Newsletter Fall 2011

Scleroderma Association of Saskatchewan Newsletter – Fall 2011


Issue #47

President/Fundraising:
Louise Goulet: Phone: 584-1950

Vice President:
Shirley Gillander: Phone: 525-6377

Recording Secretary:
Olga Sereda: Phone: 693-1936

Corresponding Secretary:
Norma Redman: Phone: 532-4449

Newsletter co-Editor:
Janet Campbell: Phone: 757-0962
janet.campbell@uregina.ca

Treasurer/Newsletter co-Editor:
Gerald Shauf: Phone: 634-3433
465 WillowBay
Estevan, Sask.  S4A 2G3
glshauf@sasktel.net

CALENDAR OF EVENTS

MEMBERSHIP FEES:  If you are receiving this newsletter by mail, please check your mailing label to see what your membership status is.  If it says PD12, you are paid for 2012. If it has X, your 2012 membership is valid as a result of a donation.  If there is nothing after your name, you have not paid for your 2012 membership.  Please remit $20.00 fees to SASK c/o Gerald Shauf at the above address if you have not already paid for 2012.  If you are receiving this newsletter by email and don’t know whether you have paid or not, please contact me.  Income tax receipts will be issued for memberships and all donations. Thank you for your support.

SPRING 2012 MEETING:

The spring meeting of the Scleroderma Association of Saskatchewan will be on Saturday April 21, 2012 at the Wascana Rehabilitation Centre.

DONATIONS:  The Scleroderma Association of Saskatchewan has received some notable donations that need to be recognised. In 2010, as a memorial donation to Diane Krafchuk, late of Estevan,SK, The Fraternal Order of Eagles – Auxiliary #4126 in Regina made a donation of $410.00.  This year, they donated $395.40.

A number of years ago, employees of SaskCentral, Credit Union Deposit Guarantee Corporation and Concentra Financial (Reginalocation) implemented a corporate-wide initiative to raise funds for charities.  In 2010, the employees donated $472, which was then matched by SaskCentral for a total of $944.  This year, the employees donated $440, which was matched by SaskCentral for a total of $880.00.

In 1950, SaskTel employees and SaskTel formed a partnership called TelCare.  The employees donate funds, which are 50% matched by SaskTel.  Since 2001, TelCare has donated $1556.00 and SaskTel has donated 50% of that amount for a total of $778.

The Regina United Wayand Donor’s Choice from Muenster, Gainsborough, Broadview and Edamhave collected donations, which have been sent to us for several years. For the past two years, Dr. Markland has held an annual Garden show.  Last year, she raised $727.00 and this year, donations in the amount of $475.00 were received.  Dr. Markland also donated $54.00, which matched the sale of Scleroderma pins and magnets.

In 2010, the Taoist Tai Chi Society of Canada–Regina, Branch, held a Chinese New Year’s celebration banquet and donated $1800 to SASK.  Several years ago, when Linda and I were vacationing at our daughter’s home in Chandler, AZ (a city near Phoenix), one of Linda’s on-line Scleroderma/Sjogren’s friends, JoAnn Snider, drove from Hemet, CA (over 5-hour drive) to meet and have lunch with us.  Since Linda’s passing, she has sent $100 every year as a memorial to Linda.

As a memorial to Linda, I have donated $1000 per year to SASK for the past 5 years.

I would also like to note that several of our members have made donations to enable us to promote research into Scleroderma. 

DISCLAIMER
The Scleroderma Association of Saskatchewan does not endorse any drugs or treatment. We wish only to keep you informed. Check any treatment with your own physician.

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 Christmas is not what you get but whom you are with.

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 Saskatchewan Parking Program for the Disabled:
Ph: (306) 374-4448

Handicapped parking permits now cost $10.00. Successful applicants receive parking placards allowing them to park in designated areas for handicapped. Applications are available at any branch of Saskatchewan Abilities, or any motor vehicle issuing office.  Ask your doctor if you qualify. Try to look at getting the placard as a way to make your life easier.

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In the eyes of children, we find the joy of Christmas.  In their hearts, we find its meaning.There is no greater gift at Christmas than to have everything you want before you open the gifts.

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Perhaps the best Christmas decoration is being wreathed in smiles.

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President’s Message

Today is the middle of November and it looks like ‘Old Man Winter’ is here to stay. For those who suffer during cold weather that is not good news.   I think we should hold a meeting in January – on beach chairs, with our feet covered in warm sand and ocean waves kissing our toes.  How about Hawaii?

Our fall meeting was on October 15th with 16 members and guests in attendance.  We welcomed Doreen, a new member who travelled all the way from LaRonge. Dr. P. C. Patel was our guest speaker.   He spoke of the effects scleroderma has on different organs in our body with particular attention to the lungs.

The Scleroderma Society of Canada Annual Conference was held in Quebec City this year.   Shirley, Roberta and I attended this event at the end of October.  We were able to attend several workshops with presenters that have worked in scleroderma research or medical doctors, particularly rheumatologists that have cared for scleroderma patients.  We hope to share gathered information at our April meeting.

