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Pediatrician with SCD gives her young patients hope
These days, thanks to transformative advances in treating SCD that have substantially improved survival, Dr. Fasipe’s mission for a new generation of patients and their families is to replace their pain and fear with relief and hope.
“If you grow up thinking that you’re going to die when you’re 18, it changes your world and your viewpoints, and it impacts your mental health,” she told this news organization.
“We are trying to make sure our children and their families know that there is a new story for sickle cell disease, and you don’t have to use any age as your prediction marker for your lifespan,” Dr. Fasipe said.
SCD, which affects about 100,000 people nationwide, is an inherited blood disorder, with the majority of patients – but not all – being of African descent. This condition is characterized by pain crises, or vaso-occlusive episodes, triggered when cells that are sickled get stuck and impede blood flow. These crises can come on suddenly and range from mild to severe.
Dr. Fasipe was born in Nigeria, where rates of SCD are among the world’s highest. She attended elementary school in the United States, where her father was studying theology, before returning to Nigeria with her family at age 11.
Back in those days, in both nations only about 50% of children with SCD lived beyond their 18th birthday. The survival rates in Nigeria and sub-Saharan Africa countries continue to be poor. In some more developed regions elsewhere, advances such as universal newborn screening, penicillin prophylaxis, pneumococcal vaccination, stroke screening, and hydroxyurea therapy have yielded substantial improvements, with 95% or more patients with SCD reaching their 18th birthday.
“With measures such as newborn screening, we can immediately start prevention measures in sickle cell disease, such as prevention of infection, which was the number one reason why children were dying,” Dr. Fasipe explained. “With global initiatives, we want that story to be the same in sub-Saharan Africa as well.”
Cousin’s early death inspires medical studies
In an essay published by Texas Medical Center that describes her childhood experiences, Dr. Fasipe recounts a pivotal event in her life: The heartbreaking death of her beloved cousin at the age of just 17, from a complication of SCD. This bereavement fueled Dr. Fasipe’s determination to pursue a medical career, to do all that she could to prevent such losses.
“Having sickle cell disease myself wasn’t the trigger that made me become a doctor. But when Femi [her cousin] died, I thought: ‘This shouldn’t happen,’ ” Dr. Fasipe wrote.
When she applied to medical school back in the United States, she declared in her application essay: “I want to cure sickle cell.”
By the time Dr. Fasipe was ready to undertake residency and fellowship applications, her essay had shifted to focus on pediatrics “specifically because I want to reach sickle cell patients before they’ve defined how their lives are going to be,” she said. “I want to give them hope.”
Hope for a cure
Fast-forwarding to this point in Dr. Fasipe’s career, she noted that her dream of a cure for SCD is no longer a distant aspiration, thanks to the advent of stem cell transplantation and more recently, gene therapy. These advancements have elevated her hope for a cure to an entirely new level.
Each new treatment comes with caveats. Stem cell transplantation requires a matching donor, leaving the majority of patients ineligible. And while gene therapy eliminates the need for a donor, treatment can reportedly cost nearly $3 million. Nevertheless, Dr. Fasipe emphasized the promise that these new advancements represent.
“The scientists that work in these spaces do appreciate these [accessibility barriers], and the expectation is these therapies will be more accessible with time and effort,” she said. “We’ve got to start somewhere, and it’s exciting that they’re making these early successes.”
Advice for clinicians
With firsthand knowledge of how it feels to be the patient, as well as on the clinician side of SCD treatment, Dr. Fasipe advises colleagues on some ways that they can improve care while boosting their patients’ hope:
Speak with empathy
Acknowledge the ‘elephant in the room’; the pain that patients with SCD can experience is real.
“When I’m managing any patient with pain, I first acknowledge the suffering because while we may not understand what that person is going through, acknowledgment is part of showing empathy,” she explains.
Seek out resources
Patients with SCD may typically seek treatment in primary care, where expertise in the disease may be lacking, and general practitioners may feel frustrated that there are limited treatment options.
“If you do find yourself treating a sickle cell disease patient, you may not have all of the answers, but there are good resources, whether it’s a nearby sickle cell disease centers or national guidelines,” Dr. Fasipe said.
Access to treatment
With research, including a recent study, showing that only about 25% of patients with SCD are prescribed hydroxyurea and even fewer – only about 5% – receive more recently approved SCD treatments, clinicians should be proactive by making sure that patients receive needed treatments.
“Clearly medicines like hydroxyurea are not as optimized in this community space as they should be, and then there are newer therapies that families, patients, and even providers may not be aware of, so it is important to be informed of the guidelines and provide all patients with comprehensive, high-quality care,” Dr. Fasipe said.
In the ED, patients with SCD are ‘care-seeking,’ not drug-seeking
Due to the sometimes rapid onset of severe pain symptoms, patients with SCD commonly wind up in the emergency department. In this time of an opioid epidemic, patients too often are suspected of merely seeking drugs.
“Sickle cell disease tends to get lumped into a category of a disease of pain, but pain is subjective and it is difficult to quantify, so unfortunately, patients can be labeled as potentially drug-seeking,” Dr. Fasipe explained, citing an article that detailed this problem.
Consequently, patients may have particularly negative experiences in the emergency department, but the use of resources such as a sickle cell disease point-of-care tool developed by the American College of Emergency Physicians and the American Society of Hematology can help improve care for those patients.
“One of the [point-of-care recommendations] before even managing the pain is that physicians show compassion by acknowledging the patient’s pain and that they understand why pain with sickle cell disease might look different than other types of pain,” Dr. Fasipe said.
Building trust
Encounters such as negative emergency department experiences can perpetuate a deeper issue of distrust between those with SCD and the medical community, which originated in long-held, well-documented racial disparities in health care.
“We know historically and even today that there are difficulties facing our families who are impacted by sickle cell disease, and they are related to structural racism and socioeconomic barriers,” Dr. Fasipe explained.
With these issues in mind, she said, “I refer to sickle cell disease as the medical representation of the Black experience in America.” However, she added, the good news is “we are now doing our best now to improve that.”
Among key efforts in building trust is the inclusion of patients with SCD and their families in as many aspects of research and clinical care as possible.
“In the global health care community, it is imperative to invite people with sickle cell disease and from the community to the decision-making table,” she noted.
“Now, when we’re talking about research for therapies, their expectation is that research trials and other initiatives for sickle cell disease must have input from the community; there are no initiatives for sickle cell disease that do not have input from the community.
“The patients and community members may not be experts on the science of sickle cell, but they’re experts on the lived experience and that’s very important when you’re thinking about new bringing in a new therapy.”
Forward momentum
Meanwhile, Dr. Fasipe observed, with the collective, advocacy-driven, forward momentum of the SCD community as a whole, things should only continue to improve.
“Because of the various barriers, some progress may not be immediately around the corner, but I do have confidence that this current generation of children with sickle cell will have improved health equity by the time they reach adulthood,” she said.
“I believe in this future, so I’m doing the work now, and it’s a promise I tell parents: I want your future adult child to live their best life, and we’re working hard to ensure that that becomes their future reality.”
Sickle cell disease awareness
September is National Sickle Cell Disease Awareness Month, and the National Heart, Lung, and Blood Institute offers a comprehensive website that clinicians can pass along to their patients, with information ranging from fact sheets on the disease and treatments to social media resources and inspiring stories of people with the disease.
