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About Louisiana Sickle Cell Disease
What is Sickle Cell Disease?
According to the Centers for Disease Control and Prevention, sickle cell disease (SCD) is a group of inherited red blood cell disorders. It is a genetic condition, meaning it is present at birth and usually detected during the routine newborn screening. Early diagnosis and treatment are important because of the increased risk of infection and other health problems.
Red blood cells contain hemoglobin, a protein that carries oxygen. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has sickle cell disease, the hemoglobin is abnormal, which causes the red blood cells to become hard and sticky and look like a C-shaped farm tool called a “sickle.” The sickle-shaped cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow.
Living with Sickle Cell Disease
Individuals living with sickle cell disease experience serious health complications, worse health outcomes, and have access to fewer resources. Historically, funding, research, social services, and other resources have not been prioritized for this condition. Each person with the disease can experience different symptoms and complications ranging from mild to severe. Symptoms and health effects usually begin around five months of age.
Common health complications with sickle cell disease include:
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Pain: Sickled cells traveling through small blood vessels can get stuck and block blood flow throughout the body, causing pain. A vaso-occlusive episode (pain crisis) can start suddenly, be mild to severe, and can last for any length of time.
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Lung problems: Acute chest syndrome, pulmonary hypertension, and sleep-disordered breathing are lung-related complications caused by sickle cell disease.
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Organ damage: Because not enough blood is reaching organs, people with sickle cell disease are at greater risk for organ damage, including problems with the liver, spleen, and kidneys.
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Circulation issues: Sickled cells commonly get stuck in blood vessels, causing many complications, including blood clots, strokes, painful swelling in the hands and feet, and more.
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Fevers and infections: Fevers can be the first sign of an infection or other health complications due to sickle cell disease.
Due to the varied health complications as a result of the disease, people living with sickle cell disease experience a shorter life expectancy, have the highest rate of returning to the hospital within 30 days of being discharged, experience a higher rate of stroke and may experience longer wait times to see a doctor and get pain medication due to a presenting complaint being initially assigned to a non-emergent condition. Further, most patients with sickle cell disease nationally are enrolled in Medicaid, and a limited number of clinicians are trained to treat patients with sickle cell disease, so obtaining high-quality healthcare services can be a significant challenge. Medical providers trained in hematology and oncology often train to treat cancers; however, few specialize in blood disorders, and even fewer in sickle cell disease.
Emergency Treatment for Conditions Related to Sickle Cell Disease
Individuals living with sickle cell disease may need emergency treatment due to the varied health complications as a result of the disease. Most commonly, people living with sickle cell disease need emergency treatment for pain associated with the disease, known as a vaso-occlusive episode. There are guidelines in place regarding the prescription of opioids, which may result in a primary care provider being unable to prescribe certain medications needed for managing vaso-occlusive episodes. This could contribute to the necessity for an emergency room visit or an emergency visit with a specialist for individuals with sickle cell disease. It is important to inform your primary care provider of any visits to the emergency room to come up with a care plan to manage future vaso-occlusive episodes.
Tips to Live a Healthy Life with Sickle Cell Disease
People with sickle cell disease can live full lives and enjoy most of the activities that other people do. The following tips will help you, or someone you know with sickle cell disease, stay as healthy as possible.
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Find good medical care: Sickle cell disease is a complex disease. Good quality medical care from doctors and nurses who know a lot about the disease can help prevent some serious problems. Often, the best choice is a hematologist (a doctor who specializes in blood diseases) working with a team of specialists.
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Get regular checkups: Regular health checkups with a primary care doctor can help prevent some serious problems.
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Prevent infections: Common illnesses, like the flu, can quickly become dangerous for a child with sickle cell disease. The best defense is to take simple steps to help prevent infections.
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Learn healthy habits: Children can, and should, participate in physical activity to help stay healthy. However, it’s important that they don’t overdo it, rest when tired, and drink plenty of water.
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Look for clinical studies: New clinical research studies are happening all the time to find better treatments and, hopefully, a cure for sickle cell disease. People who participate in these studies might have access to new medicines and treatment options. Patients can ask their physicians about clinical trials, or they can go to clinicaltrials.gov or sicklecelldisease.org/sickle-cell-health-and-disease/education-and-research/clinical-trials.
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Get support: Find a patient support group or community-based organization that can provide information, assistance, and support. For more information about SCD, including diagnosis, complications, and treatment, click here.
Sickle Cell Disease in Louisiana
Approximately 80 infants born each year in Louisiana are diagnosed with sickle cell disease. Louisiana Medicaid provides healthcare coverage to approximately 3,000 individuals living with sickle cell disease in the state each year. However, the true number of individuals living with the condition is unknown because there is currently no comprehensive population-level public health monitoring system in the state.
Sickle Cell Disease Policy in Louisiana
Nationally and in Louisiana, sickle cell disease is being recognized as a health issue that is overdue for investment and policy action. In 2020, the National Academies of Sciences, Engineering and Medicine released Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action, illuminating factors affecting patient-centered and effective care, treatment and support for individuals living with sickle cell disease and sickle cell trait (when a person receives only one sickle cell gene from a parent). The report highlighted societal and structural contributors to disease impact, such as lack of public awareness and misinformation. The report also highlighted the need for patient registries and public health monitoring systems as a foundation for improving clinical care and quality of life.
