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.
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