parenting

Lights, Camera…

It’s our 30th anniversary! And since I am stuck at home like 90% of America, I’ve had few distractions and plenty of time to review tons of media from the past 30 years. My book “Raising a Child with Hemophilia” was published in 1990, when I was 33 years old. Armour Pharmaceutical Company wished to promote the book, and I found myself suddenly on TV and doing radio interviews, sometimes scheduled at midnight. The company hired a PR firm to work with me. We traveled to Cleveland, Dallas and Philly to make videos and promote the book. And some hemophilia nonprofits also included me in their promotional videos about hemophilia. It was a whirlwind time! I had two small children at the time, more books to create in the pipeline and a newsletter to produce, all done from home.

Guess what I found? Copies of the interviews and tapes. If you’d like to see them, go to our home page, click on “See” which will take you to our gallery, and click on “Videos.” You’ll see videos from the 1990s!

I did learn a few things: I never really liked giving interviews. Maybe it was just that I was always distracted with raising children and work (now I don’t mind at all). And when the PR firm thought it was a good idea to plug the product my son was using, I ended the corporate-sponsored interviews. It was interesting, to say the least! But I never went back on my personal policy of never personally endorsing a product. Enjoy the shows!

Bubba’s Factor

Part 2 by Derek Markley

With Abbey happily moved over to a hotel, it was time for us to focus on getting Bubba to sleep. His little-kid incarceration was a concern. The main thing I remember was how incredibly horrible I felt seeing him in a hospital crib with metal bars raised on all sides. This had to be one of the saddest moments of my life. We knew he had to go to sleep, which he would not do quickly. He’d sit up and we’d have to reach through the bars to comfort him. It would’ve been easier to just push the bars down and play with him. Unfortunately, we knew he needed sleep. If we kept getting him out of bed, we’d be up all night.

I was treated to having the lounge to myself. The chair/bed wouldn’t turn out to be the strangest thing, nor the sleeping in the middle of a lounge area. There was a shower in the lounge. You can’t make showering that close to strangers feel normal. Nothing else about our day was normal or comfortable. My son was in a metal cage and my wife was sleeping in a recliner beside him. We were in the middle of St. Louis and our son had blood that didn’t clot. We were going to send our small child into surgery, and he was going to have a metal disk placed in his chest.

The next morning I went down the hall to see Bubba and Ashley. We were about to have a very long day. We’d begin by having Bubba factored up. The people at St. Louis Children’s Hospital were adamant that his factor level would be through the roof to assure no complications during surgery.

Surgery day always means that the patient will be given some type of medication. Bubba was given medication. He went immediately loopy and it was hilarious. I’ve often wondered if they give little kids their meds slightly early so the resulting goofiness is something the family can enjoy. You’re given a break from the handwringing and gut churning that has been gnawing at you.

The unfortunate truth was that he would be heading to a surgical suite and we’d be left outside trying to put forth a normal countenance that masked the fact we were both on the brink of sanity. There was no parenting class called, “How to Not Go Feverishly Insane Because Your One-Year-Old Child is Having Surgery.” My palms are sweating a little bit right now just thinking back to that time. This is one of the uncomfortable truths about raising a child with a serious medical condition. You will have to experience things that are not a part of the lives of most parents. These things will be stressful and unpleasant. These things will make you feel like you want to vomit. These things will be necessary to assure that you can help your child deal with whatever disorder, condition, or ailment has become a part of his or her life.

After roughly 32 hours (my estimation may be skewed), the physicians emerged to tell us that the procedure was complete and Bubba was headed to recovery. Everything had gone as planned and the port was in place. Later that day the nurses would have to access the port to complete his next infusion. Post-surgery infusions were crucial to assuring that the healing process began properly.

There are not many feelings in this world that allow you to decompress as fully and quickly as you do when you’re informed that your child’s surgery went exactly as planned. After what seems like forever, you are allowed to let your weapons-grade stress level return to somewhat normal human proportions.

We were taken back to a general recovery area. Ashley was holding Bubba and gently speaking to him. We just needed his eyes to open. Any small indication of being awake would’ve be great. This should’ve been one of those Disney moments when the little kid opens his eyes, sees his mother, and produces a massive smile. Then a small sparrow would land on Ashley’s shoulder, chirp happily, and a warm sunset would be visible on the western wall of the recovery room.

