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.
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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.
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.