Tag Archives: sickle cell disease

September is National Sickle Cell Awareness Month: A CLOSER LOOK AT SICKLE CELL DISEASE

September is recognized every year by the health community as Sickle Cell Awareness Month. Sickle Cell Disease (SCD) is a genetic disease that impacts families across the globe. Keeping individuals and communities informed about the struggles that come with the daily management of SCD can help raise awareness, as well as debunk stereotypes and stigmas associated with persons who have SCD.

SCD affects millions of people throughout the world.  Although SCD is most common among African Americans in the United States, it can also affect Hispanics and people whose ancestors come from countries in South Asia (such as India), southern Europe (such as Greece and Italy), and the Middle East (such as Saudi Arabia and Lebanon).

Quick Facts About SCD and SCT

  • SCD is a blood disorder that causes sickling of the red blood cells.
  • Sickle-shaped red blood cells become stuck in blood vessels and cause disruption of blood flow – this results in crises.
  • SCD affects 100,000 people in the United States.
  • SCD is an inherited blood disorder from the person’s parent, like any other genetic trait, such, as hair color and texture and eye color
  • SCT is where a person inherits one sickle cell gene and one normal gene.
  • SCT is not a disease, and the individual is generally asymptomatic.
  • SCT affects 1 million to 3 million Americans and 8 to 10 percent of African Americans.
  • Persons with SCT can pass the trait on to their children.

MinorityHealthSickleCell

SCD and COVID-19
Amid the COVID-19 pandemic, there is significant concern for individuals with underlying medical conditions. The CDC has indicated that persons with SCD and other blood disorders are among groups of individuals at an increased risk of severe illness from COVID-19.

Below are recommendations from the Sickle Cell Disease Association of America (SCDAA) for patients and families with SCD regarding COVID-19:

  • Discuss with your healthcare provider about converting all routine in-person appointments to virtual or telephonic.
  • Ask your healthcare provider for information about COVID-19 signs and symptoms and the importance of physical distancing to limit chances of exposure and infection. Discuss enhanced emotional connection through virtual or cellular-based modes.
  • Continue to seek medical help from your doctor, nurse, or hospital for fever and other signs of infection. Be sure to call first for advice on where to go safely for an evaluation.
  • Make certain you have an ample supply of all prescribed medications at home (including analgesics) to manage both acute and chronic pain.
  • Adhere closely to the correct use of your medications.

 DHEC’s Sickle Cell Program & Partnerships

DHEC is committed to educating the community and public about the availability of resources and services for individuals and families living with sickle cell disease.  DHEC’s Division of Children and Youth with Special Health Care Needs (CYSHCN), provides assistance to persons with sickle cell disease by covering services, such as: medical expenses, physician visits, durable medical equipment, medical supplies, and prescription drugs.  Assistance is offered to both children and adults who meet eligibility requirements. Additionally, CYSHCN partners with the Newborn Screening Follow-Up program to ensure infants who are newly diagnosed with sickle cell disease have a medical home to address treatment and care for their disease.

In addition to financial assistance and care coordination services, CYSHCN engages with the four Sickle Community Based Organizations in the state — the James R. Clark Memorial Sickle Cell Foundation, – Louvenia D. Barksdale Sickle Cell Anemia Foundation, – Orangeburg Area Sickle Cell Foundation and – COBRA Human Services Agency Sickle Cell Program – to ensure persons living with SCD have access to community resources and support services. Each of these organizations provide genetic screening, counseling, education, case management, and support groups to address the needs of families and individuals living with SCD. If you have questions about testing for you or your family, you can visit one of the four sickle cell community-based organizations.

Sickle Cell Has Many Faces

Sickle Cell Has Many Faces as illustrated through this video by the Center for Disease and Prevention Control. This video highlights how people of different races and ancestry could have SCD.

If you have questions about the DHEC sickle cell program, contact the CYSHCN office at 803-898-0784. For general information about sickle cell disease, visit cdc.gov/sicklecell or https://www.sicklecelldisease.org/.

June 19th is #WorldSickleCellDay

Today (June 19th) is World Sickle Cell Day!  Every year, the international health community recognizes Sickle Cell Disease (SCD) as a debilitating genetic disease that impacts families across the globe. Keeping individuals and communities informed about the struggles that come with daily management of SCD, can help raise awareness as well as debunk stereotypes and stigmas associated with persons who have SCD.   

SCD affects millions of people worldwide and is particularly common among people originating from sub-Saharan Africa, Saudi Arabia, India, South America and Central America, and Mediterranean countries, such as Turkey, Greece and Italy.

