July 2020

Hemophilia: A Woman’s Journey to Effective Treatment

Laurie Kelley July 26, 2020

by Milora Morley, MPH

©LA Kelley Communications, Inc. Reprinted with permission.

Publication: Pulse 9.19

I never thought at age 27 that I would begin a journey as a woman diagnosed with mild hemophilia A. My brother, as well as several men in my family, have all been diagnosed with severe hemophilia A. I’m no stranger to this disorder.

I grew up in the south Florida hemophilia community. As my brother’s keeper, and older sister, I had a lot of responsibility as a secondary caregiver. I knew about doctor appointments, factor brands, and clinical trials. But when the roles change, and you’re the one who may need caregiving…everything changes.

I remember I encouraged my brother to be positive and live his best life. Hemophilia was not the end of the world! Now, here I am years later, feeling discouraged and defeated, as if hemophilia were indeed the end of the world.

My personal journey has been difficult. Witnessing my brother’s experience made me hope that I’d also get the attention and treatment I need to move forward. But I was wrong. As much as health professionals may know about hemophilia, it seems that they know it only as it pertains to males. In my experience, the specialists aren’t always educated about this bleeding disorder, or maybe they truly don’t want to believe that a woman can have hemophilia.

A Surprise Diagnosis

In 2016 I lived in Atlanta, Georgia, and became my brother’s legal guardian; he had recently relocated from Miami, Florida, to complete his senior year of high school. During his transition, he lost Florida Medicaid and was also ineligible for Georgia Medicaid. He was left with no insurance.

Later in the semester, he had a hip bleed. We rushed to the ER. Thankfully, the hospital social workers helped him receive emergency Medicaid, which covered his visit. While he was being treated, they suggested I have free genetic testing for parent or guardians, so I did.

My brother wrapped up his final semester as a senior, and I moved to Los Angeles, California, in October 2017. In November my brother called, asking me to contact his nurse. When I called her, she explained that they had been trying to reach me for the last few months. I was in a bit of shock as she told me that I have a factor level of 38%—mild hemophilia A—and that I should register with a local hemophilia treatment center (HTC).

I asked her what hemophilia looks like in women. I reflected on my past and current health issues, particularly my prolonged menstrual bleeding. At age 16, I began experiencing irregular menstrual bleeding due to abnormal hormone levels, but by age 22 my periods became more irregular and very prolonged. My menstrual cycle would stop for a day or two and start all over again; this went on for months. Doctors diagnosed me with pre-polycystic ovarian syndrome (PCOS), and started me on birth control right away.

Before my hemophilia diagnosis, my ob/gyn refused to take me off birth control. We agreed that once I reached my weight goal, she would take me off so we could address the underlying issue of what was causing my PCOS or prolonged bleeding. I lost nearly 90 pounds to combat PCOS, and achieved my weight goal, only to have the doctor change her mind and tell me that I should just stay on birth control.

I felt defeated. I eventually decided to stop birth control and explore my own options. By this time, I had already relocated to LA and had just received my hemophilia A diagnosis, but I was also having prolonged bleeding. I thought it best to start the journey with the mindset that maybe this bleeding was not just PCOS but also influenced by my new hemophilia diagnosis. So I connected with my PCP and decided to get back on birth control while I figured this all out.

My PCP sent a referral for me to be seen by the local HTC in LA. However, my insurance denied it, as well as the appeal, due to “lack of medical necessity.” At this point, I didn’t have many options, so I figured I should see an in-network specialist.

“Women are just carriers”

On April 5, 2018, I had my first appointment with an oncologist who supposedly had experience treating patients with hemophilia. I told him about my strong family history and that I thought hemophilia could possibly be the real cause of the menstrual issues I’d been having, and not PCOS, as stated by past physicians.

Although he had received a copy of the official diagnosis, the oncologist told me that it’s unlikely for women to have hemophilia, and that women are just carriers. He suggested I be tested by a lab that he trusted. He also suggested I have an ultrasound performed to look at my ovaries. I complied.