That was not our outcome.

Next week: Post operation success?

Derek Markley lives in Saltillo, Mississippi with his wife Ashley and their children Abbey and Bubba. He is the executive director of two University of Mississippi regional campuses and an assistant professor in the school of education. Ashley is a fourth grade teacher in the Tupelo Public School District. Derek is author of The Bubba Factor, which can be ordered on Amazon.com.

Bubba Gets a Port

by Derek Markley

Owen, who we call “Bubba”, has hemophilia B. He had two bleeds in two months before his first birthday. Our physician made a recommendation that would change our world.

“Bubba” Markley

Bubba was going to get a port. A small circular piece of metal would be placed in Bubba’s chest. There is a line running from the port that would be inserted in a vein. We’d use the port to infuse him with clotting factor. We’d be able to do the infusions at home after being trained by a home health nurse.

I had so many questions. I furiously Googled terms to get a better idea of what was going on. The port images I found had an external line, a piece of plastic tubing outside the body. It didn’t look like it would be a great option for a little kid.

We were informed that the port would not have an external line. The only time Bubba would be accessed would be when we were infusing. The port is accessed any time a needlestick is done to push medicine into the port. Our physician also told us that we had the ability to use a low-profile port. This meant that the port was thinner than other models and would not stick out as far when implanted.

Even now, more than eight years after the surgery, I still have no way to accurately describe our feelings about what happened. I can describe the day in detail, but I can’t put my feelings into words. We had to be in St. Louis the night before the surgery at the St. Louis Children’s Hospital (SLCH). Bubba’s surgery would be early in the morning. He’d need to be in the hospital so they could give him enough factor to make surgery possible. We’d never spent the night in a hospital with a small child. But the decision was made. The Markleys—my wife Ashley, Bubba, daughter Abbey and I— were surgery bound!

Ashley’s mother had made the long trip from Washington D.C. to take care of Abbey throughout the process. She’d found a hotel room and Abbey would stay with her the night before the surgery. I would be spending the night in the room reserved for family of patients. The room had reddish-orange chairs. What appeared to be an ugly, uncomfortable chair was actually an ugly, uncomfortable bed. With the pull of a lever the front of the chair extended forward as the back simultaneously laid flat. Bam! It’s a bed—in the middle of a room where other strangers might choose to sleep, read, or otherwise hang out.

Ashley was going to sleep in the room with Bubba. The surprise came when we saw Bubba’s bed.

We were introduced to the Baby Hannibal Lecter line of beds. It was immediately noticeable that Bubba’s bed looked more industrial than the standard crib. It didn’t take long for a nurse to step in and show me exactly how this bed differed from most. Ashley and I were shown a demonstration of how the metal bars, which started on the bottom, slid upward and locked in place. It was a Bubba jail.

It looked like Trent Reznor designed it in the early 90s during his industrial phase. The experience was going to turn our kid into a little Goth in training. We’d need to get some black hair dye and a magnetic nose ring. He’d just mope around the house talking about things like the darkness in his stuffed duck’s eyes or the repressed aggression of the Backyardigans being the root of their imaginative adventures.

… and he looked absolutely pitiful when we put him in it the first time.

By pairing incarceration with surgery, I was certain that we’d taken two strong steps toward assuring that Bubba would never forgive us.

We would have a number of meetings that day. The people at SLCH were seasoned veterans and addressed many of our questions before we even had a chance to ask them. By far the most stressful time came when we sat down with the two physicians who would be performing the surgery.

I also had a moment in which I realized that I was not a child anymore. One of the physicians looked suspiciously young. Like most people I’d always visited doctors who were significantly older than me. It hit me like a ton of bricks. I was turning into the person that says, “I’m not letting that kid operate on my child.” I was a mere step from complaining about underage intruders on my lawn.

Once the doctors explained it to us, the process itself did not seem complicated, compared to other surgical procedures. My concerns were still firmly in place. You’re operating on my child who very recently celebrated his first birthday. The child going under the knife also has a small issue with blood not clotting. There is bleeding involved during surgery.

