Heidi’s MPN Story – An Increasing Trend in Patient Advocacy

by Heidi Cascarano

Heidi on bike

Enjoying a bike ride!

“Watch and Wait…”

What other cancer, in the 21st century, is so misunderstood that your local doctor will struggle to diagnose you, possibly tell you he has a few other patients with it, then advise you to do nothing but get a therapeutic phlebotomy and take a baby aspirin?  You get online to learn that you (supposedly) have a few years to live, but your doctor tells you that you are fine – people can live decades with Polycythemia Vera, and probably have a pretty normal life.  Well, that was my experience.

I will not forget how much it scared me.  I was diagnosed in 2008, at 40 years old.  It just happened to be the same week I lost my health insurance.  I was living alone, in a state far away from any family, and wasn’t working at the time.  After the initial abnormal blood test, but before further testing, I got online and figured out what I had.  It was a pretty easy guess, because it was very clear-cut.  But the positive JAK2 mutation results made it official, so I was prepared when the hematologist told me I had PV.  He immediately prescribed Hydroxyurea, which I took home and stared at.  I was afraid to touch it because of all the warnings on the bottle.

PV Lies Lurking

As is true with many patients, it was apparent that I had probably had the disease for a few years before my diagnosis.  Looking back at old lab results, my counts had taken an abrupt turn upward in 2003, but the doctor I had seen was not aware enough to look further into it.  I had asked him about my high counts, and he dismissed them as being due to my competitive fitness training.  I happily accepted his dismissal.  I only mention this because even the best doctors are very unaware of MPNs, and they are easily overlooked for years.  I have met many patients with similar experiences.

The information I initially found on the Internet was troubling.  Life expectancy was not very good, and risk of stroke, hemorrhage, etc., was chilling.  Most scary was the chance of progression to acute leukemia, which seemed more likely with the Hydroxyurea.  I took one pill and quit after spending long hours on my deck contemplating my mortality.  After some wallowing, I found a new doctor who was in agreement with not taking the medication, and prescribed phlebotomies and baby aspirin.  I was officially on the “watch and wait” protocol.

Finding Support Online, You’re Not Alone

As I adjusted to my new life, I was very fortunate to find some online support groups, as well as the MPN Research Foundation, and other great sources of information.  I started educating myself and learning about other treatments, such as Pegasys.  I read that it had the possibility of reversing fibrosis in my bone marrow.  Of course, I had no idea if I had fibrosis, since my doctor didn’t perform bone marrow biopsies, and didn’t really encourage them.  He was very nonchalant about the disease.  He told me he had other patients with it.  When I asked about the Pegasys, he said the side effects would be much worse than the disease symptoms.  I chose to trust him on not taking medication at the time—that plus the fact that I still didn’t have insurance quenched that idea.  I was paying cash for all my visits.

I had started working full-time at that point, but I was cleaning houses, and the fatigue was beginning to wear on me.  My counts were increasing and my iron was very deficient from the phlebotomies.  My WBC was as high as 24,000 and my platelet count at 800.  I needed phlebotomies every few months.  Almost everything on my CBC was abnormal.  My spleen had been enlarged from the time of diagnosis, but now reached over 20 cm.  I had terrible itching and worsening fatigue.

A Return to Church brings Peace

Heidi and husband Pino

Heidi and husband Pino

All that sounds terrible looking back, but not all was negative.  The realization that my life would likely be shortened had spurred me to return to church.  I started developing a close relationship with Christ that gave me an unexplainable peace.  My new church family supported and prayed for me.  I likely would not have been able to joyfully persevere without all the prayer I received.  I also met my husband, Pino, and dated him since 2009.  In 2011, we married, and I felt assured that we would make it through all the trials of my MPN together.  It was soon after this that I felt that my disease was progressing.