I have included a letter from Dr. Murray Baron, M.D. who was instrumental in starting the Canadian Scleroderma Research Group.  If you have not joined this registry and wish to do so, please contact me at (306) 584-1950.  I encourage all those with scleroderma to do so.   I believe we must be our own advocates in seeking ways to help ourselves and strive to learn as much about this disease as we can.

I wish you many Blessings and Joy during the Holiday Season.  Be sure to rest, relax and take time for yourself.   Keep warm and stay healthy.

Louise

CSRG  GRCS

Canadian Scleroderma Research Group –Groupe de recherche canadien sur la sclérodermie

October 28, 2011

Dear Scleroderma Society of CanadaAnnual Conference Attendees,

Once again, the Canadian Scleroderma Research Group (CSRG) will be holding their scientific meeting alongside the meeting of the Scleroderma Society of Canada.

Our goals have always been to seek answers for you.  We have approached the problem of scleroderma, both from a clinical and a laboratory approach.  Since we started in 2004, we have enrolled over 1,350 patients in the CSRG.  We have trained many students and hopefully have planted seeds that will grow into new scleroderma researchers in the future.  We have published numerous scientific papers in respected journals and presented dozens of papers at important international scientific meetings.  Our Group has become one of the most important players in the world in this field of research.  The generous support of the patient organizations such as Scleroderma Society of Canada and all its provincial Chapters, Scleroderma Ontario, Sclérodermie Québec & Cure Scleroderma Foundation have been essential ingredients of our success.

We have several laboratories working on the very basic aspects of scleroderma in an attempt to unravel its causes and hopefully find a cure based on a true understanding of the disease.  At the same time, we recognize that such a cure may be years away and we are also working hard on trying to find ways to help patients better cope with this illness.

In particular, the Scleroderma Society of Canada has been a key player in our development.  They were there from the very beginning.  Without this organization’s help and support we never would have been able to reach the goals that we have already achieved.

And of course without the help of the patients who have participated in the CSRG projects we also would still be at square one.  Instead, with your help we have formed an incredibly productive and one-of-a-kind partnership.

As always, we are very happy to be able to have our meeting alongside yours and to be able to share with you some of the work that we have done in the past year.

Enjoy the conference!

Dr. Murray Baron, M.D.

CSRG Director & Division of Rheumatology Chief
SMBD – Jewish General Hospital
3755 Cóte Ste-Catherine Road, Room A725,
Montreal, QC, CANADA
H3T 1E2,
Fax: 514-340-7906; Tel 514-340-8231

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Minutes of October 15, 2011 Meeting

The fall meeting of the Scleroderma Association of Sask. was held at the Wascana Rehabilitation Centre in Regina on Oct. 15, 2011 with 16 in attendance.

President Louise Goulet called the meeting to order at 10:25 a.m. and asked for brief introductions around the table.

The agenda was adopted with two additions, to discuss where to donate our funds this year, and what to give as an honorarium to our guest speaker.  Louise Goulet declared the minutes of our spring meeting adopted as read.

In Business arising from the minutes, the Scleroderma banner was repaired and displayed in Regina in July instead of June.  It is likely that most people who see the banner don’t know what Scleroderma is, and as it faces east, it probably isn’t as easily seen now that 11th Avenue is a two way street.  No bill for the display has been received to date from the City of Regina.

No one has contacted Global TV about interviewing someone from our group.  Other possibilities for getting some exposure are by Public Service Announcements on CKCK, Access TV and perhaps the Leader Post.  Roberta Nichol will take charge of notifying these outlets of our meetings.

There has been nothing definite re: collaborating with the Lupus Society in fund raising efforts.  The Scleroderma Society of Canada has not decided on use of the sunflower as a national symbol.  Ovarian Cancer Canada uses the sunflower as its symbol.

The Treasurer’s report shows a bank balance of $20,038.21.  In response to a question, Gerald Shauf explained why we have several accounts.  Moved by Gerald Shauf, seconded by Joyce Kellington that the report be adopted as printed.  Carried.

There was no correspondence.

In new business, the subject of how we can better spread awareness about Scleroderma was discussed.  Members are finding that even medical personnel know nothing about our disease.  Dr. Markland will be asked if she could speak to medical students about Scleroderma if she is not already doing so.  It was suggested that we could take part in volunteer fairs.

Suggestions for disbursement of our funds were to donate to CSRG and SSC.  After some discussion it was moved by Ken Goulet, seconded by Andy Sereda that we donate $10,000.00 to CSRG.  Carried.

Louise Goulet asked us to respond to several questions from SSC.

#1 – What are the needs for Scleroderma patients in each province?  We need medical people who are more informed and also more specialists.

#2 – What is the role of SSC?  They must be advocates in charge of awareness; they must lobby for more funding for research; they should reinstate the national newsletter; they should have someone to co-ordinate provincial efforts acrossCanada.

#3 – What is the role of board members?  They should be responsible for information sharing from both sides – national and provincial.

#4 – What should the relationship between SSC and support groups look like?  SSC should co-ordinate information through newsletters on what kinds of fundraisers are occurring and what groups do to spread awareness.

