death, stroke, increased chance of blood clots and trouble getting pregnant. I lost a baby in 1999, probably due to this. My first child was 3 months early, and very small, we think it is because of this, even though we knew nothing about it then, in 1995.

There are a few things that I have found difficult to do since becoming sick with the Scleroderma. It has been a long hard struggle for me to realize I cannot do the same things that I used to be able to do. I have made a list for those who are interested and those who may come seeking to know what may happen when they are diagnosed:

Put change in a pay phone or small slot
Button buttons
Pat out hamburger patties
Sew or cross-stitch (I used to love cross-stitching)
Fit my hands in small spaces
Pick up small things (like coins) off of counters, tables or floors
Use a can opener
Open (separate) a plastic bag
Pick up or hold large, heavy or awkward objects because I can't open my hands
Hold small objects for long periods (like seeds - I love to garden)
Use a pen or pencil
Peel potatoes
Use a standard PC mouse (I have to use a roller-ball mouse)
Turn or separate papers (as in a book or reading a letter)
Use certain silverware
Cut up my own meat
Put in contact lenses
Use tools (like screw drivers, pliers, scissors)
Open a CD case
Put on makeup or lotion
Push small buttons (like on a remote control or phone)
Put on gloves (I have to wear mittens)
Cannot wear finger nail polish (it actually makes my fingers sore)
Open an envelope

And the biggest thing of all:
My sense of touch and feel has been affected.

~~~Scleroderma-Advice, Information And Guidance~~~
~From The Arthritis Foundation, Disease Series Pamphlet~

WHAT IS SCLERODERMA?

The word Scleroderma means "hard skin." Scleroderma is a chronic (long-lasting) disease that can affect your skin, joints blood vessels and internal organs.

Scleroderma is a rare disease. It affects women three to five time more often than men. The disease usually starts between the ages of 30 and 50. It is sometimes seen in children and the elderly.

There are two forms of Scleroderma: localized and generalized (also called systemic sclerosis).

Localized Scleroderma
Localized Scleroderma mainly affects the skin. It can also affect muscles and bone, but does not affect internal organs. This form usually is not as severe as generalized Scleroderma. The two types of localized Scleroderma are morphea and linear.

Morphea refers to the type of Scleroderma in which hard, oval-shaped patches form on the skin. The patches usually are whitish with a purplish ring around them. They usually occur on the trunk, but can occur on your face, arms, legs and other parts of your body. This type of Scleroderma often improves itself, over time.

Linear refers to the type of Scleroderma in which a line of thickened skin form in areas such as the arms, legs or forehead. It can occur in more than one area. The line can extend deep into the skin and affect the bones and muscles. This can affect the motion of joints and muscles as well as growth of the affected area.

Linear Scleroderma usually occurs in childhood.

Generalized Scleroderma
Generalized Scleroderma affects many parts of the body. It may affect your skin as well as blood vessels, esophagus, stomach, bowel, heart, lungs, kidneys, muscles and joints. In rare cases, Scleroderma may affect only some internal organs, leaving the skin and joints untouched.

There are two types of generalized Scleroderma: limited (also called the CREST syndrome) and diffuse.

CREST stands for a combination of symptoms: calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly (sklare-oh-DACK-till-ee) and telangiectasia (tah-lan-jec-TAY-shah). (Each is defined later.) This type of Scleroderma usually has a slow onset, with the first symptoms appearing 10 to 20 years before the full syndrome occurs. It usually affects the skin on the face, fingers and hands. Later it may affect internal organs such as the esophagus, the lungs and the bowels.

Diffuse Scleroderma occurs throughout the body. It usually affects the skin on the arms, thighs, chest and abdomen in addition to the face, hands and thighs. Other body parts, such as your lungs, kidneys, heart, bowels, blood vessels and joints, also can be affected. This type also cause problems such as high blood pressure, muscle weakness, trouble swallowing or shortness of breath.

Diffuse Scleroderma may progress slowly in some people and more rapidly in others. However, it usually can be controlled.

WHAT CAUSES SCLERODERMA?

