Exciting News in the Fight Against Scleroderma

                                                        March 10, 1941 to October 16, 2006
                                              Rachelle (Shelly) Hollander in an undated photo

Sexuality and Scleroderma

Sexuality is much more than having sexual intercourse.  A person's sexuality is part of how they think about themselves and their desirability to others.  It encompasses the person that you think you are, your body, how you feel as a man or a woman, the way you dress, move, and speak, the way you act, and how you feel about other people.  In other words, sexuality is not only physiological, but also psychological, involving both the body and the mind.  Along with all of the other things that scleroderma may have taken away from you, your ability to have satisfying sex might seem to have been taken away too.  Let's be honest - tight skin, dry mouth, curled fingers, painful joints, heartburn, and fatigue can make it difficult to feel sexy.  However, there are ways for those living with scleroderma to have pleasurable sex despite the symptoms of the disease.

Pleasurable experiences of any kind help increase your quality of life.  Pleasure decreases pain, increases self-confidence, and increases optimism.  Satisfying sex can do even more by increasing exercise, endorphins, healthy sleep, and blood circulation to the extremities.  It is still possible for sex to be enjoyed comfortably if a couple takes advantage of the times when the person with scleroderma is feeling good, and by making whatever adaptations are necessary to relieve the physical aches and pains.  Communication between partners is essential.  It is important to convey sexual feelings whenever they do occur in order to maintain sexuality in a relationship.  Even if that sexuality is only expressed in affection, it is imperative that both partners express their love any way that they can, and realize that a decrease in sexual activity does not mean a decrease in love.

The physical symptoms that most affect sexual activity are the tight skin of the hands and face, pain, fatigue, dry mouth, and vaginal dryness.  For men with scleroderma, erection problems can occur due to the decreased blood flow to the penis and penile malformation due to tight skin.  The psychological issues include a decrease in sexual desire due to the emotional distress and mental preoccupation related to living with the disease, depression, changes in body image, and anxiety that sex will be painful.  The male partners of women with scleroderma can also be affected by the attitude of increased anxiety and retreat from sex for fear of causing pain and discomfort in the women they love. 

There are other physical changes, unrelated to the genitals, that can affect those suffering from scleroderma.  There can be stiffness of the muscles and joints which can limit sexual movement and position.  Stiff fingers can be clumsy and insensitive.  If the fingers are curled or bent, fondling and caressing may be very difficult.  Thin lips, small mouth, or protruding teeth can make kissing less pleasurable.  Self-consciousness due to changes in the texture of the skin and body contours can lower sexual desire.  A woman might feel unattractive and sexually undesirable, which can lower her sexual desire.  If her partner is worried about her, or depressed about her illness, he may be too preoccupied with her problems and may conceal his desire for sex.

There are many ways for those living with scleroderma to get their sensuality/sexuality back.  To address the psychological issues, resources such as couples therapists, counselors, and physicians specializing in sexual dysfunction can be explored.  Male erection problems, which can be due to psychological or physiological causes, can be treated by psychotherapy, testosterone therapy, or mechanical erection aids.  To reduce vaginal dryness for women with scleroderma, either estrogen replacement hormones in a pill form or an estrogen-containing vaginal cream can be used.  A warm bath can be used to help loosen up stiff joints.  Counselling can be effective way to deal with the depression, anger, fear, anxiety, isolation, and loneliness that a person living with scleroderma may be experiencing.  Again, communication between partners is the most important aspect of having a healthy sex life, with scleroderma or without.

It is also important to know that sometimes the very medications that are needed to treat the symptoms of scleroderma can get in the way of physical intimacy and pleasure.  Talking to your doctor about these side effects that may be affecting your sex drive or physical comfort may lead to possible remedies. 

The following are some ways that you can help ensure that you are physically comfortabel enough for sexual activity:

• Attitude is everything.  Intercourse is not the goal;  being together and feeling close is the goal.  The sexiest part of your body is your mind.
• Communication between you and your partner is key to experimenting with new ways of making love.
• Think about having intimacy for intimacy's sake.  A cuddle can be almost as loving as sexual intercourse.
• Get away for a couple of days so that you can reconnect and get away from everyday life.
• Take your medications at least 30 minutes before.  This can add to your comfort and reduce your worry about your physical symptoms.
• Take a warm bath, light candles, put on music.  In other words, set the mood.
• Use Replens or KY Jelly for moisture.
• Use helpful devices for sex the same way use devices to help you in other aspects of your life.
• Stretch the vagina with lubricant.

