What's Up Doc?
(originally published in "Scleroderma VOICE,"
March 2007)
The Scleroderma Foundation receives questions on a
daily basis from patients seeking answers to various
health issues. Although scleroderma is a highly individualized
disease, we have found that some concerns are very common
among those affected. We think this forum provides a
service to you, our members.
We hope you find this section informative. However,
we understand that individual circumstances are unique
and ask that you always seek the guidance of your healthcare
professional to obtain the treatment plan that best
suits your specific health history.
Can a gastric pacemaker help with my scleroderma-related
gastrointestinal problems?
Gastrointestinal problems are extremely common in systemic
sclerosis and 95% of patients have some gastrointestinal
symptoms. Fifty to 77% of patients have some decreased
stomach movement (gastroparesis) and may have symptoms
such as bloating, getting full very easily (satiety),
nausea and vomiting. In the stomach, as elsewhere in
the GI tract, the basic problem probably arises from
blood vessels that do not nourish the nerves. This,
in turn, results in decreased ability to move food,
acids etc. along the GI tract as well as eventually
resulting in replace-ment of muscle by fibrous (scar)
tissue. Treatments such as metoclopramide (Reglan),
erythromycin (which stimulates the bowel) and octreotide
are often successful. However, when these do not work,
a new treatment is occasionally used. This is called
“gastric pacing” or “gastric electrical
stimulation” and is like a pacemaker for the heart,
except it paces the stomach, causing the stomach muscles
to push food out of the stomach into the rest of the
gastrointestinal tract. This method is only useful for
very severe problems and is not yet fully proven in
systemic sclerosis but can, occasionally, be very helpful.
– Daniel E. Furst, M.D.
|
I have persistent and very painful
digital ulcers. What are the latest treatments for
this problem? I have heard that Revatio and Bosentan
may help. What about hyperbaric treatment that diabetics
sometimes use? |
The
digital ulcers in scleroderma are due to poor blood
supply and are often precipitated by trauma. Although
some of them can be quite resistant to treatment, there
are good general measures that can help. Good wound
care, dressings that avoid further trauma, debridement
(removing dead or contaminated tissue) are part of the
solution. For those who still have difficulty healing,
several options are available. Both Revatio and Bosentan
are drugs that can improve the blood supply, and there
is some evidence that they can be of help. Coverage
of the wound with bioengineered skin is underutilized,
although readily available. Hyperbaric oxygen has been
around for a very long time. Like the other options,
it will not help unless one is also implementing optimal
wound care.
– Vincent Falanga, M.D.
What are the current treatments for pulmonary fibrosis
associated with scleroderma, and does Revatio help?
Most scleroderma patients have some pulmonary disease,
with pulmonary hypertension (PH) and interstitial lung
disease (ILD) or pulmonary fibrosis being most common.
From 25 to 90% of scleroderma patients will have some
pulmonary fibrosis, and it is notoriously difficult
to treat. However, a recent study suggests that taking
cyclophosphamide (Cytoxan) for 12 months is of benefit.
The study suggested three important things: that cyclophosphamide
may slightly improve lung function, decrease breathlessness,
and improve quality of life. Another medication under
active study is mycophenolate mofetil (CellCept). While
sildenafil (Revatio) may benefit patients with pulmonary
hypertension, there is no information to suggest that
it directly treats the pulmonary fibrosis.
– Kevin K. Brown, M.D.
|
I have systemic scleroderma and
would like to have a cosmetic procedure to help
with the lines around my mouth. I have heard that
Restylane is good for this. Can I have this procedure
if I have scleroderma? |
Restylane
is a dermal filler that is commonly used to improve
the appearance of deep wrinkles and lines (for example,
those around the mouth). It can be quite effective when
properly used. Patients have to understand that the
improvement is not permanent, that it needs to be reinjected
after a few months, and that there is some discomfort
and pain associated with the injection. Those who are
prone to “cold sores,” a common manifestation
of herpes infection on the lips or around the mouth,
should probably receive prophylactic treatment with
an antiviral medicine before the injection. It is my
opinion that it is safe to use in scleroderma patients
and can improve their appearance.
– Vincent Falanga, M.D.
The Scleroderma Foundation wishes to thank Drs. Daniel
Furst, Vincent Falanga and Kevin Brown for their help
in providing answers to these questions.
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