November 2018

China: Aggressive Growth, but Hemophilia Lags

Laurie Kelley November 25, 2018

There’s no describing China adequately: it’s massive, it’s spectacular, it’s impressive, it’s ancient. I visited on holiday from November 1 to November 17 as part of a Viking Cruise, part of which took us all down the Yangtze River, to view the Three Gorges and the incredible Three Gorges Dam, the world’s largest power station. And everywhere we looked there is construction. Gigantic tower cranes dominate the skyline in all the cities we visited, like mechanical birds dipping down into a field of high-rise apartments that jut up and on forever like a field of metal grass. I’ve seen some things in my travels to over 3o developing countries, but nothing like this. As we all gazed at the endless rows of enormous apartments, stacked like a line of dominos, I felt like we were in a Blade Runner sequel. Each city stunned us more than the previous one.

China recently surpassed Japan as the world’s second largest economy. You can see economic activity everywhere; it is a country on the move. And yet, I found myself wondering about medical care for those with hemophilia. How does China care for its patients?

Because it was a vacation, after all, I didn’t research anything about hemophilia. But I did get a chance to visit a school, supported by Viking Cruises. It is beautiful and the children treated us to a lovely performance of acrobats and dancing with fans. When I asked our guide if there were any children with disabilities at the school, he quickly said no. Might there be some children allowed sometime in the near future? Not likely.

And despite all the construction, there are no handicapped facilities. People with handicaps are seen much as a burden, in other words, like in any developing country. I do know that due to the one child rule (recently changed to allow two children per family), parents who give birth to a child with hemophilia may be tempted to drop the child off at an orphanage. We’ve seen in the last 20 years many children coming from China with hemophilia, now being adopted into American homes.

While I was in Shanghai, for the final days of the vacation, I contacted an old friend and colleague, Delin Kong. Delin contacted me back in the 1990s as he wanted to start China’s first patient organization. At the time, my company provided leadership training and supported patients like him in developing countries. We assisted, gave China its first funding for a hemophilia patient organization, and Delin and his team did the rest. We provided factor donations up until recently.

It turned out Delin lives only a few blocks from the Westin, so he visited on our final day. How good it was to see him after so many years! However, I noted his severely crippled joints. He had great difficulty walking. Luckily, he gets government assistance, and used to be a financial trader and did quite well for himself! But he is in pain.

Laurie Kelley with children at Viking-supported school

I asked about hemophilia and was surprised to know that patients in China still do not have home therapy. For every bleed, they must go to the hospital, according to Delin. This is always a major problem in developing countries. No matter how much factor gets donated, patients will suffer long term if they cannot infuse as needed at home.

And a knee replacement? Delin is on a waiting list for 6 months or more. He has asked repeatedly, but is put off by the medical team. From not enough factor to not enough medical help, Delin, despite China’s economic standing in the world, still suffers like others in worst off countries. My hope is that combined with patient advocacy, the national or provincial government may at long last meet the needs of its people with hemophilia.

Visiting China opened my eyes to its beauty, history and aggressive growth, but medical care still lags, at least for those with hemophilia.

Hemophilia For Young Readers, Part 2

Laurie Kelley November 18, 2018

Originally published in PEN November

written by Richard J. Atwood

Here are more books about hemophilia for younger readers. In the August issue of PEN, we surveyed books with fictional main and supporting characters who happen to have hemophilia. Other literary genres, such as biography, time-travel fantasy, as well as some unexpected references, also include hemophilia. Something for everyone.

Follow your curiosity by exploring different genres of literature for younger readers. I suggest the following books for your enjoyment and learning.

Biography

The Uncanny (Sterling, 1998)

Peter Hepplewhite and Neil Tonge

The Unexplained series of children’s books for young adults includes this story of how Rasputin, or the Mad Monk, used his powers to heal a joint bleed that Alexei Romanov, son of the Russian tsar, experienced because of his hemophilia in 1907. The text teases the reader, but doesn’t supply enough information on the full story of Rasputin.

