of Follicular Lymphoma and Paraneoplastic Pemphigus

Stories of Survival


My name is V Kingsley. As one of the first long-term survivors of PNP with non-operable cancer, I think I have a bit of insight from both a medical advocacy position and a survivor standpoint. People come from all over the world to read my experiences as I battle these diseases and I am here to help in whatever ways I can.

I began having symptoms in 1996 when I presented with severe gastrointestinal pain in the upper left quadrant. On ten separate occasions over 6 years, this intense pain was resolved with morphine in the Emergency Room. I had ultra sound scans, blood panel tests and a CT scan that reportedly showed no apparent cause of the pain. I was told that I had intestinal gas and should take an over-the-counter medication like Gas-ex.

In August of 2002, I presented with mouth lesions and ocular conjunctival scarring. I saw several physicians from Internists to an ENT (Ears, Nose and Throat specialist), a Rheumatologist, an Oral Surgeon, an Optometrist, an Ophthalmologist and two Dermatologists. I was screened for sexually transmitted diseases, strep throat, thrush and vitamin deficiencies. Most doctors were baffled and could not understand why prednisone, cortizone, eye drops and mouth washes were ineffective. After two months, the lesions were much more widespread to most of the soft mucosa in the eyes, mouth and vaginal canal. I began Cyclosporine  (immune suppressive therapy) when Dr. Molly Shields (my heroine / Dermatologist) suspected that I had Pemphigus Vulgaris or Erythema Multiforme. I was, along with my partner and our 5 year old son,  informed that it could be life threatening.

In addition to traditional medicine, I chose massage therapy for stress reduction. The International Pemphigus Foundation suggested early on that I keep a medical journal so that I could be my own medical advocate. This proved the best advice I have received to date. Believe me - after 6 years fighting cancer, I am NOT a fan of advice. I do have the notion that the power of positive thought, intercessory prayer (from any and all faith backgrounds) and community involvement could go a long way toward improving my quality of life. For me, this meant my family and friends, the Unitarian Universalist church community and the lesbian nation of Michfest.

When my skin showed no improvement with high dose prednisone and immune suppressive therapy, Dr. Shields ordered a new CT scan, suspecting that I had Paraneoplastic Pemphigus (also known as PNP, the more rare - and almost always fatal - form of pemphigus that usually has an underlying cancer). A biopsy was taken and sent to the dermapathology laboratory at Johns Hopkins in Maryland where a test for PNP would take a few months to show results. Meanwhile, the locally administered CT scan showed a 15 x 12 cm mass Lymphoma (Non-Hodgkins) in my abdomen  in the upper left quadrant. I was referred immediately to an oncologist who started me on chemotherapy the next day. This was December of 2002. I was told that "it will be easy - a cake walk" to get rid of the lymphoma.

My first chemo was Rituximab and because I had an allergic anaphylactic reaction (uh-oh), this course was discontinued immediately. I started 2 CdA chemotherapy, allopurinol (an anti-gout medication) and IV saline because of severe dehydration from being unable to eat or drink due to the lesions. I lost a lot of weight rapidly. I was treated for burns with silvadene.

Due to an allergic reaction to either the allopurinol or the silvadene (uh-oh), I developed Stevens-Johnsons Syndrome which compounded the blistering skin reactions and rash. With a majority of my skin severely involved and desquamation of my lips and oral mucosa,  I was prone to infection and in excruciating pain. I was also going blind due to severe dry eyes. I sought advice from the International Pemphigus Foundation where, in 2003, I met a representative from Advantacare, a San Francisco Bay Area home infusion company, who informed me that IVIG (also known as IGg or intravenous gamma globulin) had offered positive improvement in people with Pemphigus Vulgaris. No doctor had mentioned it so I put the business card in my pocket for later.

In addition to my local Oncologist, Ophthalmologist and Dermatologist, I saw Dermatologists and Ophthalmologists at UC Davis, UC San Francisco, and the Proctor Eye Clinic (where they were thrilled with the textbook scarring of my eyelids but offered no hope for relief with my impending blindness).

Please note that whatever rash that is seen on the outside is just the tip of the iceberg. The soft mucosa in every area of my mouth, parts of my throat, lungs, vaginal canal and eyes were  (and still are sometimes) ravaged.

By the fall of 2003, the very worst of the skin blistering had been been stemmed but the damage done to my eyes caused severe dry eye that worsened with time, damaging the cornea and causing an inability to open to open my eyes for any practical amount of time.  As a quilter and a mother, this was devastating. I took Restasis eye drops (a feeling akin to dropping acid in my eyes) due to the ocular involvement of the PNP. It was an attempt to increase tear production. Unfortunately, it is almost impossible to increase tear production when there is no tear production. This is an intensely painful situation and Restasis did nothing to help.