In late 2012, I realized that I had not needed a phlebotomy in several months.  I was just becoming aware of the bone pain and night sweats, though they were mild.  I asked my local hematologist for a manual CBC (since they had all been automated for a while) and he agreed.  When I got the results, I noticed that I had a moderate number of teardrop-shaped blood cells and some other abnormalities that indicated a bad trend—to me anyway.  I told my doctor I thought I was progressing to MF, and he said there was “no way.”  I listed my symptoms of bone pain and night sweats, and the fact I hadn’t needed a phlebotomy in months, and my hemoglobin was 11.5.  He was not moved.  I guess it didn’t seem likely after only 4 years with PV, and only 44 years old.  Plus, I was very active, because I pushed myself hard.

An MPN Specialist and Treatment Plan

I immediately felt driven to find an expert.  I came across my current doctor at Northwestern.  I felt so reassured going there, and finally having real confidence in someone who really knew MPNs.

He took his time to answer any questions I had, and, unfortunately, agreed with me that I was probably progressing to Myelofibrosis.  A bone marrow biopsy confirmed it.

I was pretty sure even before the results, as it was a “dry tap” (meaning no liquid was drawn out).  I already had moderate fibrosis (2+/3+).  My doctor recommended Pegasys, because he, too, believed it might reverse the fibrosis— it was early in the disease.  He said I was still in the “grey” area between PV and MF, but that my disease was aggressive.

It was scary starting Pegasys after all the things I had heard.  We started very slowly, and worked up to a moderate dosage.  My spleen had shrunk a little initially, then grew, and continued to grow.  We increased the dose hoping that my spleen would go down.  My counts had gone down quite a bit—were pretty normal.  The fatigue wasn’t really worse than before treatment.  The day after the injection I was a little achy and tired.  My night sweats and bone pain were gone.  But the itching was terrible! My spleen grew to it’s largest (23 cm), and my liver was also moderately enlarged, so we both reluctantly agreed, after 10 months, to switch to Jakafi in October 2013.

The relief I had from Jakafi was tremendous!  My energy increased and my itching was gone.  My spleen went down quickly to normal!  But I had a sinking feeling.  I had read that there was a small chance Jakafi could reduce fibrosis, but not as much chance as with Pegasys.  Each drug had helped me in different ways, and I felt that together my symptoms might be eliminated.  I begged my doctor to let me take the two drugs together.  I knew of two people on the “combo,” but didn’t really know much about their story or how they were doing, besides that they were doing well.  Every doctor visit I asked for the combo, and he would say it might be an option, but he was very “reluctant.”  This went on for quite a while.  At the time there was a belief by many that the drugs might cancel each other out because they have opposing mechanisms.  Pegasys stimulates your immune system and Jakafi inhibits it.  I went to Mayo for a second opinion, and I tried to persuade an MPN expert there, and he said he would not be against trying the combo, but I was in good hands at Northwestern.  Don’t misunderstand—I was not at all dissatisfied with my doctor.  I respect him greatly, and know he had my best interest at heart.  He was conservative in his treatment, but also open-minded, so I continued to work on him.  My WBC had never come down near normal with Jakafi, so that was what I used to persuade my doctor.  In July 2014, he agreed to let me add Pegasys back in, at a very low dose.

Pegasys and Jakafi combo brings encouraging results

Pegasys Jakafi comboI have been on the combo now for 16 months.  Since then, they have started the trial in Europe.  I have now heard of others on it, and having positive results.  So this is how it stands at this time:

  • all my counts are normal
  • my spleen is barely enlarged
  • my liver is normal
  • all my blood chemistry is normal (liver and kidney function)
  • I have no other symptoms to speak of, or side effects from the drugs
  • I recently had my second bone marrow biopsy and my fibrosis has subsided to a mild “1”
  • The few side effects I had with Pegasys seemed to have been eradicated with the addition of Jakafi

Sharing Results to help others

My positive results have made me eager to share my story with others, in order to help them avoid some of the pitfalls I have faced.  I never would have imagined sharing such private details about my life, but I have grown in my strength and been blessed to discover the fight that we are all capable of.  I have had the opportunity to participate in roundtable discussions with experts to encourage others patients along the MPN journey.  I have been amazed at the strength of other patients who share their knowledge and hope with others.

