Happy Mother’s Day !

A bleeding disorder diagnosis brings out the mama bear in most
mothers who have a child with a bleeding disorder. Maybe even more so in
those who have children with inhibitors. Below, Cazandra MacDonald, a
personal friend and wonderful mom of two boys with hemophilia (and
inhibitors) shares her thoughts on inhibitors in hemophilia. 
Happy Mother’s Day to all the incredible moms who have children with bleeding disorders!  

What’s in a Number?
Written by Cazandra Campos-MacDonald
Originally published in PEN May 2017



Numbers, numbers, numbers. Our society is flooded with numbers. From Social Security numbers to birthdays, PINs, passcodes and checking our weight, we can hardly get through a day without numbers. When you are living with a bleeding disorder, you monitor the assays of your factor, track the number of bleeds per month, check how many doses of product are on hand, and measure the circumference of a swollen knee. But when you live with an inhibitor, there’s another number that can become the focus of treatment: the Bethesda unit (BU).
The Bethesda inhibitor assay is a test that measures the titer (strength) of the inhibitor, described in Bethesda units. Inhibitor titers may range from less than 1 BU to thousands of BU. Knowing this number will help determine how bleeds are treated. If the inhibitor registers as low titer (less than or equal to 5 BU), bleeds may be treated with high doses of standard factor concentrate. If the inhibitor registers as high titer (greater than 5 BU), standard factor concentrates are ineffective and special factor concentrates called bypassing agents are used instead. Attempting to treat bleeds in the presence of inhibitors is less effective than treating bleeds without inhibitors—so the goal is to eradicate the inhibitor. If the inhibitor registers as less than 10 BU, this is when many providers will have patients begin immune tolerance therapy (ITT), also called immune tolerance induction (ITI), a treatment protocol designed to eliminate the inhibitor.1 Knowing your BU is crucial in order
to take the next step in working toward that goal.
It’s easy to put your faith completely in the numbers. Knowing your current BU is important, but know first that every individual is unique and there are several different ITT protocols. Each person does not react to ITT in the same way. One body may accept ITT easily, and his BU will come down in a short time. Others on the protocol may take years to get the same results. Numbers do not dictate that the treatment for one person will be the same as for another. For example, two brothers, both with severe hemophilia and inhibitors and with the same parents, can live very different lives with an inhibitor. My older son, Julian, was one year old
when he was diagnosed with a low-titer inhibitor; it never rose above 5 BU. He immediately had a port inserted, and he started ITT for two and a half years. He tolerized, meaning his inhibitor dropped to zero, and he has never had an inhibitor resurface.
My younger son, Caeleb, was 11 months old when diagnosed with a high-titer inhibitor that registered over 2,200 BU. His titer dropped to 0 BU at one point after ITT, but now he is living with a low-titer inhibitor, and he receives factor daily
to maintain his tolerance. My sons both reached 0 BU after ITT, but they had different outcomes.
The numbers can be promising and sometimes disappointing. But ultimately, the numbers are a key component to treatment.
Everyone who tracks his BU has an ultimate goal in mind: to lower the titer to zero. If your titer is 323 BU, your goal may first be 299 BU, then 250 BU.2 Another person may be hoping to get to double digits, and another to single digits. Of course, when you’re tracking your BU, you want to get to zero and stay there. When you reach 0 BU, you may think that the inhibitor is now a thing of the past—but not necessarily. Once 0 BU is attained, the next step is to monitor the half-life of the factor. To be successfully considered tolerized (this is also called complete tolerance), the following must be maintained:
• The inhibitor titer can no longer be measured.
• Factor recovery is greater than 66% of normal.
• The half-life of factor VIII is greater than six hours.3



