I am writing on behalf of my mother who was diagnosed with PV back in 2002. The symptoms started with her in 2001 but it took over 1 year for my mother to get to the right hospital, a specialized Hematologic hospital of the Ho Chi Minh City and she was put on HU since then, until recently, as a BMB showed that she had Myeleosis Fibrosis grade 2 post Polycythemia Vera, with following NGS: JAK2 vaf 78.9%, TET2 vaf 39.1%, TP53 vaf 4.3% and ASXL1 vaf 6.8%. She is now 63 yo.
The situation in Vietnam is quite different than what I have seen many seniors here with MPN, espeically PV and MF post PV in the US and UK have experienced. We are quite far behind in getting the new drug.
My mother had her first BMB in 2002 but only recently, in April 2022 doctor decided to gave her another BMB to find out that she was MF second grade post PV. It was not until last 3 months when my mother complained about her recent motion sickness when driving car to the hospital (which never happened before), a substitute doctor (younger) decided to get her to check NGS and advised her about taking Ruxolitinib. Ruxolitinib have been available in the US since 2011, FDA approval but only in January 2021 that Novartis got permission from the Ministry of Health to distribute this drug, with sponsorship of 50% (social insurance included), and it came out about 1400 USD monthly for the use of this drug, paid by patient. And only recently a doctor just got to know about it and advised my mother to use as her disease has progressed. And only about 20 patients got to use this Rux in entire Vietnam, for a population of 100 million, that is quite very few, given that there are many patients who also have MPN but mainly they are not yet informed and/or are not capable of paying such huge amount each month, considering this new drug has not been covered by social insurance entirely.
I came to learn that even though the doctor said she has bad prognosis (the doctor did not use any model of prediction like DIPSS or anything like that) due to the TP53 and ASXL1 mutations. I have done my research work, and, with a bit of background in gene and genetic diseases, I learnt that all three extra mutations my mother has beside the starter JAK2 are non driver mutations, so if everything is managed well, it will not be bad like main driver mutation CARL or the other ones.
I mentioned that my mother is a case of interest because I would love to hear from you all, those who are having PV, and MF post PV, about your routine blood test result. My mother ever since she was diag with PV, she only has her RBC very high, while her WBC and PLT are normal. Prior to Covid last year, her blood test was under control, everything, except for MCV which can change from 88-103 and maintain in the range, and HCT under 50. However, later in the year, her WBC started to jumped up out of the range, to 9 k/UL and subsequently up to 21 k/UL (post BMB). I was really panicked during the time when no doctor could pin point exactly what she was having, all just said she might have an infection going on. This was around April 2022 and lasted toward the year end. My mom most of the time felt very tired and felt like she could leave me any time soon. However, earlier January 2023, the condition resolved on its own. Her WBC returned to normal, under 7 k/UL and maintained above 3.8 k/UL. At the time, she got some bleeding in the teeth, which was something she got on and off throughout the time with HU, and she got to a good dentist who helped her to cure the infection and it has not returned since. I do not know if the rapid raise in WBC was due to the bleeding in the mouth but as she had endured mouth ulcers on and off for many years and we have never seen her WBC to be above 13 k/UL, not mentioning to 21-22 k/UL this time.
My mother has a large spleen (I could not take photo of the result yet) and high acid uric level (400+ umol/L) She was taken blood test before the start of Rux in mid July. Doctor gives her starting dose of 20mg / twice a day, so thats 40mg a day. And in Vietnam, there are only 2 dosages to choose for Rux, 15mg and 20mg tablets. There is not any other options to choose.
This week has been her 3rd week on the drug, so far everything has been much better for her. Before she had night sweat on the back, really terrible back pain that makes her difficult to sit down and stand out, quite some mouth ulcers and tooth bleeding (which is now resolved) and tiredness, etc. some other symptoms similar to most who have PV. Now she told me that her back felt lighter, enables her to do more work and longer exercise time. I have to thanks Hunter to bring up the post about Rux and Grape fruit the other day because when I asked the doctor who prescribed Rux to my mom what to be avoided, he did not say to avoid anything. Clearly, the doctors here are also behind. To be honest, 3 weeks that I have been on this forum gives me a lot of knowledge and to a point I think I know about this drug more than the doctor who prescribed it to my mom.
