Tag Archives: blood cancer

A Mother’s Story: Children DO Get MPNs-Our Loss May Save Your Child

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In memory of Jordan

In 2014, our daughter Jordan, age 14, complained of headaches and neck pain. Ultimately the doctors diagnosed her with mastoiditis, with a rare complication of cerebral venous sinus thrombosis (a 5% chance of that complication). The doctors then fixated on a diagnosis of migraines before, during and after shunt evaluations, placement and revisions.

In January 2015, unbeknownst to us, one doctor mentioned polycythemia vera as a possible diagnosis. He requested a hematology consult, but no action was taken. Due to the CVST, Jordan developed papilledema and required shunting due to intracranial hypertension. She was at risk of losing her vision.

Jordan suffered from headaches, nausea, vomiting, blurry vision, joint and bone aches and pains, and itching which was diagnosed as an allergic reaction to medication she had taken. Nobody looked at the bigger picture. One doctor said her issues were the result of complications due to migraines. After which,  every other doctor followed suit despite the symptoms, abnormal imaging, and abnormal lab results. All of this happened over a three year period.

In February 2018, Jordan, now 17, was diagnosed with portal vein thrombosis. Her symptoms remained unchanged for three years, yet the doctors still fixated on migraines. At this point, I started doing my own online research and requested testing for polycythemia vera (PV). We were told by a hematologist that tests including a bone marrow biopsy, were not necessary as her labs were normal. They were not.  She was reluctantly tested for the JAK2 mutation on February 21, 2018. Nobody read the results.

On March 4, 2018, we were told Jordan could possibly have an MPN and a bone marrow biopsy was needed. The biopsy was performed on March 5, 2018. Two days later, our Jordan passed away on March 7, 2018.

The medical community failed our daughter. We were told on March 4 they didn’t test her earlier because “this isn’t seen in children.” We told them this was no excuse not to test, diagnose and treat. Their expectations resulted in our daughter’s death. Instead of trying to find out why our daughter was sick, and doing further investigation into her MPN symptoms, they labeled her with a default diagnosis that did not fit.

Afterward, we were not told of her official diagnosis. No doctor called us. Patient Relations refused to speak to us and suggested we request our daughter’s records and have our primary care doctor review and advise. We found out six weeks after she passed away she suffered from primary myelofibrosis.

If Jordan were tested for JAK2 and or had a bone marrow biopsy in January 2015, when one doctor requested hematology do an exam because of suspicion of PV she would still be here. She could have had treatment. She could have had a stem cell transplant. Due to lack of awareness, ignorance, ego, expectation or a combination of these, she was left to suffer. We lost our beautiful daughter because they didn’t want to look beyond migraines. The sad part is that she was at a well-known level 3 tertiary care children’s hospital in southern CA.

We have now become advocates for awareness. We want doctors who see children not to be swayed by “expectation” as MPNs can affect anyone regardless of age, gender, or ethnicity. We want more research. The medical community needs to be educated. We advocate for funding. We advocate for Jordan. We are sharing her story on Facebook in the hopes of raising awareness, www.facebook.com/jordansstory .

MPN Advocacy & Education International’s Pediatric and Young Adult MPN initiative advocates for young patients. As part of that effort, we are in the process of creating a private, online, space for parents to communicate with each other and seek input from our Pediatric MPN advisors, Dr. Nicole Kucine, Weill Cornell and Dr. Linda Smith-Resar, Johns Hopkins Medicine. Click here to learn more about our Pediatric and Young Adult advocacy efforts 

 Click here to download MPN Advocacy & Education International’s Pediatric & Young Adult Booklet

Join Us for the 2ndAnnual Pediatric & Young Adult MPN Program on May 16 in New York City

 

 

Watch Behind the Mystery: Living with Polycythemia Vera

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The Lifetime Channel’s The Balancing Act featured a story this week on polycythemia vera (PV), with experts Dr. Richard T. Silver, a professor of medicine at NewYork–Presbyterian/Weill Cornell Medical Center, and Dr. Srdan Verstovsek of the MD Anderson Cancer Center, who discuss the latest inpatient care and clinical trials for PV, as well as the future for those living with PV as a chronic illness. Learn More

