Hydroxyurea dose! About right or too much?

[jointpain]

My darling wife still in hospital, no further towards getting better. Still vomits what little food she consumes. Since Wednesday she has been given hydroxyurea 1000mg once a day, as she now weighs short of 50kg, can I ask if this is a correct dose? Her diagnosis is myelofibrosis JAK2 positive. Platelet count of around or above 1000. She still has the portal vein thrombus, causing quite a bit of pain and discomfort. She is on 0.6 mg clexane twice a day now. Though no change in her symptoms. I have asked for her to be referred to a name given to me from my last post, but no word as yet. Is it even allowed? To change to a different hematologist and a different hospital and a different country??? England instead of Wales!!

[Aime]

So sorry to hear you are still struggling to get relief for your wife. I know it’s not affordable for everyone but could you see a consultant privately, just to get her treatment, etc sorted out? If you are unsure about dosage of meds, please insist in getting your questions answered. I know it is perhaps seen as an impolite thing to do but if you don’t stick up for your wife, then no one will. I’m so much more assertive since my diagnosis.

My thoughts are with you both, kindest regards Aime xx😺

[Garden987]

Hi JOintpain. I am so sad to hear of your wife's situation. I live in Wales and have ET and CFS. Unfortunately it is very difficult to get a referral to a hospital outside Wales which is why I am moving to Gloucestershire. You. Outdoor try Macmillan - sadly closed until Monday - but I urge you to insist that your haemotologist refers you urgently to another hospital immediately. Look on the website. The best ones are unfortunately in USA. Write to your MP. Look up private hospitals, I hear Gloucester Oncology are good but I don't believe you will find expert treatment in Wales - from personal experience. Cancer Support appear to have lots of knowledge so you could try them. I so wish there was something I could do to help you. If you would like to ring me MAz could give you my number. make as much fuss as you can to anyone you can. I send you my heartfelt pain and pray that you can find a solution. God Bless you and your wife. Mary

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[Ebot]

I’m so sorry to hear of your ongoing situation. I think you are in a bit of a bind because your poor dear wife is in hospital and therefore possibly not very mobile and transportable. I cannot see why you would not have the right to ask for a second opinion and to be transferred to the care of an MPN specialist. Are the rules different in Wales?

But first you need to know who you want to see and where they consult. And if there is someone reasonably close to home. Maz will be able to advise. Certainly while there are excellent MPN specialists in the USA there are also world renown specialists here not least Prof Harrison at Guy’s in London. I’m aware people come from all over to consult with her. You really want to be under the care of someone who is part of the UK MPN network - they talk to each other. Once you get the name of a consultant e-mail them for guidance. Ask if they would be happy to see / talk to you. Some MPN specialists may have private clinics (Prof Harrison doesn’t). I suspect you are going to have to be bullish about this.

As for dosage, it doesn’t sound unreasonable. Perhaps others have insights. The key thing is hydration. If your wife is not managing to eat I’m assuming she is on IV fluids etc.

Please let us know how you get on. Wishing you both the very best.

[JSKly]

I’m so sorry to hear about your wife and you are so worried naturally.

I am in Gloucestershire. I don’t agree with the recommendation to see someone privately as my experience for myself and others is that because these disorders are somewhat rare the greatest experience and knowledge is within the NHS. But In any system

Knowledge and experience varies doctor to doctor.

I was recommended two doctors when we moved here: Frewin and Lush. I see another but know that my situation is discussed by the team so I feel I have the best. I understand the leading expert in the country is Claire Harrison who is in London. I wonder if there is a way for you to speak to her, even a phone call. Maz might be able to help.

I certainly agree with pushing and demanding help.

I have taken hydroxy in the past. It isn’t possible to say what dosage is correct for someone else.

Please let us know how you get on.

[catkinspolymer]

I cant add much to what,s already been said , 1000mg of HU is pretty typical , what is her platelet level is it in the normal range. Prof Harrison I agree is your best bet.

[jane13]

The rules for second opinions etc are different in Wales and I am pretty sure that u have no "right" to a referral outside Wales.

As ebot said, the HU dosages doesn't sound unreasonable, but a drip for hydration sounds essential. Have u asked why HU rather than Ruxolutinib which is generally seen as "best" treatment for high risk MF?

Given your missus can't leave hospital at the moment, u may need to make do with your local haematologist for now at least. I doubt u will manage to get your wife transferred to another hospital, though it is, I suppose, possible? And I doubt any haematologist will come in to see her. Perhaps u could get her notes urgently reviewed by a n other haematologist; I don't see why this shouldn't happen.

So how to make the best of the local haem: may be a structured list of questions? Get in touch with the complaints department who should be willing to send a senior nurse to a meeting with you and the haem (and your wife). They should b able to help get a review of your wife's notes quickly too if u want this. If the complaints dept don't respond quickly - go and find them, don't wait for the rigmarole of exchange of letters - find where the Chief Exec or Director of Nursing has their office and go and ask their secretary for an appointment. (I am ex-NHS management).