The 2011 National Conference is in Quebec Cityon Oct 28th and 29th at the Hilton hotel.  Specialists will speak on topics such as lung complications, various therapies and CSRG reports.

Janet Campbell attended Dr. Markland’s Garden Show in July.  Attendance was disappointing, mostly Lupus Society members as it was a joint fund raiser this year.  We received $445 from Dr. Markland and Janet collected another $30 there.

Members expressed appreciation for our newsletter composed and printed by Janet Campbell and Gerald Shauf.  Volunteers will contact people on the membership list and ask if they wish to receive the newsletter.  There are over 100 on the list, 41 who are paid up members, and it is costly to mail to those who aren’t interested.

The meeting adjourned for lunch at 11:50 a.m. and reconvened at 1 p.m.

Fund raising possibilities for 2012 were discussed, such as a Steak Night perhaps with a 50-50 draw and/or Silent Auction; Pizza night; Walkathon.  A decision will be made at the April 2012 meeting.  Regarding a Walkathon, one possibility would be to have a nation-wide one with all provinces having the same T-shirts, perhaps with the Arthritis Society or with corporate sponsorship of some kind though it would require a lot of work.

During sharing time, a member commented on experiencing a lot of fatigue and wondered if others are affected to the same extent.  Fatigue appears to be common but not necessarily severe.  Another lady suffers severe pain especially if she bumps into something, but finds it is well controlled by prescribed narcotics.  Other pain control options were discussed.  Another lady wanted other’s opinions on healing after surgery so as to know what to expect when she has surgery.  The antibiotic ointment Bactroban (also known as Mupirocin) seems to work well for healing of finger ulcers.

Guest speaker, Dr. Patel arrived and was introduced by Louise Goulet.  He specializes in Respiratory Diseases, Internal Medicine and Sleep Disorders and spoke on how Scleroderma affects the lungs and Pulmonary Arterial Hypertension.  He said Scleroderma has many effects on the lungs but not all patients suffer all effects.  The main concerns are recurrent pneumonia and bronchitis, and impaired swallowing ability leading to aspiration.  The main symptom in these is a cough.  As the disease progresses there is shortness of breath because of scarring, leading to less lung capacity.  If the esophagus is affected by Scleroderma, swallowing is impaired perhaps resulting in aspiration pneumonia.  Scleroderma’s effects on the lungs can be impacted by allergies, anemia and co-existing conditions involving the heart, kidneys or liver.

Basic assessment of the patient includes blood-work, kidney function tests and chest X-ray.  Other tests that might be done are CT Scan, Bronchoscopy and Lung Biopsy.  What these tests show is whether there is Infiltrate (scarring or fibrosis from Scleroderma) or Infection.  Treatment depends on the patient’s condition.  If there is inflammation and not infection, steroids may be helpful, or newer drugs – Cytoxan and Cellcept which impair replication of cells.

Recurrent infections can result in bronchiolitis – inflammation of bronchioles.  If the lining of the lungs, the pleura is affected by Scleroderma, excess fluid may occur and needs to be drained off, but in this case, there should be no inflammation or infection present.

To determine the extent of Pulmonary Arterial Hypertension, blood pressure in the right heart which directs blood to the lungs is recorded.  This can be done indirectly by Chest X-ray and ECG to assess pulmonary artery diameter.  Direct measurement is by pulmonary artery catheterization.

PAH eventually causes shortness of breath to the extent that the patient can’t carry out normal activities of daily living such as dressing oneself, etc.  Diagnosis is made from pulmonary function tests, echocardiogram, history and X-ray.  Therapy includes Warfarin to prevent clots forming in smaller blood vessels.  Follow-up and reassessments are important.  Keeping active, especially walking or swimming is highly recommended.  Dr. Patel answered many questions after which he was thanked by Janet Campbell.  He continued talking with members during a coffee break.

Gerald Shauf reported that our old photocopier needs servicing again.  After some discussion it was decided that we should purchase a new printer/copier that will meet our needs.  Our organization has no web-site at present.  Gerald Shauf offered to approach a designer through his son-in-law that may do this complimentary with only a small charge for renting space on a server.

Our next meeting is booked for April 21, 2012 in the Wascana Rehab boardroom where we formerly met.

We will continue having coffee and snacks available before the meeting at 10 a.m. with Roberta Nichol and Joyce Kellington bringing the snacks for this meeting.

Norma Redmond showed a Hazelwood necklace, which she said, has helped her heartburn problem.  It is available at www.tinyteethers.com.  Janet Campbell cautioned that magnetic bracelets can wreck computers if worn when using them.  A product called Manuka honey + 20 has been found helpful in healing finger ulcers.

Gerald Shauf moved that the meeting be adjourned at 3 p.m.

Andrew Campbell offered to help members produce video testimonials for use on Youtube.  His email address is: acampbell500@gmail.com

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Christmas is a time when you get homesick – even when you are home!

He, who has not Christmas in his heart, will never find it under a tree.

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SASK Fundraising & Awareness Items:

 We have the following available:

Floral note cards:
There are 8 different floral designs in each package. Scleroderma contact information is printed on the back of each note card.  They are very pretty cards to use or give as gifts.  The cost for a package of 8 notes and envelopes is$6.00.