A month later, I returned to this doctor, who said, “Your results came back, and you have mild hemophilia A.” This was now my second diagnosis. He told me that I needed to book another appointment in three to four weeks for him to give me a plan for treatment.

Feeling a bit frustrated, I asked, “And what about my menstrual cycle?” The doctor acted confused, as if I’d never talked to him about my irregularities and PCOS. He told me nothing could be done about my irregular bleeding. I asked him, “Aren’t you alarmed that I have prolonged bleeding when off birth control, and isn’t that a symptom of hemophilia in women?”

He then became defensive, and I became even more frustrated because two things became clear: First, the doctor hadn’t listened to me during my first visit, due to his assumptions about women with hemophilia; nor had he properly prepared for my visit. When diagnosing me, he spoke as if we had never discussed my family history of hemophilia, or any of the things I’d brought up.

Second, the doctor had never had a female patient with hemophilia. When I asked if he would be open to consulting with Hemophilia Foundation of Southern California or the local HTC to determine a treatment plan for my menstrual cycle, he declined, stating that he could do his own research. I left the office in tears and didn’t feel comfortable returning to this doctor. I was still left looking for answers.

In November 2018, I switched insurance plans through a new employer and tried the process again. This journey to finding the underlying issue is important for me. My new PCP, who was also a hematologist, admitted that he didn’t think he’d be the most suitable hematologist for me because he was out of practice in that specialty and not familiar with women with hemophilia. He asked me what I wanted to do, and I said I wanted to go to the local HTC. He agreed to send in a referral through our medical group.

Again, my referral to be seen by the HTC was denied by insurance because they believed they had specialists more than capable of providing me the care I needed in-house. I obliged once again, and decided to see a specialist they referred me to in-network. But this time I was a bit more optimistic, because the specialist was a woman and this was one of the best health systems in greater LA. There was no reason I wouldn’t be able to find the care I needed…right?

I was excited to meet this new specialist on January 7, 2019. She was an oncologist, but she also had experience with bleeding disorders. However, what this really meant was that she was experienced with treating women with von Willebrand disease (VWD). During my first visit, she suggested that my factor levels be tested once again. Although I had provided two prior diagnoses, I agreed. This time, my third diagnosis came back with much lower levels—23%. Most likely, this was due to no longer being on birth control.

Breakthrough

I really wanted to work with this specialist to figure out the best options for me. A friend of mine connected me with the Women’s Bleeding Disorder Coalition, which helped educate me about what hemophilia looks like in a woman.

I thought it would be a good idea to connect my new specialist to the coalition. Surprisingly, she agreed, and I thought, wow, this is great! They provided my specialist with more information on hemophilia in women. I was happy and excited to hear that my specialist had taken that step on my behalf.

But during a follow-up visit, I was taken aback when my new specialist (who I had been bragging about) made comments invalidating the information that was shared with her through the Women’s Bleeding Disorder Coalition, because they weren’t “medical professionals.” I was crushed.

Sadly, I left that appointment with no treatment plan to address my menstrual bleeding, and the only medication offered to me was one most commonly used in women with VWD. Here we are, April 2019, and I still have irregular or prolonged menstrual bleeding. I’ve been fortunate to see an endocrinologist, who has been working very hard to determine my underlying issue. But at this point, he can’t pinpoint the actual cause. The reality is that he is not a hematologist, so he can’t help me access factor and attempt a trial treatment to determine whether my prolonged bleeding is in fact hemophilia.

I have also been working with Hemophilia Foundation of Southern California to get access to the GHPP (Genetically Handicapped Persons Program) insurance, which would cover my visits to an HTC. I am currently awaiting a decision and crossing my fingers for a positive outcome. Now, at 28, I just want answers. I just want to know what’s the true culprit behind my prolonged menstrual bleeding, and what I can do to control it.

