July 2024

Know This Superhero in Hemophilia

Laurie Kelley July 28, 2024

This weekend saw a record breaker. Not the heat. Not the Olympics. Not politics. It was the movie Deadpool and Wolverine, which grossed an astounding $205 million for its opening weekend. I confess I adore Deadpool, followed closely by Wolverine. My two favorite superheroes.

Someone on Facebook recently wondered if the newer generation of people with bleeding disorders will remember the superheroes who once broke records during our time: the late 1980s and early 1990s, fighting for justice and compensation from HIV infection in the blood supply. Our world is safer, thanks to them.

Our favorite researcher and archivist from North Carolina, Richard Atwood, shared with me a review of a book by one of our superheroes. Elaine DePrince, someone I met long ago, inspired me with her advocacy and energy. But her personal sacrifices, generosity and compassion reaches new level after reading about this new book, written by Elaine, called Mikey Speaks Out. You’ll see how Elaine welcomed the challenges of raising children with disabilities, even adopting them. The fighting for justice for them, and authoring two books about her experiences. Just incredible.

Richard writes:

Michael-Noah, called Mikey, was the first child born to a 21-year-old mother whose father had hemophilia. Mikey had not only severe hemophilia A, he also was deaf and had a cleft lip and palate. His mother gave Mikey up. He spent months hospitalized for his surgeries and medical issues. Fortunately, there was a hemophilia center located in the hospital. A foster mother in Buffalo, New York sedated Mikey daily with liquid Valium, which slowed his development. When Mikey was eighteen months old, a psychologist diagnosed him with autism, mental retardation, deafness, and blindness.

Charles and Elaine DePrince, from New Jersey, brought Mikey home with them. Elaine was a special education teacher. Mikey now had two older brothers. Mikey acquired a hearing aid, a padded leather helmet, and glasses, all of which were improvements for his senses and protection. At two, he acquired metal leg braces, that allowed him to walk for the first time. He developed bruises from his hemophilia, and began home treatment, rather than having to visit the hospital, for his factor VIII infusions. When he was three, Mikey attended a handicapped pre-school for half-days. Due to poor motor skills, he had trouble using sign language, yet he could read lips quite well. Audiologists could not determine his hearing loss. At age five, Mikey attended a communication handicapped school for two years. His family adopted two younger boys, named Teddy and Cubby, who also had hemophilia A. In 1986, Mikey, not wearing a seat belt, was in a school bus accident. He suffered a severe skull fracture that resulted in partial paralysis, damaged eyes, lost speech, and diabetes insidious.

In 1988, Mikey, Teddy and Cubby all had HIV tests. At age nine, Mikey, who read body cues of an audiologist, left the deaf school after faking some hearing tests. He began home schooling. During a CT test and an audiogram, Mikey was found to be totally deaf due to missing inner ears. His intelligence was not limited; in fact, he was a speed reader. Eventually, Mikey, Teddy and Cubby became sick with AIDS. The Children’s Hospital AIDS Program was 90 miles away. The monetary award from the school bus accident lawsuit helped to purchase an indoor, in-ground swimming pool and an RV. Mikey learned of prejudice, bigotry and hatred from reactions by misinformed adults. Cubby died of AIDS in 1993. The DePrince family helped to feed the homeless in Philadelphia. Mikey acquired measles, and later toxoplasmosis and cytomegalovirus encephalitis, that led to a seizure, a high fever, and eventually his death. Elaine DePrince, his adoptive mother, wrote Cry Bloody Murder (1997) as the legal theory for HIV transmission in hemophilia. At age 51, Elaine enrolled in law school and took part in the wrongful death lawsuits for her adoptive sons. Following Mikey’s wish, Elaine adopted six girls from Africa!

Elaine and Charles had two biological sons and adopted nine children. Sadly, the three adopted sons died of hemophilia and AIDS. Charles died of Parkinson’s disease in 2020. But nothing slowed Elaine down—her newest book is testament to that. To me, she’s a super mom and superhero. I hope our community remembers her.

Order Mikey Speaks Out here.

Keep Your Lower Extremity Joints Moving!

Laurie Kelley July 21, 2024

Michael Zolotnitsky, PT, DPT

Editor’s note: I’ve been doing a lot of hiking in the mountains lately, and my quads and joints are feeling it! I do basic exercises to keep them in shape. Below, Mike shares what you can do to keep your joints supple and strong.

