A Patient’s Story: What Box Do I Fit In And Does It Even Matter?

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Linda, Grandmother and MPN Patient pictured above with her granddaughter.

I’m 58 and the proud mother of five beautiful children, their families and seven grandchildren. In 2017, I was diagnosed with essential thrombocythemia (ET).  Looking back, I believe my symptoms began in 2011. I worked in a college library where I did research and helped organize lectures on various subjects for students and faculty. I also taught voice at night at a music school and sang at charity events and with various bands. Life was good. One day I got up from my desk and went to help students in the computer lab when all of a sudden everyone was a complete blur. Later, I experienced similar problems when teaching breathing techniques at the music school.  I was in perimenopause so I wrote it off. 

On Mother’s Day in 2011, I was out enjoying a band at a restaurant and while they were setting up their speakers, one blew right by my ear. I felt like I was underwater for an hour. Later that week I got a cold and had a loud heartbeat sound (pulsatile tinnitus) in my left ear. I then began a journey of symptoms that have not changed to this day. Early on I was diagnosed with various possibilities, Meniere’s Disease, MS, Vestibular Neuritis, Vestibular Migraine, maybe Lyme- so many ideas were entertained.  I tried working for years in a reclined chair at my job. If I got up quickly without thinking, I would often see black spots.  I would get odd brain fog at times and blamed it on the various drugs I was taking.  After getting bounced around from neurologists to ENTs to cardiologists, I was finally diagnosed with atypical Vestibular Migraine.

MPN Advocacy & Education International is gathering information on MPN patients who suffer from migraines, click here for more information.

In 2017, my platelets started climbing and my local neurologist, who had spent hours with me testing my blood pressure in different positions, felt I had a form of dysautonomia called POTs and needed more testing.  He repeated an electromyography (EMG) study which showed severe neuropathy in 2012 and it came back the same in 2018. Eventually, I got to the point that the feeling of fainting was so strong I couldn’t stand. I tried to hide it whenever I could because it was so inexplicable even to myself. I was anxious because I never knew when a symptom would occur when I had to be up for any length of time and I looked normal on the outside and was embarrassed. My family and friends were frustrated with me because I went from being an active mom and grandmother to being disabled and limited in what I could do. My local neurologist sent me to a hematologist who diagnosed me with ET, CALR 1 mutation.  He told me I would need a biopsy to confirm which I did at Sloan Kettering.,

As scary as it is to get diagnosed with a rare blood cancer, I felt slightly relieved that it might explain some of my symptoms and was told there was hope on the horizon with these blood cancers.  It seemed that my neurological symptoms could not all be explained by the MPN only.  and I probably have something else going on.  I noticed that is a common complexity of MPN patients, we usually have other things going on and have been to many types of specialists.  Being treated as a whole person can be challenging for us. I noticed that a lot of the symptoms were shared by the other groups I belonged to especially the Vestibular Migraine Group and Pots.  It occurred to me that if these different chronic illnesses could be studied together maybe drugs used to help one could be used to help another especially if you are in a “watch and wait” situation.  I’m sure this is being done all over the world.

I realized after joining some of the social media groups, that I am not alone in this feeling especially when it comes to the atypical migraines, brain fog and dizziness.  Being in a box, is not so important anymore.  Especially in the MPN world where you can have one type one day and potentially can learn it progressed or changed to another.  We are in this together. No matter what.  I’ve been lucky to have been referred to the Cleveland Clinic where I’m being evaluated by neurology and oncology to come up with an answer.  I’m inspired by that institution and the kindness of everyone from the shuttle drivers, to the technicians and doctors who work there.

If I had any advice to share it would be to be your own advocate. Not believing everything you read in the groups is also important because it may never be part of your story and there is so much being researched and studied.  If anything happens to be written that is inaccurate, you can put yourself in a state of fear even if you try to tell yourself otherwise.  Also, there are wonderful friendships to be made with people who know what you are going through.  I’m looking forward to finally meeting a fellow MPN patient, who I have been communicating with for a year at MPN Advocacy & Education International’s program in Cleveland this November. I’m realizing the importance of yoga and nutrition and I still try to keep busy for as long as I can stand before I give myself permission to rest when I can’t.  I’ve since learned that life is unpredictable and can change in a moment. All in all, I try to be optimistic and feel most people are kind, loving, and caring, but no one knows what you feel better than yourself.  I’ve also learned I have the best family, friends, and people in my life who provide love and support.