Blood Red Snow White: A Novel of the Russian Revolution (Orion, 2007)

Marcus Sedgwick

Though this children’s biography is based on documented facts, it could also be considered a spy novel. In 1913, Arthur Ransome, a writer of children’s books, leaves his family behind in England to become a newspaper correspondent in St. Petersburg. There he learns of Alexei, who has hemophilia, and of Rasputin with his healing powers. Ransome marries the private secretary of Leon Trotsky (1879–1940; a leader of the Russian Communist Party after the 1917 Bolshevik Revolution), becomes a British agent, and returns to England in 1942 to write more children’s books.

Germ Stories (University Science, 2008)

Arthur Kornberg

These 10 profiles of germs are written for young readers as poetry, with catchy rhymes accompanied by dynamic illustrations and photographs. The story of HIV mentions the fictional Bill, a second grader with hemophilia. The references to hemophilia and AIDS seem dated, but readers may not notice.

Patrick’s Wish (Second Story, 2010)

Karen Mitchell with Rebecca Upjohn

In this biography for young readers, with accompanying family photographs, Lyanne tells the story of her older brother, Patrick Fortin (1978–2001), who had hemophilia and AIDS in Canada. Patrick’s wish was a cure for AIDS, and before her brother died, Lyanne promised to tell Patrick’s story.

The Quiet Hero: A Life of Ryan White (Indiana Historical Society, 2015)

Nelson Price

This young adult biography summarizes the accomplishments of Ryan White (1971–1990), who became a national celebrity because he had both hemophilia and HIV. Ryan wanted to continue attending school in Indiana even with serious medical conditions. Taking advantage of all the publicity, Ryan became a spokesperson for AIDS education. Unfortunately, while hemophilia was controlled, AIDS was deadly. This book helps us remember Ryan.

Time-Travel Fantasy

Travel through the space-time continuum is a popular topic for young readers.

The Curse of the Romanovs (Margaret K. McElderry, 2007)

Staton Rabin

Alexei Nikolaevich Romanov, 12-year-old son of the Russian tsar, has hemophilia. He uses visualization techniques taught by Rasputin to save his own life by escaping Russia in 1918. Alexei travels through time and space to New York City in 2010. There he meets Varda Ethel Rosenberg, his 15-year-old distant cousin whose father with hemophilia died of AIDS. Varda saves Alexei by traveling back to St. Petersburg in 1918. Ages 12 and up.

Secrets of the Survivors (Xlibris, 2008)

Mark L. Eastburn

This young adult fantasy novel, written by an author with von Willebrand disease, tells the story of 10-year-old Alex Hidalgo and his younger sister Katherine, from Philadelphia. The siblings are contacted by survivors of an intelligent ancient reptilian civilization from 65 million years ago. In their struggle to save planet Earth, Alex and his sister enlist three other classmates, one of whom has hemophilia.

The Queen Must Die: Chronicles of the Tempus (Atlantic, 2010)

K.A.S. Quinn

The author admits that she “tinkered” with the facts in this children’s novel, part of a trilogy that involves travel through time and space. Katie Berger-Jones falls asleep in New York City while reading the letters of Queen Victoria’s daughters, and then wakes up in Buckingham Palace in London, 1851. There Katie meets Prince Leopold, who has hemophilia, before she struggles to return home on the same day she left. Leopold was actually born in 1853.

What’s Up with Louis? Medikidz Explain Hemophilia (Medikidz, 2011)

Dr. Kim Chilman-Blair and Shawn deLoache

This storybook comic features five Medikidz who live on the planet Mediland. When Louis, on Earth, is mocked by his friends for having hemophilia, the Medikidz teleport him to their planet. Once he discovers his potential, Louis returns to Earth knowing how to properly treat his hemophilia.

Risked (Simon & Schuster, 2013)

Margaret Peterson Haddix

This science fiction novel tells the story of 13-year-old Jonah Sizemore and his younger sister Katherine, from Ohio, who help rescue 36 famous missing children from the past. These missing children, now using aliases, include Anastasia Romanova and Alexei Romanov, who has hemophilia. With the aid of a time-travel device, the children attempt to alter history by traveling back to Russia in 1918. Ages 8–12.

