This is a nasty little booger to deal with believe me. But, the good thing is that as it has become more diagnosed, it is being more understood.
The first time we heard of this diagnosis was after my Uncle Harry developed a blood clot in his leg after nasal surgery. At that point, three of his five older brothers were dead from heart attacks, so his doctor set out to see what was going on. My mom was still living at that point, and she signed release papers so Harry's doctor could review my dad's file. Ultimately, the diagnosis of Protein S Deficiency came down the pike.
My next annual physical was coming up so I brought it up to my PCP. He did some quick research since he knew nothing about it and advised I would need to see a blood specialist for testing and diagnosis. Later that week his nurse called me, having already made an appointment with an oncologist for me. She said that my platelet count was low and since I needed to have the other testing done, Dr. Graham was going on and referring me to TN Oncology.
Now, here I am going "oncologist?" That could only mean one thing to me - of which I was too scared to think. I went to the public library that very day and looked thru a book about cancer published by the American Cancer Society. I will never forget how weak my knees went when I read that leukemia is usually diagnosed after an inital blood test shows a low platelet count which dictates further testing.
My babies were 7 and 9 at the time - I thought I was going to die - literally. I didn't say anything to anyone but I got my butt into the appointment. I spent a least an hour with the doctor that day - she was good at explaining what was known at the time. AND, my platelet count was low because my platelets had clumped! Whew, what a relief! They had clumped because of the Protein S problem. There was no cancer at all!
You are born with all the Protein S your body should ever need. You don't produce it and at this point, it is not synthetically replaceable, so, in our bodies (it is a hereditary condition) somewhere after puberty starts, our bodies destroy the Protein S and sometimes Protein C that we have. Protein S works in your bloodstream as a natural algae eater, if you will. It keeps the insides of your blood vessels cleaned up - kinda like the roto-rooter that nature provided. But, if you've lost most of your Protein S there is plaque build up in arteries and this is not related to cholesterol at all. Couple the clumpy platelets with narrowed blood vessels and you have a recipe for a blood clot. NOTE: When my dad died, his body had vascular breakdown completely through out his system - but his cholesterol was less than 160. Even when I weighed 601 pounds, my cholesterol was 161. Doesn't make sense does it?
There are some things a person with Protein S Deficiency should never do - like take birth control pills. I had done that in the early 80's, before the first blood clots in my left leg, and had to discontinue them. Surgeries should be accompanied with anti-coagulation before and after, hormone replacement therapy is never an option, even dental work needs to be discussed between dentist and PCP.
Diagnosing Protein S Deficiency does require specialized labs that need to be ordered and read by hematologists. It's not something your family doctor will be able to diagnose nor treat. I was positive and my sister Amy was tested too - she is deficient. That has probably been the root of her miscarriages.
The kid's pediatrician was aware of this possibility so Renee was tested once at 14 - everything ok at that point. When tested again at 19, she was a 'low normal'. That bloodwork needs to be repeated this year. At this point, Lynn hasn't been tested. Neither have Mike or Larry - something that seems to run with the men in the family. My cousin Mylinda has been tested and is deficient, her brother and all our male cousins refuse to be tested.
Now that I also have an irregular heartbeat, anti-coagulation full time is necessary. At this point, I am on a monthly blood check for my INR - or Pro-Time - clotting time - check. I am to stay between 2.0 and 3.0 - and usually do this with 5 mg of Coumadin 6 nights a week, 2.5 mg on Friday nights only.
Lots of things can affect clotting - for instance - I am not to drink green tea and limit the green leafy veggies I eat. I love turnip greens, eating a big bait of them will definitely thicken my blood because of the Vitamin K in them. ALso, sickness, colds, vomiting, diarrhea - any of these can change your count and may require a weekly check of the INR. Antibiotics - well they bring their own special brands of hell - and you'll be getting blood drawn once a week for a month or two to recover and maintain after them.
Alcohol plays a whole other part - I don't drink anything at all now because of the meds I take. My blood is thin enough and alcohol will thin your blood even more - so it could get very dangerous. Your blood can get so thin, you will begin to seep blood thru your skin pores. That is a crisis and would require a 911 call if that ever happened.
For the persons who are Protein S Deficient, it is critical that they wear some kind of medical alert ID - and that workplace and family members know medical treatment, etc. in case of an emergency where you are rendered incapable of speaking.
But, knowledge is power. All of these things I know and they've just become part of my regular routine - I don't even really think about them.