The Captain at my office put me in a bit of a spot. He called me asside to translate some medical stuff for him (that is from standard English to words he more clearly understood). It seems his wife has been diagnoised with Scleroderma.
He has to tell her and was quite upset about it. Scleroderma is an automimmune disease that seems to be untreatable and has no known cause. In a quarter of case it progresses from the skin to the internal organs, resulting in death.
So anyway, the Captain asked me to shop around (while I am home here in the US) for any OTC skin creams that might reduce her discomfort.
OK, gang, now I am not looking for a cure here. I just want to find anything that would help the poor lady and discharge my obligation to the Captain.
(I hope this does not violate any medical advice policy on the Board. If so, please feel free to delete this.)
I’ve generally given my patients the recommendation to try eucerin/minerin or aquaphor creams along with OTC 1% hydrocortisone cream for their skin and see if it helps. Of course, this should be combined with a treatment plan designed by a rheumatologist. My scleroderma patients see a rheumatologist at least once, and then I’ll manage them, and refer them back if needed.
Sometimes ointments work better than creames in terms of preserving skin moisture. So one should consider getting a 1% HC ointment instead, along with moisturizing ointments.
[CMA]Both those products are OTC, but should be used in conjunction with a physician’s treatment program for such a serious condition as scleroderma. [/CMA]
Well, some rheumatologists are trying things like plaquinil, methotrexate, and other immune modulators to see if perhaps it can slow the disease progression. They have not been demonstrated to be effective, but I don’t believe they’ve been shown to be ineffective yet either. Theory says they could help, and such meds certainly do help for other rheumatologic conditions.
If it were my spouse or other loved one, I’d make sure they saw a rheumatologist at least once, to confirm the diagnosis and discuss what’s state of the art and what isn’t.
Meanwhile, I’d push the skin care and reflux prevention aspects.
It’s not even called Scleroderma anymore. The new name is Systemic Sclerosis which is either diffuse (formerly known as Scleroderma) or confined (formerly known as CREST syndrome).
Treatment is designed to reduce symptoms.
If this lady has Raynaud’s disease, handwarmers and prescriptions for vasodilators might help. If she had GI symtoms, drugs to reduce acid secretion and improve motility could be prescribed. Specific therapies such as Sildenafil for pulmonary hypertension exist. Most patients seem to be on therapy to deal with their symptoms as they arise, rather than therapy designed to alter the course of the disease itself.
I think the main problem is that none of the immuno-suppressant therapies have been shown to be effective in altering the prognosis, and all of these drugs can have fairly awful side effects. Rheumatologists are the one who know best how to deal with this, and what might be worth trying in each specific situation.
Be warned though- this is every rheumatologist’s least favourite disease, because there is so little that has actually be shown to slow or prevent progression.
Trying to get a rheumatologist who is willing to look at this optimistically and aggressively, someone who is willing to try new and innovative therapy rather than from a nihilistic, pessimistic point of view that any attempt to change the prognosis is pointless, might be half the battle.