I served in the Army during a more recent war than many of the veterans who have been diagnosed with an MPN.  I was diagnosed with Essential Thrombocythemia (ET) in early 2013. My son was only a year old and I had spent two weeks in a VA hospital while hematologists tried to rule out other illnesses. I was put on Hydroxyurea (Hydrea) almost immediately due to the aggressive rise of my platelet count. It was eventually controlled and I was sent off with a permanent Hydrea prescription.

My background in health care and my university education prompted me to research this illness and I learned a lot.  I read about alternative drugs, but the drug that stood out to me the most was peginterferon alfa-2a (Pegasys). It’s what was advised for young females of childbearing age, as it isn’t harmful to a fetus. I also learned that Pegasys is the only known drug that gives a glimmer of hope for remission. That’s when I decided I wanted to be on Pegasys. Hydrea was giving me terrible side effects.

I joined several MPN support groups on Facebook. This is probably the best thing I ever did for my condition. The wealth of information in those groups is astounding. I met many people who were very proactive in finding the correct treatments that alleviated some of their symptoms.

I was fortunate to have the wherewithal to find this out on my own.  I’m sure there are many veterans who do not or cannot advocate for themselves and are probably too tired, too sick or too confident in the VA to research this for themselves. Or maybe many of them had but had been denied, like I was.

At my visit with my VA oncologist, I mentioned Pegasys and my desire to be on it. I was told that Hydrea is the standard drug for ET and that all other patients are on it. I was adamant to get on Pegasys and was told that only Melanoma patients received it. Luckily, I had a receptive and understanding oncologist who said he’d research it first before giving it to me. After doing his own research, he agreed. I think I was the first ET-VA patient in Central Texas to be put on Pegasys. To this day, I believe I’m still the only one.

I was very lucky.  I’m sure this does not happen often with other VA physicians.  My other mission was to be seen by an MPN expert.  Making all these discoveries on my own really showed me how rare MPNs are and how unaware many VA hematologist/oncologists are about the best current available treatments.  This is my life…but not just mine, my son’s too.  I owe it to him to improve my health and extend my life.

My hematologist/oncologist agreed to submit a request to get seen at MD Anderson in Houston. I was placed into the Veteran’s Choice program and repeatedly given appointments with local civilian physician.  After letting the program know time and time again, that I wanted to go to MD Anderson, my oncologist told me it had been rejected because the VA doesn’t approve “second opinions.”

I was disappointed but grateful that, at least, I was on Pegasys. About a year later, I decided to prepare to have another baby. My VA gynecologist advised me to let my oncologist know of this. At my appointment, I told my oncologist my plans and he said he’d put in another request for the VA to cover a visit to see an MPN expert. I didn’t get my hopes up.

Sometime after, I got a call saying I was approved for two visits at MD Anderson. I saw an MPN expert who basically told me I was on the right medication (Pegasys) but that I was taking it too often because my white blood cell count was low and I was very fatigued (among other disturbing symptoms). He decreased the frequency and I felt much better with my white blood cell count being within normal range for the first time in three years. MD Anderson also tested me for mutations that the VA couldn’t perform. I had many questions answered and was finally at peace with how my condition was being handled. I was ready to move forward confidently, I felt closure.

None of this would have happened if I had been a man. The VA only approved my MD Anderson visit after learning about my intention to get pregnant. As a female, I’m a minority at the VA. There are many male vets who aren’t benefiting from a visit with an MPN expert.

If I hadn’t done my own research on ET, I would still be on Hydrea today. The side effects were very trying. Pegasys wasn’t even on their radar. Even if the VA approves prescriptions for Pegasys, Anagrelide, Jakafi or something else, the patients could be suffering from dosage/frequency problems, like I was. An MPN expert can help sort this out, and so much more.

I encourage MPN patients and their oncologists to do their own research. Veterans have the right to know about alternate treatments. Veterans also need to see knowledgeable experts and have access to blood tests that are relevant to their condition.

My daughter has always been a very active, happy child. She is very creative, loves animals and art. She has always participated in sports. Currently, she runs cross country and plays field hockey and loves her ceramics and art classes. If you look at her, it is impossible to see a sick child, you’d never think she’s different or that there is something wrong with her health.

When she was 8 years old our family moved to Kentucky. Her new pediatrician requested a blood test, which was something she said she did for all of her new patients. My daughter had never had blood drawn as part of a normal check in any of the states that we had previously lived. At first it felt very invasive, but we agreed. To our surprise, it was discovered that her blood platelet count was at almost 2 million. The pediatrician of course requested a second test for validation and again the results were the same.

