As an update from my earlier post, we are now back in Australia after 3 months in South Africa.
This whole platelets thing raised its head about a month before we left.
I saw the haematologist 2 days before we left in late Jan. She put me on Aspirin 100mg daily and phoned me 3 weeks later in SA with the mutation results that I had the JAK2 mutation.
I had blood taken the day after we got home and my platelets haven’t really gone up during our time abroad. I will get my BMB done under twilight sedation in a week.
I’ll then see the specialist in a month to see what information it gives.
I told her today I was hesitant to take a chemo drug tho she explained it pretty well to my Husband and I. She said it is not really like main stream chemotherapy and the small risk is worth it to give me a good quality hopefully long life free from a debilitating stroke etc.
After reading up information while we were away, I must confess it was hard to deviate my mind from taking chemo drugs and what may be ahead. It is a rare condition, tho amazingly my SIL has ET too tho she has no mutation.
My SIL told me how she was told to wear disposable gloves each time she took the pills (she said she just tips hers into the lid and then into her mouth, that way she doesn’t touch them) and I read the article about taking care with interaction with others. Tho the information the doc gave me today on the drug here didn’t make such an issue of handling the pills or bodily fluids.
So my husband and I are feeling more at ease tonight 😊
#Hydrea
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Pachena
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My hematologist gave me information on Hydroxyurea. You can print it on Chemocare.com.
I also do not touch the capsule. I slide it onto another lid. You need to take with full glass of water…I swirl some around my mouth and then tip the pill in and drink rest of the water. This is to help prevent mouth sores. It says to drink 2 to 3 quarts of fluids daily to get rid of toxic effect. I do minimum 64 ounces of fluids daily.
Let us know how you do with your bone marrow biopsy.
Thank you, Eileen. I appreciate your reply and advice. I do need to up my water intake. I drink unpremeditated cups of tea a day so it’s best I change that to water.
I’ll fill some bottles to keep on the bench to remind me. Thank you 🌸 Also for the website info x
it would be great to include the coffee tho my doc said that are caffeinated drinks actually dehydrate so best to count on water. I quite enjoy my soda stream water tho I find it does give me some bloating.
A soda stream is a kitchen gadget that adds bubbles to your water to make it like soda or mineral water. It has a gas cylinder in it. Makes it cheaper if you use a lot of carbonated water in the family.
I just researched the subject of coffee and tea counting as fluid on Webmd.com. He said they do count. Look up the subject. I think they do flush you which is I think is the idea when taking HU…getting rid of toxic effect
Glad to hear that you are feeling more at ease with dealing with ET. It is certainly a manageable condition. I have been managing a MPN for 31 years now and am doing fine.
I just saw and posted an interesting piece on the mutations that cause MPNs. One of the recommendations is to use the more sensitive tests to detect JAK2 and the other mutations. Some of the tests give a false negative for people with lower allele burdens.
It is important that you be comfortable with your choice on how to treat the ET. The primary choices are aspiring-only, HU + aspirin, and Pegasys + aspirin. It is helpful to do a through review of each choice, looking at the benefits and the risks (pros/cons) of each choice. It is rarely a black & white decision. It helps if you are clear on what your treatment goals are (quality of life, length of life, etc.). It also helps to be clear about what your risk tolerance is. Which side effects are you more willing to risk or tolerate? There is also the issue of your treatment preferences. If you do opt for cytoreduction, do you prefer chemotherapy (HU) or an immune modulating therapy (PEG)? Based on the current research, do you want to target reducing your allele burden as a part of your treatment strategy? Do you want to consider participating in a clinical trial for treating ET if one is available?
You have choices to make in deciding how to treat the ET when the time to make a decision comes, Shared decision making = you have an equal voice in making the decision. Ultimately, no decision can be made without your consent. If you ae comfortable with HU and wish to pursue that option - great. If you would prefer Pegasys, then that is your option to advocate for. Likewise for the choice for aspirin + monitor. The decision is yours to make.
Here is some information about HU to get you started. Some of the same resources can be used to look into Pegasys and other medications.
