My name is Robert, I am 54 years old and live in Northern Sydney. My story starts at the beginning of February 2014 when I started to notice some strange changes on my body. I have been fortunate in life as I have had very few illnesses and enjoyed good health by having a sensible diet and exercising each morning to try and remain fit. I have had my own consultancy business for over 25 years now which has always been busy but in the latter quarter of 2013 and into 2014 had become extremely busy.
It was on the first Sunday in February that my wife noticed a large bruise on my thigh. I had no idea why this was there as I had no recollection of bumping it and thought that I must have unknowingly done so whilst mowing the lawns and put it down to that. I had also noticed that there were small amounts of blood whenever I blew my nose, even just lightly, and there seemed to be small red dots on my lower legs but I convinced myself that maybe I was just imagining these as I felt perfectly normal.
Over the course of the week I noticed that I was getting more of these large bruises which I kept these hidden from my wife as she was concerned with the ill health of her Grandmother and also one of my children was not well and so I didn’t want to worry her.
It came to a head for me on the following Saturday when I arrived home after spending the day in the office. I had promised my son that I would take him and his friends to the basketball game in the city and so I went upstairs to have a shower about an hour before we were to leave. As I took off my shirt I was stunned to see a large, jet black bruise just below my rib cage which wasn’t there that morning. I was still feeling fine and as I didn’t want to let him down, I decided to take them all to the game and go up to Emergency at the Royal North Shore hospital when I got home to find out what was wrong.
Needless to say my mind was on other things as we watched the game and it was not until 11:30pm that I arrived back home. I woke my wife to show her the extent of the bruising and told her that I was going to take myself up to the hospital. All sorts of thoughts were in my head (namely leukemia) on the short drive but I hoped that I would just be given an antibiotic and sent home again.
After the initial examination I was quickly sent through to the nursing station where I had some blood taken and then waited for the results. Sometime later the doctor came and took me to a room where she said that I had a platelet level of only 9,000 and that the initial indications were that I had ITP, a condition that I had never heard of before. In the early hours of Sunday 9/2/14 I was then admitted to the Oncology Ward and my treatment begun.
A hematology specialist saw me in the morning and I was given a dose of 100mg prednisone a day. Amazingly my platelets went from 9,000 to 25,000 and then to 60,000 in 3 days and I was discharged from hospital. I had a bone marrow biopsy done as an outpatient the next day and the results largely confirmed the ITP diagnosis.
I was then told that I had to reduce the prednisone dosage over the following weeks and have weekly blood tests done to monitor my platelet levels. The next week on 75mg my platelets were at 160,000 which was great so I felt that I had dodged a bullet and life returned to normal. I reduced the prednisone to 50mg the next week, had the blood test and then received a call from my specialist to tell me that my platelets had plummeted back to 13,000.
This was when the real fun started. My prednisone dose was increased back to 100mg, I was given Transamic Acid to aid clotting and booked in the next day to start 5 daily transfusions of Intragam at the RNSH Cancer Care unit, a process which takes about 4 hours per session. I woke up on the morning of the first session to find that overnight I had developed a number of large blood blisters in my mouth, tongue and lips which, whilst unsightly, did not hurt. Little did I know then but these blood blisters and the later ulcers, would soon become the bane of my life.
At the completion of my treatment my platelets had dropped to 2,000 and I was then admitted to the Hematology ward at RNSH where I would stay for the next 2 weeks and be given all the other drug options available to them. I had Dapsone, Cyclosporine, 4 doses of Rituximab (also known as Mabthera) and Dexmethasone. I was sent home to continue my treatment with the strict instructions not to injure or cut myself and not to bump my head. Throughout all this my platelets hovered below 5,000 and on one day even reached ‘0’ and I was considered a ‘Severe Refractory’ case with my immune system showing no signs of letting up. I even had an MRI scan to see if I had anything that could possibly be causing the problem along with another bone marrow biopsy but these all came back negative – I was in good health other than I had no platelets!
I now seemed to have only 2 options left – to remove my spleen or a try a relatively new drug called Romiplostim which makes the bone marrow produce more platelets than can be destroyed by the immune system. With my low platelet level an operation was considered too risky and so my specialist managed to convince the hospital to purchase a limited quantity of Romiplostim to try and increase my platelets to 50,000 so I could have my spleen removed. Romiplostim is an expensive drug which costs $3,000.00 for 1.5mls (not a misprint) and is made for those people who have had their spleen removed but this has not solved their problem – apparently removing the spleen has only a 70% success rate.
Anyway I was taken off all my other my other medications, started on this new drug and prepared for the operation by having vaccinations against some serious infections like meningococcal which can be a major issue without a spleen.
Romiplostim is also very potent and so they start you off slowly to gauge your bodies reaction – I started on a Level 1 dose (0.18mls) with no effect, a Level 2 dose (0.36mls) still no effect, then Levels 4, 6 & 7 the same – I was still below 5,000 and wondering if I would ever get better. (Level 10 is the maximum allowable dose)
Everything changed in late May when my body finally reacted and my platelet count came in at 33,000 – even my specialist confessed her excitement at this achievement! For the first time in 3 months I was above the dangerous 10,000 limit and suddenly all the blood blisters and ulcers disappeared and life became somewhat normal again. In the interim my specialist had managed to get me a 24 dose supply of the drug which changed her strategy as she would rather give my immune system a longer chance to settle down rather than remove my spleen.