In a comment, Lewis Hsu, MD, PhD, chief medical officer of the Sickle Cell Disease Association of America, underscored the uniquely important contributions of people like Dr. Fasipe, in providing inspiration to patients and clinicians alike.
“I have worked with several physicians, nurses, psychologists, and public health specialists who have sickle cell disease,” said Dr. Hsu, who is a pediatric hematologist who also serves as director of the Sickle Cell Center and professor of pediatrics for the University of Illinois at Chicago.
“They are ambassadors who have the trust of both patients and healthcare providers,” Dr. Hsu said.
In addition to providing inspiration of resilience, such care providers can serve as “communication bridges,” he explained.
“When they are conference speakers, everybody wants to hear them; when they sit on advisory committees or focus groups, they can help find the compromise or set the priorities.”
“Their impact on the whole sickle cell community is very large,” Dr. Hsu said.
These days, thanks to transformative advances in treating SCD that have substantially improved survival, Dr. Fasipe’s mission for a new generation of patients and their families is to replace their pain and fear with relief and hope.
“If you grow up thinking that you’re going to die when you’re 18, it changes your world and your viewpoints, and it impacts your mental health,” she told this news organization.
“We are trying to make sure our children and their families know that there is a new story for sickle cell disease, and you don’t have to use any age as your prediction marker for your lifespan,” Dr. Fasipe said.
SCD, which affects about 100,000 people nationwide, is an inherited blood disorder, with the majority of patients – but not all – being of African descent. This condition is characterized by pain crises, or vaso-occlusive episodes, triggered when cells that are sickled get stuck and impede blood flow. These crises can come on suddenly and range from mild to severe.
Dr. Fasipe was born in Nigeria, where rates of SCD are among the world’s highest. She attended elementary school in the United States, where her father was studying theology, before returning to Nigeria with her family at age 11.
Back in those days, in both nations only about 50% of children with SCD lived beyond their 18th birthday. The survival rates in Nigeria and sub-Saharan Africa countries continue to be poor. In some more developed regions elsewhere, advances such as universal newborn screening, penicillin prophylaxis, pneumococcal vaccination, stroke screening, and hydroxyurea therapy have yielded substantial improvements, with 95% or more patients with SCD reaching their 18th birthday.
“With measures such as newborn screening, we can immediately start prevention measures in sickle cell disease, such as prevention of infection, which was the number one reason why children were dying,” Dr. Fasipe explained. “With global initiatives, we want that story to be the same in sub-Saharan Africa as well.”
Cousin’s early death inspires medical studies
In an essay published by Texas Medical Center that describes her childhood experiences, Dr. Fasipe recounts a pivotal event in her life: The heartbreaking death of her beloved cousin at the age of just 17, from a complication of SCD. This bereavement fueled Dr. Fasipe’s determination to pursue a medical career, to do all that she could to prevent such losses.
“Having sickle cell disease myself wasn’t the trigger that made me become a doctor. But when Femi [her cousin] died, I thought: ‘This shouldn’t happen,’ ” Dr. Fasipe wrote.
When she applied to medical school back in the United States, she declared in her application essay: “I want to cure sickle cell.”
By the time Dr. Fasipe was ready to undertake residency and fellowship applications, her essay had shifted to focus on pediatrics “specifically because I want to reach sickle cell patients before they’ve defined how their lives are going to be,” she said. “I want to give them hope.”
Hope for a cure
Fast-forwarding to this point in Dr. Fasipe’s career, she noted that her dream of a cure for SCD is no longer a distant aspiration, thanks to the advent of stem cell transplantation and more recently, gene therapy. These advancements have elevated her hope for a cure to an entirely new level.
Each new treatment comes with caveats. Stem cell transplantation requires a matching donor, leaving the majority of patients ineligible. And while gene therapy eliminates the need for a donor, treatment can reportedly cost nearly $3 million. Nevertheless, Dr. Fasipe emphasized the promise that these new advancements represent.
“The scientists that work in these spaces do appreciate these [accessibility barriers], and the expectation is these therapies will be more accessible with time and effort,” she said. “We’ve got to start somewhere, and it’s exciting that they’re making these early successes.”
Advice for clinicians
With firsthand knowledge of how it feels to be the patient, as well as on the clinician side of SCD treatment, Dr. Fasipe advises colleagues on some ways that they can improve care while boosting their patients’ hope:
Speak with empathy
Acknowledge the ‘elephant in the room’; the pain that patients with SCD can experience is real.
“When I’m managing any patient with pain, I first acknowledge the suffering because while we may not understand what that person is going through, acknowledgment is part of showing empathy,” she explains.
Seek out resources
Patients with SCD may typically seek treatment in primary care, where expertise in the disease may be lacking, and general practitioners may feel frustrated that there are limited treatment options.
“If you do find yourself treating a sickle cell disease patient, you may not have all of the answers, but there are good resources, whether it’s a nearby sickle cell disease centers or national guidelines,” Dr. Fasipe said.
Access to treatment
With research, including a recent study, showing that only about 25% of patients with SCD are prescribed hydroxyurea and even fewer – only about 5% – receive more recently approved SCD treatments, clinicians should be proactive by making sure that patients receive needed treatments.
“Clearly medicines like hydroxyurea are not as optimized in this community space as they should be, and then there are newer therapies that families, patients, and even providers may not be aware of, so it is important to be informed of the guidelines and provide all patients with comprehensive, high-quality care,” Dr. Fasipe said.
In the ED, patients with SCD are ‘care-seeking,’ not drug-seeking
Due to the sometimes rapid onset of severe pain symptoms, patients with SCD commonly wind up in the emergency department. In this time of an opioid epidemic, patients too often are suspected of merely seeking drugs.
“Sickle cell disease tends to get lumped into a category of a disease of pain, but pain is subjective and it is difficult to quantify, so unfortunately, patients can be labeled as potentially drug-seeking,” Dr. Fasipe explained, citing an article that detailed this problem.
Consequently, patients may have particularly negative experiences in the emergency department, but the use of resources such as a sickle cell disease point-of-care tool developed by the American College of Emergency Physicians and the American Society of Hematology can help improve care for those patients.
“One of the [point-of-care recommendations] before even managing the pain is that physicians show compassion by acknowledging the patient’s pain and that they understand why pain with sickle cell disease might look different than other types of pain,” Dr. Fasipe said.
Building trust
Encounters such as negative emergency department experiences can perpetuate a deeper issue of distrust between those with SCD and the medical community, which originated in long-held, well-documented racial disparities in health care.
“We know historically and even today that there are difficulties facing our families who are impacted by sickle cell disease, and they are related to structural racism and socioeconomic barriers,” Dr. Fasipe explained.
With these issues in mind, she said, “I refer to sickle cell disease as the medical representation of the Black experience in America.” However, she added, the good news is “we are now doing our best now to improve that.”
Among key efforts in building trust is the inclusion of patients with SCD and their families in as many aspects of research and clinical care as possible.
“In the global health care community, it is imperative to invite people with sickle cell disease and from the community to the decision-making table,” she noted.
