Hey readers! Don't know how many readers of this blog there will be, given that PAI-1 deficiency is a fairly rare - like one in a million - bleeding disorder. Too much PAI-1 isn't good - it's a sign of inflammation and a sign of danger for stroke - but too little gives you a tendency to hemorrhage.
But I wanted to start something for people who might have this disorder but have questions or discuss problems related to bleeding disorders.
Plasminogen Activator Inhibitor-One Deficiency is a problem, essentially, not of clotting, but of not keeping the clots around large or long enough. Some of the indicators are:
--uterine or nose bleeding
--easy bruising
--cuts that bleed a little bit at a time, for days
--anemia
--abnormal bleeding from surgeries and injuries, such as dental work or head trauma.
There are not enough of us with PAI-1 deficiency for doctors to really know how to treat it. Two possibilities are:
--blood transfusion with plasma (the factors we are lacking are in the plasma, not the red blood cells)
--A drug that helps clotting called Amicar. This is not a guaranteed fix - doctors and researchers basically think it helps stop our kind of bleeding, but not enough research is available to prove this as a failsafe method.
I myself was tested for this by a top hematological oncologist in Seattle after I nearly bled to death during a routine operation. It was very scary, especially since I had been tested for all the usual stuff - platelets, hemoglobin, PTT and they had all come in normal. It wasn't until my brilliant doctor noticed my TPA was off that she ordered - out of a specialized blood lab - a test for PAI-1. It's not a commonly done test, so if you live in a small town, or not next to a major research or university hospital, it may not be available. However, I urge you to continue to pursue the tests even if your platelet counts are normal. Go find a hematologist who knows platelet disorders.
Please comment if you have this disorder or are interested in discussing it.
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I am doing research on PAI-1 deficiency. If you want to be involved in this research be so kind and contact me by email or call me.
ReplyDeleteThanks in advance,
Jerzy Jankun, D.Sc., Ph.D.
Professor
Director Urology Research Center
Department of Urology
Mail Stop 1091
Health Science Campus
The University of Toledo
3000 Arlington, Toledo, OH 43614-2598
phone (419) 383-3691; FAX (419) 383-3785
I am very interested in this research. My daughter, 13 yo., was diagnosed with PAI-1 deficiency about a year ago. Her nose bleed is stopped with Amicar that she uses as needed but has had very strong and somewhat scary bleeding episodes. If anyone has additional information, I can be reached at 917 509 5593.
DeleteThank you,
Angela
I am in Doylestown Ohio. I was diagnosed MTHFR homozygous and PAI-1 deficient. I have been seen in the past by an oncologist, but was getting nowhere. I can be reached through email annabarnettonline@gmail.com if you are still doing research.
DeleteHi Jeannine,
ReplyDeleteI've also had this diagnoises. Would you mind sharing the name of your doctor and the lab that did your blood work?
Dear Jerzy,
ReplyDeleteI'd love to get in touch - can you give me your e-mail address? I'd love to know more about your research as well! Many of the papers I've come across involve mice, not humans...
Sailorett - I'd be happy to back-channel you the info - can you leave an e-mail address for me to send you the info? I didn't want to post it in case this particular doctor didn't want to be inundated, but my understanding (from other hem/oncs in other states) is she is one of the best in the country.
Dear Jeannine,
ReplyDeletePlease send me email: jerzy.jankun@utoledo.edu or call me at 419 383 3691
Thanks in advance
JJ2
Jerzy Jankun, D.Sc., Ph.D.
Professor
Director Urology Research Center
Department of Urology
Mail Stop 1091
Health Science Campus
The University of Toledo
3000 Arlington, Toledo, OH 43614-2598
phone (419) 383-3691; FAX (419) 383-3785
Are you still doing this blog? My daughter has a severed pai-1 deficiency. I have been desperate for the past 18 months to talk to someone else with the disorder.
ReplyDeleteTeressa Wright
twright595@juno.com
My daughter and brother have PAI-1 deficiency. From what our hematologist told us, Amicar is most effective on oral bleeds, generally not other bleeds.
ReplyDeleteIn addition to Amicar and plasma, if she has a life threatening bleed she needs Novo 7- which is SUPER expensive!
Hi!
ReplyDeleteJoining the PAI group, I've been diagnosed with PAI deficiency by 3/4 of my hematologists. I am currently looking for treatment options but because my hematologists are concerned that I'm having renal bleeding I'm not a candidate for Amicar (apparently it's hard on the kidneys?). Could you please pass on the information about your hematologist? I would really appreciate it. Right now we're being referred to Mayo by my current hem but it couldn't hurt to have another doctor's name. Thanks! It's so good to actually read about other people who are PAI deficient. There aren't many of us out there!
I now this post is two years old but ask your doctor about tranexamic acid. It was recently approved in the USA. I cannot take amicar either because of a bad reaction to it(I'm alergic to everything!). I hope this helps.
