Help Me Figure This Out...

[juliegee]

Hey All-

I was hospitalized for a half week, hooked up to IV blood thinners, last time this happened. My doctors were convinced my heart had a hole in it & was throwing blood clots. Tests proved otherwise & I was released with no answers (as usual .)

Earlier this AM I couldn't feel a portion of my left index finger. I lost all feeling in it. It was frozen & I absolutely could not get any blood (or feeling) to it no matter what I did. When I finally looked at it, it was black. Here is a picture- several hours after the event:

http://gallery.me.com/juliegee#100024&bgcolor=black&view=grid

Note the black/bruised appearance on my left index finger above the middle joint. What is this??? I hesitate to go to my physicians as I seem to not find help & I sometimes get held hostage in the hospital . I have severe Reynauds, but this is different as the dark color persists beyond the vasospasm of the blood vessel. A capillary or blood vessel seems to have broken with no trauma other than the Reynauds itself.

WHY? Is this just more connective tissue madness? I do have 2 brothers with vascular craziness: one with a burst aorta & the other with a dissected carotid artery. Is this my mini-version?

Anybody else have anything like this happen? Any thoughts?

Thanks in advance-

Julie

[juliegee]

Oh, I meant to add- don't my hands look awful? I am only 48, but my hands look 70. That is one sign, BTW, of a connective tissue disorder, especially EDS- Vascular Type. (I've had that ruled out- phew!) A vascular surgeon (when I was last hospitalized) took one look at my hands and said "You have scleroderma." The other symptoms haven't added up yet.... I think my aged hands are a part of the puzzle. The rest of my doesn't look that old Anybody else with a connective tissue disorder have old-looking hands?

[lieze]

I'm so sorry.

I don't even know what to tell you.

All these weird things that come up for us are so maddening.

You are a trooper.

I do wonder if you should get it checked out?

[comfortzone]

I am curious as to the consults you've received as regards heritable diseases of connective tissue.......Were the consults with those that specialize in these alone as part of a genetics practice? Or were they EDS specialists? I had never heard before about hands looking a certain way with vascular eds like aged - in fact some EDS peoples skin looks more supple and soft and youthful compared to other non-EDS folks ~ but it is kind of alarming to read your shared family history with ruptures of hollow organs like arteries.... which can be the story with vascular eds...

I know you would absolutely hope to get someone to accurately diagnose .... either the underlying disorder (did a doctor say you had a connective tissue disorder like scleroderma & agree to follow you so you have a doctor to treat?) Has neuro been involved as you have lost sensation to your finger ~ I guess I hope you have copies of all your medical records and tests in a binder because it sounds like a complicated case - with symptoms that are changing... Would you be bruising easily because of the blood thinners?

I just wish I guess that someone would be there for you when stuff like this happens - someone to have a good working relationship with that knows exactly what you have and what treatment schemes would/will work best... I hope you feel better soon (& I hope you get a nice doctor that can follow you through all of this - hospitals aren't fun - but they were worried about you and I think they need to make sure you get good follow up)

Feel better

[Chaos]

Have you seen a vascular doc? Seems like that might be a good source. We have one locally who's very good. He thinks Reynauds is not given nearly enough emphasis and not treated well by most docs. Given your family history especially, it seems like there's definitely some type of connective tissue disorder that's affecting the vessels. I'm sure it was a relief to rule out the EDS-4 stuff but then that leaves you with yet another unknown.

Is it just artifact on the picture that makes it look like there is some discoloration on your right index finger too- up toward the nail?

Do you get swelling or pain with this? Besides the numbness and discoloration?

Sorry you had this flare up again. What a drag! Sending you hugs, but unfortunately not much helpful info!

[lissy]

Hi,

I would suggest seeing a blood specialist maybe a blood disorder going on...???? A few come to mind antiphospholipid syndrome,Thrombotic thrombocytopenic purpura .

Maybe I'm totally off but worth checking good luck.

Lissy

[juliegee]

Thank you everyone for your input. I'm especially excited about your ideas, Lissy. (Can't wait to check them out!) What's happening is unusual & none of my doctors have been able to figure it out yet. Frustrating

In the past, I've seen a geneticist, vascular surgeon, cardiologist and my internist about this. I've had many many tests. Most are normal. Each doctor is puzzled. In response to your question, Nowwhat, the geneticist that my son & I saw at Emory DID Dx us with an "unknown connective tissue disorder" based on symptoms and family history. He did rule out EDS-IV and Loeys-Dietz Syndrome with blood work. Aged hands ARE a well-known symptom of the vascular type of EDS. Many informational sites online describe that, here's one:

http://www.ehlersdanlosnetwork.org/vascular.html

The soft velvety skin that you describe is more common with the other types. I DON'T have anyone following this issue & it's scary when it happens.