But someone may live with 0 BU for many years without these three characteristics. This is called partial tolerance. For example, if your child has 0 BU and a three-hour half-life of factor in his body, he will probably continue with the same ITT therapy, which may be daily infusions. ITT is not always successful: an ITT attempt in which inhibitor titers fail to decrease at least 20% over three to six months, or remain over 5 BU after three to five years, is considered a failure. This example shows that not only is BU important, but monitoring the number of hours for the half-life is critical to treatment. So how does a family live with the numbers?
“Lab work disappointment” is a phrase Kari Atkinson’s family used when the numbers were not what they had expected for their son. “We had so much hope that the inhibitor would go away.” But now, says Kari, “we are not as concerned about the number because we can tell when [the BU is] up and down by how our son bleeds.” How an individual’s body reacts to treatment is the ultimate measure of success. If you’re living a full life with few bleeds and an active inhibitor, the important thing is that you are healthy, happy, and thriving. Eric Frey’s son, age seven, has lived with an inhibitor for over five years. “After time, we learned two things: First, we already knew what the results [BU] were going to show by the way our son was bleeding, bruising, and behaving. Second, the Bethesda number is far less important than how our son was bleeding, bruising, and behaving.”
Despite living full, healthy lives with an inhibitor, many families still worry about the numbers. “Making peace” with the inhibitor is something that most people don’t want to do. It can feel as if you’re giving in and accepting that the inhibitor will always be present. In order to live a life where hemophilia is not the center of everything, making peace is crucial. “We have had enough experience that we know if the inhibitor is under 7 BU, we are living pretty good,” says Kari. Her family is not focusing on 0 BU, but for now, they know that anything under 7 BU is acceptable. “It’s really hard to not focus on the numbers, especially when you have the active inhibitor and either you need to get below 10 BU to start ITT, or you are doing ITT and trying to get down to zero,” says Eric. “We understand how hard that is. Focus on health. Focus on wellness.”
Numbers are essential for people living with inhibitors. Keep track of bleeding episodes because this is a significant tool to see if your treatment is appropriate. Continue your regular blood draws according to your provider’s recommendations. Even if you’re not a slave to the BU, it’s vital to monitor the progress of your inhibitor. The key is to enjoy life. Savor every moment. When things aren’t going well, try to remember that life will get better. And when life is good, soak it in.



Cazandra Campos-MacDonald is a motivational speaker, educator, and patient advocate for families with bleeding disorders. She writes a blog chronicling the journey of her two sons with severe hemophilia and inhibitors, and has written articles and blog posts for other publications. Cazandra’s older brother, Ronaldo Julian Campos, died of complications from hemophilia as an infant. Cazandra lives with her family, Rev. Joe MacDonald, Julian (20), and Caeleb (11), in Rio Rancho, New Mexico.
Click here to see Caz’s Ted Talk
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1. ITT is a proven treatment toward eradicating inhibitors. Larger-than-normal doses of factor are given in the hope of overriding the inhibitor. ITT protocols can differ in frequency of infusing, depending on the physician’s and individual’s needs.
2. Once you achieve 10 BU, it doesn’t matter if the BU gets lower, because all infused factor is inactivated in minutes. Even so, families living with an inhibitor will find emotional relief when the numbers get closer to zero.
3. D. M. DiMichele, W. K. Hoots, S. W. Pipe, G. E. Rivard, and E. Santagostino, “International Workshop on Immune Tolerance Induction: Consensus Recommendations,” Haemophilia 13(2007): 1–22.

Discover the Brand New LivingWithHemophilia.com!

I made a pledge this year to get all of LA Kelley Communications’ books updated and back in circulation, because I love educating people about hemophilia. And information needs to be updated in this ever-changing bleeding disorder community. Please check out Bayer’s newly revised website LivingwithHemophilia.com to learn more about hemophilia and to learn about its highly effective and successful leadership program, Leadership U.  

The following content is sponsored by Bayer.

Discover the Brand New LivingWithHemophilia.com!

There is no community quite like the hemophilia community—which is why Bayer set out to create an online destination unlike any other.

Introducing the all-new LivingWithHemophilia.com, reinvented and reimagined to engage patients and caregivers like never before. This is where you’ll get the scoop on everything from the role of genetics, to information on pain management, to tips for traveling—and all things in between—with content that’s engaging, easy-to-find and relevant to you.

It’s the real talk you want, served up the way you want it. For example, the “Living With Hemophilia On Your Own Terms” video series is designed to help you understand often confusing terminology related to hemophilia—so you can feel more informed than ever.

