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Author Topic: I.T.P,,,,T.T.P,,,Evans Syndrome,,,,Heads-up.
Dustoff 1
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Parents, individuals, and Veterans of Foriegn Wars..Also, anyone exposed to toxic chemicals in the work place...Better read up.

When the first diagnosis of I.T.P came in yr. 2000 this disease changed our lives..Coupled with a diagnosis of Cardio Myopathy, severe Atrial Fib and congestive heartfailure, well, I guess you could say I was one sick puppy..

The I.T.P advanced to T.T.P Evans syndrome, after the splenectomy....

I was a very athletic type man, and gifted with above normal physical strength...Many years of Surfing and very hard work at Sea had really built me up....I never would of made it without that base.

Folks, this calamity happened so fast the doctors are still scratching their noggins..How could you not know how sick you were, I remember one of the first Dr.'s saying...I made a wise crack about a hangover feeling, but that was about it, the elephant had already sat on my chest at Sea, commercial Fishing for Albacore Tuna..
I had a fresh market order for a few thousand pounds of late season Tuna and didn't want to bother with a crew, so I ran the trip myself....That was dumb! Wouldn't ya know it, thats when my ticker went haywire bigtime, about 70 miles offshore..
Got the boat back to port, and into the red and white job I went. Bang! End of a long and exciting life style and some fine profits from the Sea.

Point here? pay attention to yourselves, you can lose it all in a heartbeat..

Veterans and others: Nam Vets! Get registered with the VA under the Agent Orange program....Civilian Doctors gave opinions to the VA that my condition was caused by Agent Orange exposure, so far the VA has been so busy keeping me alive I haven't really pushed a claim with them...The VA Dr's don't like to comment on the cause, they concentrate on whats in front of them now. Have your Blood checked, and get a baseline! This late in the game after Nam people like me are starting to show up with some mighty wierd stuff..Have your children's blood checked and get a baseline..

Here's the disease.....I am out of remission and it's touch and goe again...The Prednisone at this high dose level again is a real pain in the arse...But ya gotta do what ya gotta do...Life is worth the battle folks, no matter how louzy these damn drugs make you feel and look, the alternative? Well, for me I'm fighting!So there is not one..Doc's say I can get another 20 yrs...

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Evans syndrome
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Contents [hide]
1 Description
2 Those Affected
3 Signs and Symptoms
4 Possible Causes
5 Diagnosis
6 Treatment
7 Prognosis
8 The future
9 External links
10 References



[edit]
Description
Evans' Syndrome is an autoimmune disease in which an individual's antibodies attack their own RBCs as well as their platelets. Its overall pathology is therefore effectively a combination of the two autoimmune induced conditions: autoimmune hemolytic anemia and immune thrombocytopenic purpura.

Autoimmune hemolytic anemia is a condition in which the red blood cells that normally carry oxygen and carbon dioxide are destroyed by an autoimmune process. Immune thrombocytopenic purpura is a condition in which the platelets in the blood are destroyed by an autoimmune process. Platelets are a component of blood that contribute to the formation of blood clots in the body to prevent bleeding.

[edit]
Those Affected
The incidence of Evan’s Syndrome is not precisely known. The syndrome is reported to be a complication affecting 4-10% of those persons with a particular type of thrombocytopenia known as autoimmune thrombocytopenia purpura. The syndrome is more prevalent in children than in adults.

[edit]
Signs and Symptoms
The signs and symptoms of Evan’s Syndrome will be a combination of the signs and symptoms of the two underlying conditions. In autoimmune thrombocytopenia purpura the following may be found: Bleeding of skin or mucus lined areas of the body. This may show up as bleeding in the mouth, or purpuric rashes (look almost like bruises), or tiny red dots on the skin called petechiae. Laboratory results will show low levels of platelets

In autoimmune hemolytic anemia the following may be found: Fatigue, Pale skin color, Shortness of breath, Rapid heartbeat, and/or Dark urine

[edit]
Possible Causes
The cause of the signs and symptoms of Evan’s Syndrome are directly related to the low levels of red blood cells (RBC) and platelets in the blood. These low levels are a result of circulating antibodies that bind to the blood cells and destroy them. Antibodies are made under normal conditions against foreign substances in the body and are therefore very useful in warding off infection. In conditions that are referred to as “autoimmune” the body makes antibodies against itself. In the case of Evan’s Syndrome, it is not currently known what triggers this reaction to happen.

[edit]
Diagnosis
The diagnosis of Evan’s Syndrome is based primarily on laboratory findings, as well as the corresponding physical signs and symptoms. A complete blood count (CBC) will confirm the presence of anemia and low platelets. Additional studies may include a peripheral smear and a Coombs test. A peripheral smear of the blood may reveal evidence of red blood cell destruction or reticulocytosis. Reticulocytes are immature red blood cells and are usually abundant in Evan’s syndrome where there is a need to replace ongoing losses. There are also distinct shapes to certain cells that may be found when a sample of the patient’s blood is viewed under a microscope. In patients with Evan’s syndrome the red blood cells may appear small and globular shaped (then called spherocytes) but will not be fragmented. A Coombs test is used to detect the presence of antibodies against the RBC and is usually positive in immune mediated haemolysis.