My mom has been taking Amlodipine 5mg once daily and the past 2 days before breakfast, she feels a bit of dizziness and blood pressure check tells that her heart rate is 58-59 bpm (never before), and low blood pressure. So for the moment being, she has stopped taking Amlodipine and see if tomorrow the symptoms appear again. I do not know how long Rux has started to be effective in the body, and it is different for everyone else but I have read from some members here that itching ceases overnight so it must be that Rux can be quite fast to be effective. My mother told me it feels like her blood is reducing, as she is familiar with this feeling before when on HU. We are going to see the doctor again next Tuesday for a blood test. I have read on the instructions that comes with the box that in the trial it takes 1.5 months for anemia to show, but of course, it does vary.
I am a bit wary of the withdrawal rate in the studies and cohort trials of Rux but given the trials include less than 1500 people, I think it is a bit of stretch to represent for the whole MPN community, considering I did not read anywhere that trials include Asian, and which ethnicity. Living environment does play a big part and so does ethnicity when it comes to immunity and health strength in general. Considering my mom is among the only 20+ people to start Rux now in Vietnam, I would like to ask if any patients out there find themselves in my long post to chime in.
I learnt that momenitilib is due to approval in September this year but even if it is approved, it will be a very long time for the drug to show up here in Vietnam and for sure the price tag gonna be hefty, so my best bet now is to hope my mom will respond well to the drug and the efficacy will last longer than 2-3 years like what was shown in the trials.
My mom was tested HLA for a possible SCT but as she is 63 yo and both her brothers are over 60, doc said cannot get donor cells from my uncles and maybe they will post the HLA result to search for a match from community though it will be very tough in Vietnam as people here do not usually do regular check up, not mentioning to check for HLA to register in the community bank for later possible donor.
Sorry for the long post but I just want to provide as much as information as possible so that maybe knowledgeable members in here can read and comment on my wonderings here
- What else should I be cautious of when using Rux? I hope the grapefruit was the last thing that I caught up with
-Are you, PV and MF post PV, having your WBC and PLT high about the range, and not just RBC? I have read the other day that one member has her PLT to the 1050 k/uL level, which is unbelievable for me because I have never known that PV can lead to raise in all three counts, not just RBC. Her last year BMB conclusion was: increase in three counts, but if anything ever raised, it was RBC and her previous doc always focus on her HCT to be below 50, with the incident of WBC rising I described above.
-My mom RDW is 18.1 % (range is 11.5-14.7%) and she has Microcytic +, Hypochromic +, Macrocytic +++, Blasts ++, Left shift +. What I understand (from her MCV >90) is that her red blood cells are abnormal sizes, both huge and tiny and some are just premature and still circulate around. Is there something else that I am missing from this result?
-As now my mom is MF grade 2, with blood test like this and her PLT before taking Rux is 188 k/uL. , WBC 4.96 k/uL and RBC 4.06 k/uL (HU 500mg twice a day). As for prevention of Anemia on using Rux, has anyone her try using Gineng, especially the Red Ginseng? As I come from an Eastern country and have some background in Eastern medicine, I am surprised and not surprised that no study has incorporated some Eastern med in. It is like a clash, like you use Western or Eastern, no combine. Yet I think I read through a case of using acupuncture to relieve the pain here in MPN group. As for blood clot prevention, has anyone here use Natto? It is the fermented beans from Japan but its main thing is Nattokinase which dissolves blood clots — which helps maintain good blood vessel structure, improves blood flow, and lowers the risk of heart disease. It can also help lower your blood pressure, reducing strain on the heart that can lead to heart attacks (webMD). I have been eating natto on and off for a while before Covid strike and drove the price up a lot. I also told my mom to use but as she started Rux now, we wait maybe at least 3 months to see if her counts are stable before taking other supplements. For blood creation in Eastern med, besides Ginseng, we also use Panax pseudoginseng, and you could look up on GG to see how the plant is like, but for the history of Eastern med, it has been used for women for stable periods, since it has pro-blood property like Ginseng and much less expensive. Per webMD and other sites, the Panax pseudoginseng is used to stop or slow down bleeding. It is sometimes taken by people who have nosebleeds, vomit up or cough up blood, or find blood in their urine or feces. Panax pseudoginseng is also used to relieve pain; and to reduce swelling, cholesterol, and blood pressure. So my plan is, if my mother has anemia, these will come in handy, but I hope I do not have to use them with Rux.
That is for now. I am truly thankful for those who take time to read until this very end and find it helpful at some degrees to you. I hope to receive advices, and new insights to health information of my mother that I did not get to ask the doctor here (he actually does not answer much, and it is the way it is here)
Thank you and God bless you all!
Steve,
Anyone on Jakafi 10+ yrs?
Polycythemia and Jakafi
