PVNS: experience and causes

You are fortunate indeed. I never had the slighted indication of any issue until one day the knee blew up while I was sitting for a long period of time. When I started to get up the pain was indescribable. My knee had enlarged by 4.5 inches and was fluid. Of course it turned out to be blood. I then had 3 scopes with each taking 200 cc plus removal by needle 5 times with 65 to 75 cc each time. All of that in less than 3 months. 30 rounds of radiation followed ( regret every allowing that to happen) a synovectomy and tkr. All this time I was aware they couldn't remove all of it as there is a little bit in a bundle of nerves and I could lose My leg if they messed up the surgery. Its still eating and I've had a revision and several other surgeries due to staph. According to medical people you are in the age group that's susceptible (19 to 45). I was 65 when things blew up and now at almost 81 I'm like a time bomb just waiting to explode again, probably for the last time. Its caused a bad limp and back trouble. I go in 10 days for another MRI to try and figure out why the pain can't be controlled.

Not to belabor the issue but my wife has advanced Parkinson's disease coupled with severe depression, anxiety and dementia. I am her primary (only) care giver. Nearly 60 years of marriage and a promise of not putting her in assisted living keeps me trying to make it happen.

K

Like I said early on, you are very fortunate that yours was dis covered how and when it was and the ibjections were there and given. I certainly hope you can keep up with your life going the same direction.

I talked to a surgeon from a children's hospital. He was telling me of a 16 year old that had 9 surgeries and was already slated for another. The disease was coming back in more than one joint. The poor girl was lying there screaming in pain waiting to undergo the synovetomies understanding they would only be temporary.

Good luck and best wishes to you. Thankds for sharing Your success story.

Hi cryogen,

Its great to hear a positive outcome with diffuse pvns, but I have to say you're very fortunate that the Yttrium injections worked. My pvns was identified back in 1988, and after 10 or so failed arthroscopic synovectomies, I too had a Yttrium 90 injection done - 1994. That didn't make any difference as the disease progressed unabated, but the Yttrium had - as my surgeon forewarned - aged my cartilages & ligaments significantly.

I went on to have another 20 or so open Post/Ant synovectomies over the next 15 years, and still the disease did not abate. I've always been a very active person, never one to let pvns hold me back, and so eventually - because of the aged cartilage - I wore out the knee to the point that I'd ground through my medial side articular cartilage, with a high degree of bone loss.

I was in serious pain by then, and needed a full length unloader brace to walk. The damage in my knee was too bad to have a standard knee replacement - which shouldn't be done if you still have active pvns anyway. So in 2014, I opted to have the whole joint removed. I had a Distal Femur resection done, and a full prosthetic femur & joint implanted. That was a raging success and with hard work, both before and after the implant op, I have been able to achieve better strength & range of motion than many people have with a good knee. I'm now back to being very active, with no more pvns, and am able to enjoy life more than I have for decades.

So I'm glad you've been able to rid yourself of pvns, but do be careful about how much hard work you ask of your knee in the years to come. I wouldn't be doing a lot of high impact work like running/jogging etc.

Best regards, and congratulations on beating pvns & living an active life again.

Roger

Hello Roger. Thanks for the response!

I had the same concerns before getting the injection, and my Rheumatologist who injected the Y-90 mentioned that: because Y-90 has a half-life of around 2 days and because the dosage is controlled, it will only be able to disintegrate the thin Synovium (and hopefully the leftover PVNS) with no damage elsewhere (she did mention about some standard side-effects such as infection, radio-necrosis, etc. which are rare). As I am monitoring my condition in the medium-term, looks like she is right. I hope she is right in the long term as well.

I was given a PDF document by the doctor containing all details of the procedure including before and after care; here is a link: https://1drv.ms/b/s!As_tSFoxXaIElDh4VQoURKud_6z6

By the way, in my case, even if it did some damage to the cartilage and ligaments, it would be less when compared to intrusive surgery (which requires "shaving" the synovium manually), or if the PVNS was left as-is.

The key, based on my experience of suffering from the disease, is probably to get the Y-90 soon after the Synovectomy to kill-off any leftover PVNS.