DeleteI have had bleeding issues for years and was just now told that this is what I have...can anyone give me additional information??? I have four children and so far I know that three of them are showing signs of bleeding. My oldest had now had two miscarriages, and her cycles are bad. My oldest son has bad nose bleeds for years. My youngest daughter was told she had low factor 8. However I fear it is this PAI-1...as I have. I was told that is all I had to for years until I almost died three times in different surgeries. Please send me information. Sheila Shipley dragonladyship33@yahoo.com
ReplyDeleteMy periods were awful. I have 6 losses. My doctor put me on Heprin to help. After having every test under the sun it came to be PAI-1. My blood was suffocating the baby.
DeleteAlexindra - I'm happy to pass on the info about my doctor, but I didn't see an e-mail. I'll try to get in touch with you if you leave a contact. I'm trying to get back with everyone as quickly as possible.
ReplyDeleteSheila - I've sent you an e-mail and hope I can help.
Pseudonym - I hadn't heard that about Amicar but it's good information. Any links or anything I can post?
Hi! I don't know if you are still doing the blog. I just found out that I am PAI-1 deficient when my OB checked me. Now I am taking Lovenox for the remainder of my pregnancy. I am shocked at the lack of information on this disorder! My doctor was tipped when I told him my father had an undiagnosed bleeding disorder. I was shocked to learn that I actually had something too. I have never had much trauma and have a very limited surgical history limited to a couple ENT related surgeries. The only thing I have had severely wrong is unbearable migraines since I was young. I'm wondering know if it could of been caused by the PAI-1 from small blood clots in my blood stream passing through my small cerebral vessels. I'd love to discuss anything that you all may have found from your own diagnoses. Thanks!
ReplyDeletejustine.drockton@gmail.com
Hi!
ReplyDeleteI don't know if this blog is still going, but I was diagnosed with PAI deficiency when I was 18 after yrs of being missed diagnosed as a hemophilia. After four different specialist and months of testing. A Cancer specialist tested for PAI-1. After that they didn't really do much, just that every surgery I have had sense then,(even wisdom teeth) I have had to get plasma transfusions prior to. I have also been diagnosed with a Autoimmune Disorder and recently was told I might have Fibromyalgia, Because of my Server Migraines that have ended me in the ER a handful of times over the years. I have always felt that my Autoimmune, painful joints, and migraines are some how related to PAI-1. The only thing is I have no real idea what that is, or what it means, all I do know is that most Doctors are scared to do anything because I mention PAI-1. I would Love to get some more info and talk to a specialist about this, Im 26 now and married and have been told when I was younger having kids would be very difficult to keep (lots of miscarriages) and the actual labor could be life threatening to me and the baby. Please Please some one get back to me, I would love to figurer out if any of these issues are related.
thanks,
reeceflarsen@gmail.com
Hi Reece! Sorry for my delay in responding. I was also told that having kids would be dangerous, if that is any help to you, and I've had multiple autoimmune diagnoses. I think they may be related as well, though my hematologist says there is no research yet on a connection. Many folks with PAI-1 defiency have to be treated pre-and-post surgery, and plasma is one way they treat it, along with medications (like amicar and tranexamic acid.) Good luck! Jeannine
DeleteGood afternoon everyone!
ReplyDeleteMy name is Christina and I am a 35 year old mother of 4 boys! I recently (yesterday) found out that I have this PAI1 deficiency. I recently had a stroke (or several mini strokes) this October 2011. I was hospitalized at Baptist Hospital where a team of Neurologists and Hematologists ran every test known to man on me for 5 days. I was released from the hospital with the diagnosis of a Cryptogenic Stroke (which means, they don't know what caused it). The only abnormal lab at the time was a Factor VIII level which little is known about as well. I was placed on Aggrenox and Simmvistatin as most stroke victims are and sent home; however, most stroke victims have a reason for their stroke.
Several thousand dollars and a few doctors later and I am sitting in the local Oncology office yesterday being told that one of the tests that was ran in the hospital (that no one had even checked before now) was this PAI1 factor and mine was deficient. As I stated before, I have 4 beautiful boys. But to comment on Reece's concerns about becoming pregnant, I have had four miscarriages. This physician seems to think it was all related.
I had my first son Gage with no problems or complications. I miscarried twice after him and I was told by a specialist that I had low progesterone levels and was placed on prometrium. I carried my next two boys with no complications either and took my medicine faithfully. Then I became pregnant again and the prometrium did not prevent the third miscarriage. Our fourth Gabriel was born January of this year. To our surprise we shortly found out we were expecting again. Unfortunately in August at 14 weeks along, the baby's heart stopped. I was given no clinical explanation as to why this occurred and had to go to surgery for a DNC. It was in this surgery that I bled excessively. I have had other surgeries in the past; however, this was the first time that the bleeding had to be countered with heavy clotting medicines. Then in October the stroke occurred.