I hope that one day, it won’t be so difficult to be connected to a doctor or specialist who really knows hemophilia in general and how to treat women with hemophilia. I also hope that soon, women will have the option to be seen at an HTC as opposed to being forced by insurance companies to see an oncologist.

Women all over the world are gaining a voice through social movements and in politics. It’s time for the medical field to give us a voice—and answer, as well.

Milora Morley lives in Los Angeles, CA. She is employed as a business data analyst for Cedars-Sinai Medical Center. She uses her free time productively, as a small business owner of Paramedical Tattoo Services.

Wild Child

Laurie Kelley July 20, 2020

Remember the 2014 movie “Wild”? About a woman, self-named “Cheryl Strayed” (as in strayed from the path), who decided to hike the Pacific Crest Trail from California to Oregon, a journey of 1,100 miles. Recently divorced, her life in emotional tatters, the trip is supposed to help her figure out who she is and where she is going in life. With no hiking experience at all, the journey is filled with danger and discovery. The movie received positive critical reviews.

I was reminded of the movie this weekend after driving to Connecticut, to a trailhead on the Appalachian Trail (AT), to catch up with a young man with hemophilia who is now undergoing his own “Wild.” Will Addison is hiking 2,200 miles, southbound (“SOBO”) from Maine to Georgia, to raise money for the nonprofit I founded, Save One Life. That in itself is astounding. But consider this: he is not even 20, and is traveling solo.

Doug and I arrived at the trailhead around 5:30 pm, and immediately saw members of our local community, there to cheer Will on. Then Will’s parents arrived, David Addison and Victoria Kuhn, and their daughter Grace, Will’s twin sister, all from Maine. These are true outdoors people, who love camping and adventure. They came fully prepared to restock Will with food and supplies.

For Will, this was day 44, and mile 718. We parked by the side of the road, waiting for Will to appear. There were freshly baked chocolate chip cookies from Victoria, beer, and lots of laughs. Some of us came equipped to camp overnight with the Addison family, and to see Will off in the morning. We learned from Victoria that Will would not arrive till about 9 pm, or later, so we decided to hike the 1.5 miles on the AT ourselves to find the camp site, and to set up camp. It was a lovely hike in the 90° heat, shady and moist. My biggest fear was the plethora of Virginia Creeper everywhere lining the trail. I am highly allergic to this plant: even a brush of it against my skin will leave a rash like a second degree burn for two straight weeks. And I was wearing shorts!

Bleeding disorder community waits for Will

Once at camp, we quickly set up tents, spread out our sleeping gear, and then hiked back again to wait for Will.

Around 9:30 pm, as we milled about, watching the magical and mesmerizing green flashing lights from the multitude of fireflies deep in the woods, out of the forest, out of nowhere, came Will! He literally burst into our little latern-lit gathering at the edge of the woods as if it was just a little stroll; in fact, he had just completed his longest day, at 28.5 miles. Just imagine… while carrying a pack weighing about 30 lbs.

He looked great. He is tall—6 feet 1 inch—with blond hair growing longer by the day, serene brown eyes, and lanky legs. He immediately sat down and began eating! We had a lovely visit with him and the community members, who peppered him with questions about his trip so far. Victoria, ever resourceful, brought a Save One Life sign, and we took a group photo. And we all signed his banner, which he is bringing with him to Georgia, the way Chris Bombardier did when he accomplished each of the Seven Summits. This sounds like a trite thing, but when you are limited it what you can carry, and you are traveling for 105 days solo, every ounce matters. It’s a huge display of respect and commitment for Will to carry the banner.

We had cheesecake in honor of a milestone birthday for the twins, just two days before!

By 10 pm we had to call it a day. The visitors drove off, and the final team hiked to camp: Victoria and David, daughter Grace and son Will, Doug and I, and Paul, father of a son with VWD. Now it was dark, with fireflies twinkling like little green stars above, so we used our headlamps to light the way on the trail.