[edit]
Treatment
The treatment for Evan’s syndrome will involve close monitoring of hemoglobin and platelet counts. To increase the number of platelets and RBCs in the blood, the patient may be given a corticosteroid such as prednisone. Prednisone will reduce the destruction of the platelets and the RBCs. A certain level of prednisone may need to be taken for the patient's entire life to prevent the levels of platelets and RBCs from dropping. Further treatment may be necessary with immune system suppressing drugs to block the production of the antibodies. To specifically treat the anemia of the syndrome, it may be necessary to provide the child with new RBCs by a blood transfusion. It has not been shown to be effective to transfuse platelets in a similar fashion to treat the thrombocytopenia. In more severe cases that are unresponsive to treatment, it may become necessary to remove the spleen. The spleen is the organ that is responsible for removing the platelets and RBCs from circulation in the blood once they have been marked with the antibodies. A person can live a normal life without their spleen but needs to avoid injury as much as possible.

[edit]
Prognosis
Given the fact that the signs and symptoms of this disease are related directly to the abnormal laboratory findings, the prognosis depends on the patient’s response to treatment. Spontaneous remissions of each of the individual component conditions have been reported. If the child responds well to the treatment and the levels of platelets and red blood cells increase, the child can expect to live a normal life. Medications will be needed life long, and laboratory tests will need to be constantly monitored to detect any abnormal changes so that treatment can be adjusted.

Evan's Syndrome is rare, serious, and has a reported mortality rate of just under 18%.

[edit]
The future
In a patient with refractory disease, an allogeneic hematopoietic stem cell transplant (HSCT) resulted in complete clinical and serologic remission for more than 30 months. Allogeneic HSCT may be the only current curative therapy for Evans syndrome, but may also be complicated by significant toxicities.

[edit]
External links
http://www.evanssyndrome.net
[edit]
References
http://www.madisonsfoundation.org/content/3/1/display.asp?did=81
http://www.rarediseases.org
http://autoimmune.pathology.jhmi.edu/diseases.cfm?systemid=5
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1 1781654&dopt=Citation
Evans RS, Takahashi K, Duane RT, Payne R, Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. Arch Intern Med 1951;87:48-65. PMID 14782741.
Mathew, Prasad M.D.; Chen, Gang Ph.D.; Wang, Winfred M.D. Evans Syndrome: Results of a National Survey. Journal of Pediatric Hematology/Oncology. 19(5):433-437, September/October 1997.


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Posts: 10729 | From: oregon | Registered: Feb 2005  |  IP: Logged | Report this post to a Moderator
Upside
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Damn Dust,
Your life sounds like a Jimmy Buffett song. Hope all goes well for you.

Posts: 5729 | From: Wisconsin | Registered: Sep 2003  |  IP: Logged | Report this post to a Moderator
Dustoff 1
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Ah' nuttin to it...Life is life, as long as we are looking down at the ground and not up through it!

Hey, just wanted to splain' I will be disapearing again periodicaly for treatments and such...

MOST IMPORTANTI IS...Others pay attention to getting a baseline on your blood, and that of your children, it can save a loved ones life, including your own....

I'm just small potatoes upside, I just hope that all of this can do some good for someone else...

Posts: 10729 | From: oregon | Registered: Feb 2005  |  IP: Logged | Report this post to a Moderator
jordanreed
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you touched me, Dust...


You are in my thoughts..

--------------------
jordan

Posts: 5812 | From: st paul,mn | Registered: Feb 2004  |  IP: Logged | Report this post to a Moderator
Dustoff 1
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Thankyou Jordon....
Posts: 10729 | From: oregon | Registered: Feb 2005  |  IP: Logged | Report this post to a Moderator
Mr. Lava
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Best regards and blessings on your treatment. It sounds like a terrible affliction.

--------------------
I came here to teach you about the enchanting rhythms of the universe...

Posts: 222 | From: Earth | Registered: May 2006  |  IP: Logged | Report this post to a Moderator
Dustoff 1
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Thankyou Mr. Lava...Sometimes it can get real tuff for my wife and daughter......But, again people need that baseline...
Posts: 10729 | From: oregon | Registered: Feb 2005  |  IP: Logged | Report this post to a Moderator
Mr. Lava
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Well, as they say, what doesn't kill ya makes ya stronger. And thank you for your service to America. It's too bad veterans have to pay such a dear price for their sacrifices.

--------------------
I came here to teach you about the enchanting rhythms of the universe...

Posts: 222 | From: Earth | Registered: May 2006  |  IP: Logged | Report this post to a Moderator
   

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