Lapel pins:  The attractive lapel pins are dark blue with Scleroderma printed on them in white letters.  The cost is $3.00

Magnetic ribbons. These are similar to the lapel pins.  They are 8 inches x 2.5 inches and are $2.00 each.  Put them on your car or your refrigerator.

Please contact Gerald Shauf or Janet Campbell for more information or to place an order.

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PLEASE donate your empty computer printer cartridges and old cell phones to our group.  

 Help the environment and support Scleroderma research!

For more information, please contact:
Gerald Shauf   Ph: 306-634-3433
Ken and Louise Goulet   Ph: 306-584-1950.

All money goes towards research in Canada.  Ask your family and friends to donate their computer printer cartridges and old cell phones.

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CSRG (Canadian Scleroderma Research Group) study:

If you haven’t already joined, your participation is very important for Scleroderma research.

All Saskatchewan study patients must see:
Dr. Janet Markland – Rheumatologist
#302 Midtown Medical Centre
39 – 23rd Street East
Saskatoon, SK.   S7K 0H6

Phone: (306) 244-1424
Phone and make an appointment with Dr. Markland and let her know you would like to join the study. You will continue to be followed by your own physicians and see Dr. Markland only for this research study.  Dr. Markland will do a thorough examination plus order necessary tests. Test results will be used from any recent tests already done by your own physicians. Results from study tests will be made available to your own physicians. This is also a wonderful opportunity to get a second opinion.  Since 2004 there have been 86 registered, with 65 active participants.  Once a year at each study visit you will be offered $25.00 to help cover travel and parking expenses.

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Useful Web Sites:

Scleroderma Society of Canada
http://www.scleroderma.ca

CSRG – Canadian Scleroderma Research Group
http://www.csrg-grcs.ca

Arthritis Society    Ph:1-800-321-1433
www.arthritis.ca

United Scleroderma Foundation (USA)
http://www.scleroderma.org

Scleroderma Research Foundation (USA)
http://www.srfcure.org

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A little smile, a word of cheer,
A bit of love from someone near,
A little gift from one held dear,
Best wishes for the coming year.
These make a Merry Christmas!

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You Are Your Own Best Advocate!

By Michelle Mayer, R.N., M.PH., Ph.D.

Onewould think that I would be well qualified to navigate the healthcare system. I began my professional career as a nurse and later became a Ph.D.-trained health services researcher. I am also married to a physician. However, despite all of my and my husband’s training and experience, it has been incredibly hard to find my way through the maze. From scheduling appointments to pay­ing for services, the process of obtaining high-quality medical care is fraught with difficulties.

In my opinion, this process is even more difficult for persons afflicted with relatively rare and incurable diseases like scleroderma.

A Struggle from the Start

Without a doubt, my story is like those of many other scleroderma patients. Despite having diagnosed myself with scleroderma one month after the onset of my symptoms, it took 14 months and three doctors to confirm the diagnosis.

Several scleroderma “experts” cautioned me against attempting a pregnancy, although the small existing medical literature had already demonstrated that the majority of scleroderma pregnancies have a good outcome.

And, of course, I have endured all the usual inconveniences and frustrations: long waits to get appointments, followed by long waits in the patient waiting area; cold patient rooms; poor pain control, especially after postoperative procedures; and providers who didn’t look me in the eye or shake my hand, let alone listen to my questions or understand my needs.

Not all my experiences with the healthcare system have been negative, but many of them have.

Chronic Diseases Fall Through the Cracks

Much of the system is oriented toward treat­ing a disease rather than a patient. For those among us with incurable diseases that affect many aspects of our lives, this “curative” focus is not helpful in our patient care.

I think that many physicians, faced with an incurable illness like scleroderma, recoil in defeat. They fail to realize how helpful they can be in preventing or minimizing complications and disability, preserving function, and eliminating pain.

First, Properly Orient Your Thinking

I have often wondered how people without the benefit of clinical or research training navigate this difficult system. So I have developed some tips to help you get the most out of your care.

Begin by understanding that scleroderma has the potential to affect your life negatively in two ways:

I) cutting it short; and
2) making it less enjoyable.

Therefore, until a cure for scleroderma is discovered, your medical care should focus on lengthening your life and making your life more enjoyable. Pain control and physical therapy – that is, dealing with the disease’s impact – deserve as much of your attention and investment as treating the disease itself.

Practical Tips from My Experience

Now that you understand what your focus needs to be, decide what is important to you. What makes your life worthwhile? What can you not live without? Your medical care should be organized in such a way as to pre­serve your ability to live a fulfilling life according to your own standards.

For example, I wanted to have children in spite of my scleroderma, I found that many physicians failed to appreciate the importance of this issue in my life and, consequently, dismissed my needs inappropriately.

1. Learn all you can about scleroderma.  Inform yourself about the disease, existing effective treatments, ongoing research trials, symptom management, etc. The Internet can be a wonderful resource, as can other patients with scleroderma. You can also access the medical literature through the National Library of Medicine’s web site at www.ncbi.nlm.nih.gov/entrez/query.fcgi.