The cause is unknown. It is known that in Scleroderma the body produces too much of a protein called collagen. Excess collagen is deposited in the skin and in body organs. This causes thickening and hardening of the skin and affects function of internal organs. Scientists think the body's immune system plays a part in causing these excess collagen deposits.

In addition, small blood vessels are damaged by Scleroderma. There may be a connection between the build-up of excess collagen and blood vessel changes.

WHAT HAPPENS IN SCLERODERMA?

Scleroderma affects everyone differently. It can be mild or severe. Following are examples of how it may affect the body.

Skin Changes
Skin changes that can occur include:

Hardening and thickening of your skin, especially on your hands, arms and face;
Ulcers on your fingers;
Decrease in hair over the affected area;
Change in skin color.

Swelling
Swelling or puffiness on the hands and feet often happens in the morning. You may notice:

The skin on your fingers and toes looking and feeling swollen;
Your skin taking on a shiny appearance;
Usual skin creases disappearing;
Tight skin that may make if difficult to make a fist.

Sclerodactyly
Sclerodactyly means "harness of the digits" (fingers and toes). It usually occurs after the initial swelling goes away. You may notice:

The skin on your fingers and toes becoming hard and shiny;
Difficulty in bending your fingers;
Contractures in your fingers (the skin and tissues around a joint become tight and hard, causing the joint to tighten into a bent position).

Raynaud's Phenomenon
Raynaud's phenomenon is a problem of poor blood flow to fingers and toes. Blood flow decreases because blood vessels in these areas become narrow for a short time in response to cold or to emotional stress. If you are affected by Raynaud's phenomenon you may notice:

Your fingers, toes and sometimes the tips of your ears, nose or tongue being very sensitive to cold;
Your fingers turn bluish or very pale;
Tingling, numbness or a cold sensation in your fingers.
When your hands warm up, the blood vessels open and the skin color returns to normal as the blood supply to your fingers improves.
Note: If you smoke, stop! Smoking can trigger attacks of Raynaud's phenomenon.
Telangiectasia
Telangiectasia happens when tiny blood vessels near the surface of the skin become dilated and show through the skin. It involves small reddish spots appearing on your fingers, palms, face, lips and/or tongue. The spots are not harmful and can be hidden with cosmetics.

Calcinosis
Calcinosis happens when small white calcium lumps form under the skin. This is due to Scleroderma and is NOT caused by too much calcium in your diet. Calcinosis involves the formation of hard white lumps under the skin on you fingers or other areas of your body.
The lumps may break through the skin and leak chalky white liquid. They can become infected when injured or drained.

Arthritis And Muscle Weakness
Arthritis and muscle weakness also may be symptoms of Scleroderma. Arthritis occurs when joints become painful and swollen and when Contractures occur. You may notice:

Pain, swelling, warmth and tenderness in your joints;
General fatigue;
Weak muscles, often in your shoulder or hip.

Sjogren's Syndrome
Sjogren's (SHO-grens) syndrome is a condition leading to a decrease in secretions from the tear ducts, salivary glands and other areas of your body, such as the vagina. This happens if Scleroderma affects the glands that produce these fluids. If you have Sjogrens syndrome you may notice:

Dry eyes and mouth;
Dryness in the vagina (females only) or other areas of the body;
An increase in dental cavities.

Digestive Problems Scleroderma can weaken your esophagus and bowels. It can cause a build-up of scar tissue in your esophagus, which narrows the tube. You may notice that you happen to:

Have difficulty swallowing;
Have heartburn;
Experience bloating;
Feel nauseous or need to vomit;
Experience weight loss;
Experience diarrhea or constipation.

Heart And Lung Problems
Scleroderma may cause the heart to slow down and, in some case, can lead to heart failure or other problems. When the lungs are affected, they cannot function as well as in a healthy person. If you have these problems you may experience some of the following:

Shortness of breath;
A persistent cough;
Chest pain.

Kidney Problems
Scleroderma can cause high blood pressure and kidney failure. If not treated, this can be a serious problem. You should be aware of the kidney problems listed here. You may happen to notice sudden onset of:

Severe headache;
Shortness of breath;
Visual disturbances;
Chest pain;
Mental Confusion.