 

Treatments for MPNs During Pregnancy With Birth Rates and Maternal Outcomes

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Question

Are use of aspirin, heparin, interferon, or combinations associated with live birth rate and adverse maternal outcomes in pregnant women with myeloproliferative neoplasms?

Findings

In this systematic review and meta-analysis of 22 studies, reporting on 1210 pregnancies, the live birth rate was 71.3%; most studies reported on pregnancy with essential thrombocythemia. The use of aspirin and interferon—but not heparin—was associated with higher odds of live birth.

Meaning

Moderate-quality evidence suggests that treatment with aspirin or interferon is associated with higher odds of live birth in pregnant patients with myeloproliferative neoplasms.

Myeloproliferative neoplasms (MPNs) are increasingly being identified in women of childbearing potential. Pregnancy in women with MPNs is associated with maternal thrombosis, hemorrhage, and placental dysfunction leading to fetal growth restriction or loss.

Objective

To evaluate the association between the use of aspirin, heparin, interferon, or combinations and live birth rate and adverse maternal outcomes in pregnant women with MPNs.

Data Sources

Systematic searches of MEDLINE, Embase, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, and the MEDLINE Epub Ahead of Print and In-Process and Other Non-Indexed Citations from inception to July 19, 2018, with no language restrictions, was conducted. Key search terms included myeloproliferative disorders, polycythemia vera, essential thrombocythemia, and primary myelofibrosis.

Study Selection  

A study was eligible if it included pregnant patients with MPNs; interventions included aspirin, heparin, and/or interferon; there was a comparison group in which patients did not receive the intervention; the study reported on at least 1 of the study outcomes; and it was a randomized, case-control, or cohort study or series of at least 10 pregnancies. Data were extracted in duplicate; 0.5% of identified studies met selection criteria.

Data Extraction and Synthesis  

The review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines and reported in accordance with Meta-analysis of Observational Studies in Epidemiology (MOOSE) guidelines. Data were pooled using the Mantel-Haenszel approach.

Main Outcomes and Measures

Outcomes were the number of live births and maternal complications, specifically, arterial or venous thrombosis, hemorrhage, and preeclampsia.

Results

Twenty-two studies reporting on 1210 pregnancies were included. The live birth rate was 71.3% (95% CI, 65.1%-77.6%). Use of aspirin (11 studies, 227 patients; unadjusted odds ratio, 8.6; 95% CI, 4.0-18.1) and interferon (6 studies, 90 patients; unadjusted odds ratio, 9.7; 95% CI, 2.3-41.0) were associated with higher odds of live birth. Addition of heparin to aspirin was not associated with higher odds of live birth (6 studies, 96 patients; unadjusted odds ratio, 2.1; 95% CI, 0.5-9.0). The most common adverse maternal event was preeclampsia, with an incidence of 3.1% (95% CI, 1.7%-4.5%).

Conclusions and Relevance

Most studies reported on pregnancy with essential thrombocythemia. Few studies reported on pregnancy with polycythemia vera and none with myelofibrosis met the inclusion criteria. Most studies were retrospective and early pregnancy losses may have been underreported. Moderate-quality evidence suggests that aspirin or interferon is associated with higher odds of live birth in pregnant women with MPN.

Learn more

View Dr. Hobbs Presentation on Fertility, Child Bearing and Beyond

at the 5th Annual Women & MPN Conference

Learn more about Women & MPNs

Health Insurance: What You Should Know

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By Pam Trexel, American Cancer Society, Senior Vice President, Alliance Development and Philanthropy

Pam Trexel

Access to health care is a significant determinant in whether an individual diagnosed with cancer will survive. Uninsured individuals are more likely to be diagnosed with cancer at a later stage and more likely to die from the disease. The American Cancer Society Cancer Action Network (ACS CAN), the public policy arm of the American Cancer Society, believes all Americans should have access to affordable, quality health insurance.

