Hi, my wife has MF and has been having RBC transfusions reasonably often, I did ask her consultant if he had ever checked her baseline EPO levels, I didn't get an answer. He says she has a chronic bleed, but there is no evidence of passing any blood at all. I have done some research which indicates that low levels of EPO prevents blood cells maturing into red cells, and produces higher numbers of white cells and higher numbers of platelets. I'm probably way off the mark and my own memory is now suspect due to my own problems. I'm asking, could I be right in thinking her loss of a kidney and lower function of the other could affect the ability of her adrenal gland to keep her EPO levels up? Recent FBC suggest her platelets and white cell counts are rising while her Reds are reduced. Which gives her consultant reason to increase hydroxycarbamide doses and frequency of RBC transfusions.
EPO testing: Hi, my wife has MF and has been... - MPN Voice
EPO testing
There are a number of questions that are evident in what you present. You are asking a reasonable question. EPO is produced primarily in the kidneys. It is the trigger that causes the production of red blood cells. Monitoring EPO is part of understanding what is going on when someone experiences erythropenia. It is not clear why you are not getting an answer when you ask about this.
What is the status of her kidney function? Chronic kidney disease can affect EPO levels. It may be part of the overall picture.
If she has an internal bleed, where is it? How is it being treated? An internal bleed would explain the decreased RBCs and increase in platelets, but not the increase in leukocytes. Is the consultant thinking that there is evidence of the MF progressing? If so, what is the appropriate treatment plan? Would she respond better to one of the JAK-inhibitors or another treatment option?
What other symptoms is she experiencing? What is the status of splenomegaly? What role are her overall health issues playing in how her MF presents? Proper MPN care is holistic and looks at these issues in an integrated fashion.
If you are not getting satisfactory answers to your questions, suggest it is time to get a second opinion from a MPN Specialist. Getting a second opinion is always warranted with any significant medical issue or decision. mpnforum.com/list-hem./
Wishing you success in getting all of your questions answered.
Thank you hunter for your reply, her kidney function is quite low as her creatinine is often over 120, or as low as 105. She has no splenomegaly, her spleen burst 5 years ago and when removed was 6 times normal size! The chronic bleeding has never been addressed, though when she had acute bleeding they never found the source, but would top her up with Upto six bags of blood. As for a referral to an mpn specialist that's a no go area, as her consultant says he is able to reference the top mpn specialists in the country.
This sounds like a situation where better answers are needed. It is her prerogative to seek a second opinion if she wishes to do so. It always possible to pay out-of-pocket to a doctor in private practice. I believe that the GP can write the referral to a MPN Specialist if the hematologist is not willing to do so. Seeking a second opinion is a common practice Most doctors do not mind, unless they are putting their own ego in front of this decision. cancerresearchuk.org/about-...
I am not aware of any reason to withhold ruxolitinib after someone has had a splenectomy. You are correct that RUX can help reduce splenomegaly, but that is not its only purpose. You are also correct that RUX cost is much higher than hydroxycarbamide. RUX = $14,300/60 tabs. Hydroxy = $25/60 tabs. While the cost should not bear on the decision about which medication to use, the reality is that it sometimes does.
The cost of an EPO test is approximately $79.00.
Epogen (injectable EPO) cost - injectable solution: goodrx.com
2,000 units/mL (1 vial, 1 mL): $39.00
3,000 units/mL (3 vial, 1 mL): $161.00
4,000 units/mL (4 vial, 1 mL): $280.00
10,000 units/mL (4 vial, 1 mL): $686.00
20,000 units/mL (1 vial, 1 mL): $348.00
It seems very unlikely that the costs associated with testing for or providing EPO would have any bearing on the decision. It seems like a reasonable thing to at least rule out.
I would also ask about her von Willebrand Factors. They may have already been checked. Given the propensity for bleeding, they are certainly checking her prothrombin times (ptt/aptt-INR). That would be something worth knowing as a point of reference.
You are asking very reasonable questions and have legitimate concerns. The questions and concerns need to be addressed. Another concern is that hydroxycarbamide can cause renal impairment. Adverse effects are dose dependant. Consultation with a nephrologist on this issue would be warranted.
Wishing you both success in finding answers to your questions.
I would continue to push for a test of EPO levels. Mine was low and once I had started injections I didn't need transfusions.
Thank you Otterfield, I do wonder if there is a cost element involved with the consultant's thinking. He often says he's not bothered what treatments the NHS provides cost. Yet she is on hydroxycarbamide (at 50p a tablet) and Anagrelide which from memory is costing £12 a day! I remember him toying with using Ruxolitinib, but didn't, we don't know if the cost was prohibitive in his eyes or it really wasn't suitable as she doesn't have a spleen, which I think it stops getting too big. The weeks she has spent as a patient on the wards due to massive bleeds and the blood given to her are quite expensive to the NHS. So if he's penny pinching on the treatment he's losing more in hospital inpatient costs. Do you know the cost of EPO injections? Thank you.
If he says he's not bothered about the cost to the NHS, I suggest you take him at his word and ask again for the test. If the NHS can do it for me, they can do it for your wife, unless there's a good medical reason not to. I don't know what the actual injections cost, but we have all paid in taxes for medical treatment so if we need it we should get it.
Thank you Otterfield, it will be done, he often says I overthink things, but I'm fighting for my wife. I'm so glad for you, getting the SCT and hope you continue to improve. I'm not sure about asking for such a thing for my wife, what with her lack of a kidney and spleen, it's sounds a bit too much to ask for a SCT.