[jointpain in reply to jane13]

Thank you so much for your insight as to what can be done, I will get on to it asap, though I imagine Monday will be the earliest I can get hold of the complaints dept. But I will try today.

[Ramy22]

I am so sorry that all this is happening. I can’t add anything more to what has been said apart from my thoughts and prayers.

[Stevesmum42]

This is a nightmare for you both. I can't believe that you are still in this situation. As has been suggested why can't your wife's notes be sent to an mpn specialist. ? It's ridiculous that things are segmented like this, with all the the good ways of communicating now. You should have access to who you need to see, its your wife's health here that is the most important thing.

We don't appear to have joined up medical services in the uk. What is best for the patient is the overriding concern here.

I am so sorry I am unable to give you answers just want to let you know, as others have, that we are thinking of you both and sincerely hope things improve for your wife soon.

With very best wishes to you both Sandy x

[Norman45]

I can’t understand why you can’t cross the border for treatment, after all it’s a national health service open to all UK citizens and free at the point of delivery.

If you can, psychologically you would both feel better. Ward 19 at Birmingham Heartlands Hospital is a centre of excellence for MPNs and SCTs. Try it if you can. Drs Ewing, Murthy, Lovell and Panesha are good starting points. I have email addresses if you need them.

I believe patients have the right to choose their hospitals so go for it.

[fee13]

They are probably concerned with getting her platelets down first, hence the hydroxy. Is there a chance your haematologist could video link/chat with an MPN specialist and discuss your wife's case?

[Tico]

Sending u & ur wife so much love & pray everything go's well 4u. Will be thinking of u. xxx. Ps.jane13 talks so much sense i wud follow her advice!x. Atb,tico

[Aime]

Totally agree with Jane13,s knowledgeable advice. That’s one of the great strengths of this forum - we have people on it from all different walks of life.xx😺😺

[Rachelthepotter]

One thing you can do is to use the hospital complaints system , now, while your wife is in hospital. There should be a system in place called PALS where you can say how concerned you are about your wife's treatment and ask for an urgent explanation . Or you can email the Chief Exec. Hospitals have a patient experience team ( In Wales as well as England) who have to respond. It may be that the right things are happening now in terms of treatment, but you need an explanation.

If you do it in an email, they will then have a record of what’s happening, and you will have a record of their response.

Once the immediate crisis is over, you can look at finding a better consultant , or having the local one liaise with a specialist who know whats what as far as MPNs go.

I managed to get my own care ( I have MF) transferred by first finding out which consultant and hospital I wanted to go to, seeing him privately once to see if he’d be agreeable ( he was) and then getting my GP to make the referral. Once that was done and I had my first NHS appointment at the new hospital booked ( and in my case it was as much hospital as doctor that I wanted to change) I could then release the follow up appointment I had at the original hospital.

I have the appointment at the new hospital next week - we’ll see how it goes.

My heart goes out to you. Having a beloved wife with this rotten disease is one thing, but having avoidable suffering and stress caused to both of you by incompetence or indifference is something else again.

Do keep posting. All my love to you both.

[katiewalsh]

Hi. You’ve received lots of good advice so far. But I strongly encourage you to talk to Maz (the head of this forum) because she knows so much and can be very helpful. Congratulations on the great job you’re doing under extremely difficult circumstances. Please let us know how things work out & remember you’ve got a lot of people here who care about & are supporting you and your wife. Katie

[MaggieMagpie49]

Reading this my heart goes out to you and your wife. I cant add anymore to this wonderful advice , concern and support but touched to send all good wishes and strength to you both. I hope she feels better soon x x

[jointpain]

My wife was sick again last night, whether it was because of the morphine they are now giving her or the blockage of her gut which would be due to the portal vein thrombus being now so large it is making her whole colon swollen. All this is plain to me, my wife, and some nurses. Though a nurse said this morning, that because of the clexane they wouldn't operate anyway. She now wishes she died on May the 1st.

[Aime]

So sorry to hear your wife is not improving. Please insist on getting help on whatever she needs. She won’t be meaning what she’s saying about dying, she must be feeling low with all that’s happening.

My thoughts are with you both, please speak up though ASAP. As someone has already said you are doing fantastic but now you need to summon more strength. Kindest regards Aime x

[jane13]

What can we say? I am sure that everyone on this forum is thinking of you. From my own experience I entirely empathise with your wife's feelings. But you may get through this and out the other side.

I am obviously totally out of my depth clinically and hesitate to comment further. It seems that no doctor has sat down and had a conversation with you about the treatment plan, prognosis and options? You can push hard for this tomorrow via Complaints/senior management. The ward should have a record of the consultant responsible for your wife's care and that should be the place to start, though this sounds like a multi-disciplinary case.

I can only think of 1 constructive suggestion for today: you might ask the senior nurse on your ward for your wife to be reviewed now by the senior doctor to which s/he has access? This doc will probably not be very senior nor even the right specialty but will be able to call consultants for advice. You could ask if your wife needs an emergency transfer to the regional centre for Interventional Radiology (to put in a shunt around the thrombus), or an assessment by your hospital's Intensive Care Unit to see if she should be getting support from there. That might stir things up ready for action tomorrow. But I am grasping at straws.