I have no idea what the future brings.  I am aware that the Jakafi could stop working, as it does for some.  But I also know that the combo has bought me time.  There are so many great drug trials going on that would be available if I needed a new treatment.  And I know I feel very good—better than I have in years.

Walking the Fine Line

I have learned to walk the fine line between acceptance (which brings peace) and fighting for myself, like nobody else can do.

You must educate yourself on the disease process (because too many doctors know less about it than we do!) and also on the advances in research and treatment. You must also see an expert—at least once—and, hopefully, to plan a treatment and consult with your local doctor.  I now go to an expert as my regular doctor.  I only have to go every four months, and he is great about emailing and monitoring my test results from a distance.

Most importantly, you must be your own advocate, and fight for what you believe in, if you have educated yourself. Don’t give up and just go along in order not to rock the boat.  This is your life, and your doctor will never have to personally live with the decisions you make together.  You have to remember that the doctor may be an expert (and I respect my doctor greatly) but he works for you.  Be your own best advocate.  I refuse to watch and wait!


Heidi’s MPN Story: How She Advocated for Better Care

 


About David Wallace

Founder of PV Reporter, a resource for Myeloproliferative Neoplasm (MPN) patients and caregivers. After being diagnosed with Polycythemia Vera (PV) in 2009, I utilized social media to connect with “informed patients” and develop a better understanding of emerging treatment options. My philosophy on patient care is straight forward – “educating the patient is essential, so the patient can guide their physician to meet his or her needs.” PV Reporter is a comprehensive resource hub giving visitors vital tools to become “empowered patients.”

Comments

  1. Marilyn Brown says:

    The article was very good, but I wondered, Heidi, how do you find it possible to pay for these drugs, even with insurance?

  2. Glynis homer says:

    So informative I’m finally starting to understand thank you so very much for sharing your journey. It is my hub and that has pv jak 2 possative.

  3. Heidi Cascarano says:

    Marilyn,

    I use Chronic Disease Fund for helping to get the Jakafi. My co-pay is only $30. That goes toward the out-of-pocket max. When that is met, I can get the Pegasys. It’s a matter of timing it right. I did have to pay more when I first went on Pegasys. There are other programs to help patients with medication also. It is also important to have a great doctor who is used to handling the denials and sending appeals to the insurance companies. Pegasys is almost always denied the first time.

    • Marilyn Brown says:

      Thanks so much, Heidi, for a very informative response. I had no idea these drugs would be possible for me to afford. I will definitely bring this up to my Dr., and see if he can go to bat and get approval for these. The combination results for you sound just totally amazing! I am so glad for you!

  4. Nick Vojnovic says:

    Go Heidi! Thanks for sharing your story. I am just beginning my journey and you give me hope.

    • Heidi Cascarano says:

      Thanks Nick! I’m glad you liked the article, and even more happy if it helps!

  5. Thank you so much, Heidi for sharing your story! I was diagnosed with PV a year ago and have been on an emotional roller coaster ever since. Your story gives me so much hope!

    I am researching the use of interferon and have read studies that have shown that interferon is more effective if patients only have 1 mutation (the JAK2 mutation). Do you know if you additional mutations beyond the Jak2 mutation? I ask b/c I have learned that I have several mutations and wonder how effective it would be for someone with more than one mutation.

    • Heidi Cascarano says:

      Hi Faye,
      I wish I knew if I had any additional mutations, but I haven’t had any genetic testing since I was diagnosed. Of course, the other mutations were not discovered until later. Additionally, I did not ever have my allele burden checked, which I would love to do, so I would know what has happened with my treatment. I am just worried insurance might not pay for it, and it’s expensive.

      I wish you the best on your journey. Keep doing your research!

      Heidi

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