Sexuality can be affected by scleroderma in both men and women, however, it is not often spoken about because of the embarrassment.  A high quality of life includes sexuality and sensuality, as well as pain control, disability control and reduction of acute symptoms.  The same skills that you have learned to cope with scleroderma, such as gathering information and reaching out to support groups, can also be used to help you improve your sex life. 

I would like to acknowledge that the information for this posting came from the Raynaud's & Scleroderma Association (http://www.raynauds.org.uk/) and from Elaine Furst, R.N, MA, BSN (http://www.scleroderma.org/medical/sexuality/furst06_2.shtm).  Please visit these websites for further information.

Scleroderma Message Board

I wanted to take this opportunity to thank all of you who have read my posts.   I have been receiving wonderful comments and hearing stories of both triumph and tragedy from others who have also been touched by this disease.  I have also started a message board at http://www.boards2go.com/boards/board.cgi?&user=ljs0415
It would be great if more people would share their own personal experiences, or even ask questions.  Please  share this blog and message board with your friends.  And most importantly, if you or someone you know has suffered from Scleroderma, or you just empathize with those who have, please make a donation to the Scleroderma Foundation.

Thank you all for your support.

Kids Get Scleroderma Too.

While the average age of a person who is diagnosed with scleroderma is in the forties, the disease can develop and is found in every age group, from infants to the elderly, according to the Scleroderma Foundation (http://www.scleroderma.org/medical/overview.shtm).  The systemic form is more common in adults, while children are usually diagnosed with localized scleroderma.  That means that the disease is most often localized to the skin, muscles, bones, and/or joints, and does not usually affect the internal organs.

Factors other than sex, such as race and ethnic background, may influence the risk of getting scleroderma, the age of onset, and the pattern or severity of internal organ involvement. The reasons for this are not clear. Although scleroderma is not directly inherited, some scientists feel there is a slight predisposition to it in families with a history of rheumatic diseases.  Doctors say that there are more exceptions than rules to scleroderma - even more so than other diseases.  The first step is to get a proper diagnosis, including which form of the disease the child has.  The localized form rarely, if ever, turns into the systemic form.  While scientists do not yet know what causes scleroderma, it is known that it involves an overproduction of collagen. 

Most patients do not have any relatives with scleroderma and their children do not get scleroderma. Research indicates that there is a susceptibility gene which raises the likelihood of getting scleroderma, but by itself does not cause the disease. 

There are three types of localized scleroderma:  morphea, linear scleroderma, and scleroderma en coup de sabre.  Of these, morphea is the most common.  It consists of irregular patches of skin that appear on the arms, legs or body.  These patches usually start out small and pinkish in color and are not noticeable until they reach the size of a penny.  Since many superficial skin infections or irritants can cause pink lesions, a variety of creams can be tried begfore the child is referred to a dermatologist.  However, over time, the center of the lesions may become pale, dry and hard.  This often prompts a referral to a dermatologist and a diagnosis of scleroderma.  In most cases, these lesions are primarily cosmetic.  Morphea does not usually appear on the face, and most are easily hidden under clothing.  As a result, morphea is more of an emotional issue.  The long term prognosis for children with morphea is excellent.  Initially the lesions may continue to enlarge and increase in number, however, they will soften and darken over a period of years.

Linear scleroderma is a condition where the areas of skin involvement seem to spread out along lines.  Instead of a round area of skin, like with morphea, there may be a streak of involved skin.  The involved skin can be on the arm, leg, hand, foot, chest or abdomin.  Some children have both linear scleroderma and morphea.  Areas of linear scleroderma that cross a joint, such as the elbow, wrist, shoulder, knee, ankle, or finger, can cause permanent damage.  As the skin becomes tight, and the lesions harden, they can limit the ability of the joint to move.  In a young child, when large areas are involved, the arm or leg may not grow appropriately.  Some children with linear scleroderma also have muscle inflammation, called sclerodermatomyositi.  The prognosis for children with this type of localized scleroderma is also excellent, provided that they receive the appropriate treatment.  There still could be permanent damage if there was either no treatment, or the disease did not respond to the treatment.