I did have a lab appt. to have my white count checked again today so I popped in to see my internist and described the incident. My finger's still bluish-purple. He's ordered oodles of blood work, including cryoglobulins, ANA, and serum proteins. He thinks it is NOT a blood vessel disease, but that I am making blood clots and may need to be on blood thinners (which I am not on currently- with the exception of 81mg aspirin.) If he can't figure it out he's sending me to a rheumatologist- which I've never seen before. I am hopeful I can get an answer. With my family history, this is disconcerting to say the least.

THANK YOU everyone for the input, advice & caring. I'd be lost without my autonomically dysfunctional DINET family

Julie

[comfortzone]

I'm happy you got someone to look at that finger - and that they ordered labs and such.... Rheumatology sounds like an excellent idea - even if the internist gets the answers he's looking for -- for some reason it seems rheumie's do really well with unusual health issues and are never shy in ordering a plethora of tests to nail things down. I've seen four of them since my 30's and 3 out of 4 were truly exceptional people...hope if you go the same occurs for you in your visit. Thanks for the link from yet another EDS website who rightly teaches about vascular EDS and what to look for....I do think there's a fair amount of crossover in signs/symptoms and it is helpful to get a specialist in connective tissue disorders to get dx correct. I know Dr. Lavallee is in South Bend, Dr. Francomano in Baltimore and Dr. Tinkle's in Cincinnati....Even among them - sometimes it helps to see more than one - as some deal more with kids and others more with adults. Some others in the US deal with other types more than EDS types and on it goes. Sounds like you do have a good plan in place and that it's just a matter of time before you get some clarity as to the nature and cause of this finger thing and the clots etc.... I've been on Coumadin 4 times in the past and didn't have any issues with it for surgeries....but maybe if they find out the cause for sure you won't even have to have it. Only the best to you!

[juliegee]

Hi All-

Just a quick update on my finger. ALL of the tests my internist ordered (CBC, serum proteins, cryoglobulin) were normal- as usual & he was ready to drop this. I asked for a consult to a vascular doctor. On my first visit- weeks after the event- the vascular doc was ready to hospitalize me for IV blood thinners He was 100% convinced that this was a embolic vascular event (blood clot!!!) and that it IS a part of my connective tissue disorder. He ordered an arterial pressure test on my arms/fingers. It was pitiful. The pulse at my bicep was great, worse at my forearm, almost gone at my wrist & immeasurable at my fingers. Worse on my left side (where the clot was) than on the right. I have an MRA & echocardiogram coming up on Monday as he suspects I have a larger blood clot somewhere in my upper body that is throwing off the smaller clots- Yikes.

The worst part is, because of the holidays & his vacation schedule, I don't get results till 12/27. I hate the waiting!!! He's put me on oodles of Plavix and a calcium channel blocker to open up my arteries. My hands & feet still aren't warm, but they are pinker (usually purplish/blue) & look amazingly better. I have an appt to see a Rheumatologist near ATL after the 1st of the year. He wants me to go to Mayo (docs love Mayo!) to see someone about the connective tissue disorder. He's trying to rule out fibromuscular dysplasia, arterial thoracic outlet syndrome, or a structural heart defect for now.

THANK YOU, nowhat for the connective tissue geneticists you recommended. I've been thinking about seeing Dr. Francomano about this anyways. This may finally prompt me to sort it all out. So if anyone gets a blue/black finger or toe, IT IS A MEDICAL emergency & possible harbinger of a bigger problem.

Thank you everyone for your support & input. I have learned that it takes an autonomically dysfunctional village to DX me

Julie

[crowebirds]

Hi All-

Just a quick update on my finger. ALL of the tests my internist ordered (CBC, serum proteins, cryoglobulin) were normal- as usual & he was ready to drop this. I asked for a consult to a vascular doctor. On my first visit- weeks after the event- the vascular doc was ready to hospitalize me for IV blood thinners He was 100% convinced that this was a embolic vascular event (blood clot!!!) and that it IS a part of my connective tissue disorder. He ordered an arterial pressure test on my arms/fingers. It was pitiful. The pulse at my bicep was great, worse at my forearm, almost gone at my wrist & immeasurable at my fingers. Worse on my left side (where the clot was) than on the right. I have an MRA & echocardiogram coming up on Monday as he suspects I have a larger blood clot somewhere in my upper body that is throwing off the smaller clots- Yikes.

