| Doctors differ in their approaches to many things, time travel, Daleks ... and ET. |
This is a long post, so bear with me. At the end of are three simple questions to your hematologist to see if you and your doc are on the same page about your treatment goals.
Until recently ... Doctors treating ET patients were mostly worried about clots--deep vein thrombosis, strokes, clots in the heart and lungs, rare conditions like Budd-Chiari (clots clogging the veins of the liver), and small clots in the hands and feet causing ischemia (loss of blood flow). These are all things doctors remain vigilant about. Aspirin and hydroxyurea (Hydrea) and anagrelide (Agrylin) have worked well to address these problems and have been the treatment of choice for decades.
With the discovery of the CALR gene ... Doctors quickly learned that ET-CALR acts somewhat differently from ET-JAK2; ET-CALR patients have less tendency to develop clots even with very high platelet counts. As a result, hematologists treating ET-CALR may may let platelet levels ride up to a million before starting chemo if a patient has a relatively low clot risk. Figure your risk here. After that, acquired von Willebrand (AVW) disease is a danger. It can cause bleeding episodes. So keeping platelets below the AVW danger levels has been important. Again, hydroxyurea and aspirin have been the first line therapy against AVW.
JAK inhibitors ... For both JAK2 and CALR patients, the development of JAK inhibitors such as ruxolitinib (Jakafi), has offered exciting new possibilities in that it blocks the effect of both genes. These drugs also come with an exciting price tag--$10K per month for Jakafi--and insurance companies may be loath to pay for the drug because it is not officially approved for ET. JAK inhibitors do not cause remission, but some patients get remission-like benefits: lower platelets, reduced spleen size, more normal LDH levels, and fewer symptoms. Many patients also report significant weight gain, which may be a problem for those with diabetes or high blood pressure. Research continues with other JAK inhibitors.
Enter interferon ... If you are an older patient like me, you remember the 1970s and 1980s, when interferon was the Big New Thing in cancer research. It had some pretty bad side-effects, but it was hoped it would cause long-term remissions and cures in a variety of advanced cancers. It did not turn out to be the wonder drug everyone hoped, but researchers have kept tinkering with it, looking at different drug strains and dosage levels.
Pegylated interferon (Pegasys) is being used to good effect in some ET patients, and the word "remission" is being used; about 15-20 percent of patients taking Peg show no sign of mutation after a time. But how long these "remissions" last remains to be seen. I posted about this awhile ago.
The controversies: Some hematologists are still mostly preoccupied with platelet levels regardless of your type of ET. They are using aspirin and Hydrea or as the mainstays to bring these levels down and reduce clot risks. Their JAK2 and CALR patients are treated pretty much the same.
Other hematologists are discussing interferon more with their patients, particularly those under 60. Research suggests that those over 60 tolerate the side effects of interferon less well, so some doctors are cautious about using it for older patients. Hematologists may urge female patients in child-bearing years to try interferon because it is safe to take in pregnancy. (ET increases miscarriage risks.)
Some hematologists--noticing that a large number of ET-CALR patients (those with CALR type 1) usually progress to myelofibrosis (MF), the most serious type of MPN--see ET-CALR, type 1, as a separate disease altogether. They prefer to think of this group of patients as preMF. These doctors are often more concerned with the level of fibrosis in CALR patients' bone marrow (found through a bone marrow biopsy and sometimes indicated by an elevated LDH) than with platelet counts, though they are still monitoring platelet counts to guard against AVW.
Docs concerned with fibrosis in ET-CALR patients may want to start interferon as soon as any fibrosis is detected. They believe that this gives patients the best shot at preventing or delaying progression to MF. They also believe early treatment gives patients the best chance at remission.
Before starting interferon, some cautious doctors do psychiatric screening. Interferons have been associated with mood swings, increased depression, and anxiety. In addition, there are a host of other side effects that some patients cannot tolerate. For those who are not good candidates, the fallback drug is good old hydroxyurea or ruxolitinib in some cases.
The secondary cancer concern... Some doctors, despite the lack of definitive research, believe that hydroxyurea causes secondary cancers when taken long-term. As a precaution, they don't want to start hydroxyurea sooner than necessary. Other doctors who have concerns about hydroxyurea also prescribe interferon or ruxolitinib whenever possible. The patients of these doctors can become rather vehement in their rejection of hydroxyurea and scare the bejeezus out of other patients, telling them that they will get leukemia, without realizing that hydroxyurea is still the best med for many people. Most older patients will not be on hydroxyurea long enough to develop a secondary cancer in any case.
In addition, patients may tell those who take older chemos that they will not get to go into remission if they don't take interferon. While it is true that hydroxyurea and anagrelide are not associated with remission, it is also important to remember that interferon "remission" happens for fewer than one in four ET patients.
What to do?? I am not promoting or knocking any particular chemo. It's excellent news for all of us that there are more and more drugs available to address various types of ET. Here are three questions I will ask at my next hemo appointment to help me figure out whether my doctor and I are on the same page and what will be the best chemo for me when the time comes:
1. Am I at greater risk for progression to MF?
2. If so, how will you track that?
3. What medications would you recommend for me if fibrosis is detected? Why?
If your doc mentions interferon or Pegasys, it is very important to tell him or her about any history of depression, anxiety, or other mental illness. If your doctor seems puzzled by these questions, or if the answers you get don't tally with your own treatment goals, you may want to seek a second opinion through your primary doctor.
Be well!
Related: Interferon, another ET chemo
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ET is a serious disease that requires specialist care. Discuss anything you read here with your doctor. No comments promoting "alternative" or "natural" cures (yes, this includes Rick Simpson's Oil) will be published.