Learn more about MPN Clinical Trials

A Patient’s Story: How I Diagnosed Myself

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My journey with essential thrombocytosis (ET) began in May 2016.  Although I am sure I had it for at least four years prior to to that.  I self diagnosed myself after noticing my gums were bleeding when I brushed and flossed.  I am a dentist, so how could this be?  I have immaculate oral hygiene, floss and brush at least two times a day and get my teeth cleaned every three months.  A little voice from one of my lectures in dental school went off in my head;  I recalled my professor’s words, “in the absence of gum disease or dental issues, bleeding gums can indicate a blood cancer and you should refer your patient to their doctor immediately.”  I didn’t think I would be the one to need the referral.  After some research on Dr. Google I put the puzzle pieces together.  I had tingling fingers and toes for at least a few years and had actually gone to a neurologist who tested me for carpal tunnel syndrome.  Again, being a dentist, that is not unusual to get carpal tunnel. However, after that diagnosis was negative, I just brushed it off.  I had also had major hives a couple of years ago all over my legs,  I had gone to an immunologist and she said it was allergies and put me on allergy shots.  After they didn’t go away, I had gone to a primary who told me my symptoms were stress related, “psychogenic” as he called it and I needed to manage my stress.
Six months before my diagnosis, I had a case of vertigo.  Again, I went to my doctor who reassured me it was a viral infection and it would go away in a few days.  I never had vertigo again so I believed him.  No blood work or additional testing was done.
However, the bleeding gums and my professor’s voice was what made me suspicious.  I started researching blood cancers and put all my symptoms together for what pointed toward ET.  I took charge of my own health and went to a hematologist and asked him to test me for JAK2 mutation.  Sure enough that came back positive and my bone marrow biopsy confirmed it.  Platelets were in 900 range and I just didn’t feel like myself.  He insisted I start on Anagralide that day and that I was going to stroke any minute.
I wasn’t comfortable with his rush to treatment as I had read a lot on ET prior to my diagnosis, so I knew that Anagralide was definitely not first line of treatment and neither is Hydrea if you are at low risk like me.  I am otherwise very healthy, work out religiously, grow my own vegetables and juice fruits and vegetables at least 5 times a week. I am not a smoker and I don’t  drink. I have a normal BMI and have no other health conditions.  I ran out of that office and went to UCSD in search of a doctor that actually listens.
My new doctor suggested that I start on a low dose Hydrea immediately and I declined.  I had research and information on my side-I was under 60, platelets under one million and otherwise very healthy with no other cardiovascular issues or dispositions.  So I declined once more and she agreed to monitor me.  After 18 months, I had a lot of headaches and could hardly feel my feet and my hands felt horrible, almost numb.  Again, I am a dentist, so this scared me and I agreed to do treatment but wanted to try Pegasys instead of Hydrea.  My doctor was very reluctant about Pegasys because she believed the side effects are not worth the benefits.  I produced a lot of studies and literature on how it works better for some people and in my mind I would rather be on Immunotherapy rather than Chemotherapy.  I did take Hydrea for one month while awaiting insurance authorization for Pegasys and I knew immediately that I was right in my intuition. It wasn’t the right treatment for me. I was nauseous all the time, couldn’t sleep, had major brain fog, red dots on my chest and legs, my nail beds even hurt.
The answer to my symptoms by a different doctor at UCSD was to take more drugs.  One to help me sleep, and an anti-nausea medication.  I am very much against taking drugs if I don’t have to so this didn’t sit well with me.  Not to mention, they wanted to increase my dose every week since my numbers weren’t coming down the way my doctor wanted them to.  For the first time since my diagnosis, I broke into tears because I knew I wouldn’t have a quality of life if I increased my dose of 2000 mg a day.  I have a demanding job and need to have a clear brain!  When my insurance authorized the Pegasys, my doctor agreed to let me try it and I have been doing great on it at 90 MCG per week and in one month my numbers are down to 920.
I am not suggesting Interferon is for everyone because you all know we are all different and respond differently to different medications.  In some studies that compare Pegasys to Hydrea the dosing of Pegasys was so high and toxic that patients dropped out, I would too.  But at a low steady dose, it is working for me. Someday, I may have to go back to Hydrea. No one knows how our bodies respond to certain drugs long-term but for me personally, I wanted Pegasys as my first line of treatment.  I am 50 years old and 20-30 years of Hydrea ahead of me was not going to be my first choice.
I guess the lesson I have learned and continue to learn is to be your own advocate, research and study your disease.  Doctors are busy people with perhaps thousands of patients.  I only have one disease and one patient; MYSELF.  I will continue to fight for what is right for my body and luckily I have a great doctor that listens to my wants and needs. If I didn’t, I wouldn’t hesitate to switch till I found the right doctor.
 

A Veteran’s Story: The Frustrations of Filing a Claim with the VA

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By Wayne E.

MPN Patient and Vietnam Veteran Wayne E.

I served in the USAF Security Service, 6924th Security Squadron, stationed in Da Nang, Vietnam for one year (1970-1971) and was exposed to the deadly Agent Orange/Dioxin. In 2007, after a simple pre-op blood test, I was diagnosed with essential thrombocythemia (ET). Upon further study I was told I had an incurable, but manageable, blood cancer, coupled with a gene mutation (JAK2). The word cancer scared me. I had never heard of ET and I was at a loss for what to do. I didn’t know where to go next. After much reading about these potentially deadly diseases, I found out I was one of many Vietnam Veterans who had an MPN.

In 2011, I filed my first claim with the VA. Until this filing, I was unable to get any substantial information from my primary care physician (PHP) or my hematologist/oncologist, as to what may have caused or contributed to my ET. They knew virtually nothing about Agent Orange. I contacted the National Institutes of Health, The Centers for Disease Control, and as many online medical sites as possible, all ending with a bigger question mark. Nothing could be explained to satisfy my inquiry.

It was by chance that I connected with a most remarkable group, MPN Advocacy and Education International. I could never thank them enough for the compassion and the understanding they extended to me.

After my initial rejection from the VA, I filed three more times and each time I was denied because MPNs are not on the “presumptive” list of Agent Orange-related illnesses. The same message I kept getting was I needed “clinical rationale” to support my claims. My doctors have not been able to provide me with this needed information. I don’t know what to do today. I understand there are many Vietnam vets that have won their appeals and now get benefits, but there are many others who were not approved and just gave up. I don’t plan to give up.

To my fellow Vietnam Veterans who may be dealing with one of these MPNs, don’t give up. If you have been denied, file an appeal. There is hope, comfort, and assistance available. With the help of MPN Advocacy and Education International.

 Learn more about filing a claim with the VA

 Learn more about Veterans and MPNs