Because I have had two TIA’s already, the specialist is not keen to just leave the work to the aspirin. She said the risk is high and a stroke could leave me with a formidable recovery.
I said that I’ve started getting reflux which I assumed was from the aspirin. She said it was likely and to see how I go and next time she could give me a tablet if it wanted. I was on Nexium ten years ago and got myself off that over time. I don’t really want to go back on it again. The aspirin I started on here in Oz was 100mg yet in South Africa they only sold 82mg. The Heamatologist said she would like me to resume the 100mg when these run out tho my doc said he prefers the 82mg to be kinder to my stomach. I have the aspirin with a meal to help protect my gut.
I told our children tonight. They were shocked as is understandable tho happy to know I’m in good hands.
Re the aspirin, I was put on the standard UK dosage of 75mg daily when first diagnosed last August, along with Hydroxy which I am tolerating well so far. After about 4 weeks I had very uncomfortable digestive symptoms of bloating and heartburn, despite taking daily omeprazole. My haematologist put me on Clopidogrel (Plavix) 75mg instead. The symptoms disappeared after a week or so and I stopped needing to take omeprazole. Wondered if this might be an option for you?
I found water soluble aspirin gave me reflux, I was on clopidigel but ran out and bought coated aspirin which I take after breakfast. No issues with it. Professor Harrison said the aspirin if you can tolerate it was better.
That's really interesting ciye. I would prefer to be on Aspirin if possible. While I was on the water soluble aspirin, the headaches went away. As far as I can tell, Clopidogrel has only one function in PV and that's as an antiplatelet, whereas aspirin has a wider range of benefits like anti-inflammation. I've never had a stroke or a bloodclot and my platelets are well within normal range now. I'll ask my haemo about coated aspirin.
Those two are on my A-list too. The article I really miss is the notation that used to be on the NIH Toxnet. It was one of the most balanced and informative assessments I read. Sadly the Toxnet is not longer available.
With a history of two TIAs cytoreduction is certainly indicated. It will come down to a choice of which medication you prefer. If you do decide that PEG is preferable, be aware that it is much more expensive and may be harder to get authorized.
Note that for higher risk patients, some docs actually prefer the lower dose aspirin 2x/day. Some on the forum have also found that the chewable form of aspirin works better. Something i did for a while was to cut a chewable low dose aspirin in half and take 1/2 tab 2X/day. I have also tried one of the newer forms of aspirin, Vazalore, that is supposed to be easier on the GI system. It is a lot more expensive so I switched back to the enteric aspirin when I did not find the Vazalore any better. Suggest you review all of the varied options with your care team. FYI - I also have reflux and have to take Nexium daily.
Glad to hear you told your kids. They do need to know. It seems you may have a Familial MPN variant. That is also the case in my family. My daughter also has the JAK2 mutation with a current diagnosis of ET. My son has an idiopathic erythrocytosis. Familial MPNs are an active area of research into better understanding the epigenetics of MPNs.
thank you, Hunter, interesting to know about the aspirin and the diff types. I was only aware that there was an extra one that was coated to help the stomach which is what I take. So I’m glad there are more options.
The info on MPN’s is confusing. Some say no family connection, others don’t. Like u said they are still investigating the link. Your family has been unfortunate there 😔
The specialist made it all sound quite routine and nothing to worry about. My husband walked away saying ‘oh it’s really nothing to worry about at all in the end’.
You will find references to the JAK2 46/1 (GGCC) MPN-predisposing haplotype in the literature. My daughter and I are both positive for this haplotype as well as the JAK2v617f mutation as well. Genetics do matter. Some people appear to be more likely to acquire the JAK2 mutation than others.
Whether ET is a big deal or a routine health matter is a bit of a conundrum. It is not a big deal if properly managed, that is unless it becomes a big deal due to the symptoms or because of progression. Finding the right treatment plan is what can keep it from becoming a big deal. That is one of the most important things I have learned managing a MPN for 31 years.