Once at camp, we stuffed our food into a bear box, then quickly got to sleep in our sleeping bags on the ground in the woods, serenaded by the hooting of a barred owl that eventually flew off in search of prey.

Morning came fast, as birds lit up the woods in chorus at 5:30 am. By 7 am we had packed everything and readied for breakfast. Will showed me how to use my “Pocket Rocket,” a gas-powered cooking device. As I am a danger in a normal kitchen with a gas stove, this 5-inch device that looked like a little bomb scared me to death. But Will was a good teacher: I boiled water for tea successfully.

Laurie Kelley and Will Addison: sharing a love of hiking and contribution

This was a great time to ask Will about his journey so far. He is young, only a teen, but mature beyond his years. Still, where and how did he get this idea and ability? He is an athlete, participating in track at school, and soon to start cross-country track. He is a Boy Scout, “Third generation!” Victoria proudly added, a program which no doubt has provided him with the training and skills needed to camp out for 105 nights straight, and get him all the way to Georgia. He infuses often, every other day, to ward off bleeds. He has zero body fat (and lost about 10 pounds on the trail so far) but has muscle tone, so he is in excellent shape. He has an outdoor-oriented family, so the woods are not new to him. He seems to thrive in them like a true wild child!

He’s raising money and awareness for Save One Life, our international child-sponsorship organization. Why Save One Life? He wanted to make a difference in the world, beyond the borders of Maine, his home state. I sense he wants adventure in the great, wide world too. Perhaps one day Will and I will climb Kilimanjaro together, and he can also see our families in Kenya and Tanzania, how they struggle and how much they need our help. His eyes lit up at this idea.

Will spends most days alone on the trail, quiet, thinking, listening to music through his iPod. It can be lonely. He is only a kid, really. How does he keep it together? My most important question was this: what have you learned about yourself these past 45 days?

He thought for a moment, while munching on a baguette. “I think I’ve learned that I am more independent than I thought I was.”

Powerful statement. As parents of children with hemophilia, we want our children to be independent. This might be an extreme example, but a teen who can hike an average of a marathon each day for 105 days solo, while needing to infuse every other day, is beyond impressive. It’s stunning. He needs abilities like discipline, goal setting, planning (“He planned everything himself,” Victoria adds), preparation, focus. Independent? More than most adults I know. This young man, William Addison: watch for him. Donate to his hike’s cause, Save One Life (he wants to reach $10,000!). Follow him on Facebook. Learn some things from his amazing journey. He’ll be hiking till September.

William Addison is the type of young person we want to see more of in the world–with or without hemophilia. He is the future… I hope, with all my heart.

Wild child, full of grace, savior of the human race…. “Wild Child,” The Doors, 1969

Mental Health During Coronavirus

Laurie Kelley July 12, 2020

Debbie de la Riva

The collective pursuit to control the spread of coronavirus has resulted in an enormous challenge for the bleeding disorder community. The economic fallout of sheltering in place has affected our need for a steady income, health insurance, access to medical treatment, and—equally important—our access to each other. The degree of impact on our families is hard to determine, but it’s safe to say this pandemic has been very stressful.

But stress in not a new concept for the bleeding disorder community. In fact, our community has been dealing for years with the emotional angst of fighting for what is needed to manage our medical conditions. Remember our fight for safer products, or our fight for laws to protect us from job discrimination? Today’s battle, for our community, is to deal with the stress resulting from the pandemic. So let’s follow the same steps we have taken so many times before.

Let’s get informed, find our resources, and stick together.

