Well, what started out as something quite simple (a banged knee), has turned into a very rare condition – so rare that they don’t even have a name for it! To give you an idea, my go-to doctor (the one who admitted me to the hospital) is a blood specialist, or hematologist – not an orthopedist or GP.
On September 16th, 2011, I noticed a bump on my left knee – on the inside of the knee, and it was kind of painful. Odd, I thought – I didn’t remember banging it on anything. Next couple of days I noticed the following:
1. The left knee started bruising – so I took it for granted that I must have hit it on something despite the fact that I did not remember doing it.
2. I started getting a lump on my neck – on the left side, right under the chin. It didn’t really hurt, but it started getting big enough to see. No discoloration though.
3. My right calf muscle started hurting as if I had had a leg cramp… the feeling you get in the muscle after you get a cramp – but I did not remember getting a leg cramp anytime in the last few months.
4. I started getting a lump on the calf muscle near the top – like a big ol’ knot.
Ok, I understand, I just turned 48 and I’m falling apart – sure, I got it. Fine.
As the lump on my neck grew and my concern grew with it, my gait began to suffer as I unconsciously began favoring it as I walked. The first day of pain bad enough to recall was the day after my birthday. We had taken Joy-Hannah around the block for a walk but came back after it began to rain. We did not get very far, but I figured that with a sore calf muscle I just shouldn’t have gone on a walk in the first place.
So the pain in my right calf steadily got worse as the bruising on my left knee slowly dissipated and the lump on my neck disappeared. As is probably fairly common among people around my age – those who are in between being young and being old – like adolescents who are no longer children, but not quite adults – failing sometimes to accept the physical or emotional limitations that they are moving towards, and clinging to that part of their life they are leaving behind — I figured that like the knee and the neck, the right calf would fix itself and heal and everything would be wonderful and hunky dory.
But when the pain did not go away in my calf, the knot got bigger and it became harder to walk each day, I realized that I had to go see the doctor. So if the pain was not improving by Monday, 9/26, I planned to just show up at the doc’s office and get it all fixed up, once and for all.
So that’s what I did. I went to my general practitioner who looked at my bruised left leg and the right leg which was starting to swell, and asked, ‘what happened to you?!’ When I replied, “nothing – no accident or anything”, they got concerned. Immediately I was sent over to the hospital for an ultrasound on my right leg to make sure there was no clot that could cause the lump in my calf. It came back negative.
So now they figured the lump was definitely a hematoma, which is where a blood vessel bleeds out into your tissue and clots there, essentially causing a bruise. If it’s close enough to the outer layer of skin, you see it as a bruise or black and blue. If not, then it’s a strange lump. So that would be the reasoning behind the lump on my left knee and neck.
Still, without a trauma of some kind to cause the bleeding out in those spots, they did some blood tests to check for any abnormalities. So they let me leave with instructions that if the leg has not improved in two weeks (two WEEKS!?) to call them, or if it gets worse, to call them immediately; and they would get the blood test results to me.
So the next afternoon, the doctor’s office called while I working. They had the blood test results. Since it was after their regular hours, I figured it was bad. They said I had some clotting abnormalities – that I should discontinue any aspirin products and all nsaids (like ibuprofen or the Aleve the doc wanted me to take) – basically anything that would thin the blood. They would have their referral coordinator get with me the next day (Wednesday) to get me an appointment at the blood specialist (hematologist).
That evening, I had my leg up on pillows as I lay in the couch, and I noticed what looked like some dirt on the inside of my foot, by the heel. Upon closer inspection, I saw that the discoloration was underneath the skin – and that it was blood. So, I called the doctor’s office and they told me they wanted me to go to the ER and get ‘evaluated’.
So, we went to the ER, they did some blood tests, which came out a bit worse than the tests they took the day before at the doctor’s office. But, the leg did not appear to be in any great danger, so they sent me home with instructions to follow up with my GP the next day and the blood specialist.
Next day, the pain continued to get worse and the lower leg continued swelling. They made an appointment for me for that Friday, so off I went. They took more blood, again the leg did not appear to him to be in any danger. He told me that the results from the first two blood tests showed an abnormality in the clotting factors, which mean that my blood, for some reason, did not clot like it should, which could cause bleeding out (spontaneous bleeding) with little or no cause, in any part of my body. The concern, I believe, is not so much bleeding in my leg, but what if that happened in a critical organ?
