Where are you Hunter?

[Kelly2]

Where are you Hunter?

[hunter5582]

Hi Kelly2! I am currently wrapping up a couple of weeks at Camp Brainy Ridge brainyridge.com/ where I have been working with youth with chronic medical conditions. We have been working on learning leadership skills, self-advocacy, and collective advocacy for people with chronic conditions. We have also been having a whole lot of fun!

These are an amazing group of young people who manage significant chronic medical issues like epilepsy, heart disease, diabetes, neurofibromatosis, autistic spectrum, cerebral palsy, and more. They are truly an inspiration. They are also our future. Someday, they will be the ones to help find cures for the diseases that affect them and us.

You can see a bit more of what we have been up to on our Facebook page. facebook.com/p/Brainy-Ridge...

Hope all is well in your part of the world.

[Kelly2 in reply to hunter5582]

Hi Hunter! I wanted to tell you that I had my kidney surgery and hopefully all went well till now! In one monh they will get the pigtail out and then I have to check in six months and one year. I had a terrible time keeping my platelets down with hydroxy as finding the right dosage was and is extremely difficult! "Take two a day" they went 120 and had the surgery. Then take onex3 a week they went 800! Now they told me "one a day every day! Which I know it will soar up again. So I take 1 every day and two in week ends on my own. I think I really need one and a half everyday. The haematologist saw my struggle with keeping them right and said we must see the Besremi in September. As I have hypothyroidism ( my endocrinologist isn't familiar with Besremi as it is a "new drug") I am really concerned about it! I wanted your reassurance! What about the side effects?

[hunter5582 in reply to Kelly2]

My own experience has been that Besremi is much easier to tolerate and has fewer side effects than hydroxyurea. I think it is worth noting that when managing PV, it is managing the erythrocytosis is more important than thrombocytosis. It is also worth noting that when recovering from surgery, reactive thrombocytosis is to be expected.

We are each different regarding how we respond to the medications used to treat MPNs. We also each have different cooccurring conditions that must be considered. It is reasonable to have a concern regarding Besremi in the presence of hypothyroidism; however, just because something can happen does not mean that it will happen. It does not mean that Besremi cannot be considered, but does mean that caution and close monitoring is indicted.

Suggest that you review each of your options with your MPN Specialist. Compare and contract Besremi, Jakafi, and hydroxyurea. Consider efficacy, contraindications/cautions, and adverse effects. Your treatment goals and risk tolerance are the key considerations.

It is worth noting that hydroxyurea does come in doses other than 500mg when you use brand name formulations. Droxia comes in 200, 300, 400 mg. Siklos comes in 100mg. If you prefer to stick with hydroxyurea, you could use a dose between 500 - 1000 mg.

Noting that hydroxyurea can cause renal impairment, the decision involves comparing the relative risks and benefits of each of your choices. None of our options is risk-free. Each of the options has potential benefits. I would certainly consider either Besremi or Jakafi as an alternative hydroxyurea in your situation.

Suggest that a detailed review of all options is the best course of action. This will allow you to feel comfortable that you are making the best decision. You can always try, monitor, and alter course if needed.

Wishing you success moving forward.

[Kelly2 in reply to hunter5582]

Thank you very much! Without reading anything about it or talking to anyone, I knew inside me that there was a connection between the start of Hydroxyurea and the start of the stenosis! These two coincided in time. I will try the besremi and not jakafy as a haematologist told me that it is not a good medicine! I will keep close monitoring with my hypothyroidism and see what happens. Keep having a good time and thank you again. I will keep you posted in September.☺️

[hunter5582 in reply to Kelly2]

I suspect that the renal stenosis is a separate issue from the hydroxyurea. HU can directly impair kidney function. One of the dangers is that the damage caused by renal stenosis could be compounded by the HU. Moreover, anyone with both a renal stenosis and a MPN would be considered high risk for additional complications.

In an odd sense, it is possible that the HU could be the trigger for finding a renal stenosis. The enhanced monitoring of kidney function that is part of the HU monitoring protocol could detect a previously unknown problem.