I am happy that you are enjoying your active life. Happy times!

I am glad that you have such courage to deal with your condition and take care of your wife at the same time. Courage certainly gives rise to hope.

I hope a cure to this disease is found soon, so future generations do not have to go through these invasive procedures.

Hi cryogen,

Yes, I agree that if you can knock out pvns early on with the Yttrium, its definitely a better option than years of surgery. Sadly for me, it didn't stop the disease whatsoever. Each case is different, but one thing is certain in my case. The morbidity that I was forewarned about certainly occurred. I dismissed those concerns & refused to moderate my activities & kept on jogging, water skiing, snowboarding etc, and by my late 40s, the Articular - main - cartilage had ground away, and indeed a lot of medial side bone head too. The right side knee is absolutely perfect and hasn't given me any problems at all. In fact I can still even bend my right heel back into my butt.

You know, the worst part of all of this for me has been, trying to explain pvns to people. Most people don't seem to grasp the nature of this thing, and tend feel that you just have 'problems' and need to see their brother, mother or cousin's surgeon or something.

When the ongoing fight shapes & influences your choices, your life gets shaped too. At 47 I had to accept a redundancy from the company I worked for because I just couldn't cope physically anymore. Now that I'm finally free of the disease, I've clicked over 50 and have become chronically unemployed. Hey, but there're people who have worse, so I just keep enjoying the simple things and enjoying a pain free knee. 

All the best anyway. Keep on keeping on!! 

Roger

If its PVNS you will have only 2 choices, surgery or radiology. My advice is under no circumstances allow them to do radiology. My radiology consisted of a 90 minute scope, much longer that the usual. I had so much bleeding that had to be cleaned up before they could continue. I was hospitalized for 3or4 days and laid up at home for a week. Again, I was 65 when it hit and pain and swelling was unbelievable. Originally, through various procedures, they had to drain over 1000 cc of blood in 2.5 months. I was bleeding internally back into my thigh,groin and lower abdomen. Most people that have reported on here have not talked about bleeding but have talked about the pain and its intent s ity. Even with the 11 surgeries, 3 different prosthesis and radiation they can't get all of the tumor as a small portion is embedid in a nerve bundle and surgery could cause me to lose my leg. On the flip side of the coin, I'm the only person that I've ever communicated with whose had the disease that's ever had all of these systems bundled in the same package. The typical PVNS patient that I've ever seen profiled is a Caucasian of either sex, between the ages of 16 to 45. I visited with one Dr from a children's hospital that had a 9 yr old and my surgeon had a case with a 73 y/o as the patient. Some of the medical people that I've talked to seem to think it was my age that created pain and bleeding. You might go on the Web site of Stanford Medical center in CA and look at their data. They have been looking at a large data sample size and have been doing some research. One of the problems I've personally detected, its a rare disease. One in nearly 2 million. Its a enough to be a problem but apparently too small of a problem to spent much money on research.

What I have said shouldn't be considered something to frighten you but just inform you of what I've found in 15 years of struggles. One very recent contributor from Australia, I believe has had other results from early detection and injections with a drug I'm not even remotely aware of, even by name.

Best if luck and stat in touch. There are enough of us in here with the disease that your experience is very welcome info.

BTW I'm in the Kansas City area in the US

In case your PVNS is confirmed, I do not recommend impact exercises at any stage as it would worsen the joint. That is the sad truth. Surgery may stiffen the joint which may affect range of motion. In my case, it is around 80-90% of the original, plus I cannot do the yoga sitting position.

Sorry to hear about your condition. Wishing you luck.

Hi there,

I'm really sorry to hear of your troubles with this disease. I fought the 'Diffuse' form of pvns disease from my 20s in 1988 until 2014 with in excess of 30 operations - mostly open anterior/posterior synovectomies. I believe I would have beaten the disease eventually, but unfortunately I had a Yttrium 90 injection in 94 - which all my surgeons have since concluded as an ineffective and harmful treatment. A side effect of this radiation treatment is to mortify cartilage, and thus over time, an active life will wear it out completely. This happened to me by my late forties to the point where the knee joint was totally destroyed and I had it completely removed and a prosthetic joint implanted - which fortunately works extremely well.

The very aggressive nature of my pvns was quite rare, and usually the disease can be beaten in between 5 and 10 ops. Don't let anyone con you into a Yttrium 90 injection. It was considered leading edge treatment in the mid 90s - when I had it - but not any more. I urge you to do some research into it before you even think of going down that path.   

http://ard.bmj.com/content/65/10/1384

Look hard for experienced surgeons who really know this disease, and have treated many cases surgically. Surgery will of course create scar tissue in removing the joint synovial lining, but that has absolutely no impact on the life of your joint cartilage and therefore your knee joint. Y90, however, will definitely accelerate the cartilage degeneration over time, wearing out the joint.

Take this on as a fight, and get on the front foot with it. I never let my knee issues interfere with my living. I was still water skiing, snowboarding, trail walking and jogging even as I was having the surgeries. I worked hard on the recoveries with cycling & swimming maintaining good muscle tone in that leg - which is important in the fight. Unfortunately the radiation degraded my cartilages and that's the real reason I lost the joint - confirmed 100% by the very experienced surgeon who did my distal femur implant.

 I wish you all the best of success with the fight. You will beat this!! 

I agree. I believe that if I hadn't had the horrendous bleeding At the start I would have had a chance but when your leg turns blue, black and purple from your lower abdomen to below your knee within a few hours the fear overwhelms you, then couple that with a bunch of docs that have never seen it leaves you completely willing to do the 1st thing that's suggested.

Big mistake on my part.....the radiation ruined my chances of ever walking properly or being completely out of pain again. As I always say: a million dollar leg that still doesn't work properly. One of the more cynical medical types has told me: you'll leave it on the tray of the crematorium. A little morbid but probably realistic.

Hello Roger.

Looks like we had very different experiences with Y-90. It worked for me but certainly did not work for you based on your experience. Also new to me is to know that it had degenerated the cartilage over time, wherein I am confused as Y-90 has a half-life of 2 to 3 days. My doctor in Sydney (Richard Boyle) has an opinion that Y-90 is a temporary treatment as PVNS will return (and he did not mention about any side-effects of cartilage degeneration during consultation), and I disagreed with him based on my treatment.

I do agree with you that it is important to take expert opinions from experienced doctors. Preferably with various backgrounds (I recommend surgeons, oncologists and rheumatologists). Their opinion may not be the same but will help you explore various available options. As one of my surgeons who did my orthroscopic synovectomy put it after the PVNS returned: "As a surgeon, I like to deal with everything using surgery. So get an opinion from other doctors as well, as it is very hard to reach all the corners of your joint to completely remove the diseased synovium; at the same time, as we use a fluid to keep the joint clean during orthroscopic surgery, there is a risk that the PVNS will be carried into other parts of the joint". When I visited an oncologist, he wanted to perform a completely open surgery (from the front side) so as to have a good view of the tumour, as diffused PVNS is known to return in many cases. In the end, I decided to get Y-90 based on a second opinion from an Orthopaedic surgeon as there were "small pockets" of PVNS left, along with a slightly thicker synovium.

Also worth noting that I only have medium term data (16 months after the first injection) and lack long term data (>5 years). So I could be wrong (will try updating this thread over time).

By the way, I have a newborn who was conceived 6 months after the first injection and we were very afraid if she would have any side-effects of that radiation (our concern was: has the sperm been irradiated?) - thankfully she is born healthy and normal, and now at 7 weeks old.

Nevertheless, this forum is meant to share everyone's experience, and so did I.

By the way, here is some recent research, that supports my personal experience:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4290066/

Hi there,

Yeah pvns is a weird animal to deal with. It can be dealt with in as many as a few ops, or sometimes its just so aggressive you're in for a fight. In my case it was 27 years, three relationships, curtailed career options and a sh_t load of expense.

The surgeon that commenced my treatments in 1988 was using arthroscopic means to do the synovectomies, but - as you alluded - he couldn't get every bit of it, because its diffuse  ...everywhere & all over the synovium & other structures. We tried the Y90 in 94, but it was ineffective & Peter didn't want me to have any more radiation exposure.

So after about 7 or 8 of those, he went to the open technique - posterior and anterior - to get more of it out. He told me he scared himself in the last op he did because the back of the knee is a very very busy vascular & nerv highway. So he handed over to a more accomplished musculoskeletal reconstructive tumor surgeon who had a lot of experience with this condition. That was in 2000, and Mark did one, sometimes two per year for the next 14 years and still pvns came back, but slower and less of it.

I pressed on with my life relentlessly & defiantly being 'Action man' but eventually when the cartilage in that knee ground away & the joint was too destroyed for even a normal total knee replacement - and no surgeon will do one if you still have active pvns anyway - I was offered amputation. Only at the very last moment, and after I'd succeeded in getting a t&pd claim from my super's insurance - Metlife - was I offered another last resort in the distal femur implant, which I had done in May 2014. And that, my friend was an absolute life saver!!

It was a BIG effort, but worth it. I spent 3 months swimming 5km a week in the local pool - doing leg strokes - prior to surgery. Then the 5 hour op, and then worked my guts out in recovery. I now have better range of motion in that knee than most people who've had a normal knee replacement op, and I routinely do up to 30k steps a day getting stuff done. I could only do about 7k by Jan of 2014.

I understand your worries about conceiving children after the Y90 totally. The Docs reassured me it was fine, but It really worried me too. It was a moot point anyway as my 3 relationships never reached that point. Now I'm married to the 'right' girl, its too late - and she can't have children anyway.

Its good to swap notes and experiences in such a forum - as you say. Its the only lifeline for people when they find out they're fighting this thing. I actually met one of my best friends in the early naughties looking for answers on pvns. She lives in Perth and we've become great friends & regularly catch up. Michelle had around 10 ops, and two Y90 doses, and finally beat it. But she has a really badly stiff knee now. Michelle and her husband were actually at our wedding and we went away with them on Hols after too.

Good luck with your knee. Hopefully the fight is done now and you can get on with enjoying a great family life. And congrats on the healthy newborn too.

Hey OFG,

Yeah pvns is such a sh_t anyway, and the last thing you want is to make it worse with some failed treatment. I'm sorry to hear of your situation. Mine was similar in the way I accepted the Y90 injection. I knew no better, and quite frankly, nobody else did at the time either.

Too late now anyway, the damage was done in 1994 and in the end I had the entire joint resected. Good news is that the prosthetic implant is fantastic. I really fixed me up. I was a last resort, as they apparently usually only do these ops for cancer patients or major trauma cases  ...and older - 70 to 80 ish.

It sounds like you could do with the very same thing. Just cut the whole grumpy painful diseased joint out from a third the way up your femur, to the top of the Tibial plateau, and hurl it in the bin. The prosthetic works so effectively, you forget you even have it. I went from only being able to do 7k very painful steps a day, to now easily 30k painless steps per day and a new life with no more ops or treatments

It might just be worth exploring??

Best of luck though, whatever you do. And keep up the fight, keep on posting because you can at least help others as you have already.

Best regards,

Roger

i have not had any recurrence of the disease since the afte 4 years. Unfortunately I have just been diagnosed with gout in both knees, elbow and shoulder, which I have had for 3 years. This is not related to thr PVNS. I did not have radiation treatment.

The truth is that the disease seems to be different for each person. Early diagnosis is good and there seems to be a little more knowledge about this rare disease now. I live in rural England where the population is low and the hospitals remote. The cities are more likely to have surgeons who have come across this before.

Best of luck - it may not turn out too badly for 

I didn't make matters a any better w with my own actions. When I had to have a revision 9 yrs after my original tkr I thought I knew the routine and could fly through it because of experience. Morning after surgery I was out of bed, on my feet, so to speak, off to the the bathroom and my 1st potty break. Then the little stroll around the room and out of bed time in the big chair. I'm doing great so I decided to wash and shave. That being done I thought I'd slip on some underwear. I got the good leg on and the bad leg partially on but couldn't get them up while sitting. All the while very little pain as I had the femoral block in place. I needed to stand to pull the shorts up...big mistake. I didn't ask the nurse for help and when I got up I looked down and my foot was starting to roll inward. I had a tray table in front of me but couldn't get in position to stop and down I went. When I got my senses, my head was toward the opposite end of the bed, my legs in a figure 4 and I had broken my femur in a torque or twisting type break. I looked around and all I saw was feet. I don't know where all those people came from but there must have been 10 surrounding me. Somehow they lifted and turned me putting me back in bed. In less than 30 mins they had my leg in a trapeze and elevated higher than my head. In less that an hour they had me in and out of Xrays. I had twisted the femur into at a 20 degree angle, from my knee toward my hip. Next morning I'm back in surgery getting a piece of steel inserted next to the bone from hip to knee and being held in place with four 2" screws in the knee and three screws that went nearly through the femur. 10 months later that was removed and my real problems started. Staph infection, reopening the wound and flushing the opening,8 weeks of twice a day antibiotic infusions. 10 months later the staph returned, this time removal of the prosthesis, cleaning, inserting a plastic spacer with antibiotics, closing the wound placing me in a hip to ankle soft cast, inserting another PICC line and starting another 8 week, twice a day antibiotic protocol. After that 8 weeks I had to wait 2 more months for testing to make sure the infection was completely cleared up and then my 3rd prosthesis was inserted. All the while, I am trying to be the caregiver for my wife who has advance Parkinson's disease. One year later she fell and broke a hip and had to have a partial hip done. Now dementia has taken its toll and I'm about ready to put one of us in assisted living because I can no longer take care of both. I joke about one of us as she is fighting me at every turn about her having to leave her home and have someone besides me take care of her. Like the old saying goes, if I had known I was going to live this long iwould have taken better care of myself. Nearly 60 years if marriage and just waiting for the next phase. Sorry for the length of this boring saga but it does have a moral: keep your feet under you at all times AND marry someone a lot younger. However, outside of the blasted PVNS I wouldn't trade it.

Hi again OFG,

I've read bits you've previously posted, and know its been a chronic ride for you, but I didn't realise you'd actually had a spiral fracture in your femur   ...followed by several bouts of nasty infections and metal & plastic strengthening plates.

I know that spiral fractures are the enemy of any kind of healthy bone healing, and indeed, if I ever have an accident with my prosthetic knee joint, I'll likely end up with a spiral fracture and then having the leg amputated as the integrity of the metal bone union will be lost. Nothing substantial & strong enough to work with.

So I'm also guessing that - unfortunately - that's possibly why this type of thing hasn't ever been suggested as an option for you?

On my weekly Brochure letterboxing run, I have gotten to know another fellow - about my age - who also has a chronic knee issue. He has shattered the Tibial plateau of his left leg, and it's no longer strong enough or viable enough for him to be able to have any kind of replacement done. The medico's have done all they can over the last ten years, but its still a painful & useless thing. So he's come to the point where he's now considering having an amputation above the knee. There will be a net benefit to him from doing that. Our bodies are really so fragile and, sadly, medicine cannot always find a way to make things improve.

Keep up your fight and your spirits. These things define us and give us reason in life.

Best regards,

Roger 

So far ythe healing has been complete but of course I still fight pain in the leg. Right now I have been hit by a severe case of sciatica and will have an MRI a week from today. Pain med doc won't do any more injections till he sees what's going on inside. Pain is steady 8-10 so may need another decompression surgery. Had one 7 years ago and it was a miracle. Having to care for my wife has become my biggest objective. Fortunately I don't have to actually lift her so I'm not putting pressure on the leg or back.

I have no reflexes in my left (bad) leg and about 50% in the right. The Dr tries not to laugh when he pops me with The rubber hammer but its hard not to when the leg doesn't move.

You're right about the fragile nature of the body and yet the strength is unbelievable as well. We just keep on going as long as we can. I'm not ready to call it quits yet.