HOW IS SCLERODERMA DIAGNOSED?

The first step in diagnosis is a thorough physical examination and history of past and present symptoms.

Laboratory tests and other studies may be needed to help determine if you have Scleroderma. Tests may include a biopsy, in which a small piece of your skin is removed and examined under a microscope. Your doctor may check your esophagus to detect digestive problems. Tests to see whether your immune system is working normally are usually done on a small sample of blood. There also are tests that help detect early lung problems and heart involvement.

HOW IS SCLERODERMA TREATED?

Medication
Although there's not yet a cure for Scleroderma, there are many drugs that help control it. They include the following:

Aspirin may be used in large doses to treat joint pain and swelling.
Nonsteroidal anti-inflammatory drugs (NSAIDS) work like aspirin. They may be used to treat joint pain and swelling.
Steroids are man-made forms of a hormone that occurs naturally in the body. They sometimes are used to treat muscle problems.
Antacids may be used to treat heartburn and to protect the esophagus. You may also be given drugs to decrease stomach acid, protect your stomach or improve intestinal motion.
Blood pressure medication may be used to treat high blood pressure.
Drugs that increase blood flow to your fingers and toes may be used to treat Raynaud's phenomenon.

Exercise
Regular exercise helps improve overall health and fitness. For people who have Scleroderma, it also helps keep the skin and joints flexible, maintain better blood flow and prevent Contractures.

Joint Protection
Joint protection means protecting swollen and painful joints from stresses and strains that can make them hurt more. Joint protection includes learning to perform daily activities in ways that will help your joints rather than strain them. Joint protection also may include resting individual joints in removable, light-weight splints to help control inflammation.

Skin Protection
The goal of skin protection is to keep a good supply of blood flowing to your skin and to protect your skin from injury. Dressing warmly will help do this. Keeping your body warm helps open the blood vessels in your arms, hands, legs and feet. Other ways to protect you skin in include:

Use a cold-water room humidifier to keep the skin moist.
Avoid using strong detergents or other substances that irritate your skin.
Try soap, creams and bath oils that are designed to prevent dry skin.
Do not attempt to remove calcium deposits.
Enlist help from family and friends.

Stress Management

Get enough sleep. You may need to take short naps during the day.
Try to keep feelings of anxiety and fear from getting the best of you.
Express your fears and anger about what is happening to you. It often is helpful to confide in family, friends, your doctor or a counselor.
Ask you doctor to refer you to a social worker, counselor, support group or community mental health center.
Look into biofeedback training. It may help control Raynaud's phenomenon, reduce pain and promote relaxation.

WHAT ABOUT THE ISSUES OF INTIMACY AND SEXUALITY?

Scleroderma should not stop you from having a loving relationship or having sex. If fatigue is a problem either before or during sex, try making love at different times during the day. Also pace your self while making love in order to save energy.
If joint pain is a problem, try using different positions while making love. A warm bath beforehand and/or a waterbed or electric blanket also may be helpful.
Some men may have problems with impotence due to the illness. Talk to your doctor about ways to treat this problem.

WHAT IS RAYNAUD'S PHENOMENON?

Raynaud's (ray-NODES) phenomenon is a condition in which poor blood flow results in pain and skin color changes in affected parts of the body. Those parts of the body most commonly affected include the fingers, toes, ears or tip of the nose. Raynaud's phenomenon usually occurs in several episodes, with each episode lasting a few minutes to an hour or more. Symptoms generally don't occur between episodes.

When this condition occurs by itself, it is called primary Raynaud's phenomenon. When it occurs along with other diseases, such as Scleroderma, systemic lupus erythematosus, polymyositis, dermatomyositis, Sjogren's syndrome or overlap syndrome (mixed connective tissue disease), it is called secondary Raynaud's phenomenon.

This condition can begin at any age, but it usually begins between ages 15 and 40. It occurs in three to five percent of the population and is much more common in women than in men.

There is no cure for Raynaud's phenomenon, but in most cases it can be controlled with proper medical care.

WHAT HAPPENS IN RAYNAUD'S PHENOMENON?