Fortunately, in recent years there has been an increase in the number of Americans who have health insurance coverage. Since 2014, Americans have had access to comprehensive coverage that includes key consumer protections vitally important to cancer patients. These protections include: prohibiting insurance companies from denying coverage or charging more due to a consumer’s pre-existing conditions, restrictions against insurers imposing arbitrary caps on coverage, and a requirement that all insurance offered to individuals cover a broad set of benefits called essential health benefits.

Yet there are still challenges. Many cancer patients have difficulty finding specialists who participate in their insurance plan’s network, affording their prescription medications, and understanding their out-of-pocket expense liability. Recent regulatory and legislative approaches on both the federal and state levels have the potential to weaken current patient protections, segment the insurance market, allow for more insurance plans with inadequate coverage, and reduce access to healthcare for cancer patients and survivors.

Federal Activities

In 2018 the administration finalized a rule that would expand access to short-term, limited-duration (STLD) policies. The rule allows STLD products to be sold for a coverage period of up to 12 months and be renewed for three years. ACS CAN urged the administration to withdraw the rule due to concern that these policies are exempt from many of the key patient protections that ensure individuals with cancer and survivors have access to quality health care needed to treat their disease.

Additionally, the current administration has repeatedly reduced enrollment education and outreach funding,which limits efforts to inform consumers about open enrollment and plan options. Concerns remain about enrollment trends in future years and the abilities of non-governmental groups to continue outreach and enrollment efforts.

State Activities

Faced with uncertainty from the federal government, some states have implemented policies that seek to either strengthen or weaken the individual health insurance market.

Short-Term Limited-Duration Policies

As federal regulations try to expand access to STLD policies, some states are trying to prohibit or minimize their expansion. For example, New York state law permits the sale of short-term limited duration policies, but requires these plans abide by the consumer protections required for ACA-compliant plans.Other states are considering legislation that would limit STLD policies to a coverage period of less than three months without the option for renewal.

State Individual Mandates

The federal individual health insurance mandate penalty ended January 1, 2019. In response a few states have begun considering state-level individual mandates requiring state residents to maintain health insurance. Massachusetts has had a state individual insurance mandate since before the implementation of the ACA and never rescinded it.   New Jersey has also enacted legislation to impose an individual mandate requirement.

Non-Comprehensive Coverage

Following administrative actions encouraging creation of association health plans (AHPs) – plans wherein small businesses join together to purchase health coverage – some states are considering legislation that exempts AHPs from state regulation. These plans are already exempt from the important patient protections provided under the Affordable Care Act (ACA). ACS CAN is concerned these plans will be able to discriminate against people based on their health status and will syphon off younger, healthier people, leaving older and sicker people in the state’s individual market (which would increase premiums).

Utilization Management

Cancer patients often need to choose a health plan based, in part, on the plan’s prescription drug coverage. Utilization management programs are health insurer practices used to control spending.   These practices may include:  prior authorization or approval of a drug by the patient’s health insurer before a prescription can be filled; and step therapy which requires patients to try, and fail, on an insurer-chosen prescription drug before gaining access to the drug that was prescribed by their doctor but may be more expensive.  ACS CAN is concerned that if used inappropriately, utilization management may delay care or impede access to prescription drugs for cancer patients.  Several states are considering legislation to ensure that utilization management practices are timely, efficient, clearly described for both patients and doctors, and allow for appeals and exceptions when appropriate.

Click here to learn more

 

 

 

Study Unveils Family Risk for Certain Types of Blood Cancers

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Individuals with a parent, sibling or child with blood cancer appear to have a higher likelihood of also being diagnosed with the disease, according to study results published in Blood.

Moreover, age of diagnosis; whether the relative is a parent, sibling or child; and the number of affected first-degree relatives were substantially associated with familial risk for certain blood cancers.

“Although many hematological malignancies are individually rare, collectively they contribute significantly to the overall cancer burden in the population,” the researchers wrote, adding that the etiological basis of most blood cancers is poorly understood.