Linear scleroderma en coup de sabre is the diagnosis when children have linear scleroderma on the head and/or face.  These children sometimes have a deep furrow along the scalp with tight, hard skin that often extended onto the forehead.  Sometimes, as in the case of children that have Parry Romberg syndrome, in addition to the lesions seen in linear scleroderma, may have involvement of the whole side of the face, and sometimes the tongue.  The prognosis for this type of scleroderma is mixed.  If the lesions are confined to the scalp and forehead, they often evolve similarly to linear scleroderma and the effect is mostly cosmetic.  The same is true for isolated areas of involvement on the face.  However, Parry Romberg syndrome can present mre serious difficulties because the bones may not grow properly.  It is very important for parents to remember that involvement of the face is going to be obvious to both the child and to others.  These children need extra support in understanding that they did not cause their conditon and in learning how to deal with the disease, and with others. 

Appropriate treatments remain controversal, and,  because the disease is relatively rare, there are few studies being done.  For children with mild morphea, topical treatment with calcipotriene cream or ointment during the active phase is often sufficient.  Linear scleroderma usually responds well to methotrexate, and may also be used for children who have widespread morphea.  Linear scleroderma that does not cross a joint line usually does not require any treatment.  However, when large areas of an extremity are involved, or the disease is crossing a joint line, there is significant risk of permanent damage.  In these cases most specialists will recommend methotrexate, which can slowly soften the involved skin.  Like all other medications, methotrexate has possible side effects, so careful consideration must be given to the expected risks and benefits.  The use of methotrexate for children with linear scleroderma en coup de sabre is still controversial. 

For children with the systemic form of scleroderma, which again, is extremely rare, the prognosis is not as good.  Juvenile systemic sclerosis (JSS) is a multisystem connective tissue disease characterized by skin fibrosis and internal organ involvement.  Skin and vascular manifestations are the most common clinical features, while internal organ involvement is is more rare.  Cardiopulmonary disease is the most frequent visceral involvement, leading to significant morbidity.  This girl, who is nine years old, has had systemic sclerosis for five years.  Tightening of the skin is obvious, and she is unable to close her lips.  In addition to cardiovascular involvement, the disease can also involve the respiratory system, the gastrointestinal system, musculoskeletal, and renal function.


For children with diseases, the psychological effects can be more devastating than the physical.   Children with chronic illness will very often wonder what they did wrong.  Open discussion and reassurance among family members may be sufficient, but sometimes professional help is warranted.  No one should be afraid or ashmaed to seek the help tht they need to deal with the stress of chronic illness in themselves or a family member.  If allowed to smolder, the stresses of chronic illness can lead to noncompliance by the patient and the can even worsen the disease outcome.  Divorce, suicide, and other negative behaviors can all occur with increased frequency, not only in the children themselves, but also in all of their family members.

Donations are needed in order to increase the amount of research for this heartbreaking disease.  Please go to http://www.jsdn.org/ to learn more about this disease and to help the cause.  This worldwide non-profit children's organization is based all on volunteers.  Zero funds go to administrative costs, and this organization does not receive any government funds.  They rely on the support of private donations.  Please give to end the suffering of children living with juvenile systemic sclerosis. 

The Face of Scleroderma

My first post on Scleroderma was an attempt to raise awareness for this unfamiliar killer.  This post will put a name and a face on this devastating disease.  The name is Rachelle (Shelly) Sandra Hollander, and she was my mother.  She lived with this disease for twenty-six years.  It finally killed her on October 16, 2006.  Scleroderma is ruthless.  It is sadistic.  Worst of all, it destroys lives. 

Shelly was diagnosed with Systemic Scleroderma in the 1980's when she was in her early 40's.  The first symptom was a persistent cough and poor circulation in her extremities.  After several misdiagnoses, she was informed that she had the fatal form of the disease.  Shelly was given five years to live.  The doctors underestimated her strength, determination, and her will to live.  She faced every new obstacle with grace and courage.  And the obstacles were brutal.