I’ve finally read through the above 3 li is, Hunter and I must admits most of it went over my head tho I still found it very interesting. Thank you for them 🤩
Glad they were interesting even if a bit technical. I think the main thing to know is that the predisposition to acquiring the JAK2 mutation appears to be genetics while actually acquiring the JAK2 mutation happens after after conniption.
Hi Pachena, I have same diagnosis as you and I started treatment many years ago on just aspirin, 75mg daily. After about 2 years I was taken off it as was experiencing stomach pains and bleeding. Then started Hydroxy, I’ve never been cautious about not touching them, I can’t see how I would open the capsules without my fingers, or wearing gloves! I don’t touch them for long, just open and swallow. Lots of water throughout the day. I decided to try Peg recently as have now been on Hydroxy for 15 years but immediately realised that my body wasn’t managing it. So I’m back on just Hydroxy now. I was disappointed and felt a bit of a failure at not being able to tolerate Peg, but I try to think about the positives daily. As Hunter says often, we are the ultimate decision makers in our treatments, we are given the options by our specialist haematologists and with their guidance and support we can choose our path. And another helpful person responded to one of my posts saying “I love my Hydroxy, it’s keeping me alive!” I found that very helpful. On the one hand I think it’s a bummer that we have to get wrapped up in all this medical stuff and take strong meds but on the other, brighter hand, thank goodness we can! Stay in touch, we are all here for you ❤️
Thank you, RazB 🌼 for sharing and reminding me of the positives 👍🏻
It is comforting to hear and learn from others. I think this fear is beginning to pass and the realisation settling in. To read the expression of that helpful person ‘I love my Hydroxy, it’s keeping me alive 🤩’ made me smile and think 🙌🏻
Hi, you’re welcome 😊 keep in touch. We all have low times (I have many) so this community is a great place to share, support and sometimes find much needed humour! X
I’m sorry Wyebird to hear your suffered a stroke at 50. Quite a shock. With relief I read you survived with no disability 🤩🌼
It’s hard to think of the possibility when I feel so well, yet that is a false comfort. No one knows what our day will bring. I am so glad that we live in this age of health care and the help we are able to receive.
Thank you all for your support and advice as my mind works through the adjustment of the diagnosis.
Since you are having stomach issues with aspirin could you ask your hematologist if you could be on a blood thinner in place of aspirin? I was already on a blood thinner for AFib so I am only on Hydroxyurea no aspirin.
I was diagnosed this April with JAK 2 mutation.My platelets platelets are around 1100. My symptoms were due to Erythromelalgia which appeared in December and are still very troublesome.
Aspirin hasn't helped but I tolerate it by making sure that I eat regularly.
I started Hydroxy a week ago 500 mg daily. About an hour after I've taken it I feel heady and slightly dizzy which does improve as the day goes by.
As well as the Erythromelalgia I have a history of Raynaud's which has got worse since I was diagnosed with ET.
I'm feeling very low at present because sleep is disturbed every night by my inflamed feet and toes which I have to cool constantly in cold water.
But hopefully in a few months time things will improve slowly.
It is worth noting that some people who experience microvascular symptoms find that they benefit from low dose aspirin 2x.day. Some also report a better response to the chewable aspirin. The good news is that hydroxy does tend to make the blood more "slippery" and may help with the erythromelalgia. If the adverse effects from the hydroxy persist, then you do have the option to switch to Pegasys or one of the other options. These treatment options may help with the Reynaud's as well.
Resolving the symptoms you report is just as important as preventing thrombosis. If you find the treatment plan is not resolving the symptoms, then you will need a different plan. Hopefully you are already working with a MPN Specialist who is familiar with the microvascular issues people with MPNs can experience. I have experienced milder versions of the issues with cold hands and burning feet. Fortunately my care plan (IFNs, improved iron levels, appropriate aspirin dose) resolved these issues.
thank you Hunter. I see the haematologist at the end of the month and she said once the BMB confirms the diagnosis of ET she will begin the prescription of Hydrea.