SCD affects approximately 100,000 Americans and occurs in about 1 out of every 365 African-American births. Individuals living with SCD suffer from both acute and chronic complications that require frequent contact with the medical system. These complications include acute sickle cell pain, fever, and acute chest syndrome (ACS), which is the term used for a number of different findings that includes chest pain, cough, fever, hypoxia and new lung infiltrates.

Here are some quick facts about SCD:

  • Sickle Cell Disease is a group of inherited red blood cell disorders. The red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle” and the cells die early, which causes a constant storage of red blood cells. Healthy red blood cells are round and move through small blood vessels to carry oxygen to all parts of the body.
  • To get SCD, the trait must be inherited from both parents who already have the SCD trait. People with the trait usually do not have any of the signs of the disease and live a normal life, but they can pass the trait to their children.
  • SCD is diagnosed with a simple blood test.  It is most often found at birth during routine newborn screening tests. Early diagnosis and treatment are important.
  • There are several treatment options available for individuals living with different complications of SCD, but the only cure for SCD is a bone marrow or stem cell transplant.  Transplants are very risky, and can have serious side effects, including death.  For the transplant to work, the bone marrow must be a close match (usually a brother or sister). 

SCD and COVID-19

Amid the COVID-19 pandemic, there is significant concern that the overlap of lung disease from COVID-19 with ACS may result in increased complications and amplification of healthcare utilization among individuals with SCD. Patients with SCD often have underlying cardiopulmonary co-morbidities that may predispose them to poor outcomes if they become infected with COVID-19. Source: hematology.org. 

Below are recommendations from the Sickle Cell Disease Association of America (SCDAA) for patients with SCD regarding COVID-19:

  • Patients and parents should be educated about COVID-19 signs and symptoms and the importance of physical distancing to limit chances of exposure and infection.
  • Patients and parents should receive counseling to continue to monitor for fever or other signs of infection. Call  hospital, doctor, or nurse first for advice on where to go for an evaluation.
  • Be sure that patients have an ample supply of all prescribed medication at home (including analgesics) to manage both acute and chronic pain.
  • Patients should adhere closely to the correct use of medications.

SCD Partnerships

DHEC is committed to educating the community and public about the availability of resources and services for individuals and families living with sickle cell disease.  DHEC’s Division of Children and Youth with Special Health Care Needs (CYSHCN), provides assistance to persons with sickle cell disease by covering services, such as, medical expenses, physician visits, durable medical equipment, medical supplies, and prescription drugs.  Assistance is offered to both children and adults who meet eligibility requirements. 

Additionally, CYSHCN engages with the four Sickle Community Based Organizations in the state — the James R. Clark Memorial Sickle Cell Foundation, – Louvenia D. Barksdale Sickle Cell Anemia Foundation, – Orangeburg Area Sickle Cell Foundation and – COBRA Human Services Agency Sickle Cell Program – to ensure persons living with SCD have access to community resources and support services. 

September is National Sickle Cell Awareness Month

National Sickle Cell Awareness Month brings attention to this crippling illness, a genetic disease that many people around the world struggle with and must manage daily.  Recognizing Sickle Cell Awareness Month helps to dismiss stereotypes and stigmas associated with persons who have sickle cell disease.  Not only does this month shine light on sickle cell disease (SCD) but also sickle cell trait (SCT).  Here is some -information about SCD and SCT.

Quick Facts About SCD:

  • SCD is a blood disorder that causes sickling of the red blood cells.
  • Sickle-shaped red blood cells become stuck in blood vessels and cause disruption of blood flow – this results in crises.
  • SCD affects 100,000 people in the United States.
  • SCD is an inherited blood disorder from the person’s parent, like any other genetic trait, such, as hair color and texture, and eye color.

Quick Facts About SCT:

  • SCT is where a person inherits one sickle cell gene and one normal gene.
  • SCT is not a disease and is generally asymptomatic.
  • SCT affects 1 million to 3 million Americans and 8 to 10 percent of African Americans.
  • Persons with SCT can pass the trait on to their children.

 

What is the Likelihood a person will inherit SCD or SCT?

Sickle Cell DHEC Infographic

DHEC’s Sickle Cell Program

DHEC’s Division of Children and Youth with Special Health Care Needs (CYSHCN) provides assistance to persons with sickle cell disease by covering services, such as:

  • medical expenses
  • physician visits
  • durable medical equipment
  • medical supplies
  • prescription drugs

Assistance is offered to both children and adults who meet eligibility requirements.  Additionally, CYSHCN partners with the Newborn Screening Follow-Up program to ensure infants who are newly diagnosed with sickle cell disease have a medical home to address treatment and care for their disease.