2. Learn all you can about your health insurance.  Whenever possible, choose your health insurance carefully. Obtain the most comprehensive coverage that you can afford. There are many issues to consider, such as:

  • What physicians are available to you in-­network and out-of-network?
  • Can you choose your own provider’?
  • Does your insurance cover ancillary serv­ices, such as physical therapy?
  • What will be your deductible, co-insur­ance rate, and out-of-pocket maximum?

Find qualified, knowledgeable physicians who perceive themselves as your “partners” in improving your health. Your physicians need to view you holistically – that is, as a whole person, not just as a scleroderma patient. They need to appreciate how sclero­derma affects your life and demonstrate a willingness to help you minimize its negative consequences. Your physicians should treat you with dignity and be willing to answer your questions in a nonjudgmental manner. If you’re not comfortable with your physicians, find new ones.

3. Ask for what you need. Redefine your concept of being a “good” patient. You are your own best advocate. Remember, your doctor works for you.

Do not be afraid to assert yourself and ask for what you need. This applies to even the simplest procedures. For example, blood draws are painful for patients with scleroder­ma. Ask your physician to describe EMLA or Elarnax, anesthetic creams that can be applied to the site one-half hour before blood draws to numb the area. You won’t even feel the blood draw.

Also, ask the phlebotomist to use a butter­fly needle, which is smaller than the needles they typically use.

4. Manage your pain effectively. Do not live in pain. There are a variety of pain med­ications available to treat everything from joint pain to nerve pain. You should not have to suffer. If your primary care provider or rheumatologist is not able to or is uncomfortable with managing your pain, ask for a referral to a pain specialist.

5. Screen for scleroderma-related complications. Work with your provider to develop a schedule of screening tests to ensure that scleroderma-related complica­tions, such as renal involvement, pulmonary fibrosis, and hypertension, are found as early as possible. Do not wait for symptoms.

6. Use therapies to minimize scleroderma’s impact. Use ancillary services, such as physical and occupational therapy and biofeedback to minimize the effects of the disease and prevent disability. Do not wait for your doctor to suggest these services. Ask for them at the time of diagnosis or soon thereafter.

7.  Read your medical bills carefully. Read your medical bills because they are often filled with errors. Do not pay bills until you’ve reviewed them carefully and com­pared them to the Explanation of Benefit statements you received from your insurer.

Empower Yourself to Lead the Life You Want!

I decided early on in my disease that sclero­derma might shorten my life, but it did not have to run it. I have managed my healthcare in such a way as to preserve as much normalcy as possible.

At times it has been difficult to find like-minded providers who would allow me to manage my care, but I have been able to assemble a cadre of doctors comfortable with this arrangement.

Despite having diffuse scleroderma with pulmonary fibrosis, I live a very fulfilling life that resembles the one I would have wanted had I never developed scleroderma: I have two healthy toddlers, work part-time, exer­cise daily, travel, garden and sew.

Had I not been assertive in dealing with the healthcare system, I feel certain that my life would be very different.

Taking control of one’s health care can be an exhausting process. If you are too sick to fight for yourself, find a family member or friend willing to do it for you. Know your needs and goals, find a provider willing to accommodate them, and be persistent.

Reprinted from Scleroderma Voice 2002 #3.  Voice is a publication of the USA Scleroderma Foundation.

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THE DOCTOR IS IN

 By Philip Clements, M.D., M.PH., Professor of Medicine Division of Rheumatology, UCLA School of Medicine

 The following questions were asked of Dr. Philip Clements at the New England Chapter’s Patient Education Seminar. When time ran short, Dr. Clements graciously agreed to answer them in writing. This infor­motion is provided courtesy of Dr. Clements and Marie Coyle, President of the New England Chapter

 Q.  At least two years before I had any other symptoms of scleroderma, I had numbing of the hands while sleeping. Was this the start of my scleroderma?

 A.  About 5-10% of people with scleroderma develop carpal tunnel syndrome as one of the early scleroderma-related events. Generally, the numbness is confined to the thumb and index and middle fingers. There may also be pain in the hands and up the forearms. Characteristically, the symptoms wake people at night.

Carpal tunnel syndrome is a fairly com­mon problem in the general population but is more prevalent in persons with rheumatic disorders. Only if the skin becomes thickened and there arc other evidences of scleroderma can we say that this was related to scleroder­ma and could have been a heralding event for scleroderma.

Q.  Can you explain the pros and cons of taking methotrexate as a treatment for sclero­derma?

A.  Methotrexate is a very effective medica­tion in the treatment of rheumatoid arthritis. There are now two randomized controlled trials of methotrexate against placebo, which together examined 100 patients with diffuse scleroderma (widespread skin thickening) of recent onset.

Both trials came to similar conclusions: there were suggestions (or trends) that patients taking methotrexate had more soften­ing of skin at the end of a year than those who were taking the placebo, and that the physician and the patient assessments of overall course favored patients taking methotrexate over those taking placebo.

This suggests that methotrexate may bene­fit patients with early diffuse scleroderma.