As the years went by, the cough became much worse.  Shelly could barely laugh anymore because, when she did, she started coughing so badly that she could not catch her breath.  The skin on her face was beginning to get pulled tight, which, to her embarrassment, made her smile appear deformed.  The skin on her fingers and toes were being pulled so tightly that they began to bleed.  Her fingers became so deformed that she had difficulty doing a lot of the everyday things.  Shelly was no longer able to use a can opener to open the cat and dog food cans for her precious pets.  She could barely turn the pages of the magazines that she had loved to read.  She could not stand the cold weather anymore because of the poor circulation in her extremities.  All the while, the cough continued to get worse. 

Shelly was taking numerous medications each day.  There was Nexium for the persistent heartburn caused by the scarring of her esophagus.  There was Oxycodone for pain, Xanax for anxiety, prednisone (a steroid) for her breathing, Procardia (a calcium channel blocker) for pulmonary hypertension, and Albuterol for asthma-like symptoms.   These are just the ones that I know about.  I am pretty sure that there were more.   None of these were meant to cure her, or stop the disease from progressing.  They were all just supposed to help her get by.

Walking became more and more difficult with the progression of the disease due to the damage to her lungs.  A CT scan showed that my mother's lungs were as black and damaged as that of a life-long smoker, despite the fact that she had never smoked a cigarette in her life.  Shelly had more and more difficulty eating because her esophagus was narrowed due to the scar tissue caused by the scleroderma.  Her heart was also greatly damaged by the disease.  A few years before she died, Shelly's doctor suggested she go on the list for a heart/lung transplant.  She would not hear of it.  I am not really sure is she was against it for religious reasons, or if she was just getting tired of fighting.  But fight on she would, for several more years.

For a few years, towards the end of her battle, Shelly would have to be connected to an oxygen machine when she was at home.  After a while she could no longer breathe without it, and had to carry a portable machine everywhere she went.  She spent more and more time in bed, and less up and about.  Perhaps the worst thing that Shelly had to endure was the pain from her fingers and feet.  The skin was being pulled so tight that it was cracking.  Her fingers were raw.  You could barely touch her hand without her recoiling them in agony.  Creams, lotions, prescription medications - none could help alleviate the pain.  Eventually we had a twice-daily ritual of wrapping her fingers in bandaids and gauze pads.  Having them covered seemed to offer some slight relief.  There are even more symptoms that I can tell you about.  Here is a list of possible symptoms that a Scleroderma patient may experience, according to the Cleveland Clinic.   My mother had them all.

·         Swelling of the hands and feet

·         Red spots on the skin

·         Excessive calcium deposition in the skin

·         Joint contractures (rigidity)

·         Tight, mask-like facial skin

·         Ulcerations on the fingertips and toes

·         Pain and stiffness in the joints

·         Persistent cough

·         Shortness of breath

·         Heartburn

·         Difficulty swallowing

·         Digestive and gastrointestinal problems

·         Constipation

·         Weight loss

·         Fatigue

·         Hair loss

·         Possibly Raynaud's Disease (narrowing of the blood vessels)

·         Possibly Sjogren's Syndrome (chronic dryness of the eyes and mouth)

Then there were the side effects of all of the medications that she was on.  The worst side effect was a result of the steroid Prednisone that she was taking to help her breathing.  Prednisone, if taken for an extended period of time, can weaken the bones.  A few weeks before my mom passed away she complained of having an even harder time breathing than usual.  She was in terrible pain and could not get any relief, even when she was completely immobile in bed.  We took her to Stony Brook University Hospital on Long Island and it was discovered that her cough had caused a few of her ribs to collapse, leaving them pressing against her lungs.  The prednisone was to blame for making her ribs so weak.  Shelly would leave the hospital after a few days with nothing more able to be done other than more Oxy. 

Now, I don't want to give the impression that, with all of this physical and mental pain caused by Scleroderma, that life was not worth living for my mom.  She would have told you that she still cherished life.  She had so much to live for.  And don't forget, Shelly was a fighter.  She had two daughters and four grandsons to love.  And she had lessons to teach.  She taught me what real courage is.  She taught me never to take anything for granted.  And she taught me to laugh, despite everything.  So we tried to joke about Scleroderma as much as we could.  I would tell her that she would make the perfect criminal since she no longer had fingerprints.  She would tell me that she would always look years younger than her actual age because the skin tightening on her face smoothed out any wrinkles that she may have had otherwise.   Some days we were able to joke about the disease.  Most often, not.

