February 2021

Making healthy habits stick, with help from BleedingDisorders.com

This is a paid public announcement from Takeda and does not constitute an endorsement of products or services. When you click on the links in this blog entry, you will be directed to the Takeda website. LA Kelley Communications always advises you to be a savvy consumer when contacting any company; do not reveal identifying information against your will.

With everything that life throws at you, it’s not always easy to make healthy habits stick. But for people with a bleeding disorder, diet and exercise are two important ways you can help keep your body—and especially your joints—healthy.1,2

Here are a few quick tips from BleedingDisorders.com that could help you maintain a healthy weight and prevent joint damage.

Eating right and staying strong

An apple a day might not actually keep the doctor away, but eating right is a key element of living a healthy lifestyle. And that might be even more true for people living with bleeding disorders.1

While there isn’t a special diet recommendation for people with bleeding disorders, many of the same guidelines from the USDA apply: more fruits and veggies, less solid fat and sugar.3 But that doesn’t mean you can’t eat the foods you love. Try looking up new recipes that include more healthy ingredients. Cauliflower pizza crust might be your new weeknight favorite!

You can get more info about healthy eating habits at BleedingDisorders.com. And before starting any diet, remember to check with your healthcare provider (HCP) or Hemophilia Treatment Center (HTC).

To keep your body healthy, keep it moving

Exercise is another key aspect of maintaining joint health and staying at a healthy weight.2 While people with bleeding disorders used to be discouraged from participating in sports, modern treatment means that many people can continue to be physically active. In fact, it’s encouraged.2,4

Keep in mind that not all physical activities may be safe for you to do. The National Hemophilia Foundation’s Playing It Safe pamphlet includes a helpful list of physical activities and the level of risk associated with them.4 Here are some examples of low- and high-risk activities.

This is another area where it’s important to talk with your HCP or get in touch with a Hemophilia Treatment Center before getting started. And if you want more info about different types of exercises and how they may help you, check out BleedingDisorders.com.

Stay connected

Feeling inspired to making some healthier life choices? Your first step might be to get in touch with your Hemophilia Treatment Center, where you can get expert advice about managing a bleeding disorder. Your healthcare provider team can also help with any questions about diet and exercise. And of course, connect with the Bleeding Disorders community on Facebook, Instagram, YouTube, and Twitter, and sign up to get the latest news and updates.

References:

  1. Thomas Smith K. Weighty matters. HemAware website. January 30, 2015. Accessed January 27, 2021. https://hemaware.org/mind-body/weighty-matters
  2. Goto M, Takedani H, Yokota K, Haga N. Strategies to encourage physical activity in patients with hemophilia to improve quality of life. J Blood Med. 2016;7:85-98.
  3. U.S. Department of Agriculture and U.S. Department of Health and Human Services. Dietary guidelines for Americans 2010. December 2010. Accessed January 27, 2021. https://health.gov/dietaryguidelines/dga2010/dietaryguidelines2010.pdf
  4. Playing it safe: bleeding disorders, sports and exercise. National Hemophilia Foundation. 2017. Accessed January 27, 2021. https://www.hemophilia.org/sites/default/files/document/files/playing-it-safe_0.pdf

Copyright © 2021 Takeda Pharmaceutical Company Limited. Lexington, MA 02421. 1-800-828-2088. All rights reserved. TAKEDA and the TAKEDA logo are trademarks or registered trademarks of Takeda Pharmaceutical Company Limited.

[US-NON-4339v1.0 02/21]

A Passion for Music

Richard Atwood

Have you heard of the Currence Brothers? This band from West Virginia played and recorded gospel, bluegrass, and country music in the 1970s. The band members were known for their musical talent, especially with the banjo and fiddle. And two Currence brothers, plus their nephew, happened to have hemophilia. These band members overcame hardships to fulfill their passion for music.

            For five generations, the musically inclined Currence family lived in High Germany on the Randolph-Upshure county line in central West Virginia. They lived in a six-room house on a small farm, four miles from a paved road. The nearby one-room school in High Germany housed 45 students in eight grades.

