I believe it all started in 2006 while I was working as a self employed carpenter. Around that time I became more and more fatigued until it came to the point that my fatigue was so severe I had to consider doing less work. Along with the fatigue I started to lose my short term memory and went into a depression progressively as I was forced to slow down my business and eventually stop.


I was starting to lose my dignity because something was wrong and I could not figure it out. Working and being productive in the community is an important aspect of my life. Suddenly that was taken away from me against my will. Before all of this happened, I had a normal life and thought I was a healthy person.

From 2006 through the middle of 2009 I visited the doctor for various reasons such as: annual check-ups, sinus infections, allergies, etc… and each time a blood test was performed. Although the WBC was high, each doctor viewed it as a result of the reason for my visit. Several times I obtained my blood work results and noticed my WBC was higher each time. I was not sick and I had no symptoms. Something was not right. I believe this is the time frame that my disease T-PLL started to develop.

If there is one thing I can recommend to everyone that reads this blog, it would be to obtain a print out of your Complete Blood Count (CBC) results with differentials every time you have a blood test. You have to be your own doctor as trends can be unnoticed by the physician. One of my blood test results had a note on the bottom alerting the doctor to abnormal results, this was never addressed.

As a result of the Leukemia diagnosis I have gone back to the local hospital where my blood has been tested for the last four years and requested copies of all my CBC’s with the differentials. The differentials are important to show the components of the WBC, specifically the Lymphocytes and the Neutrophils. Depending on the lab you go to the normal ranges can vary slightly. From my experience normal ranges are as follows:

WBC          4.8 – 10.8 (in thousands) 4,800 – 10,800

Neutrophils 1.7 – 7.7                           1,700 – 7,700

Lymphocytes 1.2 – 4.9                         1,200 - 4,900

With the help of Mike, my partner for 15 years, we graphed out all of the WBC differentials to see if we could identify when the disease first started. In August 2006 my WBC was 8.0 and Lymphocytes were 4.2, just at the end of the normal range. From this point forward the levels continued to climb and never came back down to normal.

I have included table 1A which shows my blood level trend for WBC and Lymphocytes

In August 2009 I went back to my primary care physician where I previously lived before I moved to Titusville. He took one look at my most recent blood work and told me I needed to see a hematologist. My WBC was 14.9 and my lymphocytes were 13.0. As of June 1, 2010 my WBC is 21.8 and my lymphocytes are 19.9 the highest they have been since I started tracking.

I went to the local cancer center where additional blood work was performed and a bone marrow biopsy was scheduled. My initial diagnosis was Chronic Lymphocytic Leukemia (CLL), a disease that can be lived with for many years without treatment. The doctors monitor it with watch and wait by taking blood tests every three months. After the bone marrow biopsy the diagnosis was changed to Large Granular Lymphocytic Leukemia (LGL)

As defined by Memorial Sloan-Kettering Cancer Center:

LGL Leukemia

Large granular lymphocytic (LGL) leukemia is a chronic leukemia of T lymphocytes -- white blood cells that originate in the lymph system and in the bone marrow, and that help fight infection. Rarely, LGL leukemia is a disease of natural killer cells, which are lymphocytes that normally attack tumor cells. The disease usually affects people in their sixties. Symptoms include anemia, low levels of platelets and infection-fighting neutrophils in the blood (conditions called, respectively, thrombocytopenia and neutropenia), and an enlarged spleen. Doctors diagnose this disease through a biopsy of the bone marrow, or by using flow cytometric analysis of the circulating blood or bone marrow cells, a procedure in which various types of blood or bone marrow cells are separated, identified, and counted. Treatment usually involves chemotherapy, sometimes granulocyte-colony stimulating factor (G-CSF), and possibly surgery to remove the spleen. A closely related T-lymphocyte disorder is called T gamma disease; it is essentially the same as LGL leukemia.

At this point I was referred to Dr. Sokol at Moffitt Cancer Center in Tampa, FL, since LGL is a rare form of leukemia. In October of 2009 I visited Moffitt and 23 vials of blood were taken. Seven of the twenty three were taken to be used for research on LGL at Moffitt. At this point I was doing research on the internet every day to find information on LGL so I could get educated. In my search I found a doctor in Hershey, PA, Dr. Loughran, who seemed to be the expert on LGL Leukemia. Dr. Loughran and Dr. Sokol previously worked together. Both had written extensively on LGL. I contacted Dr. Loughran and he told me about a study he was doing on LGL and referred me to the research coordinator. A month later I had blood drawn for the study in Penn State Hershey – Milton S. Hershey Medical Center.

One of the tests done at Moffitt from the 23 vials was a flow cytometry report. As written in Wikipedia “Flow cytometry (abbreviated: FCM) is a technique for counting and examining microscopic particles, such as cells and chromosomes, by suspending them in a stream of fluid and passing them by an electronic detection apparatus. It allows simultaneous multiparametric analysis of the physical and/or chemical characteristics of up to thousands of particles per second. Flow cytometry is routinely used in the diagnosis of health disorders, especially blood cancers”
My Flow Cytometry report came back with a comment of “There is a marked increase in abnormal CD4 positive T cells expressing weaker than normal CD4, suggestive of a T Cell chronic lymphoproliferative disorder”. During my April 2010 appointment at Moffitt I was told that I had both LGL and T-Cell Prolymphocytic Leukemia (TPLL). Now my head really began to spin. LGL is rare, TPLL is extremely rare. I asked the doctor what the time frame was and he said 2 to 3 years, WOW!!! Looking at my blood work trending I calculate that I have had TPLL for at least three years, maybe longer. Time became a commodity.