Q.  What about Enbrel® versus methotrex­ate as a treatment?

A.  Dr. Michael Ellman inChicago per­formed a one-year open study in which ten patients with scleroderma were treated with Enbrel. His results were not overwhelming that Enbrel was effective.

There has been no head-to-head compari­son of Enbrel versus methotrexate.

If I were to treat someone with scleroder­ma who needed a strong medication (usually someone with early diffuse scleroderma), I would recommend methotrexate rather than Enbrel.

Q.  Is it possible to have rheumatoid arthritis and scleroderma together?

A.  The short answer is “yes.” When the skin in scleroderma is very thick and tight, especially if it’s thick over joints, it is very difficult to tell whether the joint itself is swollen or just the skin.

Joint inflammation, pain, and swelling may occur early in scleroderma and may be the first sign of scleroderma.

Later (or in some cases at the same time) the skin thickens. In this case the joint prob­lems are actually part of scleroderma.

In a small percent of patients, scleroderma and rheumatoid arthritis may coexist (or overlap). In most cases, however, the joint problems are actually part of scleroderma – ­even if the “rheumatoid arthritis blood test” is positive, which occurs in about 20-25% of all patients with scleroderma.

Q.  If you have inflammation of the lung, should your doctor treat it or just wait to see what will happen?

A.  There are now multiple studies that sug­gest that if there is truly inflammation of the lung, the likelihood is that there will be ongo­ing and permanent damage to the lung.

The greatest loss of lung function in scle­roderma occurs within the first 4-6 years of scleroderma. Inflammation of the lung can be diagnosed by open lung biopsy, bron­choscopy with washing (or lavage), and/or high-resolution chest CAT scan.

If a patient with lung inflammation has had their scleroderma for less than 5-6 years. this suggests that this person is at continuing risk of losing lung function.

Having said that, it is not clear whether we yet have a medicine that will stop the damage. The National Heart, Lung, and Blood Institute. a branch of the National Institute of Health, is sponsoring a multicen­ter study of Cytoxan® (cyclophosphamide) versus placebo in scleroderma patients with lung inflammation (active alveolitis is the fancy term) who are early in their scleroder­ma course. Hopefully, this trial will settle the issue of whether Cytoxan might be an effec­tive drug in stopping lung damage.

Gamma-interferon is another medicine which is being looked at in idiopathic pul­monary fibrosis (a disease in which there is lung damage very similar to that seen in scle­roderma) because there is evidence that it may help heal the lung and improve lung function.

Q.  Should I exercise a little every day?

A.  I recommend that patients do range-of-motion exercises every day, particularly if they are losing joint motion (and that is usually most prominent in early diffuse sclero­derma).

I think warm-water pool therapy (with water temperature in the 80s and 90s), with or without pool exercises, can be beneficial, particularly since the water alleviates gravity. This may help increase flexibility.

If people are able to walk, particularly when the weather is nice, or they can walk inside an indoor mall, they should do so; and should try to walk 30-60 minutes several times a week.

If the lungs have severe damage, particu­larly if the damage is enough to require oxygen therapy, I think the level of exercise has to be reduced to a level that’s tolerated fairly easily.

This is a topic that should be taken up with your doctor and your lung doctor (if you are seeing one).

Q.  What are the criteria for bone-marrow transplantation?

A.  Although the criteria may be slightly different in different parts of the country and different parts of the world, the criteria gen­erally require that patient candidates have diffuse cutaneous scleroderma of less than 3-4 years’ duration, plus evidence of heart, lung, or kidney involvement.

The reason is that there is a significant risk from the transplant itself. In scleroderma it’s beginning to look like there is a 10-15% mortality rate in the first 1-2 years after transplant.

The criteria for entry, therefore, must reflect this risk by requiring that the patients who receive a transplant have significant scleroderma involvement. In other words, these patient transplant-candidates have a predicted 50% risk of dying in the next 4-5 years if their scleroderma is left untreated.

At this juncture, bone marrow (or stem cell) transplant is still an experimental proce­dure. The centers performing them are learn­ing more and more about safer ways to per­form the procedure. Nevertheless, stem cell transplant is a high-risk procedure, and thus patients should have high-risk disease in order to justify these heroic measures.

 Q.  What can you tell me about “throat-stretching”?

A.  Actually the stretching occurs lower in the chest than in the throat. Generally the narrowing is at the end of the esophagus (the feeding tube that leads to the stomach) just before the esophagus joins the stomach.

The reason that the narrowing (stricture or scarring) occurs is that the muscle that nor­mally closes the esophagus from the stomach gets weak, and the opening between the esophagus and stomach remains open much too long a time. This allows hydrochloric acid (which is a very strong acid) and stom­ach juices to reflux (or back up) into the esophagus and actually “burn” the esophagus, much as strong acid might burn any tissue. As a result, there may be a scar that forms at the end of the esophagus.

Esophageal dilatation (or stretching) is the correct term for the procedure. A tube is passed through the mouth and down the esophagus and actually stretches the narrow­ing at the end of the esophagus. This allows food to go down more easily.