So the hematologist sent the blood off to be analyzed and he said he would call when he got the results. He actually called me later that evening, and he said he was confused. Though not all the results were back, what he saw did not ‘fit’ into any of the models he’s ever seen in blood clotting factors. He told me to sit tight, keep the leg up, rest and he may want to get me admitted to the hospital for testing and treatment – though exactly what testing or treatment was not clear.
It was the next day before I heard from him, and he told me to go to the ER at Trident Medical Center and they will do a direct admission. He said he would meet me in the room after I was settled.
So I went in, and the Dr showed up and we spoke about the possible treatments. The first thing was to try and infuse my blood with a ton of good fresh plasma – called a ‘plasma infusion’ and because the antibodies that could be inhibiting the factor 5 from having normal clotting times are part of the plasma in some way, infusing me with all new plasma might help.
When that did not work, the next step was to start a series of blood transfusions called ‘plasma exchanges’, once per day over the course of several days, or until the levels normalized. Once this happens, the leg should basically fix itself. As far as what happens then, if I’ll have to get further treatments or testing, I have no idea right now.
As of Monday, 10/3, the doc said that if my levels showed continued improvements, he would release me from the hospital on Tuesday! And that is what happened! I came home Tuesday, 10/4 but had to continue the plasma exchanges at the hospital as an outpatient for the rest of the week.
This past Friday, 10/7, Linda had some people come over to pray over me for healing. I was annointed with oil, the whole nine yards. It was very cool and I knew that God is totally in control!
As of today, Sunday10/9/2011, my leg is showing improvement in that the swelling is slowly diminishing and the pain is subsiding a bit. My levels are moving towards normal – slowly, but they are showing progress.
The doctor has ordered treatments (plasma exchanges) for Monday, Wednesday and Friday of this week. I will update this post when I get any further info.
UPDATE 10-14-2011: Spoke to Dr today and he is referring me for a second opinion to the clotting expert at MUSC (Dr. Greenberg) in Charleston, SC. Should be seeing him next week, but I don’t have a date yet. So far the plasma exchanges are not doing much other than to keep my clotting factors in line, but not back to normal, which was the hope. My leg is getting a little better each day, thank you Lord!
UPDATE 10-17-2011: Monday – Felt nauseous, short of breath and some tightness across my chest. Dr. Greenberg’s office said to get to the ER at MUSC. I did, was admitted when they got an ebnormal EKG. They ran a bunch of tests, starved me, and I was also seen by Dr. Greenberg and his team of hematologists at MUSC. I was released the next day when they found that my heart was OK. The symptoms I was feeling could have been from the prednisone I had been taking for the previous two + weeks. (nasty stuff!) . The ‘abnormal’ ekg just happens to be ‘my’ normal. Go figure.
UPDATE 10-21-2011: Friday – Had labs and in-office visit with Dr. Greenberg. He found that what the clotting issues came down to is my Factor X (10) levels were off. In-range, they should be from 50% to 100%. Mine was reading at 14%. Oy. He ordered some IV vitamin K, and the following week for my catheter to be removed (because he said he felt the plasma exchanges were not helping me) YAY! and on Friday he ordered to have labs taken, a plasma infusion to bolster my Factor X level and then more labs to test the result.
UPDATE 10-27-2011: Thursday – Went to MUSC to have perm-cath removed from my chest at 7am. With usual hospital time efficiency, it was 11am before anything was done, and because of some miscommunication, Dr. Greenbergs orders needed to be verified because in his notes in the system he said he wanted me to have two units of plasma infused before the cath was removed, however, it was not in the order. And, I was scheduled the very next day to have the plasma infusion and labs to check it’s effectiveness on my Factor X level.
So, they decided to moved the infusion up one day, do the labs and so then on Friday I would just go in and see Dr. Greenberg and talk about things. After the infusion and removal of the cath, which went very well, I was able to leave at 4pm!
UPDATE 10-28-2011: Friday – The plasma infusion did NOT budge my Factor X level. Still at 14. So now Dr. Greenberg is scratching his head. He ordered an MRI of my abdomen to look at my liver and spleen for this coming Thursday, and to rule out amyloidosis, they came in and did a needle biopsy of my belly fat pad to look for amyloid proteins. Lovely. Test results from the biopsy will take several days to a week to get back.