“Now, when we’re talking about research for therapies, their expectation is that research trials and other initiatives for sickle cell disease must have input from the community; there are no initiatives for sickle cell disease that do not have input from the community.
“The patients and community members may not be experts on the science of sickle cell, but they’re experts on the lived experience and that’s very important when you’re thinking about new bringing in a new therapy.”
Forward momentum
Meanwhile, Dr. Fasipe observed, with the collective, advocacy-driven, forward momentum of the SCD community as a whole, things should only continue to improve.
“Because of the various barriers, some progress may not be immediately around the corner, but I do have confidence that this current generation of children with sickle cell will have improved health equity by the time they reach adulthood,” she said.
“I believe in this future, so I’m doing the work now, and it’s a promise I tell parents: I want your future adult child to live their best life, and we’re working hard to ensure that that becomes their future reality.”
Sickle cell disease awareness
September is National Sickle Cell Disease Awareness Month, and the National Heart, Lung, and Blood Institute offers a comprehensive website that clinicians can pass along to their patients, with information ranging from fact sheets on the disease and treatments to social media resources and inspiring stories of people with the disease.
In a comment, Lewis Hsu, MD, PhD, chief medical officer of the Sickle Cell Disease Association of America, underscored the uniquely important contributions of people like Dr. Fasipe, in providing inspiration to patients and clinicians alike.
“I have worked with several physicians, nurses, psychologists, and public health specialists who have sickle cell disease,” said Dr. Hsu, who is a pediatric hematologist who also serves as director of the Sickle Cell Center and professor of pediatrics for the University of Illinois at Chicago.
“They are ambassadors who have the trust of both patients and healthcare providers,” Dr. Hsu said.
In addition to providing inspiration of resilience, such care providers can serve as “communication bridges,” he explained.
“When they are conference speakers, everybody wants to hear them; when they sit on advisory committees or focus groups, they can help find the compromise or set the priorities.”
“Their impact on the whole sickle cell community is very large,” Dr. Hsu said.
These days, thanks to transformative advances in treating SCD that have substantially improved survival, Dr. Fasipe’s mission for a new generation of patients and their families is to replace their pain and fear with relief and hope.
“If you grow up thinking that you’re going to die when you’re 18, it changes your world and your viewpoints, and it impacts your mental health,” she told this news organization.
“We are trying to make sure our children and their families know that there is a new story for sickle cell disease, and you don’t have to use any age as your prediction marker for your lifespan,” Dr. Fasipe said.
SCD, which affects about 100,000 people nationwide, is an inherited blood disorder, with the majority of patients – but not all – being of African descent. This condition is characterized by pain crises, or vaso-occlusive episodes, triggered when cells that are sickled get stuck and impede blood flow. These crises can come on suddenly and range from mild to severe.
Dr. Fasipe was born in Nigeria, where rates of SCD are among the world’s highest. She attended elementary school in the United States, where her father was studying theology, before returning to Nigeria with her family at age 11.
Back in those days, in both nations only about 50% of children with SCD lived beyond their 18th birthday. The survival rates in Nigeria and sub-Saharan Africa countries continue to be poor. In some more developed regions elsewhere, advances such as universal newborn screening, penicillin prophylaxis, pneumococcal vaccination, stroke screening, and hydroxyurea therapy have yielded substantial improvements, with 95% or more patients with SCD reaching their 18th birthday.
“With measures such as newborn screening, we can immediately start prevention measures in sickle cell disease, such as prevention of infection, which was the number one reason why children were dying,” Dr. Fasipe explained. “With global initiatives, we want that story to be the same in sub-Saharan Africa as well.”
Cousin’s early death inspires medical studies
In an essay published by Texas Medical Center that describes her childhood experiences, Dr. Fasipe recounts a pivotal event in her life: The heartbreaking death of her beloved cousin at the age of just 17, from a complication of SCD. This bereavement fueled Dr. Fasipe’s determination to pursue a medical career, to do all that she could to prevent such losses.
“Having sickle cell disease myself wasn’t the trigger that made me become a doctor. But when Femi [her cousin] died, I thought: ‘This shouldn’t happen,’ ” Dr. Fasipe wrote.
When she applied to medical school back in the United States, she declared in her application essay: “I want to cure sickle cell.”
By the time Dr. Fasipe was ready to undertake residency and fellowship applications, her essay had shifted to focus on pediatrics “specifically because I want to reach sickle cell patients before they’ve defined how their lives are going to be,” she said. “I want to give them hope.”
Hope for a cure
Fast-forwarding to this point in Dr. Fasipe’s career, she noted that her dream of a cure for SCD is no longer a distant aspiration, thanks to the advent of stem cell transplantation and more recently, gene therapy. These advancements have elevated her hope for a cure to an entirely new level.
Each new treatment comes with caveats. Stem cell transplantation requires a matching donor, leaving the majority of patients ineligible. And while gene therapy eliminates the need for a donor, treatment can reportedly cost nearly $3 million. Nevertheless, Dr. Fasipe emphasized the promise that these new advancements represent.
“The scientists that work in these spaces do appreciate these [accessibility barriers], and the expectation is these therapies will be more accessible with time and effort,” she said. “We’ve got to start somewhere, and it’s exciting that they’re making these early successes.”
Advice for clinicians
With firsthand knowledge of how it feels to be the patient, as well as on the clinician side of SCD treatment, Dr. Fasipe advises colleagues on some ways that they can improve care while boosting their patients’ hope:
Speak with empathy
Acknowledge the ‘elephant in the room’; the pain that patients with SCD can experience is real.
“When I’m managing any patient with pain, I first acknowledge the suffering because while we may not understand what that person is going through, acknowledgment is part of showing empathy,” she explains.
Seek out resources
Patients with SCD may typically seek treatment in primary care, where expertise in the disease may be lacking, and general practitioners may feel frustrated that there are limited treatment options.
“If you do find yourself treating a sickle cell disease patient, you may not have all of the answers, but there are good resources, whether it’s a nearby sickle cell disease centers or national guidelines,” Dr. Fasipe said.
Access to treatment
With research, including a recent study, showing that only about 25% of patients with SCD are prescribed hydroxyurea and even fewer – only about 5% – receive more recently approved SCD treatments, clinicians should be proactive by making sure that patients receive needed treatments.
“Clearly medicines like hydroxyurea are not as optimized in this community space as they should be, and then there are newer therapies that families, patients, and even providers may not be aware of, so it is important to be informed of the guidelines and provide all patients with comprehensive, high-quality care,” Dr. Fasipe said.
In the ED, patients with SCD are ‘care-seeking,’ not drug-seeking
Due to the sometimes rapid onset of severe pain symptoms, patients with SCD commonly wind up in the emergency department. In this time of an opioid epidemic, patients too often are suspected of merely seeking drugs.
“Sickle cell disease tends to get lumped into a category of a disease of pain, but pain is subjective and it is difficult to quantify, so unfortunately, patients can be labeled as potentially drug-seeking,” Dr. Fasipe explained, citing an article that detailed this problem.
Consequently, patients may have particularly negative experiences in the emergency department, but the use of resources such as a sickle cell disease point-of-care tool developed by the American College of Emergency Physicians and the American Society of Hematology can help improve care for those patients.