The pandemic impacted everyone’s life in 2020, making it especially hard to stay healthy. While many of us began exercising from home, some of us have completely stopped exercising because of the increased stress of working from home, managing our children’s school schedules, gyms closing, expensive home exercise equipment, or just being out of routine. This isn’t good for anyone, but if you have a bleeding disorder, lack of exercise can cause major setbacks, including joint stiffening, that put you at greater risk of joint bleeds and more joint damage.

            As a physical therapist with severe hemophilia, I’m going to focus on eight imperative workouts to perform daily without any equipment at all. And I’ll explain why these are important to maintain strength and reduce areas of common joint damage!

1. Sit to Stand

Sit in a chair, cross your arms, keep feet shoulder-width apart, toes pointed forward, and stand up. Slowly lower yourself into the chair, repeating 9 more times for a total of 10. If this is challenging, use your arms for support until your legs build strength. If this is easy, find a lower surface.

Purpose: Build strength through the quads and glutes to reduce difficulty with walking and stair negotiation, while reducing pressure on the knee and hip joints.

2. Glute Bridges

Lie on your back, making sure your spine is neutral. Bend both knees, keeping your feet on the floor. With your knees slightly apart, push through your heels to raise the hips up. If this is challenging, use your arms to help push your body up. If this is easy, progress to perform one leg at a time.

Purpose: Build strength through the glutes and the hamstrings. Also great for core activation and balance if performing with one leg. Building up sufficient glute and hamstring strength will also reduce pressure on the knee and hip joints.

3. Standing Hip Abduction

Stand facing a wall, with hands on the wall, knees shoulder-width apart. Start with one leg. Slowly bring it out to the side with toes pointing forward, and then return. Begin with 10–12 repetitions (reps) and progress to 15–20, making sure your body is not rocking sideways. If this is easy, progress to doing the reps without holding onto the wall. While performing this on the right side, the left side is focused on stabilizing, so both hips will feel the workout!

Purpose: Build strength in the gluteus medius, which will keep you upright when walking and improve overall balance to reduce your risk of falling.

4. Standing Hip Extension

Stand facing a wall, with hands on the wall, knees shoulder-width apart. Start with one leg, and slowly extend the leg back, with toes pointing forward. Make sure the lower back is not arching backward or forward; squeeze the glute to engage proper activation. Begin with 10–12 repetitions (reps) and progress to 15–20, making sure your body is not rocking sideways. If this is easy, progress to not holding onto the wall.

Purpose: Build endurance to reduce difficulty in activities requiring prolonged periods, such as hiking or taking long walks. Improving glute strength and performing standing extension-based exercise is important because we are often in the flexed posture throughout the day.

5. Penguins

Stand with your toes pointing forward, and waddle side to side, standing in place. You’ll feel this in the sides of both hips. Begin for 10–20 seconds with feet 1 to 2 inches apart, and progress to 30–60 seconds with feet 3 to 5 inches apart.  

Purpose: Build further gluteus medius strength and improve balance. While you balance on the one lower extremity, the other is working. Balance training has been shown to reduce pain in the ankle and knee, and reduce effects of osteoarthritis.

6. Heel Raises

Stand facing a wall, with hands on the wall, toes pointed forward. Slowly raise your body up on tiptoes. Progress to not holding onto the wall, and further progress by standing on toes using both feet going up, and then lowering down on one. Start with 10–15 reps, and progress to 20–30 reps.

Purpose: Build strength through the calves, to improve strength during walking. Ankle bleeds are common, but ankle muscles are not commonly exercised. Having strong ankles will reduce strain that moves up through the knee, hip, and lower back.

7. Hip Flexor Stretch

Kneel on one knee, with the other at a 90-degree angle in front of you. Maintain upright posture, and keep your sternum/chest bone pointing upward. Slowly lean forward and feel the stretch through the groin. Begin by holding 15–20 seconds if you’re under age 65, and holding 30–60 seconds if you’re over 65, to help stretch the collagen. Progress by engaging the glutes to feel an increased stretch through the iliopsoas.

Purpose: Reduce strain on the quad and low back by improving mobility and flexibility of the hip flexor. Since most people are in a flexed posture and seated position for long periods, the iliopsoas tends to get super tight and is an area of increased muscle bleeds. Maintaining good range of motion will reduce the chance of target joints.

8. Hamstring Stretch

In a seated position, have your legs apart, and slowly lean toward one ankle. Begin by holding 15–20 seconds if you’re under age 65, and 30–60 seconds if you’re over 65 to stretch the muscle. Progress by reaching farther to improve the stretch.