Legislative actions in Louisiana in 2021 and 2022 echoed elements of the calls to action in the national strategic plan, many of which have been championed by the statutorily established Louisiana Sickle Cell Commission and the state’s sickle cell foundations, community-based organizations established to provide supportive services to people living with sickle cell disease.
Louisiana Sickle Cell Disease Registry
The Louisiana Sickle Cell Disease Registry was authorized by Act No. 647 of the 2022 Regular Session of the Louisiana Legislature. The registry is also known as the Skylar-Cooper Database. The purpose of the Registry, as described in the legislation, is “to function as a single repository of accurate, complete records to aid in the cure and treatment of sickle cell disease in this state.”
It is managed by the Louisiana Department of Health, Office of Public Health, Bureau of Family Health, in collaboration with the Office of Public Health, Bureau of Health Informatics. Through this Registry, the Office of Public Health will provide actionable information and data to support and inform public health initiatives and interventions that improve healthcare services and resources for people living with sickle cell disease.
Data Sharing Agreements
In Act 748 of 2024, the Louisiana Legislature mandated LDH to “facilitate an agreement between sickle cell clinics as provided for in R.S. 40:1125.31 et seq, and an association that is part of the Louisiana Sickle Cell Commission that provides information and assistance to patients diagnosed with sickle cell disease. The agreement shall allow for the transfer of sufficient contact information from the sickle cell clinics to allow an association to contact the patient.”
In order to improve outcomes for people living with sickle cell disease, increased awareness of and access to community resources is needed. The Louisiana Department of Health can assist in facilitating data-sharing agreements between health care providers and regional sickle cell foundations by offering resources to support this process. These agreements allow for patient or family contact information to be shared with patient consent.
Health care providers may establish data-sharing agreements with regional sickle cell foundations across Louisiana to help connect patients to education, resources, and support services. The Louisiana Department of Health developed a sample data-sharing agreement template that you can adapt.
[DATA SHARING AGREEMENT TEMPLATE]
Sickle Cell Disease Program
The Louisiana Department of Health, Office of Public Health's Sickle Cell Disease Program works to help diagnose and treat sickle cell disease in babies as early as possible. They connect people diagnosed with sickle cell to resources, medical care, and other services through the Sickle Cell Foundations and Clinics around the state.
This program also oversees the Louisiana Sickle Cell Commission, which is mandated by Act 117 (SB57) of the Louisiana State Legislature. Click here to find information on the Louisiana Sickle Cell Commission, including meeting dates, agendas, and minutes. For the most recent Commission report, including information on current appointments, click here.
Resource for Healthcare Providers
The Louisiana Standard for Care of Patients with Sickle Cell Disease serves as a best practices guide for treating patients with sickle cell disease. It was developed by Renee Gardner, MD, a pediatric hematologist with Louisiana State University Health Sciences Center in New Orleans and the Louisiana Sickle Cell Commission. The goal of the toolkit is to increase and improve access to reduce acute pain and improve chronic pain management. The toolkit is distributed to pediatricians, family practitioners, and other primary care providers to follow as best practices for treating patients with sickle cell disease.
In addition, emergency providers can use the Sickle Cell Disease Pain Management Toolkit. This toolkit provides information about sickle cell disease and recommendations for emergency department providers. Pain is the most common complication of sickle cell disease and the top reason people with sickle cell disease go to the emergency department. Many people with sickle cell disease report challenges and barriers when seeking treatment in emergency department settings. There are several steps that emergency providers can take to improve care for people with sickle cell disease when they are in the emergency department.
Resources for Patients and Families
The Office of Public Health can provide resources and information for people diagnosed with sickle cell on how to receive assistance and care through the sickle cell foundations and clinics around the state. Individuals can contact the Genetic Diseases Program for the Sickle Cell Foundation and clinic in their region for help.
Additional Resources
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The CDC has compiled stories from people directly affected by Sickle Cell Disease, highlighting the impact that identification, treatment, and resources can have on real people. Read their stories
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Addressing Sickle Cell Disease: A Strategic Plan and Blueprint from the National Academies, which examines the epidemiology, health outcomes, genetic implications, and societal factors associated with sickle cell disease and sickle cell trait.
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Sickle Cell Disease Association of America | Northwest Louisiana Chapter
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For more information about sickle cell disease, including diagnosis, complications, and treatment, click here.
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Sickle Cell Disease Toolkit for Schools: Students who live with sickle cell disease are more at risk for psychological and psychosocial complications as a result of the disease because symptoms and complications can cause absenteeism from school and deterioration in school performance. Children with sickle cell disease are also at higher risk for learning difficulties. But there are many small things that the school community can do to help a student living with sickle cell be more successful in school. School communities can make a difference in the lives of students with sickle cell disease by understanding key facts about the disease, how to watch out for symptoms ,and how to provide special accommodations.