As parents we were shocked because my daughter had never had headaches, bad bruising without reason or ever felt fatigued. Every question we were asked regarding any symptoms she may have exhibited, our answer was no. She never presented any symptoms. After the first visit to the hematologist, we were told my daughter needed to get a bone marrow biopsy. At this point our world was upside down.  Why a bone marrow biopsy? What are the doctors talking about?  We had little to no answers. This is when we decided to start looking for help, after all she is our daughter and we needed guidance. At our church one of our deacons is on the medical board of a university, so we reached out to him for guidance and a better explanation. He was able to ease our worries by explaining the reasons for the bone marrow biopsy was to give us a better understanding of what my daughter had.

Once the bone marrow biopsy was done, we got the diagnosis that it was a JAK2 mutation causing Essential Trombocythemia, again we didn’t really understand what we were facing, this was a rare illness for adults and even rarer in children. They wanted to start a treatment that was usually given for another illness, but that it might help keep the blood platelets under control. Our whole life changed, we had a diagnosis but no real answers about this condition. She was the first patient in our area to be diagnosed with ET and there was little to no information about it, our child didn’t look sick or feel sick, however she needed to take hydroxyurea, a type of chemotherapy.

I can tell you that I was very upset and sad, I’m supposed to protect my child, but how? Having no answers from doctors made me feel even worse. For my own peace of mind, I decided to do my own research, I spent hours on the computer and talking to our deacon trying to figure something out. Then I came across the MPN organization and MPN Advocacy and I subscribed to get their emails and magazines, I soon realized that there was very little to no information about this condition in pediatrics.

Meanwhile my daughter started the treatment and she would ask me why, I don’t feel sick, why do I have to take this? Because of her age it was difficult to try and explain everything in a way she could understand, but we did it the best way we could. We told her we loved her and we would never let her down and everything was going to be OK.

My daughter went from doing regular, normal 8 year old things to having to visit the hematologist every other week, going through all kinds of testing and taking this chemo medicine. Her platelets went down, but after a couple of months she started vomiting and having headaches, my daughter had never had headaches or presented anything like this. There were constant visits to the ER or the pediatrician every time she was feeling sick, there were constant tests and blood draws, but nobody knew exactly what was wrong. At one point her blood platelet count went down below normal and that’s when the doctor adjusted the medicine to a lower dosage and she started feeling better, the headaches still continued, just a little more infrequently but now she presented another symptom, she felt tingling in her hands and feet.

We tried to keep her life as normal as we could but the constant headaches made it impossible, it affected her school work and attendance, I would have to pick her up every time she was sick. It affected her activities so she didn’t want to take the medicine any more. We visited all kinds of specialists, a neurologist, an ophthalmologist, just to make sure there wasn’t something we were missing but every single test came back fine. Our frustration grew and so did our need for help.

My daughter didn’t want to go to the hematologist any more, she was trying so hard to be happy, yet we can see that she was sad. She had never spent so much time in bed due to headaches, she didn’t want to take the medicine that made her feel sick anymore, she would ask, why me?

At age 9 she needed to get another bone marrow biopsy, it was very scary for her, I still remember when they put her to sleep, she looked so little and so fragile. You’re supposed to protect your children, but what can I do against this?

I continued to research ET and found that MPN Advocacy was having a seminar in Boston. My husband and I decided we needed to attend it to see if we could get some answers. I contacted Ann Brazeau and told her about our daughter and that we needed help finding more answers about ET. We wanted to learn about this condition but also wanted to try and seek out help from somebody that knew more about this. Ann not only had answers for us but she put us in contact with Dr. Nicole Kucine. We attended the seminar and it was the best decision we could have made, we learned so much about the MPN conditions and the medicines used to treat it and we also got the opportunity to meet Dr. Nicole Kucine, a leading ET specialist who specializes in pediatrics. We later took our daughter to see Dr. Kucine and she was able to answer many of our questions and give us some different options and ideas that put us in a more confident state of mind.

My daughter now feels more confident when it comes to talking about her condition, she understands that we can’t keep it a secret. In school, teachers need to know that even though she looks fine and can function like any other 9 year old, she has ET and some days are going be bad for her. She can’t understand everything at her age, but she has a strong spirit and just keeps going.

The biggest difference between the first day of this journey and now is the understanding and knowledge we gained by attending the MPN Advocacy and Education’s MPN program in Boston. We learned so much about our daughter’s condition and we gained new tools to support and help our daughter. We understand there is still so much research to do and knowledge to gain about ET in children. We know that we may never get all the questions answered for my daughter, but we take it one day at a time. She continues to attend school, she runs cross country, plays field hockey and takes ceramics classes. She continues to do well in school and for better or worse when she becomes sick she has been able to adapt and can control it better and recovers quickly. But we are looking forward to the day that we can lower her medicine to a level where she can live with no side effects. We let her know that we love her and we would do everything in our power to help her. We are thankful to God, the support of friends and family, people like Ann Brazeau, the MPN Advocacy and doctors like Dr. Nicole Kucine who dedicate their time and lives to teaching and informing parents like us.

