Derek Markley

Bubba’s Outcome

Part 3

Bubba did start to wake up, and that made us very happy. He opened his eyes and was almost immediately able to focus on Ashley. That made us happy too. Then he made a very unhappy face, an incredibly unhappy face.

He started sniffling. Then he started crying.

We were a bit concerned.

The crying got louder. The crying began to include screaming.

We didn’t have the slightest clue what was going on. What was wrong? Did his chest hurt from the surgery? Was he scared because he woke up somewhere new?

Let’s take a break for a learning experience. You may or may not be aware that some small children have an adverse reaction to anesthesia. The reaction does not occur when they are initially anesthetized; it happens when they wake up. It’s called emergence delirium.

The attending nurse reassured us that this was not uncommon. She did acknowledge that it was difficult. I’m sure she’d seen more than enough parents come through and experience angry kid syndrome upon waking. We were also told that they could give medicine prior to surgery that would decrease the severity of this reaction.

The whole process lasted about 15 minutes. Bubba slowly calmed down and began to resemble the child we sent into surgery, not the angry little monster that had emerged into the recovery room. He was obviously exhausted. All of us were ready for it to be over. We were cleared to head back to his room.

Bubba had made it through the surgery and we were good to go. The reaction to coming out of anesthesia was actually minor in the grand scheme of things. Ashley and I were on our way to becoming more active in the role of Bubba’s care. With the port in place we’d be trained how to infuse him at home. All of the stress that we were currently experiencing would be more than worth our troubles in the end.

The true test would come when it came time to test the port. Being a medical newbie, I had no idea how complicated or how simple this process would be. Bubba would be given sufficient time to rest, but we all knew that a functional port was the only path to us getting home.

Later, I joined three nurses in an exam room located right down the hall from our hospital room. It might have been a procedure room. The combination of fluorescent lighting and white overload made the room incredibly uncomfortable.

The goal was to ensure that Bubba’s port would function properly so we could determine how much time he’d need to remain in the hospital. The nurses were upfront and honest about the fact that he would not react favorably to this process.

My job would be to help hold Bubba still and do my best to calm him. I wanted to do my best on both fronts. He remained accessed because the surgery site would be far too sensitive to access with a new needle. Our goal was simply to connect a line in and make sure that everything could be pushed with no problem.

Bubba is accessed, so the nurse is now ready to push saline as soon as she gets blood return.

No blood return. That’s fine. Ports can be finicky. She tries again. No blood return. Not a problem, we’ll just put his arms above his head and that’ll open things up. A pattern began to emerge. The nurse could not get blood return.

Bubba was rolled onto his side.

Bubba was allowed to sit up.

We maneuvered and finagled him around trying to get blood return. Each time the nurse drew back the plunger nothing happened.

Bubba was getting angry. He got even angrier as the nurses tried to adjust the needle that was used to access the port. Any form of sedation or local anesthetic had worn off quite some time ago. He was now a one-year-old with a needle being moved around in his chest.

The noises started as intermittent crying. The intermittent crying became more constant. The crying was then joined with some attempts to get out of our grip. My willingness to keep him on the table was beginning to fade. I just wanted to pick him up and get him out of the room. Bubba soon began screaming at the top of his little lungs. His face was soaked with tears and bright red in color.

The issue with the port simply added to our emotional ups and downs. We began as parents frightened because their small child needed surgery. The emotional upswing came when we thought about more effectively managing his condition. We were scared to death when he went back for surgery and elated when he came out. My spirits hit rock bottom again when I went back to the room to tell Ashley that the port was not functioning.

It was not long into the day when a physician approached us.

A gaggle of short white-jacketed medical students were in tow. To be completely honest my first thought was, “Ugh.” The last thing I needed was the medical neophytes peering over us as the actual doctor spoke.

The physician explained to us that it was clear the port was not functioning.

The x-rays that they’d taken showed the line from the port was pushed up against the wall of the vein. This meant that there wouldn’t be any blood return. The opening on the end of the line was simply sucked up against the wall of the vein.

The med students continued to hover. I began to fume.

We were informed that the line would need to be moved in order to make the port functional.

“So, will he need to have another surgery?” I asked in a noticeable irritated voice.

The physician answered yes.

“So, the first surgery was done incorrectly and now he has to go in again?”

This is the type of time that my wife would often describe as “Derek acting like an ass.”

I should have been more aware that there was no reason to be angry with the surgeons. They were trying to thread a line into the body of a baby. That cannot be an easy task.

The physician calmly indicated that the line placement was not optimal and that Bubba would need to have another procedure. They wanted to schedule surgery as soon as possible. The surgeons would be able to use the existing incision.

We ran into one major issue: clotting factor.

There was none. You can’t operate on a hemophilia patient unless they’re factored up. Without available factor, there would be no surgery.

We were fortunate to have an incredible point of contact for our specialty pharmacy. Her name was Julie and she was aware that we were headed to St. Louis for Bubba’s surgery. In fact, she had given me her personal cell phone number in case anything happened. It didn’t really register at the time that the number might come in useful.

Our nurse told us that it could be a few days before they could be restocked.

I was not ashamed to call Julie. As expected, I didn’t get her at first. She probably saw a weird number come up on her cell and didn’t feel like hearing about her car’s expired warranty.