Hemophilia in Unexpected Places

Authors of fiction for young readers may refer to hemophilia in unexpected ways.

Tiger Eyes (Bradbury, 1981)

Judy Blume

This young adult novel follows 15-year-old Davey Wexler when she moves with her family to Las Alamos, New Mexico. At her new school, Davey views a film on hemophilia, but she already knows about hemophilia because she read Nicholas and Alexandra in eighth grade.

Fallen Angels (Scholastic, 1988)

Walter Dean Myers

In this young adult novel, Richard Perry graduates from high school in Harlem, New York, and enlists in the army in 1967. Richard declines a bonding ritual of mixing blood with other black soldiers by claiming to have hemophilia. Another soldier notes that hemophilia is a medical deferment from military service.

Billy’s Boy (Wildcat, 1997)

Patricia Nell Warren

Included in a series on gay family life, John William Heden is age 12 in 1989. He moves with his single, lesbian mother to Malibu, California. Searching for his father, supposedly a gay Olympic track medalist who donated his sperm before he died, John William meets Michael, who is conducting research at UCLA on inherited blood diseases like hemophilia.

Summertime Blues (Oxford University, 2001)

Julia Clarke

In this coming-of-age novel for young adults, 17-year-old Alexander Harling, from London, responds to a rude comment about his mother. Alexander beats up the guilty classmate, who bleeds so much that Alexander thinks the boy is hemophilic and needs a transfusion.

The Healing Time of Hickeys (Polestar/Raincoast, 2003)

Karen Rivers

In this teen novel written as a diary, Haley Andromeda Harmony is a 16-year-old Canadian high school senior in 2002. Because she’s a hypochondriac, Haley searches internet sites for a definition of hemophilia. When she skins her knuckles, Haley then believes she is bleeding to death due to her supposed hemophilia.

Birthmarked (Roaring Brook, 2010)

Caragh M. O’Brien

As part of a fiction trilogy for young readers, this novel follows 16-year-old Gaia Stone, who lives in a divided country and trains as a midwife in the year 2409. Gaia learns about the health problems of dying children and infertile mothers due to inbreeding in the privileged city. The biggest concern is an increasing number of children with hemophilia. Sadly, hemophilia is not cured in the future.

The Truth About Celia Frost (Usborne, 2011)

Paula Rawsthorne

In this young adult thriller, 14-year-old Celia Frost is born with a rare blood-clotting disorder for which there is no treatment—or so her overprotective mother claims. When a classmate cuts Celia, she goes to the local hospital. Her mother refuses to allow any blood tests. After arguing with her mother, Celia learns the truth: her mother abducted her from a research laboratory, where as a child, Celia had

been experimentally injected with live viruses. To prevent the spreading of the viruses, her mother employed what is now called Munchausen Syndrome by proxy,¹ imposing a fake medical diagnosis on a child. Meanwhile, the research doctor tries to capture his escaped research subject, putting Celia’s life in danger. You can find hemophilia included in a wide variety of literary genres. Literature meant for younger readers can be enjoyed by all ages, including adults. Find something you and your child like, pick up a book, and read.

Unfortunately, the author never uses the label Munchausen Syndrome by proxy (MSBP), or ever attempts to fully explain the fictional mother’s psychiatric condition. In this rare condition, parents fabricate the physical symptoms in the child, producing a chronic fictitious disorder, most often without the child’s awareness or willingness. For hematology MSBP, this usually involves parents forcing their child to ingest an anticoagulant to induce bleeding symptoms.

Explore the possibilities of individualization

admin November 9, 2018

When I was raising a child with hemophilia over 20 years ago, all factor dosing was based on our child’s weight and severity of an injury. No one ever talked about PK. What is PK, you ask? Read below and find out! It’s essential to know if you want to individualize the dosing schedule for you or your loved one with hemophilia!

This is a paid public announcement from Shire 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 Shire website. LA Kelley Communications always advises you to be a savvy consumer when contacting any company; do not reveal identifying information against your will.

