This episode was taped live during the recent “Well Beyond Medicine” Day in Jacksonville, Florida at the reimagined THE PLAYERS Center for Childhood Cancer & Blood Disorders at Nemours Children’s Health, Jacksonville. The Center includes the only pediatric sickle cell program in Jacksonville which is also one of the largest programs in the state of Florida.
We were joined by Nemours Children’s hematologist/oncologist Dr. Julian Zorrilla and self-professed advocate, warrior and patient Fredrianna Copeland Webster to discuss sickle cell disease — myths, facts and support.
Carol Vassar, producer and host
Julian Zorrilla, DO, Pediatric Hematologist/Oncologist, Nemours Children’s Health, Jacksonville
Fredrianna Copeland Webster, advocate and sickle cell disease patient, Nemours Children’s Health, Jacksonville
CAROL VASSAR: Welcome to Well Beyond Medicine, the Nemours Children’s Health Podcast.
Each week, we’ll explore anything and everything related to the 80% of child health impacts that occur outside the doctor’s office. I’m your host, Carol Vassar. And now that you’re here, let’s go.
Let’s go well beyond medicine.
DR. JULIAN ZORRILLA: The center has been a great contribution for our department. It just opened up this past summer in June. So this allowed us to, one, expand on our clinic in terms of having more patient rooms so we could afford to see more patients and help out the kids that we see. Now, we have the infusion center, which also helps streamline what treatments we could give for our kids and then as well as they need blood transfusions or anything like that, we’re able to give it right next door.
CAROL VASSAR: That’s Nemours Children’s Health hematologist-oncologist Dr. Julian Zorrilla, extolling the benefits for patients and families of the new state-of-the-art facility within the Nemours Jacksonville, Florida clinic named THE PLAYERS Center for Cancer and Blood Disorders, so-called due to the generosity of THE PLAYERS Golf Championship, which made the lead gift of $2,000,000 toward the center’s complete redesign, expansion, and renovation.
THE PLAYERS Center for Cancer and Blood Disorders at Nemours Children’s Health includes the only pediatric sickle cell program in Jacksonville and one of the largest in the state of Florida. And it’s sickle cell disease we’re talking about today with Dr. Zorrilla and self-professed sickle cell advocate, warrior, and patient Fredrianna Copeland Webster. Fredrianna and Dr. Zorrilla sat down with us for an interview as part of Well Beyond Medicine Day in Jacksonville earlier this month to help raise awareness about this group of blood disorders we know as sickle cell disease. But what does that encompass exactly? Here’s Fredrianna Copeland Webster.
FREDRIANNA COPELAND WEBSTER: So sickle cell disease is a genetic disorder, meaning that you are born with sickle cell disease. Luckily, in 2004, which is the year of my birth, there was screening available for all babies in the United States with sickle cell. So, as soon as I was born, I was tested, and my mom knew that I had sickle cell disease. So, I grew up knowing that I had sickle cell disease. And I grew up knowing how it might impact my life.
CAROL VASSAR: You also had a bout of cancer when you were young. Talk about that a little bit.
FREDRIANNA COPELAND WEBSTER: The cancer aspect, I don’t remember much of because I was cured from cancer after reaching one year old. My mom lived through that. My dad lived through that. My parents, they took care of me as I was going through that. I do know that I had neuroblastoma cancer. I do know that I had surgery to remove five cancer tumors from my abdomen. And I also know that I had chemotherapy in order to treat those tumors.
CAROL VASSAR: And the two are not related, are they?
FREDRIANNA COPELAND WEBSTER: No, they’re not related at all.
CAROL VASSAR: Dr. Zorrilla, talk a little bit more about the genetic component of sickle cell disease. Fredrianna gave us a great summary of what sickle cell is. It’s a genetic disease. Talk a little bit about that more in-depth from a clinical perspective.
DR. JULIAN ZORRILLA: Yeah. I know Fredrianna brought up a good point that in more recent years that we are able to do more newborn screening and testing, and because the important thing that she mentioned was that sickle cell being a genetic disease is very important for physicians and the medical community to understand how the disease is passed on within the families and stuff like that. And that way, families can be educated as well knowing what kind of risks they have when they want to start family planning and stuff like that.
CAROL VASSAR: Talk about sickle cell disease and how it affects the body. I know that the red blood cells are actually shaped in a sickle form. But I don’t know much more than that. Tell me more.