… and then Julie called back.

I explained what was going on and how we were certain she was a factor wizard who could make our necessary medication appear with the wave of a wand. Problem number one was that the pharmacy could not ship to the hospital. We found out that insurance would cover having Bubba’s factor shipped to us in St. Louis. We’d just take possession and then hand it over to the physician.

Julie was awesome and, I imagine, is still awesome. I doubt she’ll every read this article. If she does, Julie you are a superhero in the eyes of the Markley family.

We made one major change. Bubba was given medication prior to surgery that would help us avoid him turning into a screaming little devil spawn when the anesthesia wore off. We had faced enough challenges in a short time and there was no reason not to stack the deck in our favor in any way we could.

The outcome this time was far better. Surgery went quickly, Bubba did not awake yelling and crying, and we were able to ensure that the port was functional. Our emotional rollercoaster had finally come to a gentle stop instead of catching fire, going off the rails, and slamming into an animal shelteer filled with kittens. Things were much better the second time around.

Now the work would begin. Ashley and I would be trained to administer factor twice per week via Bubba’s new port. A new chapter in our life had begun!

Derek Marley 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’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.

Gene Therapy: Who Makes the Decision?

by Derek L. Markley

It has been more than eight years since St. Jude Children’s Research Hospital, University College London, and the Royal Free Hospital began a clinical trial using gene therapy to treat people with hemophilia B.

Derek and his son, Bubba

That same year, our family welcomed a new baby, Bubba, who has severe hemophilia B. We had recently located to central Illinois from east Tennessee when I accepted a position with Eastern Illinois University. Bringing a new child into the world is a wonderful and stressful time for any family. As parents who had no experience with bleeding disorders, my wife and I were wholly unprepared for a hemophilia diagnosis.

Six months after Bubba was born, I was headed to lunch with a friend after meetings in the state capitol building. I can still tell you exactly where I was standing when my friend handed me her phone and asked if I’d seen the New York Times article about gene therapy and hemophilia B.

Fast forward a few years, and we again relocated, this time to Tupelo, Mississippi. We were very fortunate to become patients at St. Jude. Little did we know that Bubba’s physician would be one of the primary investigators in the gene therapy clinical trial. All of a sudden, the world of gene therapy was thrust directly in front of us.

We are now in a position, like many other hemophilia families, where the reality of a cure for hemophilia seems within reach. Presently, three phase III trials are beginning to test the efficacy of gene therapy in treating hemophilia B. Clinical research does not move quickly, but advancements in gene therapy over the past eight years have been amazing. The FDA has released new guidelines regarding gene therapy development, and funding for these ventures is in overdrive.

If gene therapy treatment becomes a reality, I’m forced to ask these questions: Is this a decision we can make for our son? Do the parents get to decide? How do we talk to our son about the risks and rewards of such a treatment? Bubba is only eight now, but it seems wise to begin thinking about how we’d handle this situation. Of course, the question is moot if insurers, treatment providers, and pharmaceutical companies can’t agree on a feasible reimbursement strategy. There are a lot of variables at play, yet the advancement of gene therapy treatment demands that Bubba’s mother and I begin thinking about how we will make decisions if a gene therapy treatment becomes available for our son.

It’s amazing that, of all the health conditions in our world, science and medicine have combined to put a treatment for our son’s type of hemophilia at the forefront of gene therapy.

Bubba is a very active kid. My wife describes him as “all boy.” His condition has done nothing to slow him down. Bumps, bruises, cuts, or scrapes, he weathers them all and refuses to be limited by his uncooperative blood.

We’re reaching a time where Bubba’s questions about hemophilia have become more mature, requiring us to be more introspective when talking with him. The greatest question we face is how do we determine his role in a decision about treatment if gene therapy becomes a reality? It is his life, and, like all parents, we hope that he’ll be around for a long time after we’re gone.

Bubba views infusions as an inconvenience. We know he secretly wants to play quarterback or wide receiver. He knows that hemophilia makes him different from his friends. Different isn’t always easy for kids to understand or accept.

The problem is that his mother and I have more complex questions, not that Bubba’s questions are unimportant. What if our government decides to once again unfairly punish people with pre-existing conditions? What if Bubba is in a serious accident? What if he develops a target joint? There are a million tough questions. What makes the situation even harder is that these questions are about things over which we have little or no control. There are far more questions than answers.

The best answer we have right now is that we’re thankful for the treatment presently available for our son and the prospect of what some are beginning to call a cure. There are too many parents with children afflicted by conditions that have no available, effective treatments. When we picked Bubba up after his first bleeding disorder camp last summer, he told us that some of the kids said he was lucky because he only infused once a week. I think those interactions gave him his first insight into the fact that living with hemophilia is not the same for everyone.

Science can do amazing things. As a species, we’re quite ingenious at times. I have no doubt that hemophilia will cease to exist one day, but I have no idea when that day will come. As parents, we can only put our son’s best interests first, stay informed about medical advances, and trust that our team at St. Jude will continue to help us make the best decisions based on the options available.

Bubba won’t want to hear it, but his mom isn’t going to let him play football anyway.


Derek 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 fifth-grade math teacher in the Tupelo Public School District. Derek is the author of The Bubba Factor, available on Amazon in Kindle format and in paperback.

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