Purpose: Reduce strain on the low back and knee joints by improving the mobility and flexibility of the hamstrings. If you spend a lot of time in a flexed posture and seated position, the hamstrings tend to get super tight. Maintaining good range of motion will reduce the chance of developing target joints.

A pandemic can make working out a challenge. These eight simple exercises focus on mobility, balance, and stability, and target all the major muscle groups. They can be performed at home without any equipment. I highly recommend performing these exercises daily, and after six to eight weeks, you’ll see a major improvement. Exercise is vital if you have a bleeding disorder. The time to start is NOW!

Michael Zolotnitsky, PT, DPT, is director of neurological rehabilitation at New Jersey Spine and Wellness in Old Bridge, New Jersey. He also has severe hemophilia A. He can be reached at michael.zolotnisky@spineandwellness.com

Considering Long-Term Health in Your 20s

Laurie Kelley July 13, 2024

Kevin Correa

By the time teens with hemophilia transition into their 20s, ideally they’ve built a firm foundation on which to manage their disorder. Among many skills, young adults should be able to self-infuse and negotiate health insurance. But when you’re healthy and your hemophilia is under control, it’s easy to overlook general health maintenance beyond hemophilia.

            It’s uncommon for 20-somethings to consider the long-term impact of their lifestyle choices. So what if I’ve gained a few pounds? I only smoke when I’m out with friends. I look so much better with a tan. But young adulthood is the time to develop the habits that will help maintain a healthy body in the decades ahead.

I’m Independent. Now What?

Perhaps more than any other time in our lives, the 20s are a decade of major transformation. Many young adults move out of their parents’ home. Others graduate from college. Even those who’ve diligently managed their hemophilia may be thrown for a loop by all the changes in their lives.

            Your hemophilia treatment center (HTC) may no longer be in the same town or even the same time zone. And finding an HTC is just one of the myriad issues you’ll need to address: Where will I buy groceries? How long will my commute be? When will I find time to renew my driver’s license?

            These changes may take priority, and if you’re feeling healthy, finding a local dentist or doctor doesn’t seem all that pressing. Plus, who has the time?

Not Enough Hours in the Day

Entering the 9-to-5 world for the first time is a big deal that can have a major impact on your health—and not just for those in Deadliest Catch-type occupations. For many, this is their first experience sitting behind a desk for several hours a day. A tangible and common consequence is weight gain. The Centers for Disease Control and Prevention (CDC) reports that an astounding 20% of Americans in their 20s are obese.* Not only is extra weight bad for your joints, but obesity is a contributing factor to several leading causes of death, including heart disease, stroke, and certain types of cancer.

            The effects of “a few extra pounds” may not be immediately apparent, but that’s the point. In your 20s, you need to establish good habits to protect your body for the long run. This means adjusting what you’re eating while maintaining some form of routine exercise. Neither is easy to accomplish when you’re just starting life on your own.

            “Sean,” a 25-year-old with hemophilia, took a new job recently and is slowly figuring out how to get all the pieces of his life to mesh. In college, Sean competed in triathlons, training 25 hours a week. “In school, you had the motivation of working out with your teammates,” says Sean. “And you had a relatively flexible schedule.” Now he’s struggling to find the hours to train for just a fraction of that time. And like many of his peers, Sean has let his diet suffer: “I know I need to get back to eating food that’s good for me, and not just what’s convenient.”

            Living healthy in your 20s doesn’t necessarily require big time commitments. In some cases, you just have to make better choices: When you can, take the stairs instead of the elevator. Cut back on the amount of alcohol and caffeine you consume.

And if you’re among the 1 in every 5 Americans who smoke, quit now. According to the CDC, smoking causes more deaths in the US than alcohol, illegal drugs, and motor vehicle accidents combined.

            Sure, you may not have all the time in the world, but your general health should sit atop your list of priorities.

Molehills Can Become Mountains

While you lived at home with your family, you probably had a stable network of medical resources. But once you’re on your own, you may need to rebuild that network by establishing a relationship with your new HTC, plus maintaining all facets of your health.

            Dental health is one of the most neglected aspects of overall well-being. By the time you’re in your 20s, Mom or Dad probably don’t schedule your dentist appointments, which means that nobody does…until a minor toothache becomes something worse.