Attacks of Raynaud's phenomenon usually occur only once in a while and usually don't cause permanent damage. Some people, however, may have frequent and severe attacks.

During an attack of Raynaud's phenomenon, the blood vessels in the affected areas-usually fingers and/or toes-become narrower for a short time. This prevents the blood from flowing freely to the affected area. As this happens, you may notice the following signs:

Change of skin color in that area. First it may turn white because there is little blood in that area. It then may turn blue because the blood remains stagnant in the blood vessels due to improper flow. Finally, it may turn red or purple as the blood begins flowing again. Sometimes your skin may only turn white or blue.
Numbness and/or coldness in that area as the blood flow slows. Pain may occur during episodes of Raynaud's phenomenon.
Swelling, tingling, aching, warmth and/or throbbing in that area as the blood begins to flow again.

Without treatment, secondary Raynaud's phenomenon can damage the affected part of the body. When blood does not flow adequately to the tissues, sores (called ulcers) may develop. If these sores are not treated, they becomes infected and may take a long time to heal.

This kind of damage rarely happens in primary Raynaud's phenomenon. Therefore, it is important to find out type of Raynaud's phenomenon you have.

WHAT CAUSES RAYNAUD'S PHENOMENON?

Doctors aren't sure exactly what causes Raynaud's phenomenon. They do know, however, that attacks are often triggered by cold temperatures and sometimes by emotion stress, such as nervousness or excitement. Using hand-held vibrating tools also can lead to Raynaud's phenomenon.

HOW IS IT DIAGNOSED?

To diagnose your condition, your doctor may:

ask questions about your symptoms (as already mentioned, Raynaud's phenomenon may occur as a symptom of other illnesses);
take blood samples to test for other illnesses that may have similar symptoms;
test your fingers to see if they change color in response to cold; show you pictures of hands taken during an attack of Raynaud's phenomenon to make sure that color changes you've observed are not a strong, yet normal response to cold (this also will help distinguish Raynaud's phenomenon from a condition called acrocyanosis, meaning blue extremities);
and/or examine your fingers under a special microscope to look for abnormal blood vessels.

WHAT IS THE TREATMENT?

The goal of treatment is to prevent attacks and prevent tissue damage if an attack does occur. This is done mainly by protecting yourself from the cold, by avoiding excessive emotional stress and not using vibrating tools. In some cases, medicine also may be used. Surgery usually is unnecessary.

Cold Protection
To avoid attacks of Raynaud's phenomenon, you should keep your entire body warm at all times. Follow these tips to keep from getting too cold:

Dress warmly, especially during cold weather and during changes of season.

-Wear layers of loose-fitting clothes.
-Wear a hat in cold weather, since much of the body heat is lost through the scalp.
-Cover your face and ears with a scarf.
> -Wear loose-fitting boots and shoes that woon't cut off your blood supply.
-Wear heavy socks or layers of socks.
-Wear thick mittens-they're warmer than glooves.
-Always keep a sweater or jacket with you, even during the summer. You may need it in cold, air-conditioned buildings.

Use flannel sheets, layers of blankets or an electric blanket on a low setting. Before you get into bed, turn on your electric blanket to warm the sheets. If your hands and feet get cold when you sleep, wear mittens and socks to bed.
Keep the rooms you use the most at a comfortable temperature.
Start running your bath or shower water ahead of time so you don't touch cold water. Keep the bathroom door closed. The steam will warm the room.
Enlist the help from family and friends. In the winter, for example, let someone else get the newspaper from out side or start the car on a cold day. Avoid reaching into the freezer or doing tasks that require putting your hands in cold water.
Wear thick gloves or mittens to reach into a freezer at home or at the grocery store.
Use insulated containers or wear a glove or mitten to hold cold objects such as ice-cold drinks, frozen desserts or fruit.
Rinse and peel vegetables with warm water instead of cold.

Skin Protection
Poor blood flow may make your skin dry. It also may cause cuts, cracks or sores to heal more slowly than normal. Follow these tips to protect your skin:

Use lotion with lanolin every day on your hands and feet. This keeps the skin from chapping or cracking.