Get Informed

To learn to manage stress, we need to become familiar with how our central nervous system works. Our brain comes pre-wired with an intricate system that functions to keep the rest of our body alive. This is the limbic system, which provides the “fight-or-flight” response. If the brain determines that the body is in danger, it initiates a chemical chain reaction that gets the body ready to either fight the challenge or run from it. This response begins when sensory information is picked up by a part of the brain called the amygdala. If the amygdala determines there is a threat, it signals other parts of the brain and body to release hormones such as adrenaline and cortisol. These hormones instruct the heart and lungs to increase their output in order to create the energy needed to meet the challenge. This fight-or-flight response is extremely effective when a person needs physical energy to avoid a danger such as jumping out of the way of a car. But most of today’s challenges are emotional, and they don’t require the extra energy provided by the stress response. The result is a steady supply of stress hormones circulating in the body at all times. The image that comes to mind is a person standing next to an IV pole and steadily receiving drips of adrenaline and cortisol. In others words, our body remains in a constant state of high alert.

The good news: We do have the ability to slow down the stress response. Since we now know that the brain is constantly scanning our body and our environment to determine if it should go into stress or relaxed mode, we can intentionally offer cues to indicate that we’re not in danger. In fact, this is how meditation works. The first goal of meditation is to slow down your breathing rate. This is important, because once your brain receives the signal that your breathing rate is lowered, it will interpret this to mean that you’re not in danger, and will turn off the stress response. The second goal of meditation involves your focus. You want to be focusing on the present—instead of musing about the past or anticipating the future—and you want to intentionally focus on words or images that evoke feelings of peace or happiness.

You can bring up images of when you felt safe and happy, or you can think of words that reassure you. This tool is like anything else in life: it requires practice and commitment. But eventually, you’ll find that you can truly create a sense of well-being, no matter what’s going on in your life. Sound too good to be true? Do you need proof? Ask yourself how you feel when you’re watching a scary movie, and compare that to how you feel when you’re watching a romantic comedy. In other words, what we focus on creates how we feel inside. That same principle is at work when we intentionally think about what we are grateful for, as opposed to what we lack or what we don’t like about our lives.

Learning to handle stress is more important than ever. Let’s look at the many resources that have been created since the coronavirus pandemic started.

Find Resources

National Hemophilia Foundation (NHF) and Hemophilia Federation of America (HFA) have created content to help people in the bleeding disorder community deal with both physical and emotional impacts of the pandemic. The following links will take you to the current web pages with this information.

National Hemophilia Foundation

hemophilia.org/Newsroom/COVID-19-Information

Hemophilia Federation of America

hemophiliafed.org/news-stories/2020/04/coronavirus-covid-19-what-hfa-is-doing/

You can also purchase proven self-help workbooks on stress reduction at New Harbinger Publications: newharbinger.com

And yes, there is an app for learning to relax! Appropriately called the Calm app, it has hundreds of meditations and master classes on stress management: calm.com

You can find these resources and many others by visiting the Mental Health Matters Too website: mentalhealthmatterstoo.com

Seek Out Others

It didn’t take long for our community to figure out how to be connected virtually. Though it isn’t the same as being in a room together, it is nice to see familiar faces and get a chance to let someone know you are there for them.

Take a moment to check in with yourself, because it’s very easy to feel lonely in isolation. If you find that you’re exceptionally lonely, depressed, or anxious, it always helps to talk to someone trained to help you feel understood and supported. Online platforms like Talk Space and Better Help are reporting an exponential increase in the number of requests for counseling sessions right now.

Look for Purpose

One of the best ways to combat the feeling of helplessness that comes with a crisis is to look for a way you can help others. This sense of purpose gives people some control, and helps them feel productive and useful. For me, contributing to Save One Life is one way I fulfill my need to have purpose in my own life. Each month, I have a small sense of satisfaction knowing that there are three young people with hemophilia who feel that someone else on this planet sees them and cares about them.

So, whether it’s meditating, talking with someone, or just being there for another human, there are ways to combat stress. We will get through this pandemic as a community, the way we always have. We will get informed, find resources, and seek out each other.