Through partnerships with four sickle cell community-based organizations — the James R. Clark Memorial Sickle Cell Foundation, – Louvenia D. Barksdale Sickle Cell Anemia Foundation, – Orangeburg Area Sickle Cell Foundation and – COBRA Human Services Agency Sickle Cell Program — more persons with sickle cell disease are able to obtain services and support.

South Carolina’s Efforts to Address Services for Sickle Cell Patients & Families

As a part of the agency’s commitment to educate the community and public about the availability of resources and services for individuals and families living with sickle cell disease, DHEC collaborated with the South Carolina Sickle Cell Disease Advocacy Team to develop “A Call to Action: South Carolina Sickle Cell Disease State Plan.”  This three-year plan provides a framework for addressing gaps in sickle cell disease care as well as highlights strategies and resources to support patients with SCD.

The full plan be viewed at Sickle Cell Plan_CR-012241_Final.

sickle cell plan

If you have questions about the DHEC sickle cell program, contact the CYSHCN office at 803-898-0784.  For general information about sickle cell disease, visit cdc.gov/sicklecell or http://www.nhlbi.nih.gov/health/health-topics/topics/sca.

National Sickle Cell Awareness Month

By Malerie Hartsell, MPH, CHES
Program Coordinator
Children with Special Health Care Needs

Did you know September is National Sickle Cell Awareness Month?

Efforts to recognize Sickle Cell Month began in 1983 when the Congressional Black Caucus introduced the resolution to Congress.  President Reagan signed the resolution in August of 1983 making September National Sickle Cell Awareness Month.   Since then, organizations across the globe have increased public awareness surrounding this crippling illness.

By recognizing National Sickle Cell Awareness Month, more individuals are educated about the struggles that come along with daily management of this debilitating genetic disease. Increasing awareness is one way to debunk stereotypes and stigmas associated with persons who have sickle cell disease (SCD) and further highlights risk factors related to SCD, such as having the sickle cell trait (SCT).

What is SCD?  SCD is a blood disorder that causes sickling of the red blood cells, which diminishes the amount of oxygen the red blood cell can carry throughout the body.  sickle cellsPersons who have SCD suffer from crises — episodes of intense and excruciating pain that may be in one or multiple parts of the body when sickle-shaped red blood cells become stuck in a blood vessel and cause a disruption of blood flow in that particular area.  While people are most familiar with sickle cell anemia, other variations of sickle cell, or mutations, include sickle cell thalassemia, sickle beta thalassemia, and others.

Who is affected?  The actual number of persons living with SCD is unknown in the United States, but it is estimated that SCD affects approximately 100,000 people annually. Sickle cell disease also affects millions of people worldwide and it is more common among African-Americans.

What is SCT?  SCT is where a person inherits one sickle cell gene and one normal gene from either of their parents.  Persons with sickle cell trait  usually do not have any of the symptoms of SCD, but they can pass the trait on to their children.

How common is SCT? Sickle cell trait is an inherited blood disorder that affects 1 million to 3 million Americans and 8 to 10 percent of African-Americans. Sickle cell trait can also affect Hispanics, South Asians, Caucasians from southern Europe, and people from Middle Eastern countries. More than 100 million people worldwide have sickle cell trait. (Source: http://www.hematology.org/Patients/Anemia/Sickle-Cell-Trait.aspx)

SCD and SCT are inherited conditions, which means a person may be born with the illness or trait.   SCD or SCT cannot develop overtime nor is it contagious.  SCD is inherited when a child receives two sickle cell genes from both parents. For someone that has SCT, the likelihood of having a child that has SCD or SCT is different.  If both parents have SCT, there is a 50 percent chance the child will have SCT, a 25 percent chance that child may have SCD, and 25 percent chance the child will not have SCD or SCT.sickle-cell-flow-chart

 Care and Treatment

Early diagnosis and monitoring can make a difference in the quality of life and number of years lived for someone with SCD.  The Centers for Disease Control and Prevention recommends:

  • Babies from birth to 1 year of age should see a doctor every two to three months.
  • Children from 1 to 2 years of age should see a doctor at least every three months.
  • Children and adults from 2 years of age or older should see a doctor at least once every year.

Persons with SCD should be referred to a hematologist or an experienced general pediatrician, internist, or family practitioner.