“One of the [point-of-care recommendations] before even managing the pain is that physicians show compassion by acknowledging the patient’s pain and that they understand why pain with sickle cell disease might look different than other types of pain,” Dr. Fasipe said.
Building trust
Encounters such as negative emergency department experiences can perpetuate a deeper issue of distrust between those with SCD and the medical community, which originated in long-held, well-documented racial disparities in health care.
“We know historically and even today that there are difficulties facing our families who are impacted by sickle cell disease, and they are related to structural racism and socioeconomic barriers,” Dr. Fasipe explained.
With these issues in mind, she said, “I refer to sickle cell disease as the medical representation of the Black experience in America.” However, she added, the good news is “we are now doing our best now to improve that.”
Among key efforts in building trust is the inclusion of patients with SCD and their families in as many aspects of research and clinical care as possible.
“In the global health care community, it is imperative to invite people with sickle cell disease and from the community to the decision-making table,” she noted.
“Now, when we’re talking about research for therapies, their expectation is that research trials and other initiatives for sickle cell disease must have input from the community; there are no initiatives for sickle cell disease that do not have input from the community.
“The patients and community members may not be experts on the science of sickle cell, but they’re experts on the lived experience and that’s very important when you’re thinking about new bringing in a new therapy.”
Forward momentum
Meanwhile, Dr. Fasipe observed, with the collective, advocacy-driven, forward momentum of the SCD community as a whole, things should only continue to improve.
“Because of the various barriers, some progress may not be immediately around the corner, but I do have confidence that this current generation of children with sickle cell will have improved health equity by the time they reach adulthood,” she said.
“I believe in this future, so I’m doing the work now, and it’s a promise I tell parents: I want your future adult child to live their best life, and we’re working hard to ensure that that becomes their future reality.”
Sickle cell disease awareness
September is National Sickle Cell Disease Awareness Month, and the National Heart, Lung, and Blood Institute offers a comprehensive website that clinicians can pass along to their patients, with information ranging from fact sheets on the disease and treatments to social media resources and inspiring stories of people with the disease.
In a comment, Lewis Hsu, MD, PhD, chief medical officer of the Sickle Cell Disease Association of America, underscored the uniquely important contributions of people like Dr. Fasipe, in providing inspiration to patients and clinicians alike.
“I have worked with several physicians, nurses, psychologists, and public health specialists who have sickle cell disease,” said Dr. Hsu, who is a pediatric hematologist who also serves as director of the Sickle Cell Center and professor of pediatrics for the University of Illinois at Chicago.
“They are ambassadors who have the trust of both patients and healthcare providers,” Dr. Hsu said.
In addition to providing inspiration of resilience, such care providers can serve as “communication bridges,” he explained.
“When they are conference speakers, everybody wants to hear them; when they sit on advisory committees or focus groups, they can help find the compromise or set the priorities.”
“Their impact on the whole sickle cell community is very large,” Dr. Hsu said.
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This material may not be published, broadcast, copied, or otherwise reproduced or distributed without the prior written permission of Frontline Medical Communications Inc.</copyrightNotice> </rightsInfo> </provider> <abstract/> <metaDescription>Having grown up with sickle cell disease (SCD), Titilope Fasipe, MD, PhD, codirector of the sickle cell program at Texas Children’s Hospital in Houston, knows f</metaDescription> <articlePDF/> <teaserImage>298058</teaserImage> <teaser>A Nigerian-American hematologist draws on her experience living with sickle cell disease to encourage child patients and advise colleagues. </teaser> <title>Pediatrician with SCD gives her young patients hope</title> <deck/> <disclaimer/> <AuthorList/> <articleURL/> <doi/> <pubMedID/> <publishXMLStatus/> <publishXMLVersion>1</publishXMLVersion> <useEISSN>0</useEISSN> <urgency/> <pubPubdateYear/> <pubPubdateMonth/> <pubPubdateDay/> <pubVolume/> <pubNumber/> <wireChannels/> <primaryCMSID/> <CMSIDs/> <keywords/> <seeAlsos/> <publications_g> <publicationData> <publicationCode>hemn</publicationCode> <pubIssueName/> <pubArticleType/> <pubTopics/> <pubCategories/> <pubSections/> </publicationData> </publications_g> <publications> <term canonical="true">18</term> </publications> <sections> <term canonical="true">27980</term> <term>39313</term> </sections> <topics> <term>182</term> <term canonical="true">191</term> </topics> <links> <link> <itemClass qcode="ninat:picture"/> <altRep contenttype="image/jpeg">images/2401226b.jpg</altRep> <description role="drol:caption">Dr. Titilope Fasipe, MD</description> <description role="drol:credit">Courtesy Racheal Adetayo Artistry</description> </link> </links> </header> <itemSet> <newsItem> <itemMeta> <itemRole>Main</itemRole> <itemClass>text</itemClass> <title>Pediatrician with SCD gives her young patients hope</title> <deck/> </itemMeta> <itemContent> <p><span class="tag metaDescription">Having grown up with sickle cell disease (SCD), Titilope Fasipe, MD, PhD, codirector of the sickle cell program at Texas Children’s Hospital in Houston, knows firsthand the physical pain, mental anguish, and dread that have long accompanied this condition. So few child patients lived to reach adulthood that until recently, SCD was considered a pediatric disease.</span> </p> <p>These days, thanks to transformative advances in treating SCD that have substantially improved survival, Dr. Fasipe’s mission for a new generation of patients and their families is to replace their pain and fear with relief and hope.<br/><br/>[[{"fid":"298058","view_mode":"medstat_image_flush_right","fields":{"format":"medstat_image_flush_right","field_file_image_alt_text[und][0][value]":"Dr. Titilope Fasipe, MD, codirector, Sickle Cell Program, Texas Children's Hospital, Houston","field_file_image_credit[und][0][value]":"Courtesy Racheal Adetayo Artistry","field_file_image_caption[und][0][value]":"Dr. Titilope Fasipe, MD"},"type":"media","attributes":{"class":"media-element file-medstat_image_flush_right"}}]]“If you grow up thinking that you’re going to die when you’re 18, it changes your world and your viewpoints, and it impacts your mental health,” she told this news organization.<br/><br/>“We are trying to make sure our children and their families know that there is a new story for sickle cell disease, and you don’t have to use any age as your prediction marker for your lifespan,” Dr. Fasipe said.<br/><br/>SCD, which affects about 100,000 people nationwide, is an inherited blood disorder, with the majority of patients – but not all – being of African descent. This condition is characterized by pain crises, or <span class="hgkelc">vaso-occlusive episodes, triggered when cells that are sickled get stuck and impede blood flow. These crises can come on suddenly and range from mild to severe.</span><br/><br/>Dr. Fasipe was born in Nigeria, where rates of SCD are among the world’s highest. She attended elementary school in the United States, where her father was studying theology, before returning to Nigeria with her family at age 11. <br/><br/>Back in those days, in both nations only about 50% of children with SCD lived beyond their 18th birthday. The survival rates in Nigeria and sub-Saharan Africa countries continue to be poor. In some more developed regions elsewhere, advances such as universal newborn screening, penicillin prophylaxis, pneumococcal vaccination, stroke screening, and hydroxyurea therapy have yielded substantial improvements, with 95% or more patients with SCD reaching their 18th birthday.<br/><br/>“With measures such as newborn screening, we can immediately start prevention measures in sickle cell disease, such as prevention of infection, which was the number one reason why children were dying,” Dr. Fasipe explained. “With global initiatives, we want that story to be the same in sub-Saharan Africa as well.”<br/><br/></p> <h2>Cousin’s early death inspires medical studies </h2> <p>In <span class="Hyperlink"><a href="https://www.tmc.edu/news/2019/10/a-houston-physician-with-sickle-cell-opens-up-about-treating-patients-with-the-same-disease/">an essay</a></span> published by Texas Medical Center that describes her childhood experiences, Dr. Fasipe recounts a pivotal event in her life: The heartbreaking death of her beloved cousin at the age of just 17, from a complication of SCD. This bereavement fueled Dr. Fasipe’s determination to pursue a medical career, to do all that she could to prevent such losses. </p> <p>“Having sickle cell disease myself wasn’t the trigger that made me become a doctor. But when Femi [her cousin] died, I thought: ‘This shouldn’t happen,’ ” Dr. Fasipe wrote.