Wash with a mild, creamy soap. Clean between your fingers and toes, but don't soak them. Examine your feet and hands daily to check for ulcers. If you develop and ulcer, keep it clean and covered. See your doctor right away.
Protect your nails. Use a lotion to keep your cuticles soft. Don't cut your cuticles with scissors or use a sharp instrument to push them back. Gently push them back with a cotton swab soaked in cuticle remover. Carefully cut hangnails and file your nails in a rounded fashion to the tips of your fingers.
Wear rubber gloves while washing dishes.
Be aware of activities that put pressure on your fingertips, such as using vibrating tools or a manual typewriter or playing the guitar or piano. They may cause your blood vessels to narrow, thus triggering an attack.
Wear clothes made or natural fibers, such as cotton and wool. These draw moisture away from you skin.

Other Tips

Don't smoke. Smoking causes blood vessels to narrow, which decreases the blood flow.
Try to avoid emotional stress. When possible, avoid situations that may make you too excited, nervous or frightened. Ask you doctor about relaxation techniques that may help you reduce stress. Also, look into biofeedback training, which can help you learn to control you body temperature. For more information, contact your local Arthritis Foundation chapter and request a copy of the brochure Managing Your Stress.
Be aware of certain drugs that may cause your blood vessels to narrow. Some of these drugs are listed in the following table. Always tell your doctor that you have Raynaud's phenomenon, and discuss any over-the-counter or prescribed medicines with them. For example, even over-the-counter cold remedies can aggravate Raynaud's phenomenon. Work in partnership with your doctors to determine which drugs are most suitable for you.

**Drugs That Can Aggravate Raynaud's Phenomenon**
TYPE OF MEDICINE	USE	GENERIC NAME	BRAND
Vasoconstrictor	Headaches	Ergotamine	Ergomar, Ergostat, Cafergot, Ergotamine
Beta Blocker	Angina, High Blood Pressure, Migraine Headaches	Acebutolol, Labetalol, Metroprolol, Nadolol, Propandalol, Timolol	Sectral, Normodyne, Trandate, Lopressor, Corgard, Inderal, Inderal LA, Blocadren
Adenergic Stimulator	Colds, Respiratory Symptoms, Diet Aids	Phenylpropanolamine	Nadelcon, Triaminic, Sudafed, Various Cold Tablets

Medications
In severe cases, Raynaud's phenomenon may be treated with drugs that keep your blood vessels from narrowing and help them dilate, such as nifedipine, diltiazem or nitroglycerine.

Some of these medications may have side effects, such as: headache, palpatations, light-headedness or dizziness, skin rash, swelling of the face or other parts of the body, dry cough, loss of taste, nausea, unusual tiredness or others. Contact your doctor right away if you have difficulty breathing, fever or chills, or swelling of the face, mouth, hands or feet.

Topical medications, such as nitroglycerine ointment, also may be used.
Discuss all medication and there side effects with you doctor.

Surgery
In rare cases, a sympaththectomy may be done. This operation cuts the nerves that may be making the narrowing of the blood vessels worse. This procedure usually is not necessary and may only work for a short time.

WHAT SHOULD I DO IF I HAVE AN ATTACK?

If you have an attack, stay calm and follow these steps:

Gently warm you fingers or toes as soon as you can. Placing your fingers under your armpits often helps.
Wiggle your fingers or toes. Move or walk around to try to keep your blood flowing freely.
When you hands start feeling cold, hold them above you head. Then swing them around as if you're throwing a softball.
Run WARM-not hot-water over your fingers or toes until their normal color returns. Do not use a hot water bottle or heating pad, because they may damage your skin.

FOR MORE INFORMATION
Contact your local Arthritis Foundation chapter for a list of free brochures and detailed information about arthritis and its treatments. Their services may also help you.

Services

Arthritis Self-Help Course-Learn how to take control of your own care in this six-week (15 hour) class for people with arthritis.
Warm-water exercise program-Join in the fun of a six-to 10-week exercise program in a heated pool.
Land-exercise programs-Move easier in several levels of exercise classes, or exercise at home by purchasing and Arthritis Foundation exercise videotape.
Support groups and clubs-Share your successes and problems with others, and get tips on how to overcome problems caused by arthritis.