Therefore, they analyzed data from over 16 million individuals from the Swedish Family-Cancer Database to determine the familial risk of the different blood cancer and their possible inter-relationship.

Those with a familial link to the disease represented 4.1% of all blood cancer diagnoses – higher than patients with cancers of the nervous system, kidney and pancreas. However, this was lower than those with cancers of the breast, colon and prostate, which ranged from 8% to 15%.

In total, the researchers identified 153,115 patients who were diagnosed with a primary blood cancer, including myeloproliferative neoplasms (MPN; polycythemia vera, essential thrombocythemia, myelofibrosis and MPN not otherwise specified), chronic myeloid leukemia, myelodysplastic syndrome, acute myeloid leukemia, acute lymphocytic leukemia, Hodgkin lymphoma, non-Hodgkin lymphoma, diffuse large B cell lymphoma, follicular lymphoma, mantle cell lymphoma, marginal zone lymphoma, Burkitt lymphoma, small lymphocytic lymphoma, hairy cell leukemia, chronic lymphocytic leukemia and multiple myeloma.

The researchers found the highest relative risks for familial risk among patients with certain Hodgkin lymphoma subtypes (lymphoplasmacytic lymphoma and mantle cell lymphoma) and with polycythemia vera, myelodysplasia and essential thrombocythemia.

“This information improves our understanding of the causes of – and potential inherited predisposition to – blood cancers and should inform the identification and characterization of genetic risk factors for blood cancer, as well as how we best clinically manage patients and their relatives,” lead study author Dr. Amit Sud, from The Institute of Cancer Research in London, said in a press release. “The results should also encourage conversations among families, clinicians and patients about familial risk.” (Cure Magazine)

Read more

 

Special Interview: Living with an MPN in Childhood

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Diagnosed at age seven with Essential Thrombocythemia, Portia shared her story at the 2nd Annual Pediatric & Young Adult MPN Patient Program.

Portia, a young adult MPN patient, shared her story at the 2nd annual Pediatric & Young Adult Program

Do you remember experiencing any symptoms?

I had occasional nosebleeds that would last about twenty minutes or so. But over time, the time decreased to about ten minutes. I also experience fatigue, especially when I’m sick, all I do is sleep to try and regain any energy. Also, I’m very active and I play competitive squash, so I do experience fatigue more than an average person.

How do you cope with essential thrombocythemia (ET) symptoms and/or side effects from Hydroxyurea?

I’m very lucky that I don’t really experience too many symptoms, but I usually push through any pain that I have since I’m such a wimp about medication. I have not had any side effects from Hydroxyurea. For fatigue, I don’t take any other medication, I pretty much just work hard and try to be smart about how I utilize my energy. When playing squash, I work extra hard to make up for my fatigue, but if I really can’t breathe due to lack of oxygen, I will talk to my coach and ask for a small break to recuperate. Most coaches are very reasonable and will allow a break.

Has ET curtailed your involvement in school activities?  Sports?

When I was younger, elementary and middle school age, I would occasionally have to miss school for lab appointments, so I would have to make up work. One symptom of ET is fatigue, so I do have to deal with that in sports. But I also have Hemoglobin H, which I believe has a bigger impact on my fatigue in sports than ET. But overall, I still go about my life and continue to do the things I love.

How do you explain what you have to your friends?

As I’ve gotten older, I’ve done a lot more of my own research to further understand my condition, but to my friends, I explain that I have way too many platelets, which help clot your blood when you get a cut, and because of that, I bleed for longer.

What advice would you give other younger individuals with an MPN when peers say they don’t look sick or they’re faking?

I would tell them to do their best to ignore their hateful comments and try to explain their condition by telling them it’s something internal rather than external, that’s why they don’t appear sick. And most important, find friends who won’t judge you, and people who do, clearly aren’t your real friends, for real friends should accept you no matter what.

You are very energetic and positive, when you reach out to others your age who aren’t feeling well, what do you say to encourage them?

