This is new, overwhelming. Wife is diagnosed with PV, we need support

Posted by jgjacksonmn1 @jgjacksonmn1, Mar 4 8:42pm

After a long and stable treatment for Polycythemia Vera, my wife (age 73) has been diagnosed with High Risk Myelofibrosis. Just one month ago we were hiking and and adventuring rigorously, and then her abdomen swelled and two weeks ago scans revealed her spleen is more than three times normal size. She had another scan yesterday and we don't yet know the results. She switched three weeks ago to Jakafi (from Hydroxyuria), but only 5mg twice a day becasue her platelets are low. She has tolerated it well and they are increasing to 10mg twice a day - hopefully tomorrow.

We're scared to death. Right now her breathing is limited and it's hard to sleep. Has anyone else gone through this rapid a progression? What hope can you give. What stories can you tell? And how do primary caregivers (me) get the support they need?

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hlmcneely | @hlmcneely | Mar 7 9:28am

Blessings for you and your wife. I was diagnosed in 2021 with MDS and it progressed to MF in 2025. I’m still watch and wait. Stay strong.

jgjacksonmn1 | @jgjacksonmn1 | Mar 7 10:02am

In reply to @loribmt "Welcome to Mayo Connect, @jgjacksonmn1 Well, this news sure clipped you both off at the knees...." + (show)

@loribmt

Welcome to Mayo Connect, @jgjacksonmn1 Well, this news sure clipped you both off at the knees. I’m so sorry to hear about this rapid change in health for your wife. For most people, PV (polycythemia Vera) may remain stable for many years with varying treatments. While in some patients the natural progression of PV can lead to bone marrow damage, resulting in scar tissue formation, which characterizes MF (myelofibrosis).

Having an enlarged spleen is pretty common with myelofibrosis and really, any form of blood cancer. One of the functions of the spleen is to store extra blood cells. When someone has MF, extra blood cells made by the bone marrow can build up in the spleen, causing it to swell. If there is extensive scarring in the bone marrow impeding production, the spleen may also start producing blood cells adding to the build up.

When the spleen is swollen it takes up room in the abdomen where it can push on the diaphragm and surrounding organs. So that may interfere with breathing and eating. I’ve gone through that myself and truly empathize with your wife with her shortness of breath and difficulty in sleeping. For sleeping she might try lying on her right side, her back or propped up with pillows to keep pressure off the spleen which is under the left rib cage. Sometimes a recliner can work wonders for this. Also helpful is to eat very small portions instead of larger meals. I ended up nibbling throughout the day which worked out much better.

I know this is a huge shock and overwhelming right now because the change was so rapid. This may have been progressing for some time and reached a point where symptoms began. Your wife is now taking Jakafi which has a good track record for reducing spleen size and improving symptoms of myelofibrosis. But it does take a little time for the body to process the extra blood cells. So hang in there!

What other tests have been done for your wife to diagnosis this change? Have her lab values changed significantly? A bone marrow biopsy (BMB) can be of significant value for assessing bone marrow involvement such as scarring from the MF and also for staging a disease. Has your wife’s hematologist/oncologist suggested this test?

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@loribmt - @loribmt Thanks for this encouragement! I believe we are under great care provided by a layered team. A bone marrow biopsy following some changes in the PV blood levels and new symptoms of night sweats and weight loss is what led us to Mayo. That led to the Myelofibrosis diagnosis, and CT scans of her organs. Her spleen is over three times normal size and seems to be growing quickly. The spleen pain has gown quickly from a little annoying to debilitating. We're protecting against emergency splenectomy, and hoping the Jakafi does its job in time prevent it. She is now being monitired closely on the heals of newly prescribed Jakafi. Weekly labs are monitoring blood counts of many kinds. We are working hard to manage the pain without narcotics and the notorious constipation they cause.

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