With the results from Dr. Grant Anhalt at Johns Hopkins that I had a confirmed diagnosis of Paraneoplastic Pemphigus, I insisted on trying IVIG (60g/day 3x every 3 wk) before I saw the $10,000 a day price tag. (Insurance is another story - now it’s up to $15,000 a day.) I was treated simultaneously with 60mg (!) of prednisone/day, IV saline , 2 CdA chemotherapy, Cell Cept (another immune suppressive drug), pain medications, topical steroids, topical lidocaine, anti-constipation and anti-nausea medications and occasionally antibiotics and thrush medication, depending on infections.

I needed a break from the relentless western medicine. I spent a lot of time writing, living the tenants of laughter therapy, listening to comedy and listening to music because I was blind but still used my sense of hearing as a tool to help me survive.

For the next two years, the cancer continued to shrink in my abdomen but migrated to my lungs so when it was possible, I continued with chemotherapy Doxil and Rituxin which had to be administered in the hospital due to that initial adverse reaction in 2002. Throughout the years, I tried several alternative practices including (but not limited to) herbal and vitamin therapy, acupuncture, yoga, tai-chi, Native American healing and reiki. People swore by a wide variety of drugs,  physical and mental practices, religious rites and miracle cures. Most of the 427 suggestions offered to me were not personally useful.

Although the steroids took a serious toll on my joints and gave me a "moon face", I lost 130 pounds due to the Lesions in my mouth. I was mighty glad that I started off as fat-positive, 250 -pound woman who loves life!

In the summer of 2004, against medical advice, I discontinued all antibiotics, pain medications, prednisone, immune suppressive medications and I took a break from chemotherapy, despite the remaining lymphoma. After the initial pain of an unexpected divorce and a couple of seriously close brushes with death from a pulmonary embolism and a port-a-cath infection, I had the notion that

In my opinion, the alternative healing that helped me the most were a combination of community connections,  positive thoughts and prayers, my own journaling, massage therapy, laughter and (forgive my frankness but) sexual activity. This was the combination for me. Everyone is different but I will never underestimate the power of positive interactions.

In this process, I became fully blind and began mobility and orientation training and Daily Living Skills  with the Vista Center for the Blind (formerly the Peninsula Center for the Blind). I was unable to find consistent transportation to chemotherapy but in the spring of 2005, I had my sight fully restored by the glorious doctors and engineers at The Boston Foundation for Sight, where I was fitted for prosthetic sclera lenses. They are AMAZING! Because I could see and drive again, I was able to receive chemotherapy which was especially helpful when the cancer spread to my lungs in the summer of 2005. Once again, my life was saved by people who didn’t even know me. It happened again and again in this journey. I created the “Eye Can See” quilt as a token of my undying gratitude.

Please feel free to ask any questions and to link to this page freely. I can be contacted through the contact page.  The archives and current writings of my online journal can also be found on this page. I want to help in the time I have left. Please share your stories while you can.

After a glorious one year remission between 2006 and 2007 (in which I remarried, launched the A Lot of Life! website, started home schooling again and began to enjoy my 40s with gusto), the mouth lesions returned. I have taken chemotherapy (Rituxin) and IVIG off and on since then.

After years of infections, pneumonia for which I was twice hospitalized in 2008 and pulmonary problems, I am now anti-biotic resistant. I suffered from severe sinusitis (all available areas infected) for years. At the end of 2008, I underwent surgery for a deviated septum and two surgeries that involved all my sinuses. It was grueling and painful but I hoped that it will alleviate my breathing problems. Alas, it was not to be.

As of early spring 2009, it seems as if I have Bronchiolitis Obliterans, the pulmonary obstruction disease that most people with PNP die of if they manage to survive the initial stages. My airwaves are blocked and I cannot breathe fully. I have infections that I cannot shake in my lungs and will probably just live out my remaining life with decreasing pulmonary function, hoping that I avoid fungal (or fatal) infections.

I live in heinous pain every day of my life due to the scarring in my eyes and the mouth lesions and the breathing difficulties. I could never have done this without legions of help. You know who you are and how grateful I am.

A special thanks to the runners who run in the Leukemia & Lymphoma Society Triathlon in my name.

©  2011 Copyright V Kingsley

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This was just the beginning.

Righteous Fat Chick