We know that regardless of any similarities, no two individuals with hemophilia are the same. For example, you and another person with hemophilia who are the same age and weight may require different amounts of factor, depending on how your body uses factor.¹

One of the key elements in individualizing factor treatment is understanding and utilizing pharmacokinetics (PK). This is the study of how your body uses the medicine you take,²which in turn helps predict the factor coverage that is available within your body. PK helps predict how the treatment is working with your body. Remember, no two individuals are the same, so factor is used or removed from the blood at different rates depending on your individual PK profile.³

Your PK profile is developed during PK analysis, where your healthcare provider (HCP) draws your blood at very specific times to determine your factor levels.^4,5 PK analysis helps your HCP identify and understand^4,6,7:

Highest level of factor in your body after infusion (peak)
Lowest factor level after time has passed (trough)
Amount of time it takes for half of the infused factor to be removed from the bloodstream, known as the half-life

This PK information can help your HCP determine an optimal treatment plan for you by adjusting your infusion dose and frequency (how often factor is infused), based on how your body uses factor.¹ Your HCP will also take a close look at your lifestyle and other activities, as these can have an impact on your bleeding risk. As you can see, there are many different characteristics to consider when determining the right treatment plan for you.

Individualizing prophylaxis (routine infusion of factor to prevent bleeds) may help improve the likelihood of zero bleeds while also helping to preserve joint health.⁸ Your joint health is very important; every joint bleed matters. It is recommended to start prophylaxis early, especially in childhood or adolescence, to help preserve your joints.⁹

We at Shire understand that individualized factor treatment is key. It is necessary to tailor your factor treatment to meet your unique needs. When it comes to treating hemophilia, there is no one-size-fits-all approach.¹

Talk to your healthcare provider about factor treatment— an option that lets you individualize your prophylaxis regimen to meet your unique needs. Visit www.bleedingdisorders.com to learn more about factor treatment.

References

Valentino LA. Considerations in individualizing prophylaxis in patients with haemophilia A. Haemophilia. 2014;20:607-615.
Le J. Overview of pharmacokinetics. Merck Manual. https://www.merckmanuals.com/professional/clinical-pharmacology/pharmacokinetics/overview-of-pharmacokinetics. Accessed July 17, 2018.
Collins PW, Björkman S, Fischer K, et al. Factor VIII requirement to maintain a target plasma level in the prophylactic treatment of severe hemophilia A: influences of variance in pharmacokinetics and treatment regimens. J Thromb Haemost. 2010;8(2):269-275.
Poon MC, Jackson S, Brown M, McClure W. Clotting factor therapy. All About Hemophilia: A Guide for Families. Montreal, Quebec, Canada: Canadian Hemophilia Society; 2010:1-33.
Lee M, Morfini M, Schulman S, Ingerslev J; and Factor VIII/Factor IX Scientific and Standardization Committee on the International Society on Thrombosis and Haemostasis. The design and analysis of pharmacokinetic studies of coagulation factors. International Society on Thrombosis and Haemostasis. https://c.ymcdn.com/sites/www.isth.org/resource/group/d4a6f49a-f4ec-450f-9e0f-7be9f0c2ab2e/official_communications/fviiipharmaco.pdf. Published March 21, 2001. Accessed June 1, 2018.
University of Virginia Health System. Obtaining trough blood levels. https://med.virginia.edu/neurology/wp-content/uploads/sites/235/2015/11/obtaining-trough-blood-levels.pdf. Accessed July 27, 2018.
Medical Dictionary. Definition of Cmax. Farlex Inc: Huntingdon Valley, PA; 2018. http://medical-dictionary.thefreedictionary.com/Cmax. Accessed July 30, 2018.
Poon M, Lee A. Individualized prophylaxis for optimizing hemophilia care: can we apply this to both developed and developing nations? Thromb J. 2016;14(suppl 1):65-71.
Bertamino M, Riccardi F, Banov L, et al. Hemophilia care in the pediatric age. J Clin Med. 2017;6(54):1-13.

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All rights reserved. 1‐800‐828‐2088.
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Hemophilia Fiction for Young Readers

Laurie Kelley November 4, 2018

written by Richard J. Atwood

Originally published in August PEN

Learning to read is a significant milestone in our personal development. As adults, we nudge young readers along in this quest. First we read to them, and later we encourage and supervise for age- and skill-appropriate reading materials.