            Every member of the large Currence family—including seven boys and five girls—either played a musical instrument or sang, though none was trained or ever had a music lesson. The children and their parents would listen to a battery-powered radio or hear live entertainment, then pick out the tune on guitar, fiddle, or mandolin. At home or in church, the family members sang and played musical instruments.

            There appeared to be no family history of hemophilia. That is, until one of the Currence boys, Junior (1923–1925), died at age two after he bled out from a bumped nose, and hemophilia was later suspected. Junior’s younger brothers Jimmie (1932–1992) and Loren (1934–1987) grew up with bleeding issues, but they never visited a doctor until they were teens. There was no ice for treatment and nothing for pain, though the reason for this is unclear. The brothers applied high-powered liniments from Blair products for hemorrhages into their joints.

            Jimmie and Loren seem to have visited at least one local doctor, and they were treated as “bleeders.” It’s possible that lack of money, transportation, and awareness of the need for a hematologist may have prevented visits to an appropriate specialist or hematology lab. Before factor VIII factor concentrates became available, the brothers received blood transfusions. Loren once received sixteen pints of blood for a kidney bleed. When Jimmie had a stomach bleed as a teenager, he received a pint of blood from his brother-in-law. The boys were often laid up from hemorrhages into their joints or from other internal bleeds.

            Jimmie and Loren didn’t have their hemophilia diagnosed until the early 1960s. Dr. Mabel M. Stevenson, a hematologist at Morgantown University Hospital, diagnosed severe classical hemophilia in both brothers, who then called themselves “severe hemophiliac bleeders with near-zero clotting factor.” Neither brother could ever find full-time employment or get insurance. They both received supplemental social security, and the state of West Virginia paid for their medicine because they couldn’t afford it. Both brothers married, and each had three daughters.

            Brothers Jimmie, Loren, Marvin (“Shorty”), and Buddy—along with Malcomb Pastine, a nephew who also had hemophilia—formed the Currence Brothers Band. Loren played guitar, sang, and managed the band. Jimmie played fiddle and banjo, winning banjo and fiddle contests in four states: West Virginia, Maryland, Pennsylvania, and Virginia. Jimmie won the fiddling championship in West Virginia for four consecutive years, and was undefeated grand champion in Maryland in 1970. Then his elbow bleeds forced him to switch from fiddle to banjo. Malcomb played guitar, and even did turkey calls. Buddy sang, while Marvin played the fiddle.

            The Currence Brothers began spreading the gospel by playing their music at church functions. They traveled the country, usually in their van, to play in concerts and on radio or television. The band even recorded on their own, or with notable musicians, producing several albums in a genre they called Gospel Grass, along with a mixture of popular tunes. They collaborated with musicians including Larry Groce, who they met at a talent show at the firehouse in Parsons, West Virginia; Woody Simmons, who lived in Randolph County; Clyde See; and “Country” Charlie Jordan.

            You can find Currence Brothers music on old vinyl records and in a few YouTube videos. Check out the following albums:

   • The Currence Family, Precious Memories (Lark Records, n.d.)

   • Larry Groce and the Currence Brothers (Peaceable Records, 1975)

   • Larry Groce, Junkfood Junkie (Warner Bros. Records, 1976)

   • The Currence Brothers, He’s Coming Back to Earth Again (Major Recording Studios, n.d.)

   • The Currence Brothers Sing and Play Gospel Grass (Major Recording Studios, n.d.)

   • The Currence Brothers, Ballads and Instrumentals (Major Recording Studios, 1978)

   • The Currence Brothers, Muddy Boggy Banjo Man (Major Recording Studios, 1979)

   • Woody Simmons, All Smiles Tonight (Elderberry Records, 1979)

   • The Currence Brothers, That’s the Man I’m Looking For (Augusta Records, 1981)

   • Clyde See with the Currence Brothers (Augusta Records, 1983)

            The Currence Brothers are known for their talent on the banjo and fiddle, not for having band members with hemophilia. Their hemophilia did not define them, and it never prevented them from making music. Of course, some accommodations were made—switching instruments due to elbow bleeds; not playing at some events or traveling as much as they wanted—but not at the expense of their artistry and passion. Today, other family members continue the Currence musical tradition.