            Sean tells a common tale. “I’ve been a bit derelict when it comes to the dentist. What probably wouldn’t have been a big deal had I stayed on top of it, ended with a root canal.”

            Your primary care physician (PCP) can be your best ally. You’ll turn to your PCP when you can’t kick that cough you’ve had for three weeks or are worried about anything from a wart on your foot to your sexual health.

            As Sean assembles the medical resources he needs near his new home, he sees the value in finding the right PCP. “You want someone you can go talk to about health concerns that aren’t hemophilia related,” he says. “Someone who knows you and your medical history and if necessary, who can point you to the right specialists.”

            In addition to addressing your current health concerns, your PCP will review your family history with you, assessing your risk for ailments like heart disease, diabetes, and certain cancers. Armed with this information, together you can develop a plan to reduce some of the risks.

Why Do Today What I Can Put Off ’til Tomorrow?

Throughout your 20s, medical concerns may surface that have nothing to do with hemophilia, and everything to do with the natural aging process. You’ll begin to shed the cloak of invincibility you donned as a teen, and realize that you need to act with an eye to your future.

            So manage your total health as attentively as you manage your hemophilia:

            • Eat a healthy, balanced diet.

            • Maintain your optimal body weight.

            • Make exercise a priority.

            • If you drink or smoke, reduce your alcohol consumption, and quit smoking!

            • Schedule all the exams you’ve neglected for so long.

            • If you don’t have a local dentist, eye doctor, or PCP, contact your HTC or insurance company. They can help you find one.

True, for now, you can ignore the incessant commercials for “old people” drugs like Lipitor. But let them serve as a reminder for the not-too-distant future. What’s Lipitor anyhow? It’s a drug that controls cholesterol. What’s cholesterol?

­­­­­It’s one of many topics you and your doctor should start discussing.

* More recently, according to the CDC, 40% of US adults aged 20–39 were obese between 2017–2018. However, a 2021 Johns Hopkins study found that 56% of Americans aged 18–25 are overweight or obese. 

Originally published in PEN 2.11     © 2011 LA Kelley Communications

What Kind of Leadership Matters Most When?

Laurie Kelley July 7, 2024

Leadership is an endlessly fascinating topic and one that is never more important now in the United States. The US is facing a critical leadership challenge this year, with a situation it has never encountered before. And it’s interesting when comparing leaders in government to leaders in the hemophilia community. Because this past year, we’ve seen leadership changes in our major bleeding disorder nonprofits. Change often causes constituents—like you and me—to sit up and take notice. Who exactly is running the show? How are they running it—and why?

Certain forms of leadership are likely to be more important when countries, companies or nonprofits are facing unpredictable futures. And an effective and appropriate leadership style is often contingent on the voters, market or constituents you serve.

One study from the 1990s examined this. To identify what qualities of leadership count the most under different conditions, a team of academic researchers asked two top managers of 48 large US companies to assess the qualities of their chief executive and their operating environment.

The top managers assessed the extent to which their CEO displayed the qualities of “transactional” or “charismatic” leadership. The first type of leadership describes an executive who works within the existing system and rewards employee contributions to it; the latter describes an executive who visualizes an altered future and stimulates employee passion for it. The top managers also evaluated the degree to which their firms faced markets that were dynamic, risky, and stressful.

The researchers found that charismatic leadership by the CEO tended to have adverse impact on company financial performance when the market was predictable—but favorable impact when the market and environment was uncertain.

In other words, if a country, company or nonprofit is facing a fast-changing world with an uncertain future, building a passion for the future is critical. And this means getting citizens, employees and constituents on board with a leader’s vision, through trust, communication and credibility.

Ask what’s happening right now in our country, with our nonprofits. Which leader appears transactional? Which charismatic? What vision do they offer? Are they credible? Trustworthy? Communicate their vision well? Facing an uncertain future, a charismatic leader may well command a greater sense of power, of impact. This does not mean they are the best leader, but it means they may be the best at winning consensus and achieving power, conveying a vision and having people believe in them.

Something to really think about this year: whether in US politics or the bleeding disorder community… study the topic and components of leadership as much as you study the leaders.

Source: David A. Waldman, Gabriel G. Ramírez, Robert J. House, and Phanish Puranam. “Does Leadership Matter? CEO Leadership Attributes and Profitability Under Conditions of Perceived Environmental Uncertainty,” Academy of Management Journal, February 2001, pp. 134–143.

© 1996–2001,Wharton Center for Leadership and Change Management, University of Pennsylvania.

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