The worst part is, because of the holidays & his vacation schedule, I don't get results till 12/27. I hate the waiting!!! He's put me on oodles of Plavix and a calcium channel blocker to open up my arteries. My hands & feet still aren't warm, but they are pinker (usually purplish/blue) & look amazingly better. I have an appt to see a Rheumatologist near ATL after the 1st of the year. He wants me to go to Mayo (docs love Mayo!) to see someone about the connective tissue disorder. He's trying to rule out fibromuscular dysplasia, arterial thoracic outlet syndrome, or a structural heart defect for now.

THANK YOU, nowhat for the connective tissue geneticists you recommended. I've been thinking about seeing Dr. Francomano about this anyways. This may finally prompt me to sort it all out. So if anyone gets a blue/black finger or toe, IT IS A MEDICAL emergency & possible harbinger of a bigger problem.

Thank you everyone for your support & input. I have learned that it takes an autonomically dysfunctional village to DX me

Julie

Oh, my goodness, this scares me. You are describing a part of TLC's condition. I am praying for you. Keep us updated.

[sue1234]

My hands have a similar issue, with looking "old". I am just 51 now, but a few years ago when POTS hit, my hands changed to this old, thin, wrinkly skin. My face did not. I feel this is related, also, but not sure how. I am thinking just a lack of fluid.

[juliegee]

TLC's Mom- Sorry, don't be afraid. I'm really talking about 2 different things. I have severe underlying Reynaud's- I hope that's the part that TLC & I share in common. This other "embolic vascular event" is a fairly rare occurrence for me when a segment of my finger actually turns dark purple/black & I lose feeling to it. That's a potential crisis & needs immediate treatment. Lots of time ALL of my fingers are bluish, dusky, purple; BUT the EVT is dramatic & very different. If it's ever ONE finger or ONE toe that is very dark in color- that needs immediate attention.

I have neglected treating my Reynaud's for a long time because of a bad reaction I had to a med prescribed one time. I should have tried a different med, different doctor; BUT instead I convinced myself that I was doing OK not treating it. My hands looks so much better after a few days with a calcium channel blocker that I am shocked. I am certain that I've done permanent bone damage from allowing them to go un-oxygenated for so many years. Not smart.

Sue- Do you have Reynaud's? Any history of weird vascular stuff in your or any family members? I agree that your prematurely aged hands are probably a sign of some underlying connective tissue thing. Not easy to figure out though.

Julie

[sue1234]

I may have Raynaud's--never been investigated. I just know I vasoconstrict when upright. Later, if I get hot, then my hands vasodilate, and that is AWFUL! Then, my hands are hot and red, and the fingers swell. It all happens quickly and I don't like vasodilating, as it makes me more lightheaded. So, constricting somewhat is much better for me.

[Noreen]

Julie-

Somehow I missed this the first time around. I am glad you found a doc to work on the problem.

I will be praying for you, Sweetie.

[crowebirds]

TLC's Mom- Sorry, don't be afraid. I'm really talking about 2 different things. I have severe underlying Reynaud's- I hope that's the part that TLC & I share in common. This other "embolic vascular event" is a fairly rare occurrence for me when a segment of my finger actually turns dark purple/black & I lose feeling to it. That's a potential crisis & needs immediate treatment. Lots of time ALL of my fingers are bluish, dusky, purple; BUT the EVT is dramatic & very different. If it's ever ONE finger or ONE toe that is very dark in color- that needs immediate attention.

TLC has acrocyanosis instead of Raynauds. They are very similar in their mechanisms. My husband's mother had a lot of issues with blood clots. My husband and and his mother and grandmother all had heart issues that involve the autonomic functions. She has so much trouble with her heart rate being at varying speeds and pressures. I have been concerned with the potential of her having clots because of all these factors. Quite frequently when she deals with the acrocyanosis, her feet and/or hands (including arms/legs) get tingly (like having a foot waking up from being asleep, painful and ice cold. She has lost all sensation during these episodes. I usually massage her legs to help improve blood flow and get her feet warm again. Quite frequently she will mention that her feet are cold all the time. We keep our home at 76 degrees all the time for her and to keep her warm because of the mast cell issues. It is very hot in our house, but she is freezing (seriously icy) cold.