LivingWithHemophilia.com is also home for information on Bayer Leadership U, the summer internship program for college-aged students touched by hemophilia. For more than a decade, Bayer has offered motivated, young individuals a paid, six-week internship at their U.S. headquarters in New Jersey. Leadership U interns participate in activities that sharpen leadership skills and apply these skills in a real-world corporate setting through a variety of independent projects.

For more information on the internship program, visit www.LivingWithHemophilia.com/lead. The application deadline has been extended and will be open until Wednesday, February 15, 2017.

So, that’s the story behind the all-new LivingWithHemophilia.com. Make sure you check it out!

Hemophilia Myths and Facts

Myths are stories or beliefs created to explain phenomena when scientific knowledge is lacking. Sometimes they persist even when scientific explanations are available, because people can get emotionally impacted by myths. When it comes to blood, people have strong emotional beliefs and feelings, and this may prolong myths. So read this nifty list of myths which includes the facts that dispel the myth. You can download a pdf of this with graphics here.  Enjoy!
Hemophilia is a medical condition that impairs
the body’s ability to control bleeding. There are about 20,000 people with
hemophilia in the United States and with the condition being so rare, many
people may not know much about it. Below we dispel 10 common myths to help
you better understand hemophilia.
MYTH
FACT
If a person with hemophilia gets a minor cut,
they’ll bleed to death.
People with hemophilia don’t bleed faster, but
they do bleed longer because they’re missing a protein in their blood that
assists in forming stable clots. Minor cuts are not a cause for concern and
will usually heal on their own.1
Only males have hemophilia.
It’s true that hemophilia does primarily affect
males because it’s an X chromosome-linked medical condition.1,2
However, females who are carriers can also experience bleeding symptoms.
Though extremely rare, a daughter who is born to a father with hemophilia and
a mother who is a carrier can inherit the severe form of the condition.3
People with hemophilia always have a family
history of the condition.
Nearly one-third of hemophilia cases are not
genetically based and have no family history of the condition.1
All people who have hemophilia are diagnosed at
birth.
Due to a lack of bleeding at birth or because the
family doesn’t have a history of hemophilia, some people who have hemophilia
may not be diagnosed until months after birth, as toddlers, or even later,
depending on the severity of their
medical condition.4,5
Children with hemophilia will grow out of it.
Hemophilia is a lifelong condition, a bleeding
disorder caused by the absence of an essential blood clotting protein.1 While
there is no available cure for hemophilia, research continues to progress and
shows promise. Scientists are currently researching how gene therapy can help
the body produce its own clotting factor.2
Hemophilia is the same for all patients.
People can have hemophilia A, which is defined by
low levels of clotting factor VIII (8), or hemophilia B, defined by low levels
of clotting factor IX (9).1,3 The severity of hemophilia is
categorized as mild, moderate, or severe.1 Additionally, about 15% to 20%
of people with hemophilia will develop an antibody—also called an
inhibitor—which is one of the most serious complications of the condition.6
People with hemophilia shouldn’t exercise or play
sports.
Exercise is important for people with hemophilia
because it strengthens muscles, which helps protect joints, and reduces the
risk of being overweight, which places added stress on joints. They should
work with their health care professional to create a fitness plan that’s
right for them.7
Hemophilia is a “royal disease.”
While it’s true that in the 1800s hemophilia did
affect the royal family of Queen Victoria of England, hemophilia affects
people regardless of race, ethnicity or economic status.2,4
Hemophilia causes AIDS.
Hemophilia doesn’t cause HIV/AIDS. Unfortunately,
from 1978–1985, many people with hemophilia did contract HIV when they
received treatments made from tainted blood donations. The blood supply is
now much safer due to improved donor screening and advanced purification
methods, and also, many newer treatments aren’t made from donated human
blood. There hasn’t been a documented case of HIV from the use of hemophilia
treatments since 1987.2
People with hemophilia can’t live normal lives.
With proper treatment, most people with
hemophilia lead long, full, and productive lives.4
References
1.    National Hemophilia Foundation.
Hemophilia A. https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Hemophilia-A.
Accessed November 3, 2015.
2.    National Hemophilia Foundation.
History of Bleeding Disorders.
https://www.hemophilia.org/Bleeding-Disorders/History-of-Bleeding-Disorders.
Accessed November 3, 2015.
3.    Midwest Hemophilia Association.
Myths and Facts About Hemophilia.