Our bleeding disorder community is fortunate to include notable authors who write about hemophilia. Their books for young readers are educational and entertaining. Look for short reviews of these books in PEN’s Biennial Bleeding Disorder Resource Guide.

Other authors who write for younger readers sometimes include hemophilia. I suggest the following books.

Main Characters with Hemophilia

Starring Peter and Leigh (Delacorte, 1979); The Friendship Pact (Scholastic, 1986)

Susan Beth Pfeffer

What could be better than a fictional leading character with hemophilia? Peter Sanders, a 17-year-old with hemophilia on Long Island, becomes stepbrother to 16-year-old Leigh Thorpe. Leigh, a former child actor, wants to be a normal teen, while Peter, often bedridden, wants to attend school. Pfeffer also included hemophilia in her pre-teen novel The Friendship Pact. Tracy Newfield, age 12, has a crush on a young actor, Ross Perlman. Ross is a Dartmouth graduate who gives a benefit concert for National Hemophilia Foundation and dedicates his unreleased song to Tracy. Hemophilia in these novels seems dated, yet the descriptions are accurate for the time period.

Panda Bear Is Critical (Macmillan, 1981); Picture Perfect (Severn House, 2000)

Fern Michaels

Michaels wrote Panda Bear, a suspense novel, and later retitled it Picture Perfect after making some changes. Five-year-old Davey Taylor stays with his aunt and uncle while his scientist parents travel to Florida to testify in a mob-related drug trial. Davey has hemophilia, for which he receives daily “antigen shots.” During a camping trip, Davey is kidnapped without his hemophilia treatment. He must cunningly escape, aided by his Yorkshire terrier and his CB radio, so he can be treated before it’s too late.

She Died Too Young (Bantam, 1994); All the Days of Her Life (Bantam, 1994); A Season for Goodbye (Bantam, 1995)

Lurlene McDaniel

This series of three inspirational novels deals with life-altering situations for children and young adults, ages 10 and up. In the series, teens with various medical conditions attend summer camp at Jenny House in Asheville, North Carolina. One camper is Jeff McKensie, who has hemophilia. Jeff moves from Colorado to Miami to study to be an architect, yet his romantic opportunities at camp are stymied due to his medical condition.

Diving for the Moon (Macmillan, 1995)

Lee F. Bantle

Bantle, a lawyer, tackles issues of puppy love, adolescence, hemophilia, and HIV in this novel for ages 9–12. Josh Charkey, age 12, has hemophilia and AIDS. Spending the summer at a Minnesota lake, Josh shocks his close friend Carolina Birdsong with his medical revelations, while still fostering their budding romance.

Uphill and Into the Wind (Royal Fireworks, 1996)

Willard Helmuth

In this young adult novel, 12-year-old Eric Kenton, who has hemophilia, wants to be normal. While his parents worry about hemophilia and HIV, Eric dreams of, then successfully accomplishes, riding his fat-tired Huffy bicycle almost 400 miles to a summer camp in Ohio.

Kinetic (DC Comics, 2005)

Kelley Puckett and Warren Pleece

Puckett and Pleece wrote and illustrated this storybook comic fantasy. Tom Morrell is a high school senior who has hemophilia, plus about a dozen other syndromes. Tom gains superpowers when he is hit by a truck, and the absorbed energy amazingly “cures” his medical conditions.

The Healing of Ryne O’Casey: A Novel (FaithWalk, 2004)

Scott Philip Stewart

Ryne O’Casey, a 10-year-old from Tynbee, Tennessee, is first diagnosed with “hemophilia type A” when just over a year old, and then diagnosed with HIV at age 8. Some of the medical details are questionable for 1995, but the social reactions are truly believable.

The Lorin Solo (self-published, 2006)

Chuck Edwards

This young adult romance novel follows Lorin Lenki, a music student at San Francisco State who has mild hemophilia. Lorin meets Tracy Martin, a nursing student at Saint Rita’s Hospital. Tracy has severe hemophilia, as did her identical twin sister who died. The budding romance tragically ends when Tracy dies from a brain bleed in 1986.