Shelly died on October 16 at around 6:00 AM.  I got the call from the hospital while I was taking a shower.  For the past two days I had been working with my sister to have my mother brought home with a full-time nurse.  I knew that she wanted to be home, although she was almost completely out of it by this time.  She had been in and out of the hospital for months by this time.  I did not want her to die alone, but she did.  We could not get her home in time.  That is one of my greatest regrets.  Of course, I visited her at the hospital every day, but that does not even ease the pain of her being all alone in a cold hospital room when she passed.  The actual cause of my mother's death was listed as Pulmonary Hypertension, but the underlying cause was Scleroderma.

I write this blog in the hope that you will want to help by donating to this cause.  In the hope that it is more real to you now that the disease has a name - and a face.  The face of Shelly Hollander - my mother, and an inspiration to us all.  Please visit http://www.scleroderma.org/ to help eradicate this unfamiliar killer.

Current Treatments for Scleroderma

While there is presently no cure for Scleroderma, there are several treatment options available that can enable those afflicted with the disease to alleviate some of the symptoms that it causes.  Such treatments depend on the subtype of the disease, the stage, and the affected organs, since no two cases of Scleroderma are exactly alike.  The current therapies focus on the four main complications of the disease, which include autoimmunity, inflammation, vascular disease, and tissue fibrosis. 

In order to address the autoimmunity issue, immunosuppressive therapy is most often used.  Researchers believe that the autoimmune process is causing inflammation, resulting in tissue damage and fibrosis.  They think that the fibrosis is an "innocent bystander" that is being driven by the cytokines (chemical messengers) that are produced by the immune system.  There have only been a few well designed studies into this type of therapy, which is one reason why a $1 million/year research budget is not nearly enough.  However, several drugs are currently being used to suppress the immune system.  These immunosuppressing medications include methotrexate, cyclosporine, antithymocyte globulin, mycophenolate mofetil and cyclophosphamide. A recent study suggested that methotrexate did not significantly alter the skin score (a measure of skin thickening) compared with placebo.  Cyclosporine has not been completely studied because of reports of renal toxicity. The most promising drugs are mycophenolate mofetil or cyclophosphamide.  Unfortunately, there is no placebo-controlled study to define their exact role in treating scleroderma, however, if used during the active inflammatory phase of the disease, they seem to be effective.  In very severe cases of Scleroderma, the use of aggressive immunosuppressive therapy with very high doses of cyclophosphamide or with autologous bone marrow transplantation can be used, however, this treatment has potential risks. 

The complication of inflamation can come in two forms in a patient with Scleroderma.  The more conventional type can cause arthritis (inflammation in the joints), myositis (inflammation in the muscles), or serositis (inflammation in the lining of the heart or the lining of the lung).  The most often used drug therapy for this symptom is a traditional anti-inflammatory drug, such as NSAIDs, like ibuprofen, or corticosteroids, like prednisone.  The other type of inflammation relates to the skin and other tissue injury caused by the Scleroderma process.  This type does not seem to respond to NSAIDs or corticosteroids.  These medications also have serious potential side effects, including gastrointestinal disease, fluid retention, and renal disease.  Prednisone is also known to cause bone weakening, which is why my mother's ribs collapsed onto her lungs just weeks before she passed.

The vascular disease caused by Scleroderma affects both the medium and small arteries.  Low blood flow into the skin and tissues is believed not only to damage tissue by the lack of nutrition and oxygen but also to activate fibroblasts and promote tissue fibrosis. Therefore, treatment of the vascular disease is now considered crucial to controlling the disease as a whole as well as preventing specific organ damage. There are three major features of the vascular disease that potentially need treatment: vasospasm (spasm of blood vessels), a proliferative vasculopathy (thickening of blood vessels), and thrombosis (blood clots) or structural occlusion of the vessel lumen (blockage of blood vessels).  Calcium channel blockers, like nifedipine, a vasodilator (opens up the blood vessels) is effective in helping blood flow to the skin and the heart.  Epoprostenol improves blood flow in the lungs.  There is evidence that these vasodilators directly affect tissue fibrosis.  Left untreated, scleroderma vascular disease can lead to thrombosis or advanced fibrosis, causing the occlusion of the vessels.  To deter this, antiplatelet or anticoagulation therapy using low-dose aspirin is recommended. 

