Hi all, first time poster :). My husband (55) has the CalR mutation, diagnosed 8 years ago, but we weren't given any information about it until we moved to the Fylde coast 4 years ago. We've always just gone with the flow. He's under a haematology consultant and has quarterly blood tests. Anyway, his count was around 700 in the autumn (has been as high as 1300 in the past but seemed settled for the last year or so). To prepare for a gall bladder removal he put him on hydroxy - 9 pills a week, was tired, and his gout was verrry reactive but that's all. Anyway op went well and the doc said it was up to him whether he came of hydroxy, husband asked what would happen - he said he had no idea, never taken anyone off it before.. it's been about 3 weeks now and he's much more tired than before - we are figuring it's the platelet count bouncing back? Anyone had similar experience? What happened? Also, is it common to go onto Hydroxy to prep for an operation (it makes good sense - but obviously hasn't happened in our neck of the woods)
Lastly, from reading posts I see that folks go on hydroxy with much lower platelets, we'd always thought 1500 was the tipping point, but is it the difference between Jak2 and CalR? I feel very uneducated!
Thanks all! H
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As patients we need to have confidence in those treating us and you have many unanswered questions. After that admission by your husband’s haematologist of what appears to be limited experience of Mpns I’d be asking for a referral to a Mpn specialist.
There’s Dr Nauman Butt at the Clatterbridge Cancer Centre (Wirral). I’ve noticed his name on the same panel at Mpn Voice events as specialists from Guys, Oxford, Cambridge hospitals.
Also there’s Professor Somervaille and his team at the Christie (Manchester). You’d have a journey to either but it has to be worth the effort for peace of mind and expert care. Others here who are nearer your area will have names of specialists too.
Hello and welcome. Glad you decided to post your questions.
JAK2 vs CALR. There are three identified driver mutations that cause the classic MPNs, JAK2 - CALR - MPL. All three of these gene variants are somatic mutations that affect hematopoietic stem cells (HSCs). They are similar but not identical in their impact. All three cause the HSCs to self-phosphorylate. This means the "on-switch" for blood cell production is always on regardless of the need for more blood cells. These mutations also cause an upregulation of the JAK-STAT pathway. In addition to making too many blood cells, the body overproduces inflammatory cytokines. This overproduction of inflammatory cytokines is thought to be responsible for many of the constitutional symptoms we experience, including fatigue.
The CALR mutation differs from JAK2 in that the risk of thrombosis is lower with CALR than it is with JAK2. Note that there are two types of CALR, type 1 and type 2. There are also two types of JAK2, v617f and Exon 12. You can learn more about these gene variants from multiple sources. These webinars are particularly good. Start with MPN Molecular biology. mpninfo.org/conferences/202...
It is worth noting that the higher the platelet levels go, the higher the risk of hemorrhage (excess bleeding/bruising). There is a risk at higher levels of Acquired von Willebrand Disease, a bleeding disorder. People with higher platelet levels can experience increased hemorrhage even in the absence of AcqvWBD. This used to happen to me whenever my PLTs were near 800.
It is also worth noting that fatigue is a common hydroxyurea adverse effect. Renal impairment / increasing uric acid levels is another potential adverse effect. Normally, discontinuing the HU will resolve any side effects. I have never heard of a rebound effect with fatigue when discontinuing HU, but we are all different in how we respond. The treating doctor should be familiar with starting and stopping hydroxyurea since both are common occurrences when treating MPNs. However, MPNs are rare disorders and many doctors are not experienced with treating them. This includes many hematologists. It may be the doctor he was consulting with is just not experienced with managing ET.
While starting on HU just to prepare for surgery is not a common practice, it would be reasonable to consider, particularly if his platelet levels were on the higher side. There would be a significant increased risk of bleeding if the platelets were too high. Note that post-operative reactive thrombocytosis would also be predictable. It is common to see a spike in platelet levels after a surgery. I have experienced that with every surgery I have had.
It is unfortunate that your husband was not educated about ET from the first point of diagnosis. Going with the flow is not a good idea when managing a chronic form of cancer like ET. While the course of the disease my be relatively indolent, managing it should not be taken for granted. It is very important to consult with a MPN Specialist when managing a MPN. MPNs are rare disorders and optimal care requires MPN expert provider input.
Wishing you and you husband all the best in this journey.
What a great response. So detailed & supportive. I have CALR but didn’t know there are 2 types. Had my 12 weekly consultant call last week but will ask about it next time. Thank you.
The "tipping point" by standard practice is 450-600, depending on the Dr's preference. But 1500 is well in the excess zone by accepted MPN standards. You say he was at 1300 but before starting HU he was at 700, is that right? That is a large variation absent treatment and a good result if it holds. If you're trying with and without HU your Dr should be recommending frequent blood tests to monitor the effects.
Also there are some early trials for exciting new therapies for CALR. If you have access it's worth a look.
I’m not an expert, having just started my own HU journey, but as others have already said, it would be wise for your husband to be cared for by an MPN specialist.
Dr Oakes at Carlisle Infirmary is excellent. And not a million miles from the Fylde coast.
you have some very helpful answers already on the meds side.
Just to cover all bases, has your husband had all his vitamin levels checked recently, just incase some are very low? Tiredness can be due to something as simple as a B12 deficiency. If his vit levels haven’t been tested I would suggest he requests this , GP could do this.
Ask for : Vit D, B12, Folate, Iron panel , possibly thyroid TSH, Free T3 & free T4.
Always get copy of results. Being in range is not always the same as optimal , especially with B12. I used to suffer from excessive fatigue until I corrected my B12 deficiency. My B12 was still in range but towards lower end. This is quite common and I know others here had similar experiences.
Just good to rule these things out.
Hope you find the answers to your husband’s problems.
I just want to say a holding thank you to you all for your replies, this has been a lot of really helpful perspective and information. As a partner, there is something around managing the discovery phase (late as it is!) with respect for my husband's wishes. While it's happening to him, we are in it together, but we have differing levels of curiosity and approaches. Fortunately, we work well together I've started researching referrals to achieve a more informed treatment plan. Tho tbh this has been infinitely better than the resounding wall of silence we had for the first 4 years of his diagnosis. And while I knew about them before I'd never looked at the video list, so this is great.
And this has also given me the drive to find out more about his other - let's call them 'foibles'.. and how they might relate to ET (it's a whole other post in waiting).
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