Myths and Facts About Hemophilia


Accessed November 3, 2015.

4.    Indiana Hemophilia &
Thrombosis Center, Inc. Hemophilia Awareness Month – Fast Facts for Patients.
http://www.ihtc.org/wp-content/uploads/2012/02/Hemophilia%20Awareness%20-%20Fast%20Facts%20for%20Patients.pdf.
Accessed November 3, 2015.
5.    Centers for Disease Control and
Prevention. Hemophilia Diagnosis.
http://www.cdc.gov/ncbddd/hemophilia/diagnosis.html. Accessed June 7, 2016.
6.    Centers for Disease Control and
Prevention. Hemophilia Inhibitors.
http://www.cdc.gov/ncbddd/hemophilia/inhibitors.html. Accessed June 13, 2016.
7.    National Hemophilia Foundation.
Playing It Safe: Bleeding Disorders, Sports, and Exercise.
https://www.hemophilia.org/sites/default/files/document/files/PlayingItSafe.pdf.
Accessed November 3, 2015.
Novo Nordisk is a registered trademark of Novo
Nordisk A/S.
© 2016 Novo Nordisk
          All rights reserved.
              
USA16HDM02573                 
July 2016

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In Praise of PSI

So last Wednesday night I walk into an Irish pub in Haverhill, a city about 20 minutes from my home, with Kevin. “The Peddler’s Daughter” is a wonderful little piece of the old country, in a cozy cellar location, with great food and a lovely Irish atmosphere, run by real Irish people. It’s one of our favorite places to eat. Great fish and chips; homemade ketchup.

About an hour later a young man walks into the pub and heads to the bar, and I feel like i know him. Sure enough, I do. It’s Tom M., the young man who tiled my cellar last fall. At that time was part of a work crew from a local contractor, and he surprised me, as he was putting in the tile, when he said, “Does your son have hemophilia, too?” Apparently, he figured it out from the photos I have of Paul Newman and me at Camp Hole in the Wall (can’t miss that autographed picture, front and center as you walk in). We chatted and I learned he has mild hemophilia and lives in Haverhill. He and my son Tommy even have the same hematologist. Hmmm. I have people from all over the world on my mailing list but not someone who lives 15 minutes away? Well, he and his family were not active in the community. I added him to my mailing list, gave him a free copy of my book and sent him on his way, leaving me to enjoy my new floor.

Two months later Tom stopped by my office with news. “I quit my job,” he said hesitantly. As he is almost the same age as my Tom, I guess I kind of treated him with the same care and concern I would my own son. “What are you, crazy?” I said. No job, no insurance; no insurance, no factor. What was he thinking? He didn’t like his boss. And with no college education, it would be tough to find another job. I lectured him a bit and then armed him with a mission: call your hematologist; call your home care company and very quickly, call PSI. Tom had never heard of PSI. When I explained to him that it could save his life, I guess he listened.

Off he went and months went by. I didn’t hear from him. And then, in all the bars in all the world, he walks into mine.

“Hi, Tom?” I asked, eyeing him in the dim light.

“Mrs. Kelley!” he exclaimed, recognizing me. Then, “I got a job!”

Poor kid: now he’s treating me like his mother!

I was thrilled for him. Of course, there is a waiting period for those with pre-existing conditions… not to worry. Tom amazed me when he said, “I called PSI like you said and they are covering me until my new insurance kicks in.”

Wow. Here was a kid who never attended a hemophilia meeting, went to camp once, doesn’t know anyone else with hemophilia, quit his job not realizing the dire consequences, couldn’t name his factor brand… and he did it. He got himself back on track. I was so happy! He was too.

While the photo is very dim (cell phone + pub lighting) I hope you can see that we are nonetheless beaming! With my son gone off to college, Tom M., is now officially the person with hemophilia who lives closest to me. So I do kind of feel like his mom, and hope we meet again!

Kudos to PSI: you don’t know Tom but you have changed his life. Thank you, Dana, thank you everyone at PSI!

(To learn more about PSI and how they help people with chronic disorders who lose their insurance, go to www.uneedpsi.org)

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