To learn more, read profiles of the Currence Brothers and other West Virginia musicians in Mountains of Music (University of Illinois Press, 1999), edited by John Lilly.

Have a (Healthy) Heart!

Today is Valentine’s Day, a day associated with celebrating love, affection and romance. The symbol of Valentine’s Day? A heart. Well, not an anatomical heart, but a rendering of it. The symbol we use now was popular as far back as the Middle Ages, and was popular during the Renaissance,  as seen in art depicting the Sacred Heart of Jesus. By the 18th century, it was being used in Valentine Day cards. The origins of St. Valentine’s Day (to be specific) are found in Christian and even Roman tradition.

The heart is often associated with feelings: we describe someone as being “all heart,” having a “big heart,” or being “heartless.” Young children, often unaware of the contents of their own bodies, can still draw a heart (and a stomach!) when asked to draw what’s inside of them. Feeling the heart beat, they know they are alive. (Feeling their stomach hurt, they know they are hungry) Seeing themselves bleed, they worry,  mainly about their heart not working. They learn that the heart pumps blood; when they see a bleed, they may fear bleeding to death. Especially if they have hemophilia!

So we have to be careful when we educate our children about their heart, blood and hemophilia. My book Teach Your Child about Hemophilia will help! (Order here)

And the heart has an interesting connection in hemophilia beyond making our blood travel about the body. Sarah Aldrich reported in HemAware, the magazine of the National Hemophilia Foundation, that in a six-state study of more than 3,400 men with hemophilia, CDC investigators found that after HIV and intracranial bleeds, the third most common cause of death was heart disease. This was comparable to nonhemophilic men. Hemophilia does not give you a Valentine’s Day get-out-of-jail-free card just for having hemophilia!

Subsequent studies confirmed this. Atherosclerosis, coronary artery disease… all show up in older men with hemophilia. Unlike hemophilia, these are linked to lifestyle. Stress, diet, smoking and obesity surely have a negative impact on heart health.

Be nice to you heart on Valentine’s Day. Refuse the chocolate! (I did) Start thinking of ways to exercise safely; eliminate high sodium and processed foods; cut down on alcohol; stop smoking. Especially stop smoking! And for your little ones with hemophilia, learn how they understand how the body works. It might be quite different than what you think! When they give you a Valentine’s Day card, it might be a good idea to get a conversation going about hearts, health and hemophilia!

Happy Valentine’s Day!

https://hemaware.org/life/heart-health-and-hemophilia 2.6.13

Inhibitors 101

Paul Clement

Paul Clement

For many parents of children newly diagnosed with hemophilia, the word “inhibitors” soon becomes part of their vocabulary. And although they may not know at first what an inhibitor is, they may have learned to associate the word with something fearful. But for people with hemophilia A and inhibitors, things aren’t as bad as they once were.

What exactly is an inhibitor? Who gets them? What happens when you get an inhibitor? How do you treat bleeds if you have an inhibitor? Do inhibitors go away on their own, or can you grow out of them or eliminate them?

What Is an Inhibitor?

Inhibitors are specialized proteins called antibodies. They’re a part of the immune system that protects us from bacteria, viruses, and foreign proteins—in other words, anything that the body identifies as not belonging, and as being potentially harmful. But sometimes the immune system makes mistakes: it may even attack the body itself, as in autoimmune diseases including rheumatoid arthritis or multiple sclerosis. With hemophilia, the immune system also makes a mistake: it misidentifies a helpful agent—infused clotting factor—as something harmful, and then mounts an immune response against the factor to neutralize it and mark it for removal from the body.

Inhibitors are very efficient. When an inhibitor is present in hemophilia, some or all of the infused factor is neutralized within minutes. This prevents the factor from participating in the clotting process to stop bleeding. And it means that people with inhibitors can’t use standard clotting factor concentrates to control bleeds.

Unfortunately, the alternative therapies we have for treating bleeds with inhibitors aren’t as effective as standard factor at controlling bleeds. As a result, people with inhibitors tend to bleed longer, develop target joints (joints that bleed frequently), and suffer from joint damage more often than people without inhibitors. Fortunately, for people with hemophilia A and inhibitors, treatment has improved dramatically over the past three years.