We were soon back in Bubba’s room ready to face the next challenge. How to entertain a toddler while sharing a hospital room with another family and their visitors? The answer is: Get out of the room. SLCH made a wise investment and bought plastic kiddie cars for use on our floor. They had a large handle on the back that extended upward so moms and dads could exert some control over their kid’s driving. It was the Hemophilia 500. Like many hospital floors, the hallways were laid out in a square with the large nurse’s station in the middle.

Bubba in the hospital

We went around.

We went around again.

… and again and again and again.

I began to curse the little red car.

But it kept Bubba happy and that was our goal.

Next week’s blog: Surgery!

Derek Markley lives in Saltillo, Mississippi with his wife Ashley and their children Abbey and Bubba. He is the executive director of two University of Mississippi regional campuses and an assistant professor in the school of education. Ashley is a fourth grade teacher in the Tupelo Public School District. Derek is author of The Bubba Factor, which can be ordered on Amazon.com.

Teaching Your School-Age Child About Hemophilia

Published in August 2018 PEN

With the start of the school year comes new teachers, nurses, and caretakers for your child. You may be explaining to many adults what hemophilia is and how they should properly respond if your child has an issue. But, how do you go about teaching your child about their hemophilia? In this excerpt from our latest issue of PEN, we delve into how children understand their bleeding disorders and what you can do to help present information  for them to digest. 

Teaching Your School-Age Child About Hemophilia 

One of the biggest challenges we have as parents of children with hemophilia is teaching our children about their disorder. We often use words like hematoma, factor, and deficiency; and concepts like prophy, coagulation, and heredity. But children understand these words and concepts very differently than adults do.

If you don’t know how your child’s mind works at various stages of his development, then teaching him about hemophilia becomes hit-or-miss. But when you know how he thinks, you can tailor information in a way that he can easily understand. So to teach your child about hemophilia, you need to know how he processes his world in general, and hemophilia concepts in particular.

The School-Age Child’s Thinking Tools

Between ages 7 and 11, the school-age child is in a fascinating stage of cognitive development. “Cognitive” refers to how he thinks, how he processes incoming information about his world—basically, his ability to think logically. Just as he has a skeletal structure that develops as he grows, he also has a mental structure that develops as he matures, filtering information in a way he can grasp.

Your child’s mental structure is characterized by five major thinking tools that are constantly evolving:

Causal thinking: Figuring out when something causes something else, using a step-by-step process. A preschooler doesn’t typically think step-by-step.

Internalized thinking: Moving from understanding his world mainly through his senses—where things happen outside him—to realizing that things can happen inside him.

Gradient thinking: Knowing that the world isn’t just polar opposites, like good guys and bad guys. There are now shades of gray, degrees of intensity. A good guy might do something bad. Your child can also distinguish parts from the whole.

Empathic thinking: Starting to see the world from another’s point of view.

Time: Understanding that he doesn’t exist just in the present, but that he has a past and a future.

For understanding hemophilia, the most important of these five thinking tools may be causal thinking. Your child can now try to figure out how one thing causes another. Like…What causes bleeding? A blood clot? What is genetic transmission? It’s hard to explain these concepts when your child doesn’t understand causality. These are more sophisticated thinking tools than he had as a preschooler, yet a school-age child, ages 7 to 11, is most comfortable using his new thinking tools on things and places he knows best—the tangible, visible world. So let’s see how he uses these thinking tools on various topics in hemophilia, starting with blood.

How He Understands Blood

Unlike a preschooler, your school-age child understands the concept of the whole and its parts. So you can explain blood in terms of what it’s made of. Children between ages 7 and 9 believe that blood is a red liquid, but also that it’s composed of “stuff—water, food and energy.” Children between ages 9 and 11 tend to describe blood in more abstract terms. “It’s cells. Little roundish stuff. They’re red and blue.” A child develops from concrete to more abstract thinking, so this is perfectly acceptable!

