Raised platelets, JAK 2 Alternative drugs to Hydroxy..

[Kwiatek]

I am 74 and have been diagnosed with JAK2, for 4 years. In reality I have had this condition for longer. Diagnosis only came after thrombosis and a blood clot in the leg. I am on Hydro and Warfarin. I have had a few venesections but not for a couple of years now. My platelets last time were 500. This is an increase. Now I am on 500mgs hydro for 5 days and 2times 500mgs for 2 days a week.I have not seen a consultant for over 2 years. She is on long term sickness leave. Just haematology nurses.They change the dose. I have a telephone appointment with a registrar next week, I will request a face to face appointment.

Question: What other drugs or treatments are available in the UK for JAK 2 ?

Any suggestions of a good haematology consultant in the UK ? I love near Brighton, London would be ok .

Thank you anyone who can help.

[Mostew]

Can't get better than professor Claire Harrison at Guys hospital in London

[hunter5582]

It sounds like you are diagnosed with Polycythemia Vera, caused by the JAK2 mutation. Note that JAK2 can also cause Essential Thrombocythemia and and Myelofibrosis.

There certainly are other drugs to treat PV and the other MPNs. Jakavi and Besremi are both approved for the treatment of PV. Hydroxy is actually used off-label for PV here in the USA. While in common use, it is not FDA-approved for PV. Another drug in common use for PV (off-label) is Pegasys.

Jakavi is a JAK-inhibitor. It is particularly good for certain PV symptoms. Pegasys and Besremi are both PEGylated forms of interferon. The interferons are the one treatment option that may be disease-modifying, reducing allele burden and risk of progression. Many MPN Specialists now favor the use of the interferons over other treatment options.

Note that the interferons and Jakavi are much more expensive than hydroxy. There is resistance in some healthcare systems to accessing these medication. Your care team can best explain this to you.

Your care team should include a MPN Specialist rather than only a regular hematologist. Relying solely on a regular Hematology Nurse Specialist would be suboptimal. MPNs are rare disorders and require MPN expert consultation to optimize treatment. Here is a list of MPN Specialists. As Mostew notes, Dr. Claire Harrison is one of the world's leading experts on MPNs and a great doc. mpnforum.com/list-hem./

All the best.

[EPguy]

The replies here have the essential info you need. On how we ask the question, most Drs won't discuss treating "Jak2" (actually Jak2 v617F mutation). Rather they will discuss treating the effects of having the mutation, especially blood counts.

But it gets interesting in the last few years that we actually can sometimes treat Jak2, the IFNs (interferon) PEG and Besremi can reduce the mutation for many patients while also controlling blood counts. This is good to know when you discuss your options with the specialist.

Your Dr will likely remind you that the benefits of "treating" Jak2 are still not proven, but indications are strong it's useful as discussed on the forum here.

[Kwiatek]

Thank you ,everyone for this advice. I will ask my GP to refer me to Guys hospital, Professor Harrison and her team. Is there a long waiting list ? It would have to be NHS . Will it mean I will have to go to London for blood tests , every 3 months ? Or can there be a collaboration between hospitals ?

[MPort]

Hi i will just answer your last question. I am under Professor Harrison and the Guys team. I used to go every 4 months pre Covid. Now I have more telephone consultations. I get my blood tests done at my local GP surgery. Guys nurse send them the request and then I can book an appointment. The results are sent back to Guys. It does take some prompting! Another point if you travel by train from Brighton you can change at East Croydon for London Bridge station. Guys is across the road. I use this route from East Grinstead. Good luck.

[Exeter21]

definitely as all have put in uk contact Professor Claire Harrison at Guys London for at least a telephone appointment . She is the expert . She sorted my problems and approved Interferon. Sounds bad treatment in your area but unfortunately you have to keep being firm & complain to get anywhere in UK at present . It’s your health so make sure you push for your treatment. Dr Harrison is excellent. 👍. I am in Devon & she deals with all of UK if the need .

[Hopetohelp]

Worth asking about interferons. I can recommend Pegasys. Good luck

[Kwiatek]

Thank you , telephone conversations soon, I will ask and post relevant reply