DR. JULIAN ZORRILLA: Right. Yeah. You’re correct. Normally, our red blood cells should be circular and disk-shaped. But in sickle cell, what happens because of that genetic mutation they develop a sickle shape. And because of that sickling going on, it makes the red blood cells prone to breaking apart, which could cause anemia. And that’s where it gets the name sickle cell anemia. But also, when the red blood cells develop that sickling shape, it makes them a little bit stickier in that when they pass through the small blood vessels, called the capillaries, they could start causing little blockages in people affected with sickle cell.
CAROL VASSAR: I understand it’s also very painful when a sickle cell episode occurs.
FREDRIANNA COPELAND WEBSTER: Yes. CAROL VASSAR: Fredrianna, you were quick to say yes. Talk about that.
FREDRIANNA COPELAND WEBSTER: OK. So, as Dr. Zorrilla said, sickle cell causes the red blood cells to sickle. And they cause blockages of blood flow, and this causes pain. And this pain can be anywhere in the body, from the legs to the arms to the chest, anywhere where our blood vessels are. So, this pain can be triggered by changes in temperature. It can be triggered by stress. It can be triggered by dehydration and so much more.
And there are other complications to sickle cell as well, such as organ damage, and organ failure, and things like that, because our organs they need blood supply, and they need oxygen being supplied to them. So, for example, when our brain isn’t getting enough blood. We might potentially have a stroke. When our eyes aren’t getting enough blood, then we might have vision loss and things such as that. And it really breaks my heart to know that there are people with sickle cell who are close to my age who have had to have procedures, and hip replacements, and all types of other things just so that they can live a normal life.
CAROL VASSAR: How has having sickle cell disease– you’ve known this your entire life. How has it affected your life? You– I’m sure that school, you’ve missed some school along the way. You’ve had work maybe and relationships that are impacted by the disease. Talk about that. And also talk about how you compensate for that because you are a very knowledgeable woman about what’s happening to you.
FREDRIANNA COPELAND WEBSTER: Thank you. I must say that I now have a strong hold on sickle cell disease. But at one point in my life, sickle cell had a strong hold on me. As early as elementary school, I would miss weeks of school, having to be hospitalized, given pain medications around the clock, fluids, maybe a blood transfusion. And then, when I would feel better, I would come back home. I would be sent back to school. And what was there waiting for me is a stack of schoolwork, maybe some missed tests, maybe a project I have to start on, which you can imagine to be pretty stressful, especially after I progressed in. I got to middle school, and I got to high school.
And high school, I attended Paxon School for Advanced Studies, which is a college preparatory school. So, I was taking very rigorous classes. I had a very heavy workload. And at this point in my life, I started experiencing something known as chronic pain that people with sickle cell may also experience. That meant that I was waking up in pain. I was going to sleep in pain. And this lasted for months on end. I actually ended up missing months of school. I had to be hospital homebound. I had to do my schoolwork from home while I was also in pain at the time.
Luckily, I was able to start new medical treatments, such as Adakveo, and they made my quality of life much better. I was able to graduate on time from high school. I was able to start college. I am now a sophomore in college. But that definitely did have a tremendous impact on my life. I can’t remember making any friends in high school. But now, as I am in college, my life is much better, and I do have friends now.
CAROL VASSAR: Dr. Zorrilla, let me ask you this. The treatments that Fredrianna was just talking about, what are the current treatments for children, young adults, and older adults with sickle cell disease? And how have they progressed over time?
DR. JULIAN ZORRILLA: Yeah. I would say one of the longest-standing medical treatments that we have for sickle cell disease, with the most literature out there, is hydroxyurea, which helps change your hemoglobin to prevent that sickling going on in your blood. So hydroxyurea is one of the mainstays in the treatments for sickle cell.
But like Fredrianna was saying, nowadays we’ve come to more recent advances where now we have crizanluzimab that Fredrianna mentioned. We also have voxelotor as well that’s been approved for people with sickle cell disease. There are also curative options as well, like doing a bone marrow transplant, and then as well as now on the horizons, the newest thing would be gene therapy eventually.
CAROL VASSAR: How often do bone marrow transplants come into play? And what are the circumstances that allow someone to get a bone marrow transplant that could possibly cure their sickle cell disease?