<br/><br/>When she applied to medical school back in the United States, she declared in her application essay: “I want to cure sickle cell.”<br/><br/>By the time Dr. Fasipe was ready to undertake residency and fellowship applications, her essay had shifted to focus on pediatrics “specifically because I want to reach sickle cell patients before they’ve defined how their lives are going to be,” she said. “I want to give them hope.”<br/><br/></p> <h2>Hope for a cure</h2> <p>Fast-forwarding to this point in Dr. Fasipe’s career, she noted that her dream of a cure for SCD is no longer a distant aspiration, thanks to the advent of stem cell transplantation and more recently, gene therapy. These advancements have elevated her hope for a cure to an entirely new level. </p> <p>Each new treatment comes with caveats. Stem cell transplantation requires a matching donor, leaving the majority of patients ineligible. And while gene therapy eliminates the need for a donor, treatment can reportedly cost nearly $3 million. Nevertheless, Dr. Fasipe emphasized the promise that these new advancements represent.<br/><br/>“The scientists that work in these spaces do appreciate these [accessibility barriers], and the expectation is these therapies will be more accessible with time and effort,” she said. “We’ve got to start somewhere, and it’s exciting that they’re making these early successes.”<br/><br/></p> <h2>Advice for clinicians</h2> <p>With firsthand knowledge of how it feels to be the patient, as well as on the clinician side of SCD treatment, Dr. Fasipe advises colleagues on some ways that they can improve care while boosting their patients’ hope: </p> <p><strong>Speak with empathy<br/><br/></strong>Acknowledge the ‘elephant in the room’; the pain that patients with SCD can experience is real. <br/><br/>“When I’m managing any patient with pain, I first acknowledge the suffering because while we may not understand what that person is going through, acknowledgment is part of showing empathy,” she explains.<br/><br/><strong>Seek out resources<br/><br/></strong>Patients with SCD may typically seek treatment in primary care, where expertise in the disease may be lacking, and general practitioners may feel frustrated that there are limited treatment options.<br/><br/>“If you do find yourself treating a sickle cell disease patient, you may not have all of the answers, but there are good resources, whether it’s a nearby sickle cell disease centers or national guidelines,” Dr. Fasipe said.<br/><br/><strong>Access to treatment</strong><br/><br/>With research, including <span class="Hyperlink"><a href="https://ashpublications.org/bloodadvances/article/7/13/3114/494814/The-use-of-FDA-approved-medications-for-preventing">a recent study</a></span>, showing that only about 25% of patients with SCD are prescribed hydroxyurea <span class="tag">and even fewer – only about 5% – receive more recently approved SCD treatments, clinicians should be proactive by making sure that patients receive needed treatments.<br/><br/></span>“Clearly medicines like hydroxyurea are not as optimized in this community space as they should be, and then there are newer therapies that families, patients, and even providers may not be aware of, so it is important to be informed of the guidelines and provide all patients with comprehensive, high-quality care,” Dr. Fasipe said.<br/><br/><strong>In the ED, patients with SCD are ‘care-seeking,’ not drug-seeking<br/><br/></strong>Due to the sometimes rapid onset of severe pain symptoms, patients with SCD commonly wind up in the emergency department. In this time of an opioid epidemic, patients too often are suspected of merely seeking drugs. <br/><br/>“Sickle cell disease tends to get lumped into a category of a disease of pain, but pain is subjective and it is difficult to quantify, so unfortunately, patients can be labeled as potentially drug-seeking,” Dr. Fasipe explained, citing <span class="Hyperlink"><a href="https://academic.oup.com/painmedicine/article/17/10/1793/2270349">an article</a></span> that detailed this problem.<br/><br/>Consequently, patients may have particularly negative experiences in the emergency department, but the use of resources such as <span class="Hyperlink"><a href="https://poctools.acep.org/POCTool/04082647-6422-473b-83ee-3a5b6291a415">a sickle cell disease point-of-care tool</a></span> developed by the American College of Emergency Physicians and the American Society of Hematology can help improve care for those patients.<br/><br/>“One of the [point-of-care recommendations] before even managing the pain is that physicians show compassion by acknowledging the patient’s pain and that they understand why pain with sickle cell disease might look different than other types of pain,” Dr. Fasipe said. <br/><br/></p> <h2>Building trust</h2> <p>Encounters such as negative emergency department experiences can perpetuate a deeper issue of distrust between those with SCD and the medical community, which originated in long-held, well-documented racial disparities in health care. </p> <p>“We know historically and even today that there are difficulties facing our families who are impacted by sickle cell disease, and they are related to structural racism and socioeconomic barriers,” Dr. Fasipe explained. <br/><br/>With these issues in mind, she said, “I refer to sickle cell disease as the medical representation of the Black experience in America.” However, she added, the good news is “we are now doing our best now to improve that.” <br/><br/>Among key efforts in building trust is the inclusion of patients with SCD and their families in as many aspects of research and clinical care as possible.<br/><br/>“In the global health care community, it is imperative to invite people with sickle cell disease and from the community to the decision-making table,” she noted. <br/><br/>“Now, when we’re talking about research for therapies, their expectation is that research trials and other initiatives for sickle cell disease must have input from the community; there are no initiatives for sickle cell disease that do not have input from the community.<br/><br/>“The patients and community members may not be experts on the science of sickle cell, but they’re experts on the lived experience and that’s very important when you’re thinking about new bringing in a new therapy.”<br/><br/></p> <h2>Forward momentum</h2> <p>Meanwhile, Dr. Fasipe observed, with the collective, advocacy-driven, forward momentum of the SCD community as a whole, things should only continue to improve.</p> <p>“Because of the various barriers, some progress may not be immediately around the corner, but I do have confidence that this current generation of children with sickle cell will have improved health equity by the time they reach adulthood,” she said. <br/><br/>“I believe in this future, so I’m doing the work now, and it’s a promise I tell parents: I want your future adult child to live their best life, and we’re working hard to ensure that that becomes their future reality.”<br/><br/></p> <h2>Sickle cell disease awareness</h2> <p>September is National Sickle Cell Disease Awareness Month, and the National Heart, Lung, and Blood Institute offers <a href="https://www.nhlbi.nih.gov/education/sickle-cell-month">a comprehensive website</a> that clinicians can pass along to their patients, with information ranging from fact sheets on the disease and treatments to social media resources and inspiring stories of people with the disease.</p> <p>In a comment, Lewis Hsu, MD, PhD, chief medical officer of the Sickle Cell Disease Association of America, underscored the uniquely important contributions of people like Dr. Fasipe, in providing inspiration to patients and clinicians alike.<br/><br/>“I have worked with several physicians, nurses, psychologists, and public health specialists who have sickle cell disease,” said Dr. Hsu, who is a pediatric hematologist who also serves as director of the Sickle Cell Center and professor of pediatrics for the University of Illinois at Chicago. <br/><br/>“They are ambassadors who have the trust of both patients and healthcare providers,” Dr. Hsu said.<br/><br/>In addition to providing inspiration of resilience, such care providers can serve as “communication bridges,” he explained.<br/><br/>“When they are conference speakers, everybody wants to hear them; when they sit on advisory committees or focus groups, they can help find the compromise or set the priorities.”<br/><br/>“Their impact on the whole sickle cell community is very large,” Dr. Hsu said. </p> </itemContent> </newsItem> <newsItem> <itemMeta> <itemRole>teaser</itemRole> <itemClass>text</itemClass> <title/> <deck/> </itemMeta> <itemContent> </itemContent> </newsItem> </itemSet></root>
Children with sickle cell anemia not getting treatments, screening
Fewer than half of children aged 2-16 years with sickle cell anemia are receiving recommended annual screening for stroke, a common complication of the disease, according to a new Vital Signs report from the Centers for Disease Control and Prevention.
