BMB- 16 year old daughter JAK2+ ET

[Piperpeeps]

Hello,

My 16 year old daughter was diagnosed with JAK2 + ET in 2020 and subsequently had a BMB in November 2020, confirming this. I have posed this question to you all before and received great feedback. Thank you so much!!

However, the new pediatric hematologist and the pediatric MPN specialist are suggesting another BMB over the summer. My daughter’s platelet count is stable in the 800’s range and taking 81mg aspirin daily. However, the platelet count rose to 1,250,000 when she was sick and lowered to 800’s after recovering. I really don’t see the need and I would prefer not to proceed with another BMB so soon, if it’s not necessary. Does anyone have any feedback or recommendations regarding whether or not we should commence with another BMB this summer?

Any feedback is greatly appreciated!

Thank you,

Angela

[cmc_ufl]

My input is that if the temporary rise in platelets (which subsided after her illness resolved) is the sole reason behind their request to do a BMB, then it is likely not necessary. I would ask the MPN specialist for all factors behind the exact reasoning for wanting the BMB before proceeding.

[Piperpeeps in reply to cmc_ufl]

I did have that discussion with the MPN specialist and she said it is because the generally recommend having a BMB every 2-3 years. I’m not sure if this is because she is so young? It just seems excessive to me. Otherwise, she is feeling great. Her headaches are not as frequent.

[cmc_ufl in reply to Piperpeeps]

I have never heard of this. Perhaps it is something they do specifically for peds MPN patients, but that seems excessive to me.

[Piperpeeps in reply to cmc_ufl]

Thank you so much for the feedback!

[hunter5582]

I have never heard of a BMB every 2-3 years for any MPN patient. If this is just routine screening then I would consider deferring. In the absence of a change in disease status, I would question the value of doing another BMB. There is variance in the MPN world regarding how best to monitor disease status. You may want to get a second opinion.

I would also note that at age 16 your daughter has the right to refuse to consent to a medical procedure. She is old enough to have autonomy in regards to what is done to her own body. She does, however, need your consent to move forward with treatment, I am sure she relies on your guidance in making these decisions. With your help I am sure she can participate in making a good decision.

There can be significant discomfort involved in a BMB. If you/she decide to move forward with the BMB then I would suggest considering more aggressive pain management than is often done. There are a range of options depending on which facility you use.

All the best to both of you

[Piperpeeps in reply to hunter5582]

Thank you so much for the feedback and information. I’m hoping to avoid that procedure again for her. All other bloodwork is normal with the exception of RA value, which is 21. We are scheduled to meet with the RA doctor in March.

[charl17]

What do they expect to learn and how would it affect her treatment? I don't need to know this but you need to be satisfied with their answers. I had one BMB 8 years ago at the initial diagnosis. For the last 6 years I have been managed by an mpn specialist at Moffitt Cancer Center in Tampa. He has never mentioned a second BMB.

[Piperpeeps in reply to charl17]

Thank you for the reply. I will definitely question it a bit more.

[Cja1956]

What about her other numbers? Are they pretty normal? If only her platelets are high, then a second BMB at this point does seem excessive. How often is she monitored for bloodwork? I had my first BMB at age 52 in 2008 and didn’t have a second one until 2019 because I suspected I had progressed from Et to MF. My red blood cell counts had dropped and I developed anemia. If you’re daughter is feeling good and all her other numbers check out, you might want to hold off on a second BMB for now and get a second opinion from another mpn specialist. Good luck to both of you. I know how we moms worry about our children.

[Piperpeeps in reply to Cja1956]

Thank you for the feedback. Yes, all of her out counts are normal with the exception of RA factor, which is 21. And very strange for a 16 year older. We are consulting with RA doctor in March.