I was first diagnosed with HCL in November 1992 at the age of 28 years. The reason I was diagnosed was because of a trip to change doctors to the same doctor as my wife Angela so we would have a family doctor, Angela was pregnant with our first child Zoe at the time. I had a blood test and this came back with very low counts and from various tests HCL was diagnosed.
I was first treated with interferon, but I was found to be allergic at the time. I then had my spleen removed in February 1993. Zoe was born on the April 9, 1993 and I started being treated with Pentostatin very soon after. I had 15 cycles of Pentostatin and was then deemed to be in remission.
I carried on with regular blood tests and in 1997 our 2nd child Paul was born. At the end of this year my blood counts had deteriorated to warrant further treatment. I was the treated with Cladribine on the 24/7 regime in January 1998.
I was then in remission for 10 years and in early 2008 my blood counts had again deteriorated and HCL was present in the bone marrow so treatment then started on April 14th. I remember the exact date as it was my birthday! I was scheduled to have Cladribine but when I went into the hospital to start the treatment I was told that there was a worldwide shortage of Cladribine and I was started off on Pentostatin. I again had 15 cycles and was told that I was in remission.
Regular blood tests were taken and in May 2011 my counts were again down and HCL was again filling my marrow. I had to take into account that we had tragically lost our daughter Zoe in a horse riding accident in April 2011 and was not sure how the grief and stress at this time was affecting me. By now Cladribine was available and I received this once a week for seven weeks and was then informed that I was in remission.
By January 2012 I was feeling really down and extremely tired. I was now very familiar with the symptoms and felt that all was not right. I made an appointment at the hospital for a blood test and this showed the counts to be low. I was treated with Pentostatin and Rituximab, but confirmed that this line of treatment was not working and that I had become refractory to the 1st line treatments.
At this time it was the worst I had ever felt with the condition, I was extremely tired all of the time, had no energy whatsoever and could not exert myself at all. I was having to have regular blood and platelet transfusions and had a number of infections that needed a hospital stay to overcome.
In early August 2012 it was confirmation that the Pentostatin/Rituximab regime had not had any effect and that there was still more than 90% HCL in my marrow. I was informed on the work done with Vermurafenib, although this was only on one other person but he had been refractory the same as me. I commenced treatment on September 26, 2012 with 1 tablet twice a day. At this time HCL was detectable in my blood.
After a week I went to 2 tablets twice per day and then 3 twice per day on the third week. On this week I got an infection and went into hospital for treatment with antibiotics. Treatment carried on and I was going to increase to 4 tablets twice daily. Angela (my wife) contacted my doctor with regards to the dosage as this seemed to be high and if anything was making me more ill. I had been told in the first 2 weeks that the treatment seemed to be working and that white cell counts were going in the right direction and HCL could not be seen in the blood slides.
My doctor conferred with others and it was decided that the treatment would continue with 2 tablets twice per day. The infection was cleared up and I was sent home to continue with the treatment of 2 tablets twice per day. My HGB was a little low at this point, but I put off a transfusion so we could see if the counts were getting better. I was convinced that all was well because I was feeling better every day. By the middle of November I was feeling great. Blood counts were rising and I started to attend my gym again and started regular exercise that also included riding my mountain bike. I had my life back!!
In December 2012 we went to see my doctor again for a BMA and the results came back with less than 10% HCL. Treatment was carried on at one tablet twice a day until March 14th 2013. I had a BMA again with my doctor 2 weeks previous.
In my opinion if it were not for my doctor and Vermurafenib I would not be here now. I have lived with HCL for more than 20 years and have many of the 1st line therapies and time was running out as I was refractory to all these. I was extremely tired all the time, had no energy and was losing all hope and could see no way out. This had been made worse because of the accident to Zoe and this was and is still very raw with all of the family. Angie, Paul and I were struggling to come to terms with her accident and the consequences and we were now faced with another crisis where we could see no successful outcome.
I had always thought deep down that I would one day succumb to HCL, but wanted Zoe and Paul to be at an age where they could handle this. Zoe was strong emotionally like me where Angie and Paul are more sensitive and I am convinced that Zoe would have been that emotional prop for them. This had now been taken away and I felt utterly lost and cheated. I have never smoked and only drink occasionally and never in access. I have kept fit all my life and hardly ever catch colds- why was this happening to us? I have never been one to feel sorry for myself and I did not in a strange way. I felt sorry for Paul and Angie. I could not tell them it would be all right because I thought it would not be all right. When I was in the hospital in October just after I had started the Vermurafenib and the dose was high, Angie and Paul came to see me and had bought me a new pair of trainers and I remember thinking “why have you bought these I will never wear them.”
It was a miracle- by the middle of November I was feeling great, I was eating very well and the counts wre going in the right direction- my life was returning. Paul and I started mountain bike riding again and we were having the time of our lives, nothing was a effort for me, I refurbished our bathroom and caught up on all the chores around the house that I had put off because I had no desire or energy. I felt 10 years younger. I can say that I had not felt this good since before 2008 when the HCL returned then. Incidentally I have nearly worn those trainers out!
Vermurafenib as a treatment was easy, after all it is tablet form so no visits to hospital for drips and the like. The dosage of 2 tablets twice daily was the ideal regime for me, the only side effects were my skin was a little itchy and this was treated with an emollient cream and when applied daily was not a problem. My hair all over my body went thinner and I was careful not to go into the sun without sun block.
Of all the treatments that I have had I would say that this has been the easiest and although the early days were difficult I am convinced that this was because of the advanced state of the HCL and in an ideal world treatment would be started before the HCL had got to this advanced stage.
We do not know how long the remission will last but this can be said of all the treatments and I would recommend this line of treatment to anyone with HCL. All in all, Vermurafenib has given my family their lives back and we are able to look forward and to plan future events.
I wish you all the very best in your fight against HCL and if you think I can be of assistance in any way please do not hesitate to contact me.