Question
Hi Amelia,
I don't know if you can help me or not but I thought I would give you a try!
My 56 year old sister has had Crests Syndrome for 10 years and has developed ulceration in the tip of her middle finger. She has experienced excruciating pain and discharge from the finger. Her rheumatologist has treated her with antibiotics and the finger now is very hard but still painful to touch. She works in the bank and cannot go back to work as she can not use this finger to count money as it is still painful and she is fearful of the infection reoccurring. Her specialist said he has seen nothing like this and does not know what to do next except give my sister doctor's certificates!
He has told Heather that if it gets 'too bad' to go to hospital and ask for a surgeon to admit her.
Do you have any ideas?
Many many thanks,
Janette Richardson

Answer
I'm hoping you live within a reasonable distance of one of the scleroderma treatment centers on the Scleroderma Foundation website:
http://www.scleroderma.org/medical/centers.shtm

But, even if it means an extended trip, your sister should see a scleroderma specialist.  Her local rheumatologist "has seen nothing like this" because he treats very few scleroderma patients.  At a scleroderma treatment center, doctors there have seen hundreds, or even thousands of patients, which raises the odds they will see more of the various complications and manifestations.

You probably already know about the Raynaud's Phenomenon that happens in scleroderma, where fingers lose circulation and turn colors in the cold.  What complicates Raynaud's for us that primary Raynaud's sufferers don't have is damage to the microvessels in the fingers and toes, caused by the same fibrosis affecting the skin and other organs.  

So, not only is your sis having these uncontrollable "collapses" in her blood vessels that block the blood from getting to tissues, some areas of the vessels are narrowing.  This can make them lose circulation even without a Raynaud's attack.  And when tissue doesn't get the oxygen carried by the blood vessels, the tissue starts to die. This causes an ulceration that is subsequently hard to heal because of the loss of blood flow - the tissue needs oxygen to live and to heal, and it's being starved of it in the area where the vessel is damaged.

There are some fixes her local doc can try right away.  If she's not already on a calcium channel blocker to help blood flow, she could be. It's a blood pressure medication, which can be a problem if she has normal or low BP because as it opens up the blood vessels, it lowers the BP.  She can try with a small dosage.  There likely won't be a dramatic change but it may help some.  Another item I used was nitroglycerin paste applied directly to the finger.  She'd need to avoid putting it anywhere near an unhealed ulceration, but all around it can help.  It's another thing that opens up the blood vessels to get the best possible blood flow.  It's goopy and sticky - she'll want to wear a _loose_ bandage or glove over it.

Pain is a huge problem in digital ulcerations and she needs to talk to her doc about this.  Only by knowing how much pain she's really in can he properly treat it.  I'm sure she's as scared as all of us of narcotic pain meds, with their reputation.  But this isn't usually a problem with careful prescription and oversight in a real pain situation.

Another treatment that her doc was alluding to, with the surgeon, is a sympathectomy.  In a digital sympathectomy, the nerves are stripped from areas of the hands so that the impulse to collapse the vessels doesn't reach them and blood flow continues.  This is usually only a temporary change of, from what I read, a few years.  There is another sympathectomy that involves cutting out nerves higher in the body.  Again, it may only be a temporary measure.

Research has gone into using new pulmonary hypertension drugs for digital ulcerations, and some success has been seen.  But the drug being tested is crazy expensive.  Some docs are trying other, similar drugs that are less expensive, like Viagra.  I've also known people who have had ulcerations heal up when on an antidepressant like Prozac.

This is a very serious complication and can lead to the loss of fingertips.  Your sister is right to worry about the repeated trauma of working with her hands.  She should see a scleroderma specialist as soon as possible and discuss her working options with her/him.  In the meantime, she should keep warm and keep her fingers warm.  Gloves help some but mittens work best.  Many folks use the "heater packs" you can find at the hunting supply stores by just keeping it in their pocket and inserting their hand, or using it between a ski glove liner on the hand and an outer mitten.  There's also a heated "Wristie" at wristies.com that can be added to the arsenal of warm things.

There's more on digital ulcerations on the Scleroderma Foundation website at http://www.scleroderma.org/medical/raynauds.shtm but as with everything in scleroderma, no one is alike.  It will be best to be assessed by a specialist.