First, I would listen to their concerns and possible issues, and then I would tell them to keep their head held high and know that it does get better. This is just a phase and eventually, the negative parts will fade away. Also, it’s very important to know who your close friends are and be able to talk to them since many kids would rather talk to their friends rather than a parent or even a doctor since it can be intimidating. I would also say, take one day at a time and find joy in the little things, whether it’s going for ice cream or just taking a nice walk.

If you could wave a wand and change one thing in the world of MPNs, what would you change?

Personally, I would change the medicine. I really hate swallowing pills, so I would much prefer something fun to eat or drink as my medicine. I’m also very strange and would much rather have the medicine get injected into me, which I know is an option, but the majority of people aren’t a big fan of needles.

 

 

 

A Caregiver’s Story: The Journey of a Spouse Through the Transplant Process

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In March, MPN Advocacy & Education International highlighted the story of a recent stem cell patient, Andrea (click here to view story). This month, Andrea’s wife, Denise, shares her story as a caregiver in an interview with us. 

Andrea and Denise

How did you feel about your spouse’s decision to have a transplant? Were you apprehensive? 

I have been a part of Andrea’s medical journey since her diagnosis from ET to myleofibrosis.  I recall it took me about a week to learn how to pronounce the name correctly, and it has been an education ever since.  Being present during doctor appointments and multiple clinical trials provided first-hand information in addition to our ongoing discussions.  Observing and experiencing her five years of transfusion independence was a gift.  We took advantage of the opportunity to cycle and travel together and separately. Knowing the clinical trial would either end or the drug would quit working was only a matter of time.  And knowing how she physically felt prior to and after this time period played a role in my ‘wrapping my head’ around a possible future transplant.  All the while, we adjusted and modified activities as needed in order to continue allowing her to live life to the fullest.

When medications were no longer yielding the same results, and Andrea’s blood transfusions became more frequent, the doctors felt the alternatives were to continue clinical trials and hope for the best or discuss a stem cell or bone marrow transplant. I felt she was strong enough physically to beat the odds. It was now or never. I never doubted our decision, and have had no regrets.

How did you prepare yourself as a caregiver? 

In order to prepare, I scoured MD Anderson’s educational resources to understand what a ‘typical’ transplant journey would entail.  I developed multiple spreadsheets to track medications, nutrition, recovery, therapy, etc. I left the online myleofibrosis forum readings to Andrea as she would report her findings from reading first-hand accounts.  She also talked to many individuals who had had transplants, pummeling them with questions.

Another important piece of this journey was my mindset.  I focused on the end goal – her successful transplant and healthy recovery.  I looked at the journey in three parts:

  1. Pre-transplant
  2. Transplant/hospital stay
  3. Post-transplant

My goal was to have as much of her daily care and needs become second nature to me prior to her hospital release. When she was released, the additional environmental interaction was familiar without the distraction of ‘everything new all at one time’.

What tools did you find useful as the caregiver and advocate?

I created a 3-ring notebook with tabs to manage spreadsheets, medical records/test,  and doctor questions, so I could access information easily and quickly. I created a spreadsheet to track her medication schedule.  The purpose was to help me identify what was needed, including dosage when refilling medications both inside and outside the hospital. Click here to view medication tracker template. (For an Excel spreadsheet version of the attached please email kmichael@mpnadvocacy.com).

I also knew there was a good possibility that after her hospital release and within the first 100 days, she would have a trip to the emergency room and be re-admitted to the hospital.  Therefore, medications and ‘the notebook’ were kept in one location and easily picked up and transported with us.  The notebook was with us each time we visited the care team.

I created additional spreadsheets to help track:

  • Food and water daily intake and output. This is useful for doctor & nutrition appointments.
  • Daily stats: blood pressure, temperature, pain levels, exercise, spirometer therapy.  This is useful for tracking blood pressure and temperature anomalies.  Because if her temperature rose to a specific number, she had to go to the emergency room immediately.
  • Signs of graft vs host disease (GVHD).
  • I choose to stay in the hospital 24/7 to understand how the nursing staff/care team handled her care. This experience helped me understand her routine.
  • I figured if I learned her hospital routine, it would be familiar when she was released.
  • I used my smartphone and set alarms with labels so I knew what drugs were due when. (Used upon hospital release.)
  • I utilized Caring Bridge (www.caringbridge.org) to communicate Andrea’s transplant journey to her friends and family. My intent with each post was to create an engaging story yet convey “a day in her life” so everyone could get a sense of being there.