What began next was the roller coaster ride of balancing dosage levels to platelet levels dependant on my weekly test results. From 33,000 I went to 315,000 then back to 56,000 then to 512,000. You are not given a dose if you go above 400,000 and then you watch to see if your immune system has settled down. At one stage I reached 604,000 but within the space of 4 weeks without doses I was back down to 155,000. Today (20/8) I reached a high of 1,036,000 (also not a misprint) so I dare say that I will not be having another dose for 4-5 weeks and we shall monitor the rate of platelet destruction to see if it is slowing down. I have 12 doses left until my Romiplostim supply runs out and if I have not fixed myself then unfortunately I will have to have my spleen out.
Apart from my weekly blood test and visit to the hospital for treatment, life is normal at the moment. I sometimes think about how my life changed so suddenly and inexplicably back at the beginning of February and where this will all end up but I do not dwell on it as things could always be worse. I have good support from my family and friends but when you do not look sick, I sometimes wonder if they really understand what it is like to experience this condition.
Apart from the blood blisters and any medication side effects, I have not felt any different at 1,037,000 than I did when I was at 0. I did not experience any pain from the bone marrow biopsy procedure. In regard to taking Prednisone and Dexmethasone:
- I felt foggy and forgetful at times but returned to normal once I was off them.
- I am a very good sleeper however I found that it really messed up with my sleep patterns at times.
- my heart rate increased from 65 to the high 90’s.
- it did not affect my appetite.
In regards to taking Intragam, Dapsone, Transamic Acid and Mabthera – I did not experience any side effects from these drugs at all. Romiplostim – gives me a slight headache and fatigue but not enough to worry about. I have not had any issues with body heat changes. I am conscious not to get too stressed any more as this has been mentioned as one of the possible causes of this condition. I do not drink alcohol at the moment as I find that it gives me a headache and only increases the fatigue.
And Update on Robert’s ITP Story
Further to my earlier story posted on the 20/8/14, I thought that it may be of interest to update my situation for others using Romiplostim or about to start using this drug.
After my high reading of 1,038,000 on the 20/8/14, the next week I continued to climb even without an injection and reached a platelet level of 1,084,000. At this point I was asked to take ¼ of an aspirin tablet a day due to the increased risk of clotting. Whilst the aspirin does not destroy platelets, it apparently makes them less sticky and therefore less likely to clump.
After such an incredible ability to create platelets, the next week I had the incredible ability to destroy them and lost nearly 700,000 platelets in one week and was back down to 386,000. I was given a small Level 2 dose and what happened next was quite interesting.
I started to follow the same test results pattern I had back in July when, without any doses, I went (in broad terms) 400K – 600K – 400K – 250K and then 150K. Back in July at 150,000 I was given another dose which took me back up to 400,000 but this time around I was not given another dose. When I went for my blood test on the 22/10/14, I had expected a result of around 60,000 based on the fact that I seemed to destroy between 100K – 150K of platelets a week but to my surprise my result was 193,000 which meant that despite not having had any treatment for 6 weeks, my platelet level had increased by over 40,000. The next week they rose again to 257,000 and the following week I was at 239,000 which by then had been 8 weeks without any treatment.
It is a very positive sign when you are able to maintain a platelet level of over 200,000 for a period of 3 weeks without treatment and on the 5/11/4 my specialist said that all indications were that I had gone into remission. Whilst this was a very good result, she said that there were no guarantees that it would never return and that I would continue to be monitored with my blood tests now extended out to a 2 weekly basis.
And so after 9 months and 65 blood tests, my first experience with ITP is over and the two dates of the 9/2/14 and the 5/11/14 will remain with me for a long time. My life is once again totally normal in all respects and I look back on what I had just experienced with mystery as to how it all started and why it suddenly decided to stop.
Along the way I met the nicest group of people I have ever had to deal with from the nurses and staff in the RNSH Cancer Care Day Treatment Unit and those up in the Oncology and Hematology wards at the RNSH. I also had the most wonderful specialist who showed such care and compassion during my illness and who went to such great lengths to obtain the Romiplostim needed to restore my health. I just wish that there was some way I could repay these people for what they have done for me.
Finally I would also like to thank the brilliant people at Amgen who created this wonder drug Romiplostim (Nplate) for without which I would not have been able to write such a good outcome to my story.
Notes from Robert’s experience with Romiplostim;
- The full impact of the drug seemed to come into effect 2 weeks after the injection as my results climbed the next week even without having an injection.
- The drug has an on-going effect for about 4-5 weeks after injection and I could also feel this by the lessoning of the mild headache I experienced when I went for the extended periods without treatment.
- My specialist had recently been to a conference where some Romiplostim specialists advised they were finding that patients receiving Romiplostim within 6 months of the initial ITP diagnoses seemed to be entering into an early remission and that this would appear to have been the case with me.