Diagnosing Inhibitors

How do you know if you have an inhibitor? There are usually no outward signs. Inhibitors are sometimes diagnosed during routine hemophilia treatment center (HTC) clinic visits; and sometimes inhibitors are suspected after you notice that factor infusions fail to adequately control bleeding. Your HTC should test for inhibitors at least annually and always before any surgery, and you should request a test whenever you feel that bleeds aren’t being controlled effectively with your usual dose of factor.1

 When an inhibitor is suspected, a diagnostic test called a mixing study (activated partial thromboplastin time, or aPTT) is performed: the patient’s blood plasma is mixed with normal plasma to see if this corrects the clotting time. In someone with hemophilia without an inhibitor, a mixing study results in a normal clotting time; but if an inhibitor is present, then the clotting time is abnormally prolonged. If this happens, then another test, the Bethesda inhibitor assay, is done to determine how much of the inhibitor-causing antibody is present.2 The Bethesda assay is a quantitative assay, meaning that it measures the amount of inhibitor and the results are expressed in numbers.

Note: Testing for inhibitors is a bit tricky. It’s best to have a Bethesda assay done at an HTC, because the lab techs there have more experience performing the tests, and the results are more likely to be accurate when compared to tests done at other hospitals.

Strength of the Inhibitor

To develop a strategy for treating bleeds, your doctor will need to know the strength, or concentration, of the inhibitor. The inhibitor strength is reported as a “titer” and is expressed in Bethesda Units (BU).3 Inhibitor titers can be as low as 1 BU or higher than 10,000 BU.

An inhibitor titer less than or equal to 5 BU (≤5 BU) is considered a low-titer inhibitor. An inhibitor greater than 5 BU (>5 BU) is considered a high-titer inhibitor. If you have a low-titer inhibitor, you can still use standard factor to treat bleeds, although in higher doses to accommodate for some of the factor being neutralized by the inhibitor. If you have a high-titer inhibitor, standard factor concentrates are not effective because all the factor is quickly neutralized after an infusion.

In addition to the inhibitor titer, inhibitors are categorized by how the immune system responds to infused factor. For some people, the inhibitor titer stays more or less stable and doesn’t rise after the patient is exposed to factor. If your child has an inhibitor titer ≤5 BU, and it remains at or below 5 BU for several days after an infusion, he is a low responder.

For others, when factor is infused, the immune system quickly ramps up inhibitor production in an effort to neutralize the infused factor. This results in an increase of the inhibitor titer within four to seven days of exposure to factor, peaking within one to three weeks. This ramping up of inhibitors after factor exposure is an anamnestic response (meaning a memory or recall response). If, after exposure to factor, the inhibitor titer rises above 5 BU over a few days, then your child is classified as a high responder. High-responding inhibitors are more challenging to treat than low-responding inhibitors because normal factor concentrates are useless with high-titer, high-responding inhibitors. Treating bleeds with these inhibitors requires special factor concentrates called bypassing agents, such as FEIBA or NovoSeven.

 But there’s one case where standard factor can be used to treat bleeds in high responders. In high responders, the immune system often produces fewer and fewer antibodies over time if it isn’t exposed to factor. If someone hasn’t been exposed to factor for several months, then the inhibitor titer may have decreased to a level low enough that normal factor concentrates may be used to treat bleeds successfully for a few days—that is, before the anamnestic response kicks in and the inhibitor titer increases again, making the factor ineffective.

1. You can get free inhibitor testing at federally funded HTCs by participating in the Centers for Disease Control and Prevention’s (CDC) Community Counts Registry for Bleeding Disorders Surveillance program.  2. There are several different types of inhibitor assays; the Bethesda assay is the most widely used.  3. A Bethesda Unit (BU) is the amount of an inhibitor that will neutralize 50% of factor VIII in normal plasma after 120 minutes’ incubation at 37°C.

ADVERTISEMENT
HemaBlog Archives
Categories
LKelleyAds
ADVERTISEMENT

A Note Regarding COVID-19 and Shipping:
LA Kelley Communications will continue to ship orders as long as the post office remains open. However, it's likely that orders will be slightly delayed. For any questions, please don't hesitate to contact us at info@kelleycom.com
Stay Healthy!