With a history of renal stenosis, I would not consider a medication with a higher risk of renal impairment like HU. You are well advised to switch to another medication like Besremi or Jakafi. I would have to disagree with the comment that Jakafi "is not a good medicine." Many people do much better on it than HU or other options. Like all of our choices, Jakafi comes with its own risk-benefit profile. I would certainly consider Jakafi as one of the preferred options to treat PV. I would certainly prefer it to HU.

I think you have a good plan in place to try Besremi and see if it suits you. Suggest that the low and slow approach is the safest and preferable approach. I chose to increase in 25mcg increments (not the standard protocol) and paused at each increase to give the Besremi time to do its work. I have maxed out at 175mcg as my highest tolerable dose. Fortunately, this dose is effective for me.

Wishing you all the best.

[Kelly2 in reply to hunter5582]

I am afraid that I gave you a false impression of my kidney problem. I do not have renal stenosis which has to do with the artery! Unfortunately I cannot find the translation for it. I think it is called ureteral stricture. It is stenosis from the kidney to the ureter. Something quite common unfortunately! It has been fixed with robotic laparoscopic surgery so I saved my kidney. One month ago, so I am recuperating.

[PhysAssist in reply to Kelly2]

Glad you saved the kidney- here is more informational ureteral stenosis:

cityofhope.org/clinical-pro...

I doubt HU had anything to do with it other than coincidence, but of course, I could be totally wrong too.

Best

PA

[Kelly2 in reply to PhysAssist]

Hi. I don't know what to think. The place where the stenosis occurred had chronic inflammation!!! I discovered it totally by chance, by having a computer tomography last year for something else that was bothering me !!! But it must have started last year. Now I am scared lest it is formed again! I am expected to check it.

[PhysAssist in reply to Kelly2]

Hi Kelly 2,

So, from the listed causes of stricture, it sounds like yours was from some event that irritated your ureter at that spot,- what that event was may never be clear- infection, irritation from passing an unrecognized kidney stone, or just a passing bit of bad luck.

Here's what I would say: Be happy it was found and has been well-treated, leaving you with a healthy kidney.

Do what you can to keep it that way- drink adequately and follow your MD's treatment plan.

I do agree w/ Hunter that HU may not be the best way to treat your ET given any concern for your kidneys.

Besremi may be a new drug for your endocrinologist, but it isn't really overall.

Maybe seeing an MPN specialist would help?

pvreporter.com/mpn-speciali...

mpnforum.com/list-hem./

It's just a form of a natural immune modifier [interferon] that your body already produces, albeit it has been treated to make it hang around and last longer so that you don't have to have it dosed daily or even weekly.

In fact, some patients get to a point where their disease is managed by dosing once a month if their body responds very well to it.

That being said, after almost 2 years on it, I'm still not 100% controlled at the maximum dosage of 500 mcg SQ every 2 weeks. My WBC and Platelets are well controlled, but my RBC, which were the first cell-line affected by my PV are still just a bit stubborn, with my Hct averaging 49-50,- hardly in the 'Danger Zone', but still tweaking my Heme/Onc MD's "You have to go get phlebotomy trigger..." every other month or so.

Despite being at this high of a dose, I have no side-effects and no worries either- since my pre-treatment Hct was high 50's ranging up to mid-60's and at the time I was diagnosed all three cell line counts were very elevated as well.

Don't worry unnecessarily, live your life, make treatment choices that fit your goals, and be well!

PA

[Kelly2 in reply to PhysAssist]

Very probably a kidney stone as I had terrible pain in my abdomen and back and was hospitalised three times until they discovered the cause of my pain!!! although I showed them last year's computer scanning . Maybe the pig tail let it go. I wasn't drinking much water either and was dehydrated. I drink a lot now. Thank you I needed the reassurance! I don't live in the states but my haemo is a Professor and I trust him. We will see.

[hunter5582 in reply to Kelly2]

Thanks for the clarification. I have heard of that issue before. I do not think it would be related to the HU; however, I would be very reluctant to consider HU as an option after experiencing an issue like that. There are options likely to be safer for your kidneys.

Glad to hear the surgery was successful. Having saved the kidney, the motto of "Be kind to my kidneys" may be indicated. Kidneys are nice things to have working.

All the best.

[Mostew in reply to hunter5582]

Great to see the photos . Looks like all are having fun