If I only bit my nails, I’d have something to do now…..
But, all in all, I am feeling good. In the Factor X world, my 14% represents a ‘mild’ case of deficiency. Anything 10% and up is mild, but under 1% is severe. That is good news! My leg is almost back to normal, walking does not hurt at all, and I really have no symptoms. My strength is coming back from all that time in the hospital, so I think things are looking up! (if you ever really want to feel sick, go stay at a hospital! Yuck.) I really feel all the prayers, the good thoughts and feelings coming my way from all over the country. Thank you, thank you, thank you!
Well, I saw Dr. Greenberg and his assistant today at MUSC, after they took more blood. Got the PT, PTT and INR levels back and they were all pretty high and getting higher (not good). The Factor X level was not back yet, but I will call later to find out what it is. Judging from these other levels, I’m not betting on it being any better than last time.
I got a written report of the MRI they did on my abdomen and the good news is that there were no lumps or masses anywhere, no blockages or anything with any of the blood vessels. Just an enlarged liver and spleen. So, taken with the biopsy they did of my abdominal fat pad which showed no amyloids, they are still not sure why my liver would be enlarged.
They are referring me to a liver specialist at MUSC – I will hear back today most likely with an appointment time.
They will probably want to do a liver biopsy to check there for amyloids. Evidently the fat pad biopsy is not completely conclusive because there would a higher concentration of amyloids in an affected organ, and in this case, they suspect amyloidosis of the liver, which would explain everything, they said. It would NOT be good, but it would explain the clotting issues and enlarged liver and spleen.
So that’s that. Dr. Greenberg told me the liver care at MUSC is rated in the top ten across the country, so I’m in good hands. Nice to know. Dr. Greenberg is still thinking it may be fatty liver which could have been caused by my weight or weight loss, but he’s wanting to see what the liver doctor will say. Me too.
He also cautioned me to be very aware of any bleeding – like if I notice any pain or bruising – to get to him or the ER immediately. So, no paintballing for me!
In any event, I’m just waiting and wondering when this whole thing will be resolved. It’s going on 6 weeks! Enough already!
The Liver Biopsy: Well, it was a much longer day that we had anticipated, and I am exhausted as I write this. Linda was with me the whole day, thankfully!
The appointment was for 8am and we were told that I would actually go into the procedure at 10:30am. The 2.5 hours would be to get labs, results, and most likely some fresh frozen plasma (plasma infusion) to help with clotting.
Well, the plasma infusion that was ordered was for 2 bags, and that didn’t even start until about 11am. And they had it running at 150ml/hour which is very slow. Each bag was over 300 ml, so we were looking at 5 hours to get 2 bags of ffp inside me. Crazy.
I asked if they would step that up. Last time I had the plasma infusion was just before they removed the catheter in my chest, and that ran that at over twice that rate. I told them that and the nurses checked for me. The attending doctor said no, 150 is what he wanted. OK, fine. I emailed Dr. Greenberg (the hematologist – coagulation specialist) and asked him to call the attending physician and have him step it up.
Well, that didn’t work because they never did increase the rate.
Finally at 10 minutes to 6pm after the plasma was finished and another round of blood work, they took me in for the biopsy. I hadn’t eaten since the night before at around 8. I was miserable.
The biopsy went well, though by the way they prepped me, you’d think it was major surgery!
The pain was minimal, and it went very well with no bleeding at the site on my neck, or at the liver, as far as they could tell.
(they did a trans-jugular biopsy of the liver, where they put a catheter into my neck, into the jugular vein which goes right into the liver.)
The whole thing took about an hour, and afterwards they wheeled my back to the room, and then said we had to stay there for about 2 hours so they could monitor me. Finally we got to leave at 8:45. So what we thought was going to take 4 hours or so took 13 hours. Thanks, MUSC!
They said it could take a week or more to get the results back, but I am going to start calling the liver doctor (Dr. Wilner) tomorrow to find out when they will be ready. I do not yet have a follow-up appointment with him.
Thanks for reading! Blessings to you and many thanks for your notes, cards and words of encouragement and prayers!