DR. JULIAN ZORRILLA: Right now, with bone marrow transplant, there’s still ongoing studies. And a lot of those studies have to do related to what kind of suitable donor you can have to be able to perform a transplant. Ideally, we would want a sibling of the patient who is a perfect match for them. That way, their bone marrow could accept their new bone marrow from their brother and sister. Usually, when it comes to transplant, it’s mostly for those patients who have the severe complications that Fredrianna had mentioned; like notably, patients that have, unfortunately, suffered a stroke would be an example of who might be a candidate for transplant.
CAROL VASSAR: Fredrianna, let me ask you this. What are the misconceptions of sickle cell disease? What– Let’s do some myth-busting here. What is it not?
FREDRIANNA COPELAND WEBSTER: First, let me say that sickle cell is a group of blood disorders, meaning that there are different types of sickle cell disease with different severities. So, my type of sickle cell might not equate to someone else’s type of sickle cell. So, you can’t label every person with sickle cell as the same condition, the same person with the same condition. And let me also say that, unfortunately, many people with sickle cell, typically the adults, they are labeled as drug-seekers when they go into the emergency room. And unfortunately, they steer away from the emergency room because they feel that they won’t be treated properly.
CAROL VASSAR: Dr. Zorrilla, I want to pull a thread that Fredrianna had mentioned. And that is that not every sickle cell is the same, that this is a group of diseases. Expand on that.
DR. JULIAN ZORRILLA: When she was mentioning how it’s a group of diseases, that becomes very important for families to know because oftentimes, what happens is that either parent may understand that they’re sickle trait, and they may not know that their other partner has some type of other hemoglobin issues, like beta-thalassemia, for example. And what they are often not aware of is that when you have people with sickle trait or thalassemia, that still encompasses the condition of sickle cell disease.
CAROL VASSAR: This is a genetic disease. Can it be found prior to birth? Or should parents who know that they are carrying the trait be aware that they might have a child with sickle cell prior to– should they have genetic testing prior to becoming pregnant?
DR. JULIAN ZORRILLA: Even prior to genetic testing, there is other tests that they could do that doesn’t involve genetics in terms of being like what we call a hemoglobin electrophoresis. And if that is able to pick up on hemoglobin S, then that will be diagnostic that that individual has sickle trait.
CAROL VASSAR: Let me ask this. How can we raise more awareness about this? It seems like this is a disease that we don’t hear a lot about.
FREDRIANNA COPELAND WEBSTER: I think the best way to get the word out about anything nowadays is through the media, social media, the news, any type of online platform. So I think that would be the best way, just pushing that awareness through online platforms. And I recently started my own YouTube channel for sickle cell advocacy and awareness.
And I have big plans to really educate a wider audience of people this way and also getting involved with organizations that want to help people with sickle cell specifically, such as the Richard Webster-Bass Health Institute. That is my aunt Selena Webster-Bass’ organization named after her now-deceased son Richard Webster-Bass. I wish she could have been here with me today. But I know she is a very busy lady.
CAROL VASSAR: Your aunt Selena has this organization that is, again, raising awareness about how to prevent those kinds of unfortunate deaths and see people have a longer lifespan. Are we seeing a longer lifespan of people with sickle cell disease, Dr. Zorrilla?
DR. JULIAN ZORRILLA: Yeah, definitely, compared to years prior. I mean, now we are able to push the envelope in how we could extend lives for people with sickle cell. Before, it used to be a big burden for even the toddlers in terms of their risk of infections. But now, we’re– due to preventative measures and with the disease-modifying medicines that we have, we’re able to push those boundaries and improve life.
CAROL VASSAR: Fredrianna, have you done any of this advocacy or policy work? Is that something that interests you?
FREDRIANNA COPELAND WEBSTER: In the long run, I hope to connect with some leaders in policy change and also in healthcare. Right now, I’m just focusing on raising awareness and getting the word out about sickle cell disease.
CAROL VASSAR: What advice would you give to others like yourself living with sickle cell disease or maybe their loved ones with regard to support and understanding? What don’t we know?
FREDRIANNA COPELAND WEBSTER: My advice would be to know that there is a community out here for them. The sickle cell community, we are stronger together. We are stronger unified. I would like them to know that it’s important to have a strong support system and that we are here for them. And also, for the individuals with sickle cell, I would like them to know that we are the ones who have sickle cell. We know sickle cell better than anyone else. So, we are the best advocates for ourselves.
CAROL VASSAR: What’s it been like? You said you’ve had– I think your entire life, you’ve been receiving treatment here in Jacksonville from Nemours. What’s your experience been with the folks here?
FREDRIANNA COPELAND WEBSTER: Lovely. Nemours is the best. I get the best treatment. I have the best doctors. They have educated me so much about sickle cell disease. They’re all so friendly. And I just love Nemours. And I’m actually pretty sad that I’ll have to transition to adult care pretty soon.
CAROL VASSAR: Aw. Fredrianna, talk about mentally and emotionally, how do you cope with having a chronic disease?
FREDRIANNA COPELAND WEBSTER: Sickle cell definitely takes a toll on the mental health. I actually researched, and I found that 1 in every 3 people with sickle cell will have depression at one point in their lifetime. My strongest coping mechanism is my faith and my belief in God. So every time I have a sickle cell pain crisis, and when I’m also having a mental breakdown because I’m so stressed out about it, I pray. And even when I feel that my life is on the right track, I pray to God. And also having my support system, my family, my friends, my parents. They’re always there when I need them.
CAROL VASSAR: How are you feeling generally today?
FREDRIANNA COPELAND WEBSTER: I feel great. It’s great to be here. I feel good today.
CAROL VASSAR: Good. Good. Good. Anything I haven’t asked about that you think we should include in a podcast about sickle cell disease, either from the scientific medical perspective, clinical perspective, or from your personal perspective, Fredrianna?
FREDRIANNA COPELAND WEBSTER: Yes, actually. I would like to mention the challenge that I hear about. Every time people that I know with sickle cell go into an emergency room, they usually have to wait a very long time before they’re able to see a doctor, which is incredibly saddening because imagine waiting in the emergency room at a 10 out of 10 pain level, and your pain is only progressing. And you need these strong pain medications as soon as possible.
But every time I go into an emergency room, the triage nurse they pull my charts, and they see that I have a history for cancer. And they automatically put me at the front of the waiting list, so I don’t have to wait longer than 10 minutes to be seen by a doctor. So I don’t understand why that can’t be done for everyone with sickle cell disease.
CAROL VASSAR: Something to work on.
FREDRIANNA COPELAND WEBSTER: Definitely. There should be a policy change in place for that.
DR. JULIAN ZORRILLA: Yeah. No. And one of the things that we are hoping to discuss with our ER staff is actually to have those quality improvement projects in terms of decreasing that time that our kids with sickle cell have their first administered pain medicine because what we do know is that the longer we let these pain episodes ride out, the more severe and obviously the decreased quality of life we have for our kids.
CAROL VASSAR: It becomes more dangerous, really, if the pain isn’t addressed immediately.
DR. JULIAN ZORRILLA: Yes, most definitely.
CAROL VASSAR: Fredrianna.
FREDRIANNA COPELAND WEBSTER: I would just like to say that I won’t rest until progress is made. My ultimate goal is to find a universal cure for sickle cell disease. My major is biomedical science, and I aspire to be a clinical researcher to actually go into the field, find better treatments, and hopefully a cure for sickle cell disease. And sickle cell will not stop me.
CAROL VASSAR: So this is really driving your life.
FREDRIANNA COPELAND WEBSTER: Definitely.
CAROL VASSAR: In a positive manner.
FREDRIANNA COPELAND WEBSTER: Yes, ma’am.
CAROL VASSAR: Well, hopefully, you’ll be finding that cure or somebody you know. Dr. Zorrilla.
DR. JULIAN ZORRILLA: It’s always awesome to hear that. I feel like in my time in the hematology space, I feel that sickle cell is starting to gain ground, and more awareness, and stuff like that. And so it’s awesome to see there’s more people advocating for sickle cell, and there’s more sickle cell warriors.
I would definitely advocate for people to reach out to their policymakers or lawmakers in government because I know that currently, there is the Sickle Cell Comprehensive Care Act that was recently introduced earlier this year in Congress. And so the more people we have advocating for that bill to pass, then eventually, down the line, we will be able to have improved coordinated care for people with sickle cell.
CAROL VASSAR: Is that the gist of that bill, that there’s better and more coordinated care?
DR. JULIAN ZORRILLA: Yeah. That’s correct. That is the ultimate goal is ultimately to model some other type of diseases that we treat, like hemophilia, for example, is very coordinated and comprehensive when our patients come in. And so that’s the goal, to bring that model with sickle cell disease, because, like Fredrianna was mentioning is that, it does involve a lot of symptoms and complications that often involve very coordinated medical care.
CAROL VASSAR: Dr. Julian Zorrilla is a hematologist oncologist with THE PLAYERS Center for Cancer and Blood Disorders at Nemours Children’s Health. We also heard from Fredriana Copeland Webster, sickle cell disease advocate, warrior, and patient.
CAROL VASSAR: Our roving reporter, Cheryl Munn, was also at the Well Beyond Medicine Day in Jacksonville and caught up briefly with Dr. Peggy Greco, assistant vice president and chief experience officer for Nemours Children’s Health. Dr. Greco is a pediatric psychologist by training and experience. And so, we asked her what she believes are the three most significant influences on a child’s social, emotional, and mental health. Here’s Dr. Peggy Greco.
DR. PEGGY GRECO: That’s definitely the question of the day. I think we’re all aware of the increasing incidence of depression and suicidality in older kids and teens. And I think there tends to be a focus on social media. And when you think about the fact that when you look at the graphs that overlay both use of social media, and implementation of the iPhone, and incidence of depression, you see the corresponding inclines. The temptation is to, I think, really put all of our nickels in one pot and say, this is what we need to focus on. But it’s obviously much broader than that. So I think the three biggest influences are, one, basic needs. So, when you think about Maslow’s hierarchy of needs, do kids have the food that they need? Do they have shelter? Are they being basically sleeping at regular intervals and getting the sleep that they need? The second is safety. Emotional and psychological safety is necessary for growing up in a way that you have really robust emotional health. And the third influence, and this is, again, another broad one, are really caregivers. And that’s broader than just parents because it includes teachers and other adults that are in the child’s environment that are important to them and have that influence.
When you think about those three broad areas, I think it’s interesting to think about the intersection of those with social media. We tend to think of social media use only from the child’s perspective in terms of their time spent on it. We don’t think about the impact of parent time on it. I think one of the things we can think about are, how are our kids growing up differently than they were before the advent of all of these tools, which can also be very useful.
And I think one of the most striking differences that is visible to almost anyone who’s in a public location or has their own kids or friends that have kids is that you rarely see someone without an electronic device. And part of building emotional health is learning how to tolerate and cope with emotions. And instead, the reaction tends to be; I don’t have to experience this boredom, this disinterest, this irritation. I can escape.
So, even babies. I was in a DMV a while back helping my dad, and there was lots of crying babies there. Almost all of them had an electronic device. I didn’t see any books, no manipulatives, none of the toys that we know really stimulate child development. And so I think that’s something we have to give some thought to in terms of how are we teaching our children to experience emotions, to tolerate them, and cope with them. Yeah.
CHERYL MUNN: Yeah. I worry that they’re not learning how to self-soothe either.
DR. PEGGY GRECO: Exactly. They’re not learning the fact that they can be competent in their own ability to be able to deal with those emotions. Instead, there’s this tendency to escape, and that’s reinforced.
CHERYL MUNN: If you had a magic wand and you could just say, let me take this away, what would you do to improve children’s health?
DR. PEGGY GRECO: Taking away is always a lot harder than adding. So I think to be truly successful, I would instead add– layer back in– social media is here to stay. And there are some obvious benefits, and it’s not really social media that’s the sole cause. It’s really how we’re using it and our overreliance on it. So I would layer in very purposeful education around coping with emotions. So whether that involves pediatricians who are helping parents understand what their kids need emotionally from a very early age, whether it’s school-based curriculum that focuses on emotions and how to cope, but that we’re very purposeful as a society in terms of building tolerance to emotion and ability to cope.
CAROL VASSAR: Dr. Peggy Greco is Nemours’ chief experience officer.
Well beyond medicine.
CAROL VASSAR: Thanks for listening to our conversations recorded at Nemours Well Beyond Medicine Day in Jacksonville with me, Carol Vassar, and our guests Fredrianna Copeland Webster, Dr. Julian Zorrilla, and Dr. Peggy Greco.
What do you see as the most significant influences on the social, emotional, and mental health of the children in your life? Leave a voicemail on our website, nemourswellbeyond.org. That’s also the perfect place for you to submit ideas for upcoming podcast episodes and leave feedback and comments. That’s nemourswellbeyond.org. While you’re there, check out our other episodes and subscribe to the podcast.
Thanks to Che Parker, Susan Masucci, Cheryl Munn, and Kerry Ely for their production assistance. Join us next time as we begin a three-part series on artificial intelligence in healthcare. Until then, remember, together, we can change children’s health for good, well beyond medicine.
Let’s go well beyond medicine.