By Silvia

How has your loved one’s MPN diagnosis impacted your life?

My husband was diagnosed 15 years ago while we were in our mid 30’s and the only information that was obtainable at that time was grim.  Needless to say, there were tears in the beginning…questions like, will he walk our then 6 year old down the aisle when she gets married?  Will I be a widow before my 50’s? However, as the information and medical treatments improved, so did our attitude towards the MPN’s.  In a way, our lives are so much more rich due to him having this disease.  We celebrate the little things more and don’t sweat the small stuff.  Work isn’t the driving force behind our partnership, it’s quality family time.  We now have two kids in college but remain incredibly close to them at all times.   We founded a support group and truly care about each of the 100+ members in this group, some of the members are as close as family members to us.

Do you feel like you had the knowledge and resources you needed to navigate the various challenges of dealing with this diagnosis.  

15 years ago, there wasn’t all that much information in terms of support around MPN’s.  But now, thanks to the MPN Research Foundation and MPN Advocacy and Education International, we feel like the information and support are just a click away.  Our support group meetings have been invaluable to me in terms of learning more about the disease and talking with other caregivers on how to continue to support our loved one.

What are your biggest challenges in trying to help your loved one?  

I remember back in 2005, we were at a Mayo MPN Conference in Arizona, one of the topics that was discussed was Hospice.  After listening for 20 minutes, I excused myself and went to the ladies room where I broke down and cried. A very wise, elder lady gave me a big hug and said, “Wrap it up.  You can’t show you’re weak to your partner, they need you to be strong for them.  You are their biggest advocate and you are so needed to help them win this fight, now…dry those tears and walk out with a smile on your face”.  I remember it so well.   I thought about what my husband was hearing during this talk and how frightening it must have been for him.  It shouldn’t have been his job to console me.  I needed to be there for him to console him, which is just what I did, thanks to my new friend.  Four years later, my husband had complications with his spleen and he spent 3+ weeks in the hospital.  It was hard juggling young children and being present for him at the hospital.  I knew the best decision was to have the help of friends, family and neighbors take over my role with the kids so I could be the best advocate for my husband.  I needed to be there, at the hospital, helping him navigate the complicated hospital maze.  There were discussions with the many medical team members who were rotating constantly.  My husband was highly medicated and found it hard to tell his story correctly.  There was NO better person than me to make sure his story was being told correctly so he got the proper care.  My kids got hugs and kisses from me at the end of the night, but it was my husband’s life at stake and I’m glad I made the choice to always be strong and present for him.

What thoughts or advice do you have for those just beginning a journey with a partner with an MPN?  

The journey that begins with a diagnosis of an MPN is an unexpected and unpredictable one.  But the only constant is the support that you can provide your loved one.  Being there with them at doctor’s appointments, medical conferences and support group meetings as well as occasionally checking in with them to see how they are doing are among the most valuable contributions you can provide.  Also, you may notice certain side effects of medications that the patient is unaware of such as mood swings, lack of engagement or loss of emotion, and can inform the doctor. It is the job of the advocate to pay attention to these changes and make sure steps are taken to resolve them. If you are a significant other, I believe it is best that you go on this journey together by making mutual decisions on things such as new medications and tests. My view is that every decision has potential positive or negative outcomes and you both have to live with them…so do it together.

In 2011, my husband filed a claim with the VA in hopes of having his myelofibrosis recognized as service related disease.  He believed his exposure to Agent Orange while in Vietnam caused his MPN. He died in May 2014. His claim had been denied twice. After he died, my son and I went to a hearing in June 2015 to once again have his case reviewed.  On January 13, 2017, I received notice from the VA stating my husband’s illness and death were related to his service in Vietnam and his benefits were approved. This was good news and a financial benefit for me. However, the VA has denied many more cases because MPNs are not included on their ‘presumptive’ list of service-related illnesses.

There were several things we did which I believe helped our case. For example:

• We researched the cases where benefits have been approved.  In each of the approved cases I found there were letters from the veteran’s doctor in support of the connection between the illness and their service. Keep it simple.  It does not need to be too technical.

• We were sure to include a nexus:  a statement of their job in the service and their exposure. Our letter of approval from the VA mentioned both our doctor’s letters and the nexus.

• We created an executive summary of our case, and kept it to one page and provided all back up information with it.

We hope our victory is useful to other veterans in their effort to receive benefits for their MPN.

MPN Advocacy & Education International is creating a package
for Veterans with MPNs to assist in their efforts when filing a claim.  
We will keep you posted as it is finalized.  

Click here to learn more about service-related illnesses and Agent Orange.