I asked at the chemist here for chewable aspirin and we don’t have it here in Oz. So I’ve bought the old fashioned aspirin that can be cut in half and will try half in the morning and the other half at night with meals to see how that goes. Thanks to those who suggested it 🤩
I saw my SIL yesterday and her face was peeling. She was confused why. She is a PE teacher and said she had a full sport day, it was cool and windy so maybe wind burn. I said I’ve read that you need to use sunblock and cover up when you r on Hydrea. So she went and got a hat and cardigan. You mentioned what I’d read about taking care with intimate issues etc and she said she hadn’t been told any of that. She’s been on it awhile now 🫣
I am rather surprised to hear that the chewable aspirin is not available anywhere in Australia. As far as I know, it is quite common most everywhere. If you do use a regular low-dose aspirin, it matters whether it is enteric or a regular tablet. You would not want to cut an enteric tablet in half as it would defeat the purpose of the coating. It would be OK to do that with a regular tablet. Di be sure to not take the aspirin on an empty stomach and drink plenty of water as it sounds like you plan to do.
That does sound like a windburn/sunburn for your sister. Hydrea increases skin cancer risk, which is why we have to be careful about sun exposure. While it can also cause rashes and other skin issues, we still can experience the same things as anyone else when exposed to the weather. Many providers do not warn patients about precautions with intimate relations. The risk is deemed relatively low so some providers simply do not bother warning patients. In my view, it is fine for people to decide to ignore the risk provided they understand what it is. That is what informed consent means.
Once the BMB results are in you can make your decision about what treatment option you prefer. Provided you know all of your options, you can make the choice that best suits your goals, risk tolerance, and treatment preference. That is also what informed consent means. If you believe Hydrea is the best choice for you, then it is your prerogative to accept the recommendation.
Wishing you all the best as you start the next stage of this journey.
Thank you Hunter, as always, your advice is greatly appreciated.
I’ll take note of all of the above. The haematologist has not offered any other option so when I see her again, I’ll ask what else there is to consider. She did say it wasn’t like regular chemotherapy tho each time I read a link about Hydrea, I do inwardly cringe. I know all medicines have a list of side effects tho the possible secondary cancers down the track aren’t desirable. But then as you said it is a matter of preferences and risk balancing.
The important thing is the review all of your treatment options prior to making a decision. Each choice has its own risk/benefit profile that needs to align with your goals. risk tolerance, and treatment preferences. The two primary choices, Hydrea and Pegasys, work in different ways and have different risk/benefit profiles. Some tolerate one and not the other. Some benefit more from one and not the other. We are al different.
It helps to understand some of the basics of the disease and how each of these medications works. The short version is that hydroxyurea is a cytotoxin. It works by interfering with DNA activity in hematopoietic stem cells (HSCs) and other cells in your body. It is not selective in that it affects all HSCs. Pegasys is an immune modulating drug that is an antiproliferative agent. The interferons seem to somehow target the mutated HSCs more than the normal cells. This may be why the IFNs can reduce the mutant allele burden. Both drugs have benefits in terms of cytoreduction. Hydroxy usually works faster. Interferon may have additional benefit in reducing the risk of progression (evidence pending for ET - some evidence for PV). Both drugs have risks. There is no way to predict how any one of us will respond. Both drugs also have a list of cautions and contraindications that are part of the decision.
Knowing the basics about ET and the underlying biology helps to inform your decisions and how you talk to your care team. There are some excellent webinars here. mpninfo.org/conferences/202...
It is also helps to understand how authorizations for medication works in your insurance or healthcare system. Generic hydroxyurea 500mg 60 caps = $25.00 (1-2 month supply). Pegasys 4 weekly doses = $4,200 (1 month supply). It is much easier for a doctor to get hydroxy approved. If you opt for a more expensive medication you often have to be prepared to advocate for your choice.
You may have seen this, but here is a excellent article by Dr, Claire Harrison on making the decision about how to treat ET. It really should be the model for how to make the decision. mpnjournal.org/how-i-treat-...
Hi Hunter, thank you, the article was quite a help and I feel the anxiety decreasing the more I understand. I appreciate all the time you give helping myself and others here 🌸
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