Unfortunately, dilating the opening can also then allow stomach contents to reflux back into the esophagus. Nexium® and other proton-pump inhibitors (Prevacid®, Prilosec®, Protonix® and Aciphex®) are very good at decreasing the amount of acid in the stomach juice. When stomach juice refluxes into the esophagus the juice is no longer acidic and, therefore, is much less likely to burn or scar the esophagus.

Q.  What does HDIT stand for?

A.  High Dose Immunosuppression with Transplant (HDlT). This is another name for stem-cell transplant.

Q.  Tell me something about Bosentan.

A.  Bosentan is an inhibitor of endothelin.  Endothelin is a naturally occurring substance made by the lining cells of blood vessels, which causes the blood vessels to constrict or narrow, causing decreased blood flow. This is especially important in the lung circulation where blood vessels may be narrowed and scarred from scleroderma.

This narrowing in the pulmonary circulation may lead to high blood pressure in the pulmonary circulation (pulmonary artery hypertension).

Pulmonary hypertension (increased blood pressure in the artery inside the chest which goes from the heart to the lungs) becomes a major problem in about 10% of patients with long-duration scleroderma.

Endothelin levels are high in patients with pulmonary artery hypertension; and the vaso-constriction caused by high levels of endothelin worsens the condition. Blocking the effects of endothelin should lead to relax­ation in the blood vessels and increase the blood flow into the lung.

In December 2001, Bosentan was approved by the Food and Drug Administra­tion for use in pulmonary hypertension in scleroderma. In trials, Bosentan improved exercise capabilities, but the long-term bene­fits and risks are not yet known. Only time will tell whether this drug will be of major benefit to scleroderma patients with pulmonary hypertension.

 Q.  What can be done for tight skin and itch­ing and for skin cracking under the breasts’?

A.  Sclerodermatous skin (be it from sys­temic sclerosis thickening or from a localized form of scleroderma like morphea) has lost many of its wound-healing properties and does not protect the underlying tissues in a normal fashion.

“Cracking” of the skin under the breasts is partly related to the fact that there is a warm, constantly moist environment under the over­hanging breast that promotes maceration and breakdown of the skin as well as fungus infection.

It is important that the tissues underlying the breast be given a chance to dry out and be treated with antifungal agents. Sometimes gauze-like material and dry powder need to be put between the skin of the chest wall and the skin of the breast to keep the area dry.

Once the breaks in the area have healed, the area needs lubricating oils, because that skin has lost its own lubricating oil glands.

Q.  Can diffuse skin thickening go into remission after three or four years’?

A.  The term “remission” suggests that a dis­ease goes away and is not active. In sclero­derma, unfortunately, it is very hard to know whether the activity of the disease ever goes completely away. Often there are scars left over in the form of thick skin, or lung scars or some other evidence that scleroderma has been there, As investigators, we have a very difficult time defining when scleroderma is active and when it is inactive.

More properly I should say that after 3-4 years, many people with diffuse scleroderma have improvement in skin thickening, flexibility, energy, and their sense of wellbeing. Their disease becomes much less of a prob­lem and the remaining evidences may be only Raynaud’s, heartburn, and tight fingers. So, yes, many people with diffuse scleroder­ma have a period of improvement that may last a lifetime.

Q.  Are there studies being conducted on embryonic stem cells?

A.  Stem cell transplants in scleroderma rely on purifying stem cells from the peripheral blood of adult humans with scleroderma. Embryonic stem cells are a different type of stem cell. Their use is quite controversial because of the issues related to how embryonic stem cells are obtained (i.e., from abort­ed fetuses, for example). How similar or dif­ferent the adult-derived stem cells are com­pared to the embryonic stem cells is not clear. I am not aware that there are major studies looking at embryonic stem cells in scleroder­ma, but I am well aware that there are several ongoing studies of adult-derived stem cells as a potential treatment for scleroderma.

Q.  What are specific selection criteria that might be good indicators for lung transplan­tation?

A.  Although there are patients with sclero­derma who have severe lung disease and should be candidates for lung transplantation, most lung transplant centers shun patients with scleroderma. The bias against scleroder­ma derives in part because there often are multiple organ systems involved in scleroder­ma. People with scleroderma may have mild degrees of heart, kidney, and gut disease that have not been discovered prior to transplant. Reflux (which occurs in virtually all people with scleroderma) can lead to inhalation of stomach juices and acids and cause aspiration pneumonia, and the transplant physicians feel that the presence of reflux is a major reason not to transplant people with scleroderma.

The University of Pittsburgh has the largest collection of scleroderma patients who have undergone lung transplantation. Very few other centers have more than one sclero­derma patient who has undergone transplan­tation. The experience at Pittsburgh suggests that scleroderma patients who undergo lung transplantation do as well as other people.

Unfortunately, the diagnosis of scleroder­ma seems to be a very strong reason for some centers to exclude patients. Until lung trans­plant physicians become convinced that scle­roderma is a good group for lung transplantation, this situation is not likely to change.

Reprinted from Scleroderma Voice 2002 #3.  Voice is a publication of the USA Scleroderma Foundation.