What was the most challenging part of your role and why?

Coming home and changing environments automatically kicked us back to normal behaviors and patterns. I felt we had to be more careful and diligent in preventing infection.  Even though we were home, I had to be more watchful because familiarity brings about a relaxed state.  Her immune system was developing and the risk of infection was too great.  Andrea quickly tired of me saying “No, you can’t do that or touch that.”

Once home, Andrea’s friends visited.  This was a considerable risk to her because the natural tendency is to hug and touch. To reduce her risk, we asked people to use hand sanitizer when they were around her.  We developed a routine where I would greet her friends first with a hug and explain she couldn’t hug yet. Andrea stood back a few feet to reduce her availability.

What I learned :

Every recovery and journey is different.

While it’s tempting, don’t measure your progress against someone else.  It’s your journey.  That goes for both the patient and caregiver!

The “notebook” was a great tool.  It kept us on schedule for all medications and were able to provide information to the doctors as needed.

The hospital care team staff is a critical part of your recovery.  Don’t opt for staying close to home if you feel a facility’s care team is better in another location.

Deciding where to have the transplant included researching the number of myleofibrosis transplants, versus other blood disease transplants, and the success that facility had.

Participate in the journey. I chose to shave my head at the same time Andrea did.  It sure made showering quick and easy!

Take in the outdoors and/or change the scenery.

Exercise or go for walks.  Listen to music.  Visit a friend. Go to the grocery store.  Do something to clear your head and regain perspective.

Have a confidante. It’s normal to question, or become frustrated, and to second guess.  But remember – it’s temporary.

You are the coach, cheerleader, and guardian all at once.  Embrace the many hats you will wear!

Click here to read Andrea’s Transplant Story 

MPN Patient Daily Stats 06-10-18

A Parent’s Story: Navigating the Health Insurance Maze

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By Sarah W.

Sarah is a member of MPN Advocacy & Education International’s Patient Advisory Council

I know there are good people who work at insurance companies, however, some are the bane of my existence. Every month I gear up for the fight to get my son, Jedi, his medicine. Jedi has an MPN. My hope is that my journey can be used to make this process easier and better for other patients and their families.

Hydroxyurea was the initial drug prescribed for my son. It wasn’t a difficult drug to get filled, even though it was a little overwhelming walking my son through the adult oncology unit at MD Anderson Cancer Center, to the “specialty pharmacy.” It was frightening for him to see adults that look so sickly skinny and with their bald heads from their treatments. However, the real frustration began when he was put on Pegasys. MD Anderson tried to call numerous pharmacies to his prescription filled, but they were unable to find a pharmacy that had it in stock. So we left the hospital with a handwritten script in search of the drug.

Read tips from Speciality Pharmacist/MPN Patient Jennifer Powers w/ links to treatment assistance programs

At that time, we were insured by United Health Care. This was my first introduction to a specialty drug. For background purposes, specialty drugs are a recent designation of pharmaceuticals that are classified as high-cost, high complexity and/or high touch, such as injectables. A chemotherapy that is administered orally, like Hydroxyurea, is covered as part of a normal prescription. A chemotherapy that is administered as a subcutaneous (jiggly fat) shot, like Pegasys, is not. In other words, it comes out of your deductible. For us, that means we owed approximately $4,000 for the first month of Pegasys. We had to prove to the insurance company that Pegasys had been approved for polycythemia vera. This was done by producing a memo my husband found on the internet from United Health Care specifically stating Pegasys was approved for polycythemia vera.

Then, our insurance was switched to Blue Cross Blue Shield of Texas (BCBSTX). (I will skip the conversation about trying to get our member number early so I could start the pre-approval process.  I had been told in a Facebook group that it took about six weeks to approve Pegasys with BCBS. This was cause for concern because we could only get four weeks of medicine at a time.)