Two cardiologist have refused to see her because her symptoms were too "complicated." Her heart and blood pressure are all over the place. Her resting heart rate is usually 100 - 120. When she stands, her heart rate has increased so much that you cannot even count the beats, but have been recorded as high as 194 bpm. She normally has a low blood pressure, but as her heart rate goes up, her blood pressure goes down. It is very difficult to do a manual pulse because the pressure is so weak and she has beats on top of beats. I have asked intake nurses to take her pulse and pressure both sitting and standing. They tell me that it is normal, but I see them struggle maintaining a pulse when she stands. Her Immunologist put her on Propranolol for her heart, which gives her a little more energy, so I know it helps, but she still struggles with those numbers. Seeing your story means that I have to be just that much more careful in watching her.

[Chaos]

Julie!!! So glad you finally got in to see the vascular doc. Yikes! Glad you are getting some help with the calcium channel blockers.

Good luck with your tests on Monday. Please keep us updated!

Hugs!

[juliegee]

TLC has acrocyanosis instead of Raynauds. They are very similar in their mechanisms. My husband's mother had a lot of issues with blood clots. My husband and and his mother and grandmother all had heart issues that involve the autonomic functions. She has so much trouble with her heart rate being at varying speeds and pressures. I have been concerned with the potential of her having clots because of all these factors. Quite frequently when she deals with the acrocyanosis, her feet and/or hands (including arms/legs) get tingly (like having a foot waking up from being asleep, painful and ice cold. She has lost all sensation during these episodes. I usually massage her legs to help improve blood flow and get her feet warm again. Quite frequently she will mention that her feet are cold all the time. We keep our home at 76 degrees all the time for her and to keep her warm because of the mast cell issues. It is very hot in our house, but she is freezing (seriously icy) cold.

Two cardiologist have refused to see her because her symptoms were too "complicated." Her heart and blood pressure are all over the place. Her resting heart rate is usually 100 - 120. When she stands, her heart rate has increased so much that you cannot even count the beats, but have been recorded as high as 194 bpm. She normally has a low blood pressure, but as her heart rate goes up, her blood pressure goes down. It is very difficult to do a manual pulse because the pressure is so weak and she has beats on top of beats. I have asked intake nurses to take her pulse and pressure both sitting and standing. They tell me that it is normal, but I see them struggle maintaining a pulse when she stands. Her Immunologist put her on Propranolol for her heart, which gives her a little more energy, so I know it helps, but she still struggles with those numbers. Seeing your story means that I have to be just that much more careful in watching her.

Gotcha', her circulation issues are certainly more complicated than simple Reynaud's. I can't help but think much of this is related to low blood volume- nor enough to go around- and what little there is is shunted to more vital organs. I've also read that Reynaud's is the result of an overactive sympathetic nervous system- maybe the same with acroyanosis. I think vasospasms also play a part in the blue hands/blue feet club. Do her hands feet sweat a lot? That seems to be a hallmark of acroyanosis. Mine don't, but they are persistently blue and I do get ulcers (chillblains) occasionally on my hands and more often on my feet. TLC's lucky to have such a vigilant & careful Mom. I'm sending prayers that you find better answers.

Julie

[juliegee]

I may have Raynaud's--never been investigated. I just know I vasoconstrict when upright. Later, if I get hot, then my hands vasodilate, and that is AWFUL! Then, my hands are hot and red, and the fingers swell. It all happens quickly and I don't like vasodilating, as it makes me more lightheaded. So, constricting somewhat is much better for me.

Me too- vasoconstriction when upright!!! Sometimes, they stayed constricted even when I sat or layed down. Interesting about feeling worse when they finally dilate... Your hands are almost like a barometer for you You can tell when you are vasoconstricting or vasodilating by their color. Could come in handy....

Julie

[louloutinks]

Hi Macks mum,

have you checked out for general essential telangiectasia (GET). It can follow on from scleroderma. It can start off as a tiny capillary then spread. Just a thought.

Funnily enough I am visiting my docs as I really think that I have this scleroderma. My hands are so old and I am 41 yet thet are so wrinkled and knotted. Raynauds is present too. I have been looking up about this the past couple of days thats why I recognised the look in your photo - I may be wrong.

Since being dx'd and I have looked into things, I realise now that I should have seen the doc about more things....all the small things add up to make the larger picture

[louloutinks]

Oops just seen the date of first post....d'oh! :rolleyes: