Waiting Game

I'm becoming a professional waiter and I'm not talking about the ones that serve food. It's been a month since I went to see my primary care Dr. about having flank pain. At that appointment I told him that the lateral side of my knee had been swollen for at least a year with an oblong soft mass on the back of my knee for 2 months but it didn't hurt nearly as much as the flank/back pain. Since then I've been waiting for this exam, that consult, this opinion and test results. Test results have shown pleural thickening in bilateral lung bases and PVNS in my knee. I know that it would be extremely rare but not unheard of for PVNS to be in the spine, knee and lungs. 

The docs haven't been able to identify the cause of the flank pain yet.  Now I'm waiting again until Thursday to see my pain specialist to determine if the flank pain is originating from the area in the spine in which my baclophen pump catheter is inserted. The pump controls severe muscle spasms due to previous neurological damage (unrelated to PVNS). I'm sure another MRI is in order. 

I am also waiting for my appointment on Friday with a orthopedic oncologist who is supposed to know about this "pain in the joint disease." I will be interviewing her to see if she has the expertise to treat my knee.  I'll post my interview questions under another heading. I'm very apprehensive about this appointment. I've read many accounts of this neoplasm returning after surgery and quite frankly I don't want to be included in those statistics.

JOKE OR REALITY

Three doctors are in the duck blind and a bird flies overhead. The general practitioner looks at it and says, "Looks like a duck, flies like a duck... it's probably a duck," shoots at it but misses and the bird flies away.

The next bird flies overhead, and the pathologist looks at it, then looks through the pages of a bird manual, and says, "Hmmmm...green wings, yellow bill, quacking sound...might be a duck." He raises his gun to shoot it, but the bird is long gone.

A third bird flies over. The surgeon raises his gun and shoots almost without looking, brings the bird down, and turns to the pathologist and says, "Go see if that was a duck.

Went Hiking Today

I finally looked up from my computer and realized I haven't stepped outside and smelled the fresh air for 5 days.  It's been just over a week since I found out that I have PVNS. I'm finally wrapping my brain around this disease after spending countless hours researching. Knowledge is power "so I should be lighting up like a flood light."

Prior to telling others of PVNS Diagnosis

Most people don't understand what PVNS is and why it's a life changing  diagnosis. Due to this fact they may not respond with the level of concern that this diagnosis warrants. When you tell someone you have a cold vs cancer they understand what that means and how to respond, unlike PVNS.

It's very difficult for me to tell my family of any illness or difficulty due to past events. Prior to my diagnosis of PVNS I had a five year medical history involving multiple hospitalizations and five months in hospice care due to a neurological disorder. As you can imagine it was very difficult for my children who were in high school and newly graduated at the time. People became worn out from watching me suffer and at times they just want it to end. This can lead to resentment, anger, guilt and sometimes estrangement from the person or situation. I believe this is what happened in my case. So believe me when I say, the last thing I want to tell my children is that I have a rare joint disease (awaiting confirming biopsy) that is not easily treated and that has no definitive end.

We live in an instant society where definitive endings are expected; if you have appendicitis an operation instantly cures you...bacterial infection and you're good as new after a course of antibiotics... broken bones and a cast is in order. How does one cope when their disease is long term? I'll address this topic on another post.

Getting back to telling family members about the PVNS diagnosis. First of all I need to understand this disease so that I can educate others about it. In the last five days I've read many case studies, forums, blogs, medical journals and books. I felt well prepared to tell my daughter and clinically I presented the disease and treatment well, but I left out the most important part "the human factor." I didn't tell her that I was really scared of this diagnosis, the treatment and it's unpredictability.  She asked if it was hereditary and I notified her that it doesn't appear to be. "Why can't you get something normal, why a rare disease" she asked. What am I supposed to do? If only I could have the doc choose a more socially acceptable disease that everyone understands. A disease that can be quickly fixed without additional disfigurement of my current patchwork of a body. A disease with a guaranteed happy ending. Sorry folks life doesn't work that way. I personally feel that I will be a very lucky woman if it turns out that I only have PVNS.

To do List prior to notifying people of diagnosis:

1.   Research: 

• Pigmented villonodular (vĭl'ō-nŏj'ə-lər) synovitis (PVNS)  is rare, almost always benign, locally invasive tumor of the synovium. PVNS may be nodular, diffuse or rarely a combined form. PVNS is usually diagnosed with a MRI and confirmed upon biopsy during surgery.  PVNS doesn't seem to run in families. This joint disease is characterized by inflammation and overgrowth of the joint lining.  It is usually only found in one joint but may be found in multiple joints and anywhere that there is synovial tissue. It affects the knee 80% of the time but may occur in the hip, shoulder, ankle, elbow, hand, foot, tmj and the spine. The synovium lining makes extra fluid, causing swelling and making movement of the joint painful. There is controversy whether PVNS is a neosplastic or an inflammatory process and there is a cellular difference between nodular and diffuse PVNS. Surgery is usually indicated and radiation therapy may also be useful in killing the remaining cells, but not without risks of it's own. PVS is aggressive and returns about half of the time. In the worst case scenario joints must be replaced and very rarely amputation.

2.   Differential Diagnosis: 

• hemophillic arthropathy
• soft tissue sarcoma
• fibromatosis
• synovial chondromatosis
• septic arthritis
• inflammatory arthropathies
• hemorrhagic synovitis
• tuberculosis

3.   Treatment Options:

• arthroscopic vs arthrotomy (open) surgery
• partial vs total synovectomy
• surgery only vs surgery and radiotherapy, or colloidal chromic P32 synoviothesis

4.   Choose an energizing warriors attitude: 

• Build an army with the most qualified doctors and medical facilities. This is a disease that may have many battles and may take me to foreign lands. My arsenal of knowledge will be strong, my tools and army powerful. I accept the challenge; I fight for freedom of movement, health and quality of life. 

5.  Now it is time to tell others of the challenge before me, my attitude toward it and what I may need     from them on this journey. 

Emotional Roller Coaster

I initially went to Dr. Bryan due to long term flank pain. During my appointment I mentioned that my left knee had lateral swelling for over a year and a mass behind the knee for a couple of months. My knee is only moderately uncomfortable with occasional flairs of increased swelling and pain. The doc ordered an MRI which showed signs indicative of PVNS. Great, an incidental finding that I could have lived without.

  Seriously? Now I have to deal with the flank pain, PVNS and another incidental finding from a CT that was done on the abdomen and chest showing bilateral undifferentiated pleural thickening in the lung bases. The radiologist is requesting a follow up ct in six months and the doc is wondering if I would like a referral to a pulmonologist in the interim. I'm overwhelmed and frightened to the point of near immobility, however, I understand the importance of finding a surgeon that is very familiar with PVNS in order to have the best chance of a positive prognosis. Thankfully I have the research skills to find such a person.

Do all of these findings have some type of connection? I know it's just my paranoia running away with my imagination; but what if the PVNS turned out to be malignant and it had already metastasized into the pleural cavity of the lungs and what if the PVNS is in the lumbar vertebrae causing the flank pain. Possibly plausible but very, highly unlikely. I don't believe that is the case but it is my worse fear.

I've been in a five day reading frenzy finding out everything that I can about this mysterious joint disease that may threaten my quality of life. At the very least it's going to cause even more pain due to the surgery that is required, time for rehabilitation, medical expenses increasing all while I'm unable to work. I would like to ignore the PVNS, however, the case studies have proven that would be a very unwise choice. The sooner action is taken the better chances of it not returning. Denial is a luxury that could be very costly. My attitude toward what is happening is much more powerful than the uncontrollable physical events taking place.  I will face this with dignity and grace and be triumphant as I have been in the past.           

PVNS Diagnosis

Nov 07, 2011: MRI - Lower Extremity Joint w/o contrast
History: Mild Left Knee pain, lateral swelling

Findings: 
Ligaments: The ACL, PCL, MCL and colateral ligamentous complex are intact.

Menisci: There  is globular degenerative-type horizontal pattern tear body lateral meniscus with extension to the free edge. The medial meniscus is intact. The posterior meniscal roots are preserved.

Osseous Structures and Articular Surfaces: There is no acute fracture. No focal bone marrow contusions are identified. There are changes of grade 2 medial compartment chondromalacia along the posterior inner and peripheral medial femoral condylar and opposing medial tibial plateau articular surfaces. There are changes of grade 2 lateral compartment chondromalacia along the central to posterior and peripheral lateral femoral condylar and opposing lateral tibial plateau articular surfaces. There are changes of grade 2 chondromalacia patella along the medial patellar facet and adjacent patellar medial eminence, as well as along the medial femoral trochlear articular surface and adjacent femoral trochlear groove.

Extensor Mechanism: The extensor mechanism is intact. The medial and lateral patelar reticula are within normal limits.

Miscellaneous: There is small knee joint effusion. There is a small to moderate-sized complex Baker's cyst. There is diffuse internal joint space synovitis with hypointense signal throughout the synovium, suggesting hemosiderin deposition. This appearance is compatible with underlying PVNS. There is a 5 cm x 3 cm complex cyst along the posterolateral joint line, which may reflect ganglion, synovial cyst or atypical parameniscal cyst. The popliteal neurovascular structures are maintained. There is no evidence of muscular atrophy, nor signal alteration to suggest denervation-type injury. No high-grade musculotendinous injury is seen.

Impression:
1. Intact ligaments.
2. Globular degenerative-type horizontal pattern tear body lateral meniscus with extension to free edge.
3. Intact medial meniscus.
4. Grade 2 medial compartment chondromalacia, lateral compartment compartment and chondromalacia patella.
5. Small knee joint effusion and small to moderate-sized complex Baker's cyst. There is diffuse internal   joint space synovitis with hypointense signal throughout the synovium, suggesting hemosiderin deposition. The appearance is compatible with underlying PVNS. There is a 5 cm x 3 cm complex cyst along the posterolateral compartment joint line, which may reflect a ganglion synovial or atypicl parameniscal cyst.

Index and Glossary of terms: 
What is Pigmented Villonodular Synovitis: 
Knee Anatomy Movie:


Grade Chondromalacia:  
Grade 0: healthy cartilage 
Grade 1: the cartilage has some soft spots 
Grade 2: minor cartilage tears are visible 
Grade 3: deep lesions in the cartilage that are more than 50% of the cartilage layer 
Grade 4: the cartilage tear goes all the way to the bone