Jovenes vampiros. El codice secreto (versos y trazos, 2007)

Jose Aguilar

This illustrated novel, written in Spanish, tells the tragic love story of young Marco Tulio and Isabel, who has hemophilia. The setting is ancient Italy, and includes vampires and Leonardo da Vinci.

Supporting Characters with Hemophilia

Something Different in the Bank (E. J. Arnold and Son, 1989)

Grace Moulton

Something Different in the Bank is one of a series of books about children with medical conditions. The narrator describes his cousin Michael, who has hemophilia. The treatment of hemophilia seems out-of-date even for 1989, though some of the behavioral concerns are timeless.

Monkey Island (Bantam, 1991)

Paula Fox

This teen novel’s awards include an American Library Association Best Book for Young Adults, and a Horn Book Fanfare Selection. Clay Garrity, age 11, is homeless in New York City. When Clay is hospitalized for pneumonia, his roommate, an experienced hospital patient with hemophilia, provides sage advice.

Play to the Angel (Scholastic, 2000)

Maureen F. Dahlberg

Greta, age 12, has an older brother named Kurt who dies due to his hemophilia. Set in 1938 in Vienna, this motivational teen novel with historical detail involves children from a musically gifted family who fulfill their dreams, even using the death of a sibling with hemophilia for inspiration.

Angel of the Square (HarperTrophy, 2001)

This novel for ages 10 and up tells the story of Ekaterina Ivanova, called Katya, the 12-year-old daughter of a lady-in-waiting to Empress Alexandra in Russia before 1918. Katya is a playmate of Anastasia and learns what is wrong with Alexei, who has hemophilia. Although Katya is fictional, her perspective on the Russian Revolution is believable.

A Home for Garth (self-published, 2010)

Reed

Jake Stevens, age 11, finds a lost dog that belongs to Robert Higgins, a 10-year-old with hemophilia. Jake’s moral dilemma is deciding whether to lie to keep the lost dog, or to be truthful and return the dog to its owner. Jake learns about hemophilia and how it limits what Robert can do.

The Hardy Boys Undercover Brothers, #32 Private Killer (Aladdin, 2010)

Franklin W. Dixon

This novel for ages 8–12 features teens acting as detectives to solve mysteries, and includes a female with hemophilia. Destiny Darity is the troubled daughter of the headmaster at an exclusive private school near Boston. Destiny has a rare blood type that she inherits from her mother and a mild case of hemophilia that she inherits from her father. The Hardy brothers, Frank and Joe, may not completely understand the genetics of bleeding disorders, but they determine who plays pranks on Destiny.

Blood Ties: A Blood Coven Vampire Novel (Berkley, 2011)

Mari Mancusi

This young adult novel includes a main character named Sunshine McDonald, a 17-year-old fairy princess living in Las Vegas. She loves Jayden, a mortal with hemophilia. When Jayden is bitten by a vampire, he needs blood transfusions—not for his hemophilia, but for his turning. Sunshine and Jayden travel to London and Tokyo to find a cure for vampirism by drinking from the Holy Grail, and by doing so, cause Jayden’s hemophilia to disappear.

I Hunt Killers (Little, Brown, 2012); Game (Little, Brown, 2013); Blood of My Blood (Little, Brown, 2013)

Barry Lyga

This trilogy of young adult mystery/thrillers delves into the world of serial killers, and the vivid carnage may be inappropriate for young readers, or for any readers. Seventeen-year-old Jasper Dent, called Jazz, is the son of an imprisoned serial killer. With the help of his best friend Howie, Jazz helps police identify serial killers. Howie is a “type-A hemophiliac” with an overprotective mother, so his assistance is sometimes limited when extracting Jazz from dangerous situations. Thankfully, Howie provides some comic relief to an otherwise serious topic.

***

Reading should be a life-long endeavor and source of pleasure. Take advantage of these books to help young readers learn something about bleeding disorders. And keep reading good books to your child!