Debbie de la Riva, LPC, has been an active member of the bleeding disorder community since the birth of her son with severe hemophilia 25 years ago. She served as executive director of the Lone Star Chapter of NHF, was a co-chair of an NHF Annual Meeting, received a Ryan White Award for Advocacy Excellence, and has presented on mental health issues to chapter and national organizations. In 2018, Debbie founded Mental Health Matters Too as a way of combining her degree as a licensed professional counselor with her passion for helping community members who struggle with mental health challenges. To contact Debbie: www.mentalhealthmatterstoo.com or debbie@mhmtoo.com

Red, White and in Two

Laurie Kelley July 5, 2020

The topic of flags has been in the news a lot lately, and with the Fourth of July weekend ending tonight, it got me thinking of our flag. It’s the main symbol of any country, and deeply personalized and personal to each country. It’s used to represent a country in times of peace at international conferences and meetings; and used to unite a country in times of war, to rally the troops and display on ships and aircraft. Americans love to wave the flag and refer and defer to the flag, even when we are as deeply divided as we ever have been since the Vietnam War. A foreigner visitor staying with me once said that they had never seen any country that had so many flags posted in public as the US.

The symbols on a flag are carefully chosen, as are the colors. Look at the color red, for example. The bleeding disorder community does not have a flag per se, but we use colors and symbols as our “flag,” to unite us: the red figure and the “empty” figure. I hesitate to write white, even though the figure appears white in color, because the figure really represents a figure devoid of blood, of factor. Many years ago the logo was of a white figure leaning on the red figure, showing dependency. While this is still the case for a lot of countries dependent on factor donations, the World Federation of Hemophilia wisely chose to change this to two upright figures, to show unity, support and hopeful equality.

Red is used in many flags to depict blood, or more appropriately bloodshed, for that spilled when a country was formed. And white is often used to denote peace. Red, white and blue is the standard for republics: France, Russia and the US all have common looking flags with the bold tricolors.

But these colors may have a different meaning for the US flag. From pbs.org: “Thee stripes represent the original 13 colonies and the stars represent the 50 states of the Union. The colors of the flag are symbolic as well; red symbolizes hardiness and valor, white symbolizes purity and innocence, and blue represents vigilance, perseverance and justice.”

It’s hard to know what the colors mean in reality now. The nation is torn politically, reeling from escalating COVID-19 cases, back to quarantine and lockdown for some states, and economically teetering. We are showing our true colors under the stress. When I think of hardiness and valor, I think immediately of the medical teams at ground zeros, working day and night to keep people alive. And while they struggle to do this, a nation burns and yells and topples statues. Purity and innocence seem lost. And justice is being questioned daily.

But not that long ago, the hemophilia community was also torn in two. The HIV scandal caused a deep divide when we learned that so many knew that HIV was or might be in the factor being injected into our young men, and kept that knowledge or concern hidden for the sake of profit. The community launched a severe backlash against the national foundation, which was to be our watchdog, which we trusted; the community as a whole eventually launched a social and legal assault on the government, which ignored our plight for too long, and on pharmaceutical companies. We didn’t have social media then, only our voices and feet, to march and speak out. This community united and changed history, and helped save the lives of thousands.*

Sadly, America has a ways to go before we will be united and eventually healed. But it will happen.

And what about the stars and stripes? A book about the symbolism of the American flag published in 1977 by the House of Representatives reads (from colonialflag.org):

“The star is a symbol of the heavens and the divine goal to which man has aspired from time immemorial; the stripe is symbolic of the rays of light emanating from the sun.”

Sober things to reflect on this holiday. Perhaps we can think of the flag colors red and blue as denoting the two chief political parties… and white, to denote peace, a peace we want, need and can not seem to achieve just yet.

*Watch the excellent documentary Bad Blood, by Marilyn Ness

July 2020

Hemophilia: A Woman’s Journey to Effective Treatment

Wild Child

Mental Health During Coronavirus

Red, White and in Two

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