I’m currently on weekly EPO injections as I have been very anaemic for a long time. There’s been various investigations as to whether I’m bleeding from somewhere but nothing found. My white cell count has stayed in normal levels. I had a blood test after 3 injections and my haemoglobin had come up a bit so Haematology prescribed 8 more. I go back at the end of June for the results and to see if they’ve finally got the Next Generational Sequencing tests results. Hope all goes well for you.
Thank you for replying, my wife is also on anagrelide two tablets a day. May I ask if your platelet count dropped at all after the EPO injections? And how much your haemoglobin went up? My wife often has an iron infusion along with the red blood cells when her Hgb goes below 90.
Re Ruxolitinib One thing you need to research is the current NICE guidelines. As you now know it is expensive but it can only be prescribed according to those guidelines. When I was on it it was for MF and to shrink the spleen. See if your wife's conditions are covered. However if it is a NICE restriction the consultant should have made that clear
EPO injections have certainly kept my Hb levels up for the last 4 years but don't seem, from comments on this forum, to work for everyone and it certainly took a number of months for them to have any effect for me - I nearly gave up. I unfortunately developed anaemia after starting ruxolitinib, which is a common side effect so you could ask if this is why it isn't being considered. Although for most people this resolves itself in my case it didn't. On the plus side I haven't put on weight. I've had so many transfusions that my ferritin levels are too high so trying EPO was the next course. It's not a panacea though. I had to stop it for 3 months earlier this year as it was 'fingered' as causing a bad spike in my blood pressure. My Hb levels dropped like a stone and more blood pressure drugs added before I could start it again. Although my figures are good it has done nothing for fatigue and breathlessness which at the risk of sounding flippant (and I am only too aware how serious all this is) I was very disppointed in due to the publicity about its illegal use in sport
Regarding price the NICE indicative figure for 3,000 units for 6 injections is approx £100 - this is my prescription and different strengths are priced up or down accordingly. A drop in the ocean compared to the price of rux which I've been on for over 4 years (in Scotland).
Sounds a good idea to at least find out about your wife's EPO levels.
Thank you DJK12 for your answer, my wife's ferritin levels when tested are often too low, which is why she has had at least five iron infusions in the past year, which does point to her losing blood rather than recycling the red cells which would recover the iron held within the red cells, therefore keeping her ferritin levels up. But I'm not at all sure where the iron is recovered from old red cells, if it is in the spleen or kidney, it could mean she's not losing blood from a chronic bleed, as she hasn't a spleen and only one dodgy kidney. As to high blood pressure, she does have it, it only started to rise six years ago, when the doctors now think her kidney began to die, though her GP wasn't bothered when she went to her about the sudden increase in BP.
Iron metabolism is actually quite complex. Most of the iron used to make red blood cells is recycled from aged red blood cells.
"Approximately 90% of iron needs for erythropoiesis are met by internal iron recycling from aged red blood cells. This task is accomplished by macrophages, predominantly Kupffer cells (KCs) in the liver and red pulp macrophages (RPMs) in the spleen" ncbi.nlm.nih.gov/pmc/articl....
A chronic bleed would be expected to reduce ferritin since RBCs are being lost to the bleeding and are not available for recycling.
Thanks hunter, I've been reading a little on it and got to the bit of macrophages and the liver, but didn't know of the role the spleen took, but suspected the spleen might be involved. As she hasn't a spleen and even though only a lightweight has a slightly enlarged liver, I'm wondering if she is losing a little blood from the biliary tree into the colon, as this was suspected when she had acute bleeding numerous times, but luckily they have ceased.
Bleeding into the colon would be evident in a occult blood stool analysis. My understanding it that is pretty standard assessment when any kind of GI bleeding is suspected. I would think that has already been done if bleeding into the GI system is suspected.
Welcome to Wales!!!, no such analysis has ever been done, though we are getting a list together of what needs to be done, and I will be writing to her consultant before her visit at the beginning of June
Assertive patients receive higher quality care. Passive patients do not. It sounds like it is time for some very assertive action.
Wishing you both all of the best.
Your wife is lucky to have you in her corner. It seems obvious from others' answers that an EPO test should not be a big hassle to do via the NHS. The answers your wife's current specialist is providing are setting off alarm bells for me. Having "access" to the top or "referencing" the top specialists in the country is not the same as being seen by someone who is dedicated to MPNs. Additionally, any answers he receives will be automatically filtered through his own perceptions and lack of direct experience in this area. Getting a second opinion is every patient's right and as Hunter said should not be an issue for any good doctor - a doctor who wants to make sure his/her patients are receiving optimal treatment.
The only time I have personally run into problems with referrals it has been due to the ego of the physician..and the two times it happened I promptly switched providers. We get second opinions on auto mechanics, plumbers, painters and a myriad of other service providers, but when it comes to our health we tend to just accept what our doctors tell us. They are only human and have limits as do we all. The best recognize this fact and are happy to have their work/thoughts challenged - either they turn out to be right or they expand their knowledge and everyone wins.
I would demand an EPO test as well as answers to your other questions. I would also, personally, seek a referral to an MPN specialist. Your wife is lucky that there are quite a few in the UK. Hopefully the referral would be done in coordination with your wife's specialist but if he's not willing to put your wife's needs first and his ego second then get a referral from your wife's GP and move forward.
Best of luck!
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