This new hematologist stated that the miscarriages were linked to the deficiency and stated that if we decide to have more children, then he would strongly suggest receiving shots of heparin in the stomach daily until the baby was born. I hope this gives you some guidance or insight into you having children. It is not impossible and we have four wonderful boys!
Have any of you been told that you would have to be on Coumadin for this disorder? Apparently the Plavix and Aggrenox are mainly for platelet disorders and does not affect the PAI1. I am highly concerned with this Dr’s suggested method of treatment, as this is a very dangerous drug with serious complications.
Dear Christina,
DeleteIt sounds to me like your treatments and symptoms are actually the result of too much PAI-1 - not a deficiency. Someone with PAI-1 deficiency would not ever take heparin. Miscarriages and stroke are unfortunately both common among people with too much PAI-1 in their system, which can be the result of the same kind of genetic anomaly that causes PAI-1 deficiency. But I definitely would make sure and check in with a hematologist that has experience with people with PAI-1 disorders. Good luck!
I think the dr's confused. She bled too much.
DeleteI think the dr's confused. She bled too much.
DeleteHi!
ReplyDeleteMy name is Rachel. I am a 16 yo girl and was diagnosed with PAI-1 deficiency when I was 12. I had a severe intolerance to amicar, and it only worked for a week. I tried birth conrol pills, depot provera injections all to no avail at stopping my bleeding. I was put on three to five birth control pills a day when bleeding was severe, which made me very sick. I am now on depo lupron injections which shuts my ovaries off and puts me into a chemical menopause. Along with that, I am taking 10mg of aygestin daily. I must come off of the shot in June with a side effect of major bleeding in someone who has no problems with bleeding. My hemotalagists plan to give me tranexamic acid and low dose birth control to help. I also have nose bleeds that stop after the clots break down. I am about to get my wisdom teeth removed. For this I must be hospitalized and have three days of tranexamic acid. I also have been diagnosed with cervical dystonia which is muscle spasming of the neck and shoulders. I have other symptoms that I am trying to get diagnosed right now through mayo clinic. Please let me know if anyone has had a procedure with tranexamic acid, or just general information you think will be helpful to me.
Thanks
Dear Rachel, So sorry to hear about the amicar intolerance. My hematologist has said that tranexamic acid is a good alternative to amicar, so hopefully it will help you! I have read that tranexamic acid is sold over the counter in England for heavy periods alone, so in other countries it is widely available, just not here. I also have had cervial dystonia and have had good luck with treatment (holistic physical therapist - mine specializes in TMJ but does cervical really well too.) So don't give up! Good luck with everything!
ReplyDeleteI'm not sure if this blog is still active. I was diagnosed with PAI-1
ReplyDeletedeficiency about eight years ago after I experienced excessive bleeding after a biopsy. I take Amicar immediately after any surgical procedure. I had symptoms for years, but from what I've read a test was not available until the 1980s. My father had terrible nose bleeds when he was young and he always said he was a 'bleeder'.
My email is mafitzpatrick@att.net
Thank you - hope to hear from you ..
Wow i have all those same symptoms and i was diagnosed with PAI-1 last yr. I was 30 when diagnosed but have dealt with constant migraines that have lasted up to 28 days. I was given 3 day long infusions at my neurologist. A few yrs ago feb 4th 2011 to b exact. I was diagnosed with epilepsy but i had bever had a siezure until then. I have severe joint pain sometimes so severe i can hardly move. I have been to so many specialists no one can seem to figure out the cause of my pain migraines and siezures. Im slways researching as if im on some quest to find the why in order to find the solution. I take lovanox and asprin daily but the lovanox cause such severe bruising on my abdomen its hard to do regularly. I just find it very odd that others with pai-1 have alot of the same symptoms and yet no dr has notoced the connection or found a treatment. Please if anyone has anymore info or symptoms similar to mine please contact me at (330)203-9993. Leave a messege if i dont answer but i want to find as many ppl as i can and talk to my oncologist/hematologist. Maybe we can find a treatment if we come together.
ReplyDeleteI am a 76 year old CLL [chronic lymphocetic leukemia] patient diagnosed in 2013 and about the same time had a bleeding issue from a minor elbow surgery that kept bleeding below the skin.
ReplyDeleteStanford and Palo Alto Medical Clinic did all the clotting tests that proved normal except that I was deficient in PAI-1 [less than 2 (units of the test)]. My Dr.'s discounted this information and have just called it coagulapathy.
Anyway I would like to know if there is any treatment available as I continue to have issues with bruising, bleeding, hematomas and concern over future CLL treatment. Some of the better CLL treatments like Imbruvica warn of bleeding as a side effect and so this may rule me out of that treatment.
I have used Amicar during a 2015 hernia surgery but it did not stop significant under the skin bleeding.