After the dual diagnosis I had so many questions I scheduled a follow up with Dr. Sokol at Moffitt for May 24th 2010, which is when the latest blood work and Flow Cytometry was to be available.

According to Wikipedia:
"T-cell-prolymphocytic leukemia (T-PLL) is a mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin involvement. T-PLL is a very rare leukemia, primarily affecting adults over the age of 30. It represents 2% of all small lymphocytic leukemias in adults. Other names include T-cell chronic lymphocytic leukemia, "knobby" type of T-cell leukemia, and T-prolymphocytic leukemia/T-cell lymphocytic leukemia."


As I desperately searched the internet for anything on TPLL I found several blogs that gave me hope. These blogs are written by individuals and their significant others, to document their journey with TPLL. (Dennis Pyritz, Mark Vancura, Shirley Fischer, I hope I didn’t leave anyone out). These individuals are the inspiration for me writing my blog. They are leaving a trail for people like me to have hope, I want to make a difference for those who are diagnosed after me.

Here are links to their blogs, I encourage you to visit them to get educated on the disease and to read their story.
http://diaryofanillness.com/

http://beingcancer.net/about-t-pll/

http://tpllsupportnet.blogspot.com/

http://curingvancura.blogspot.com/

http://www.cancercompass.com/

The May 24th visit revealed, via Flow Cytometry, that I only had one disease and it was TPLL. Most of the literature I found stated that TPLL had a mean life expectancy of 7 months after diagnosis. After reading the blogs it was clear to me that getting treatment was something that needed to happen sooner than later. My doctor at Moffitt ordered another bone marrow to be resulted at Moffitt. Pending the results and confirmation of TPLL, the slides would be sent to National Institute of Health (NIH) for a second look. Then he would start treatment with Campath, a bio-therapy drug.

During these last few weeks, Dr. Sokol at Moffitt was in communication with Dr. Loughran in Hershey, PA discussing my case. On May 26th I was contacted by Dr. Loughran’s coordinator Hershey, PA, Dr. Loughran wanted me to see a colleague of his that has treated several cases of TPLL. He believed I should start treatment immediately. At this point things started to move very quickly. By the end of the week I had an appointment with Dr. Epner in Hershey, PA for June 3rd.

Friday May 28th Mike and I left for a trip to Long Island, NY in our motorhome to visit both my family and Mike’s. The plan was for me to stay in New York and spend time with family until June 2nd and then travel with the motorhome to Hershey, PA for the appointment. Mike was going to fly back to Florida on Memorial Day, work Tuesday and Wednesday and then meet me in Hershey, PA on June 3rd. During the ride to NY I experience pain in my stomach, especially after I ate something. I assumed it was the spleen. I also noticed some bumps on my arms. I was having trouble sitting up for long periods of time so Mike had to do most of the driving. Everyone that knows me will agree that I love to drive, especially the motorhome, and I never turn down the opportunity to drive.

We arrived in NY at Mike’s father’s house at 4:00am on Saturday May 29th. Just after parking we noticed large bumps on both of my arms. We had never seen bumps like these so we called Moffitt to see if we needed to get to the emergency room. One of the symptoms that occurs with TPLL are rashes. The attending on call told us that she believed it could wait until our appointment June 3rd.

Picture of rashes

We really enjoyed spending time with both families however, Sunday the bumps broke and turned into rashes. This became more concerning so we made the decision to drive to Hershey, PA Sunday afternoon in hopes of seeing someone on Monday. I wanted to be close to the hospital that would be able to treat me with this rare disease if things got worse.  Monday being Memorial Day, it was not a good day to be seen as most of the hospital was off. We didn’t want to go to the emergency room since very few doctors have heard of TPLL. We walked around the hospital to get familiar with where to go and decided to wait until Tuesday morning to call the doctor. Mike canceled his flight and called his boss to let him know what was happening. We are staying at the Hershey High Meadow Campground, which is 1.5 miles from the hospital. The campground has wireless internet which allows Mike to work when we are not at appointments.

Tuesday morning we called Dr. Epner’s coordinator and explained what we had experience over the week-end and asked if Dr. Epner would like to see me at the hospital.

Several hours later we received a call telling us an order was placed for blood work, and Dr. Epner would see us after the results were received. We went in and Dr. Epner examined the rashes and felt my spleen and said it was necessary to reduce the size of the spleen to normal. He felt that treatment should start right way.

On Wednesday I had another examination and was scheduled to start treatment on Friday June 4, 2010. Dr. Epner is using two drugs to treat my disease. Campath and Cladribine. The Campath will be administered via injection three times a week for three months. The Cladribine will be administered via IV for five consecutive days, once a month for three months.

Tomorrow I get my first dose of Cladribine by IV which should take approximately 2 – 3 hours. I will also get my first dose of Campath.

My schedule for the first five days is:

Friday 6/4/10 – 2:00pm – Campath and Cladribine

Saturday 6/5/10 – 2:00pm – Caldribine

Sunday 6/6/10 – 8:30am – Campath and Cladribine

Monday 6/7/10 – 10:00am – Caldrine

Tuesday 6/8/10 – 4:00pm – Campath and Cladribine

In addition to the treatment I have been prescribed several drugs to avoid infection:

Bactrim – Friday and Tuesdays – 1 tablet twice a day to prevent infection
Acyclovir 800mg – 1 tablet once a day to prevent herpes viruses

If my Neutrophils go below 500 I will need to take the following in addition to the medications above:

Cipro 500mg – 1 tablet twice a day to fight infection
Fluconazole 200mg – 1 tablet once a day – antifungal to fight thrush