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Keeping Hands Flexible

By Elaine Sobin

You simply mustfight scleroderma’s impact on your hands. From my own non­-expert experience, the only alternative is a slow, inexorable progression to what I saw at a scleroderma meeting: a lovely lady who had both hands frozen into clenched fists still feeling pressure. Fortunately, she had a kind husband who did everything she couldn’t.

I’ve had scleroderma for some 40 years, luckily not too badly. Except for needing to have my esophagus stretched, my only prob­lems have been small mouth and curling fin­gers on both hands.

When my rheumatologist referred me to a hand surgeon, my right thumb was under the forefinger, and the skin on my hand felt so tight over the knuckles it looked as though the skin would split. Not a vein or bone was visible. It felt like I was wearing a drying leather glove, or a giant was crushing my hand.

Paraffin treatments could do nothing for the pain deep in my palm. The high-powered jets in the Jacuzzi at my gym penetrated deeply enough, but they may have helped destroy my CMC joint (below the thumb. at the wrist), which the surgeon ended up replacing.

 Making a Commitment to My Hands

After my joint replacement, I had physical therapy three times a week for a month. It was minimal, but my hand liked it.

If I could see improvement with that (l asked myself), what would happen if I com­mitted myself to all-out warfare?  We owe it to ourselves to see veins and bones in our hands, a little bending in previously frozen knuckles, soft and pliable skin, and remission from crushing pain.

Now I exercise my hands one to two times daily. If I skip a day, the skin starts to harden.

Following, I describe my hand routines.

 Heat and Massage

I like my heating pad set on High, wrapped around my hand for 15-20 minutes.

When I was getting therapy at the hand institute, for massage they used a therapeutic hand lotion. But my hands love Bag Balm in a green can, bought at a local feed store. It’s the thickest, gloppiest, most penetrating cream I’ve tried.

I divide the can into smaller plastic bottles to keep it as fresh and uncontaminated as possible.

I recommend you massage it into both hands, even if you think only one hand is “bad.” I was amazed to find (see below) the pinky of my “good” hand had less strength than the pinky of my “bad” one.

Keep massaging and wringing every cen­timeter of your hands until you can almost feel it going under your skin. Take more cream if you need it.

 Stretching and Bending Hand Exercises

Perform every motion to the utmost of your capacity, firmly but gently. If a motion feels useful, repeat it 10-20 or even 30 times. You say time is money, and you can’t afford to spend this much time? Your return on this investment will be more valuable than money.

Note: Try to alternate stretching and bending exercises.

1.         Holding your four fingers with the other hand, press the fingers away from your thumb. At the same time, stretch your thumb as far away from the fin­gers as possible.

2.         Try to bend the knuckles on all four fingers at once, as far as possible, using the other hand to apply pressure.

3.         Now try to bend the knuckles on each finger individually, as far as possible, applying pressure with the thumb on the other hand.

4.         Press both hands together, trying to straighten your fingers at the knuckles. Also try to press each hand backward at the wrist, as far as possible.

5.         Keep massaging the flap of skin between thumb and forefinger.

6.         Press a couple of fingers of one hand between each finger of the other hand.

7.         Stretching your hand from thumb to pinky (as hard as possible), twirl and stretch each finger with as much effort as possible in one direction and then the other.

 Exercising with Toys

Note: You can also do your mouth exercises while you do these.

Balls: I happen to have a pretty stiff foam­ rubber baseball and a beanie-bag-type of squeeze ball, so I’ll squeeze each ball 60-100 times with each hand.

Weights: I have the old-fashioned 2-pound iron dumbbells. Bend your arm, holding a weight in each hand, and curl your wrists only up and down slowly 30 times. Turn your hands facing the other way and repeat. Then holding the dumbbell with two fingers only, curl the dumbbell up and down 30 times; repeat till all fingers have been used.

Clothespins: Holding a clothespin between thumb and forefinger, press 30 times. Repeat with thumb and all fingers. (This is when I discovered my “good” pinky was actually weaker than my “bad” one.)

More Ideas for You

Add, subtract, and tailor the exercises above to your own needs. I  try to do some exercises wherever I am.

Examples:

  • The driver’s wheel in my car is a great place to press curved knuckles straight; when you are a passenger you can do the same thing on the elbow rest.
  • One of my favorite places is the Jacuzzi because of the heat and mois­ture (though I can only stand it 10 min­utes or so). With your palms spread next to your body, raise yourself by your hands, concentrating on straight­ening your fingers. (If this is too hard, I highly recommend Aquasize or work­ing out with a “noodle.”) Also, flatten your palms and try to raise one finger at a time.

If you have the opportunity to work with a good therapist – unfortunately, I’ve seen and heard a lot of horror stories – you’ll have a good idea of much pressure you, or someone working with you, should apply.

I’m still working on refining my routines, and trying to find what works best for me.

For me, as for all of us, hand therapy is a work in progress. But for now, I am keeping some flexibility in my hands, and the crush­ing pain is gone.

Reprinted from Scleroderma Voice 2002 #3.  Voice is a publication of the USA Scleroderma Foundation.

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Gifts of time and love are the basic ingredients of a truly Merry Christmas!

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