I started the pre-approval process with BCBS after we received a member ID number the first week of January. As expected, Pegasys was initially declined.  In the state of Texas I should be able to submit an emergency appeal and get a response in three days, but I had to get the appeal submitted first.  It is very difficult to get the appeal started. Not to mention that BCBSTX outsources the pharmaceuticals to Prime.

After a few weeks of deep frustration and the looming possibility we could run out of his medication, I finally posted the following on Blue Cross Blue Shield of Texas Facebook page:

January 15, 2019. Your appeals process is TERRIBLE. I have spent HOURS trying to figure this out. I have a 10 year old with CANCER. His medicine is out next week. I have to get it from a specialty pharmacy. His doctor called BCBS yesterday (a number I got after spending TWO HOURS on the phone last Friday) and was told that he has to wait 7-10 business days to start the process. Today I received a NEW peer-to-peer phone number. Does this start the process? No, it sets up an appointment for our doctor to talk to your doctors. Just to give you an idea. This medicine controls platelets. If his platelets go up he could have a heart attack, stroke, embolism for example. You can see my concern. My next steps are to ask my doctor to call AGAIN to START the appeals process – you know the one he tried to start yesterday? I will also have on my TO DO list to file a complaint with Texas Department of Insurance, contact my state Senator and Representative. Thank you for making this such a HARD process. Oh, by the way, the National Comprehensive Cancer Network, other BCBS insurance (different states), United Healthcare ALL have approved this medicine for his diagnosis

 I received a phone call from BCBSTX within 24 hours of posting this. I was told I had made it to the “special escalation” team because I posted on social media. On one hand, I am grateful he was approved. On the other hand, I am sad that this is what it took. I think it also helped that I had gathered the information to make it easy for them to approve. I had whitepapers, Jedi’s medical records and the details from the National Comprehensive Cancer Network. (Click here to view).

I wish I could say this was the end of the drama. But this is a story for another day. Just writing this was overwhelming. I can’t imagine what people who are sick go through. I am the caregiver, not the patient. I do not have chemo brain and I am exhausted by the process and the stress and anxiety to ensure my child receives his treatment. I hope this information will help other families move through the process a little more smoothly and I will continue to write about the trial and tribulations I experience on my website.

View Videos from the MPN Pediatric & Young Adult Program in 2018

 

 

Interview with a Patient: Making the Decision to have a Transplant

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Andrea was initially diagnosed with essential thrombocythemia after her primary care doctor noticed her platelets steadily rising. Ten years later it had progressed to myelofibrosis. She lives in Texas with her wife (and caregiver), three dogs and one cat. After working with American Airlines and Sabre Holdings for 30 years she chose an early retirement and pursued other opportunities, including working in a bike shop (she’s an avid cyclist), flower shop, consulting firm and for the last six years with Apple. 
What made you decide to have a Stem Cell Transplant (SCT)?
It was an exceptionally hard decision to make. I struggled with it up to and including the day before transplant. I had been feeling good, I was in and out of several clinical trials, the last of which had worked well for six years. But I realized my options for the future were very limited. I had become red blood cell transfusion dependent. My age was advancing, I had exhausted all relevant clinical trials and available drugs and I was physically pushing harder to do things I loved. It was clear to me that waiting “it” out and hoping for another miracle drug or a SCT were my only options.
How did you prepare for the procedure?
I like to be as prepared as possible, so I found speaking with actual survivors helped me get answers to all my questions that doctors may not have known or had time for. I spoke to several SCT recipients, especially those who had the same MPN as me. I read as much information as possible such as newsletters, blogs, and other online sources. It did not dwell on the less positive comments! Gathering as much information as possible from a variety of sources gave me an idea of what was to come. Keeping in mind that everyone is different, everyone I connected with gave me tidbits that I could refer to before, during and after transplant.
Andrea was initially diagnosed with essential thrombocythemia after her primary care doctor noticed her platelets steadily rising. Ten years later it had progressed to myelofibrosis. She lives in Texas with her husband (and caregiver), three dogs and one cat. After working with American Airlines and Sabre Holdings for 30 years she chose an early retirement and pursued other opportunities, including working in a bike shop (she’s an avid cyclist), flower shop, consulting firm and for the last six years with Apple. 

 

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