[Pingi]

Sorry to hear about your wife’s ongoing problems, have you asked to be referred to this service? They take patients from Wales - uhb.nhs.uk/crd-budd.htm

[Norman45 in reply to Pingi]

University Hospital Birmingham, also known as the QE, is in the same trust group as Heartlands and if you see my posting above is an MPN centre of excellence.

It’s worth trying them to see if they will take on your ‘poor’ wife.

[jane13]

Just to be clear about the logistics, your Health Board (HB) of residence will have to pay for your wife's care, wherever she is treated within the NHS. Birmingham, for example, may be very willing to treat her, but they have no authority to send an ambulance to collect her, nor funds to pay for her care unless this is authorised from within her HB. So this is a "push" not a "pull" system. Her GP or, at the moment, the hospital she is in need to request Birmingham (or wherever) to take her, usually with a consultant-to-consultant dialogue. This should happen readily if she needs an emergency or a specialist operation which the current hospital cannot do: she should then be sent to a competent centre wherever this is, without problems.

If these conditions do not arise I would be very surprised if there is an inter-hospital transfer. If she gets home OK then getting her referred elsewhere for outpatient care will be easier - see Rachel's post above or tell yr GP u have no confidence in the local service and why.

You could potentially go privately to Birmingham but I would suggest pursuing current lines of approach via complaints etc first.

[jointpain in reply to jane13]

I have already written to the GP stating we have no confidence in the current hematology dept or the hematologist amongst other points I need answering. I shall be going in tomorrow morning in the hope of seeing the head of nursing, with a request that the PVT is reviewed by a competent surgeon, as the 'plan' she is on at the moment is making her weaker and in more pain each day. Her hydroxyurea has been increased to a now disturbing 1500mg a day. As she vomited again last night, and had a nose bleed I doubt if the increased drugs are being absorbed.

[jane13]

Good thinking re meeting Head of Nursing, hopefully with your wife's named consultant?

Here's a few thoughts, bearing in mind that I know very little about what's happening, and am out-of-date so please don't think that I am competent to make recommendations.

Don't try and do the medics job for them - ask questions, don't tell them what's wrong and what needs doing, make them tell you.

Confirm at the start of the meeting that a note of it will be produced asap.

Also maybe focus on what is going to happen now, rather than go over old complaints, which u can do later if need be. Be constructive, try to be dispassionate - difficult I know - focus on your wife's future care..

Questions I would ask:

- what is the diagnosis: how many things has your wife got wrong with her? e.g.: MF, PVT, high platelets, missing spleen

- how do they recommend treating each element of the diagnosis eg: in some detail go through the options, risks, expected outcomes

- what is the timeline they expect for treatment as some elements may be interconnected?

- where is treatment going to take place and why? e.g. given the complexity of yr wife's case shouldn't she be transferred to a specialist centre as a matter of priority?

- who is going to co-ordinate care and through what process? you need one consultant in charge but you might expect a multi-disciplinary team of several specialists to talk regularly for a short while

- who is going to tell u what is happening and when? eg perhaps your meeting reconvenes in 2 days by which time x y and z should have happened

I do not know whether or not yr wife's treatment is correct, but the consultant should be able to talk through what is happening and why in a clear manner.

If this was my relative, when talking about PVT I would want to know why they are not getting an Interventional Radiologist to put in a shunt around the thrombus. There may be a good reason for this but what is it? If you haven't come across Interventional Radiology before, maybe have a quick look on the web tonight.

If you are still really unhappy with the treatment plan at the end of the meeting you could ask for an external doctor to urgently review your wife's notes.

Good luck

PS i have been on higher doses of HU as have others on this forum. As fee13 says, that may be the fastest way of reducing platelets, which carry a risk of stroke, but ask the question and get them to tell you

[jointpain in reply to jane13]

Hi, that you for the list of questions I should ask, they will come in handy tomorrow if I get an audience with the head of nursing! Just rang my wife and she is on the move from surgical assessment ward to a gynecology ward?! No I haven't a clue why.

PALS does not apply in Wales, one is supposed to complain or "raise a concern" with the person you are unhappy with in the first instance. How that works is beyond me. I doubt if this hospital has even heard the words Interventional Radiologist before never mind have one! Anyway a big day tomorrow. Just hope it all goes well.

[asacker]

When I had a splenic vein thrombosis, I was first diagnosed as expecting twins! Well I was pregnant at the time but it was my spleen that was the lump. If your wife has a very enlarged spleen from her MF, she could be being sick because of that. Ruxilinitib should sort that out. I have just gone on it and have no side effects only a wonderful release from all the itching after showering. I was also on hydroxyurea for about 30-40 years, sometimes on higher doses that you report. If Dr Harrison can't see/talk to you then I can recommend the MPN team at University College Hospital in London too. Good luck to you and your wife, Amanda