Now you can introduce the idea that blood has components: white blood cells, red blood cells, and platelets. While preschoolers focus on things outside the body, mainly what they can see, hear, and feel, a school-age child realizes there are things inside him that he can’t see. So he’s ready to learn about simple blood components, especially those related to his hemophilia.

How He Understands Hemophilia

Because he understands a whole and its parts, your child can now categorize things. A preschooler might describe hemophilia as “blood,” or “something I have,” but a school-age child can classify hemophilia as a “blood disorder,” or “when blood doesn’t stop bleeding.”

He also progresses from describing hemophilia as his own specific injury (“It’s when I get a hurt knee”) to seeing it as a condition (“It’s when someone gets hurt and bleeds a lot”). This is the empathic thinking tool: he knows he is not the only one to have hemophilia. He now says that hemophilia is when “boys with hemophilia have to go to the hospital sometimes.” Compare this to the preschooler reply, “When I have to go to the hospital.”

Your child also has matured from an external to a more internal focus. A preschooler might say, “Hemophilia is bruises,” but a school-age child will say, “My blood doesn’t work right.” What is it that doesn’t work right? Well, he understands the concept of a whole and its parts, and he’s ready to know that blood is composed of parts. So he can deduce that hemophilia means “something’s missing” in his blood. Some children say that they have “lost” something, or that their blood is “too thin.” These answers reflect the “something’s missing” idea. For example, “It’s when you’re missing some factors that help to make it so if you slam your knee against something it doesn’t swell up as much. You’ll have to replace the factor.”

Misconceptions and medical inaccuracies abound as your school-age child struggles to understand hemophilia. “It’s a blood disease. You lose part of your blood and you need to get more blood.” At this stage, what’s important is not so much that his answers are right or wrong, but how he arrives at his interesting conclusions.

So teach your child that hemophilia is a “blood disorder.” Teach him that blood is made up of parts, and that he is “missing” a part. There’s no need to get too specific at first, for example by mentioning factor and proteins; just stick to general concepts and ideas. To help him visualize, use a concrete example, like the falling dominoes. Remember that a school-age child is increasingly able to understand more abstract terms, but he needs the help of concrete examples.

How He Understand Genetics

Learning about heredity is a great way to exercise the “missing step” concept in a step-by-step sequence. To a preschooler, hemophilia is just something he was born with. To a school-age child, something had to happen to cause hemophilia.

What is that something? His parents are usually the missing step. Your child possesses the thinking tool of time, so he may realize that hemophilia could have started in his family many years ago, even centuries ago.

But how exactly does hemophilia get from one person to another? Most school-age children name a causal agent—the thing or event that caused hemophilia to happen. This can be a parent, blood, a chromosome, sperm, or even “vibes,” as one boy phrased it.

Understanding often differs among younger children (ages 7–9) and older children (ages 10–11). When asked how they got hemophilia, younger children may simply reply, “Mom” or “Mom and Dad.” Some children name blood. “It streams through your family, through their blood. I got it when I was two or three. My uncle gave it to me.” This child is medically incorrect, but he’s trying to sort it out logically: a family member had hemophilia, I have hemophilia, and…maybe my uncle gave it to me?

School-age children may also understand or accept some basic heredity rules, such as “mothers are carriers.” This makes sense to them, because a “carrier” is a causal agent.

From ages 9 through 11, a child’s answers and thought processes become more sophisticated. The causal agent may be chromosomes, which only a few children can discuss at this stage. Remember that school-age children are still very concrete—more comfortable with things that they see, hear, feel, and smell. Chromosomes are abstract. Some children identify an “X thing” as the causal agent, but don’t understand the idea in purely scientific terms. To them, X and Y are not parts of the cells. They’re more like “germs” that other family members “catch.” One boy explained, “Mom’s a carrier. She has two little things inside her, little Xs. They’re like little eggs. She has a good X and a bad little X in her. My brother got the good carrier and I got the bad carrier.”

Ask your child, “Where did your hemophilia come from?” and let him figure it out on his own; don’t judge his answer at first or try to answer for him. You can work on the details later, supplying more accurate information once you’ve listened to his explanation and understood his thinking.

To read more on this topic download our August 2018 PEN today at

https://www.kelleycom.com/product-category/newsletters/pen/

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.
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