For the fourth main complication of the disease, tissue fibrosis, there really is no good therapy that can be offered currently.  In Scleroderma, excess collagen is produced in the skin and other organs.  Several medications were being used, such as colchicine, para-aminobenzoic acid (PABA), dimethyl sulfoxide, and D-penicillamine, however, experts are not convinced of their effectiveness.  The search for new drugs that alter the fibrotic reaction is currently one of the most active areas of scleroderma research.  Strategies include directly suppressing the fibroblast and its ability to make collagen, inhibiting the cytokines that activate the fibroblast, and the use of agents that might break down collagen faster and promote tissue remodeling.

Much more research is crucial to, not only discovering new therapies to combat the symptoms of the disease, but, ultimately to find a cure. 

For more information, read

http://www.hopkinsscleroderma.org/patients/scleroderma-treatment-options/

http://www.hopkinsscleroderma.org/downloads/SystemicSclerosis_Chapter23.pdf

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Education, Support, and Research for Scleroderma

Scleroderma is a chronic auto-immune disease that involves the skin and connective tissue.  According to the American Autoimmune Related Diseases Association, Inc. (AARDA), “There are two types, localized and systemic. In the localized type, the skin shows one or more patches of sclerosis (thickening and hardening). The systemic type involves the skin and the connective tissue…Systemic scleroderma involves body systems such as the esophagus, intestines, lungs, heart and kidneys. Diffuse scleroderma is a term which describes systemic sclerosis and skin changes on many parts of the body. Tight, glossy skin may be present on the trunk and upper arms as well as on the face, chest and extremities”.  Scleroderma causes a buildup of scar tissue and can damage internal organs (Scleroderma Foundation).  This is a heartless, little-known disease that, while it can be found in men, typically affects women in their thirties and forties (AARDA).  My mother was one of them.  She was diagnosed in the mid-1980’s when she was approximately 40 years old.  She was told that she had the systemic type, and that the prognosis was not good.  According to Daniel Furst, M.D., those with the severe cases of the disease, like my mother, have a 50% chance of living for five years.  My mother managed to beat the odds – she was able to live with the disease for over 26 years, before finally succumbing to it in October of 2006.  By the time the disease had progressed my mother was a candidate for a heart-lung transplant because her's had been so badly scarred and damaged. 

              

  “Scleroderma is not contagious; it is not infectious; it is not cancerous or malignant. There are an estimated 300,000 people in the United States who have scleroderma, and one third of whom have the systemic form of scleroderma. Since scleroderma presents with symptoms similar to other autoimmune diseases, diagnosis is difficult and there may be many misdiagnosed or undiagnosed cases as well."  While treatment is available for some forms of the disease, scleroderma is not yet curable.  Due to the fact that scleroderma is a relatively rare disease, it has not gained worldwide recognition; therefore, the research budget for this disease is relatively small.  For example, the Scleroderma Foundation, which is a leading nonprofit supporter of scleroderma research only has an average of $1 million a year to find the cause and cure for the disease.  In fact, according to the Scleroderma Foundation, “The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), a part of NIH, provides funding for the majority of scleroderma research. Although NIH grants to scleroderma researchers have increased in recent years, scleroderma funding is still a relatively low priority.  The Scleroderma Foundation has a critically important role as catalyst—to fund and to stimulate new research and new ideas.  The Scleroderma Foundation is enlisting the support of legislators and other decision-makers to establish a higher profile for scleroderma and the needs of patients. The key to all our efforts—to ensure that productive research moves forward—is the continued generosity of our individual and corporate donors”.

  The Scleroderma Foundation’s goal of providing education, support, and research is admirable, and I know personally that it had helped my mother in the past to have an organization that helps patients and their families to cope with the disease, while helping to promote public awareness.  However, they cannot do it alone.  A yearly budget of $1 million is not going to get the job done.  Much more money for research is needed in order to find a cure.  My goal is to help spread the word about this terrible disease that I know only too well.  By getting people to understand the devastation that it causes, I hope to increase awareness and hopefully encourage people to make donations to help in the research.  My mother’s life after she was diagnosed with scleroderma was filled with pain – both mental and physical.  While she rose to the challenge like a champion, my wish is that nobody will have to go through what she went through for more than two decades