Many of these children also are not receiving the recommended medication, hydroxyurea, which can reduce pain and acute chest syndrome and improve anemia and quality of life, according to the report released Sept. 20.
Sickle cell anemia (SCA) is the most severe form of sickle cell disease (SCD), which is a red blood cell disorder that primarily affects Black and African American people in the United States. It is associated with severe complications such as stroke, vison damage, frequent infections, and delayed growth, and a reduction in lifespan of more than 20 years.
SCD affects approximately 100,000 Americans and SCA accounts for about 75% of those cases.
Physician remembers her patients’ pain
In a briefing to reporters in advance of the report’s release, Debra Houry, MD, MPH, the CDC’s acting principal deputy director, recalled “long, tough nights with these young sickle cell warriors” in her career as an emergency department physician.
“[S]eeing children and teens suffering from the severe pain that often accompanies sickle cell anemia was heartbreaking,” she said.
She asked health care providers to confront racism as they build better systems for ensuring optimal treatment for children and adolescents with SCA.
“Health care providers can educate themselves, their colleagues, and their institutions about the specialized needs of people with sickle cell anemia, including how racism inhibits optimal care,” Dr. Houry said.
She said people with SCA report difficulty accessing care and when they do, they often report feeling stigmatized.
Lead author of the report, Laura Schieve, PhD, an epidemiologist with CDC’s National Center on Birth Defects and Developmental Disabilities, and colleagues looked at data from more than 3,300 children with SCA who were continuously enrolled in Medicaid during 2019. The data came from the IBM MarketScan Multi-State Medicaid Database.
Key recommendations issued in 2014
In 2014, the National Heart, Lung, and Blood Institute (NHLBI) issued two key recommendations to prevent or reduce complications in children and adolescents with SCA.
One was annual screening of children and adolescents aged 2-16 years with transcranial Doppler (TCD) ultrasound to identify those at risk for stroke. The second was offering hydroxyurea therapy, which keeps red blood cells from sickling and blocking small blood vessels, to children and adolescents who were at least 9 months old to reduce pain and the risk for several life-threatening complications.
The researchers, however, found that in 2019, only 47% and 38% of children and adolescents aged 2-9 and 10-16 years, respectively, had TCD screening and 38% and 53% of children and adolescents aged 2-9 years and 10-16 years, respectively, used hydroxyurea.
“These complications are preventable – not inevitable. We must do more to help lessen the pain and complications associated with this disease by increasing the number of children who are screened for stroke and using the medication that can help reduce painful episodes,” said Karen Remley, MD, MPH, director of CDC’s National Center on Birth Defects and Developmental Disabilities, said in a press release.
Bridging the gap
Providers, parents, health systems, and governmental agencies all have roles in bringing evidence-based recommended care to young SCA patients, Dr. Houry noted.
Community organizations can also help connect families with resources and tools to increase understanding.
Dr. Schieve pointed to access barriers in that families may have trouble traveling to specialized centers where the TCD screening is given. In addition, appointments for the screening may be limited.
Children taking hydroxyurea must be monitored for the proper dosage of medication, she explained, and that can be logistically challenging as well.
Providers report they often don’t get timely information back from TCD screening programs to keep up with which children need their annual screening.
Overall, the nation lacks providers with expertise in SCD and that can lead to symptoms being dismissed, Dr. Schieve said.
Hematologists and others have a role in advocating for patients with governmental entities to raise awareness of this issue, she added.
It’s also important that electronic health records give prompts and provide information so that all providers who care for a child can track screening and medication for the condition, Dr. Schieve and Dr. Houry said.
New funding for sickle cell data collection
Recent funding to the CDC Sickle Cell Data Collection Program may help more people get appropriate care, Dr. Houry said.
The program is currently active in 11 states and collects data from people all over the United States with SCD to study trends and treatment access for those with the disease.
The data help drive decisions such as where new sickle cell clinics are needed.
“We will expand to more states serving more people affected by this disease,” Dr. Houry said.
The authors declared no relevant financial relationships.
Fewer than half of children aged 2-16 years with sickle cell anemia are receiving recommended annual screening for stroke, a common complication of the disease, according to a new Vital Signs report from the Centers for Disease Control and Prevention.
Many of these children also are not receiving the recommended medication, hydroxyurea, which can reduce pain and acute chest syndrome and improve anemia and quality of life, according to the report released Sept. 20.
Sickle cell anemia (SCA) is the most severe form of sickle cell disease (SCD), which is a red blood cell disorder that primarily affects Black and African American people in the United States. It is associated with severe complications such as stroke, vison damage, frequent infections, and delayed growth, and a reduction in lifespan of more than 20 years.
SCD affects approximately 100,000 Americans and SCA accounts for about 75% of those cases.
Physician remembers her patients’ pain
In a briefing to reporters in advance of the report’s release, Debra Houry, MD, MPH, the CDC’s acting principal deputy director, recalled “long, tough nights with these young sickle cell warriors” in her career as an emergency department physician.
“[S]eeing children and teens suffering from the severe pain that often accompanies sickle cell anemia was heartbreaking,” she said.
She asked health care providers to confront racism as they build better systems for ensuring optimal treatment for children and adolescents with SCA.
“Health care providers can educate themselves, their colleagues, and their institutions about the specialized needs of people with sickle cell anemia, including how racism inhibits optimal care,” Dr. Houry said.
She said people with SCA report difficulty accessing care and when they do, they often report feeling stigmatized.
Lead author of the report, Laura Schieve, PhD, an epidemiologist with CDC’s National Center on Birth Defects and Developmental Disabilities, and colleagues looked at data from more than 3,300 children with SCA who were continuously enrolled in Medicaid during 2019. The data came from the IBM MarketScan Multi-State Medicaid Database.
Key recommendations issued in 2014
In 2014, the National Heart, Lung, and Blood Institute (NHLBI) issued two key recommendations to prevent or reduce complications in children and adolescents with SCA.
One was annual screening of children and adolescents aged 2-16 years with transcranial Doppler (TCD) ultrasound to identify those at risk for stroke. The second was offering hydroxyurea therapy, which keeps red blood cells from sickling and blocking small blood vessels, to children and adolescents who were at least 9 months old to reduce pain and the risk for several life-threatening complications.
The researchers, however, found that in 2019, only 47% and 38% of children and adolescents aged 2-9 and 10-16 years, respectively, had TCD screening and 38% and 53% of children and adolescents aged 2-9 years and 10-16 years, respectively, used hydroxyurea.
“These complications are preventable – not inevitable. We must do more to help lessen the pain and complications associated with this disease by increasing the number of children who are screened for stroke and using the medication that can help reduce painful episodes,” said Karen Remley, MD, MPH, director of CDC’s National Center on Birth Defects and Developmental Disabilities, said in a press release.
Bridging the gap
Providers, parents, health systems, and governmental agencies all have roles in bringing evidence-based recommended care to young SCA patients, Dr. Houry noted.
Community organizations can also help connect families with resources and tools to increase understanding.
Dr. Schieve pointed to access barriers in that families may have trouble traveling to specialized centers where the TCD screening is given. In addition, appointments for the screening may be limited.
Children taking hydroxyurea must be monitored for the proper dosage of medication, she explained, and that can be logistically challenging as well.
Providers report they often don’t get timely information back from TCD screening programs to keep up with which children need their annual screening.
Overall, the nation lacks providers with expertise in SCD and that can lead to symptoms being dismissed, Dr. Schieve said.
Hematologists and others have a role in advocating for patients with governmental entities to raise awareness of this issue, she added.
It’s also important that electronic health records give prompts and provide information so that all providers who care for a child can track screening and medication for the condition, Dr. Schieve and Dr. Houry said.
New funding for sickle cell data collection
Recent funding to the CDC Sickle Cell Data Collection Program may help more people get appropriate care, Dr. Houry said.
The program is currently active in 11 states and collects data from people all over the United States with SCD to study trends and treatment access for those with the disease.
The data help drive decisions such as where new sickle cell clinics are needed.
“We will expand to more states serving more people affected by this disease,” Dr. Houry said.
The authors declared no relevant financial relationships.
Fewer than half of children aged 2-16 years with sickle cell anemia are receiving recommended annual screening for stroke, a common complication of the disease, according to a new Vital Signs report from the Centers for Disease Control and Prevention.
Many of these children also are not receiving the recommended medication, hydroxyurea, which can reduce pain and acute chest syndrome and improve anemia and quality of life, according to the report released Sept. 20.
Sickle cell anemia (SCA) is the most severe form of sickle cell disease (SCD), which is a red blood cell disorder that primarily affects Black and African American people in the United States. It is associated with severe complications such as stroke, vison damage, frequent infections, and delayed growth, and a reduction in lifespan of more than 20 years.
SCD affects approximately 100,000 Americans and SCA accounts for about 75% of those cases.
Physician remembers her patients’ pain
In a briefing to reporters in advance of the report’s release, Debra Houry, MD, MPH, the CDC’s acting principal deputy director, recalled “long, tough nights with these young sickle cell warriors” in her career as an emergency department physician.
“[S]eeing children and teens suffering from the severe pain that often accompanies sickle cell anemia was heartbreaking,” she said.
She asked health care providers to confront racism as they build better systems for ensuring optimal treatment for children and adolescents with SCA.
“Health care providers can educate themselves, their colleagues, and their institutions about the specialized needs of people with sickle cell anemia, including how racism inhibits optimal care,” Dr. Houry said.
She said people with SCA report difficulty accessing care and when they do, they often report feeling stigmatized.
Lead author of the report, Laura Schieve, PhD, an epidemiologist with CDC’s National Center on Birth Defects and Developmental Disabilities, and colleagues looked at data from more than 3,300 children with SCA who were continuously enrolled in Medicaid during 2019. The data came from the IBM MarketScan Multi-State Medicaid Database.
Key recommendations issued in 2014
In 2014, the National Heart, Lung, and Blood Institute (NHLBI) issued two key recommendations to prevent or reduce complications in children and adolescents with SCA.
One was annual screening of children and adolescents aged 2-16 years with transcranial Doppler (TCD) ultrasound to identify those at risk for stroke. The second was offering hydroxyurea therapy, which keeps red blood cells from sickling and blocking small blood vessels, to children and adolescents who were at least 9 months old to reduce pain and the risk for several life-threatening complications.
The researchers, however, found that in 2019, only 47% and 38% of children and adolescents aged 2-9 and 10-16 years, respectively, had TCD screening and 38% and 53% of children and adolescents aged 2-9 years and 10-16 years, respectively, used hydroxyurea.
“These complications are preventable – not inevitable. We must do more to help lessen the pain and complications associated with this disease by increasing the number of children who are screened for stroke and using the medication that can help reduce painful episodes,” said Karen Remley, MD, MPH, director of CDC’s National Center on Birth Defects and Developmental Disabilities, said in a press release.
Bridging the gap
Providers, parents, health systems, and governmental agencies all have roles in bringing evidence-based recommended care to young SCA patients, Dr. Houry noted.
Community organizations can also help connect families with resources and tools to increase understanding.
Dr. Schieve pointed to access barriers in that families may have trouble traveling to specialized centers where the TCD screening is given. In addition, appointments for the screening may be limited.
Children taking hydroxyurea must be monitored for the proper dosage of medication, she explained, and that can be logistically challenging as well.
Providers report they often don’t get timely information back from TCD screening programs to keep up with which children need their annual screening.
Overall, the nation lacks providers with expertise in SCD and that can lead to symptoms being dismissed, Dr. Schieve said.
Hematologists and others have a role in advocating for patients with governmental entities to raise awareness of this issue, she added.
It’s also important that electronic health records give prompts and provide information so that all providers who care for a child can track screening and medication for the condition, Dr. Schieve and Dr. Houry said.
New funding for sickle cell data collection
Recent funding to the CDC Sickle Cell Data Collection Program may help more people get appropriate care, Dr. Houry said.
The program is currently active in 11 states and collects data from people all over the United States with SCD to study trends and treatment access for those with the disease.
The data help drive decisions such as where new sickle cell clinics are needed.
“We will expand to more states serving more people affected by this disease,” Dr. Houry said.
The authors declared no relevant financial relationships.
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This material may not be published, broadcast, copied, or otherwise reproduced or distributed without the prior written permission of Frontline Medical Communications Inc.</copyrightNotice> </rightsInfo> </provider> <abstract/> <metaDescription>Fewer than half of children aged 2-16 years with sickle cell anemia are receiving recommended annual screening for stroke, a common complication of the disease,</metaDescription> <articlePDF/> <teaserImage/> <teaser>Annual screenings are recommended as is treatment with hydroxyurea, but only about half of affected children are getting lifesaving care.</teaser> <title>Children with sickle cell anemia not getting treatments, screening</title> <deck/> <disclaimer/> <AuthorList/> <articleURL/> <doi/> <pubMedID/> <publishXMLStatus/> <publishXMLVersion>1</publishXMLVersion> <useEISSN>0</useEISSN> <urgency/> <pubPubdateYear/> <pubPubdateMonth/> <pubPubdateDay/> <pubVolume/> <pubNumber/> <wireChannels/> <primaryCMSID/> <CMSIDs/> <keywords/> <seeAlsos/> <publications_g> <publicationData> <publicationCode>fp</publicationCode> <pubIssueName/> <pubArticleType/> <pubTopics/> <pubCategories/> <pubSections/> </publicationData> <publicationData> <publicationCode>mdemed</publicationCode> <pubIssueName/> <pubArticleType/> <pubTopics/> <pubCategories/> <pubSections/> <journalTitle/> <journalFullTitle/> <copyrightStatement/> </publicationData> <publicationData> <publicationCode>pn</publicationCode> <pubIssueName/> <pubArticleType/> <pubTopics/> <pubCategories/> <pubSections/> </publicationData> <publicationData> <publicationCode>hemonc</publicationCode> <pubIssueName/> <pubArticleType/> <pubTopics/> <pubCategories/> <pubSections/> <journalTitle/> <journalFullTitle/> <copyrightStatement>2018 Frontline Medical Communications Inc.,</copyrightStatement> </publicationData> </publications_g> <publications> <term>15</term> <term>58877</term> <term canonical="true">25</term> <term>49734</term> </publications> <sections> <term>27970</term> <term canonical="true">39313</term> <term>37225</term> </sections> <topics> <term canonical="true">225</term> <term>271</term> <term>268</term> <term>62503</term> </topics> <links/> </header> <itemSet> <newsItem> <itemMeta> <itemRole>Main</itemRole> <itemClass>text</itemClass> <title>Children with sickle cell anemia not getting treatments, screening</title> <deck/> </itemMeta> <itemContent> <p>Fewer than half of children aged 2-16 years with sickle cell anemia are receiving recommended annual screening for stroke, a common complication of the disease, according to a new <span class="Hyperlink"><a href="https://www.cdc.gov/mmwr/volumes/71/wr/mm7139e1.htm?s_cid=mm7139e1_w">Vital Signs</a> </span>report from the Centers for Disease Control and Prevention. </p> <p>Many of these children also are not receiving the recommended medication, hydroxyurea, which can reduce pain and acute chest syndrome and improve anemia and quality of life, according to the report released Sept. 20.<br/><br/>Sickle cell anemia (SCA) is the most severe form of sickle cell disease (SCD), which is a red blood cell disorder that primarily affects Black and African American people in the United States. It is associated with severe complications such as stroke, vison damage, frequent infections, and delayed growth, and a reduction in lifespan of more than 20 years.<br/><br/>SCD affects approximately 100,000 Americans and SCA accounts for about 75% of those cases.<br/><br/></p> <h2>Physician remembers her patients’ pain</h2> <p>In a briefing to reporters in advance of the report’s release, Debra Houry, MD, MPH, the CDC’s acting principal deputy director, recalled “long, tough nights with these young sickle cell warriors” in her career as an emergency department physician.</p> <p>“[S]eeing children and teens suffering from the severe pain that often accompanies sickle cell anemia was heartbreaking,” she said.<br/><br/>She asked health care providers to confront racism as they build better systems for ensuring optimal treatment for children and adolescents with SCA. <br/><br/>“Health care providers can educate themselves, their colleagues, and their institutions about the specialized needs of people with sickle cell anemia, including how racism inhibits optimal care,” Dr. Houry said.<br/><br/>She said people with SCA report difficulty accessing care and when they do, they often report feeling stigmatized.<br/><br/>Lead author of the report, Laura Schieve, PhD, an epidemiologist with CDC’s National Center on Birth Defects and Developmental Disabilities, and colleagues looked at data from more than 3,300 children with SCA who were continuously enrolled in Medicaid during 2019. The data came from the IBM MarketScan Multi-State Medicaid Database.<br/><br/></p> <h2>Key recommendations issued in 2014</h2> <p>In 2014, the National Heart, Lung, and Blood Institute (NHLBI) issued two key recommendations to prevent or reduce complications in children and adolescents with SCA.</p> <p>One was annual screening of children and adolescents aged 2-16 years with transcranial Doppler (TCD) ultrasound to identify those at risk for stroke. The second was offering hydroxyurea therapy, which keeps red blood cells from sickling and blocking small blood vessels, to children and adolescents who were at least 9 months old to reduce pain and the risk for several life-threatening complications.<br/><br/>The researchers, however, found that in 2019, only 47% and 38% of children and adolescents aged 2-9 and 10-16 years, respectively, had TCD screening and 38% and 53% of children and adolescents aged 2-9 years and 10-16 years, respectively, used hydroxyurea.<br/><br/>“These complications are preventable – not inevitable. We must do more to help lessen the pain and complications associated with this disease by increasing the number of children who are screened for stroke and using the medication that can help reduce painful episodes,” said Karen Remley, MD, MPH, director of CDC’s National Center on Birth Defects and Developmental Disabilities, said in a press release.<br/><br/></p> <h2>Bridging the gap</h2> <p>Providers, parents, health systems, and governmental agencies all have roles in bringing evidence-based recommended care to young SCA patients, Dr. Houry noted.</p> <p>Community organizations can also help connect families with resources and tools to increase understanding. <br/><br/>Dr. Schieve pointed to access barriers in that families may have trouble traveling to specialized centers where the TCD screening is given. In addition, appointments for the screening may be limited.<br/><br/>Children taking hydroxyurea must be monitored for the proper dosage of medication, she explained, and that can be logistically challenging as well.<br/><br/>Providers report they often don’t get timely information back from TCD screening programs to keep up with which children need their annual screening.<br/><br/>Overall, the nation lacks providers with expertise in SCD and that can lead to symptoms being dismissed, Dr. Schieve said.<br/><br/>Hematologists and others have a role in advocating for patients with governmental entities to raise awareness of this issue, she added.<br/><br/>It’s also important that electronic health records give prompts and provide information so that all providers who care for a child can track screening and medication for the condition, Dr. Schieve and Dr. Houry said. <br/><br/></p> <h2>New funding for sickle cell data collection</h2> <p>Recent funding to the CDC Sickle Cell Data Collection Program may help more people get appropriate care, Dr. Houry said.</p> <p>The program is currently active in 11 states and collects data from people all over the United States with SCD to study trends and treatment access for those with the disease.<br/><br/>The data help drive decisions such as where new sickle cell clinics are needed. <br/><br/>“We will expand to more states serving more people affected by this disease,” Dr. Houry said.<br/><br/>The authors declared no relevant financial relationships.</p> </itemContent> </newsItem> <newsItem> <itemMeta> <itemRole>teaser</itemRole> <itemClass>text</itemClass> <title/> <deck/> </itemMeta> <itemContent> </itemContent> </newsItem> </itemSet></root>
FROM THE MMWR