Currently, only hematopoietic stem cell transplantation (HSCT) can cure SCD; however, this is very intensive treatment and not everyone qualifies or can afford this treatment.  To help prevent complications and medicate the acute or chronic pain episodes as they occur, hematologist will start children on daily doses of penicillin at birth up until at least 5 years of age.  Blood transfusions are utilized to help reduce the risk of stroke.  Additionally, hydroxyurea, a drug that increase the levels of fetal hemoglobin has helped reduce pain, hospitalizations, and lung damage.

Visit the CDC’s website to learn more about sickle cell. You also can find information on the National Heart, Lung and Blood Institute site.

Caring for a Child with Sickle Cell Disease

By Malerie Hartsell, MPH, CHES, DHEC’s Children with Special Health Care Needs Program

Sickle Cell Disease (SCD) affects many South Carolinians across the state who either have the disease or care for someone with SCD who needs their assistance. DHEC employee Brenda Green knows all too well the impact that SCD has had on her life and the life of her son, Braden.

Brenda is an environmental engineer with the Bureau of Water in the Domestic Wastewater Permitting Section. Ever since her family was informed of her son’s SCD diagnosis, she has been a champion and advocate for Braden and his health.

To help raise awareness during National Sickle Cell Awareness Month, Brenda did a Q&A interview with DHEC’s Children with Special Health Care Needs program to share a small piece of her SCD story and how the disease has impacted her family.

 Q:  Tell us about your son, Braden Green.

A:  Braden is 10 years old and in the 5th grade at Satchel Ford Elementary School. He loves school and his favorite subject is math.  He loves to draw, paint and create art.  He plays the piano, drums and just began the strings program at school where he will play the cello.  He loves ALL sports but plays little league baseball.  He knows the stats on almost all baseball, football and basketball teams and their star players.  He’s a really smart and sweet kid.

Q: You and your husband have three children, and your son has sickle cell disease. What are some things you have to be mindful about when raising and caring for Braden that you don’t necessarily worry about with your other children?

A:  I’m more mindful about a lot of things in comparison to his big sisters. It was more difficult during the first years of his life.  He wasn’t able to tell me where it hurts and how the pain felt.  I had to wonder, when he was an infant, whether he was crying because he was hungry, teething or having sickle cell pain.

Now that he is older, we use the number scale for pain that is used in the hospital (1-10 with 10 being the worst).  He can also tell me if it’s a sharp, dull or burning pain. I have to make sure he’s hydrated especially when he’s playing or practicing baseball.

I have to make sure he understands that he has to wash his hands and try to keep his hands from his face. Germs are not our friends at all times.  The common cold can be very dangerous to him.  He is most susceptible to pneumonia and has been hospitalized several times because of it.

I have to make sure he’s not too cold in the winter or too warm in the summer.  Even though it’s warm now, I make sure he takes his jacket to school just in case the classroom is cold.  Extreme temperatures can cause pain crises for him.

I worry about him at birthday parties, especially if they are running and playing.  He gets tired quicker than the average child but he will push himself if he’s not reminded to rest.  This is the same with him playing sports.  I’m also more mindful of the types of sports he plays.

Q:  What have you found to be the most helpful for your family since finding out Braden has SCD?

A: The most helpful thing that my husband and I have found is to be very observant of him and his symptoms and to make sure we’re responsible when it comes to his medical care.  We call his pediatrician if we are concerned and we make sure he attends all routine appointments.  It’s important that we continue to learn as much as we can about SCD and be very proactive with his care.

The pain level chart, I reference earlier, has been very helpful to us.  We now understand his pain tolerance better.  He can function normally until his pain is a 5.  At that point, he will take more frequent breaks and may ask for his heating pad.  Usually a pain of 8 or greater requires pain medication.  If we’re unable to control his pain at home, then it requires medical attention.

 Q: Braden has been attending Camp Burnt Gin for several summers. What has that experience been like for him and for your family?

A:  The experience has been wonderful.  He has made friends and has enjoyed being with other children who battle the same disease as him.  It’s his opportunity to relax and have fun without mommy hovering close.  My husband and I are happy that he can go to an overnight camp where he can have fun, experience the camp life just like the healthy kids and all the while at a safe location with his hematologist, Dr. Carla and his nurse, Nurse Julia on site.  What more could we ask for?

braden-and-dr-carla-camp-burnt-gin

Braden and Dr. Carla at Camp Burnt Gin, a summer camp in Wedgefield, SC for children with physical disabilites or chronic illnesses.

Q:  What advice would you give to other parents who have just learned their child has SCD?

A: Be encouraged and know that your child is so special and will teach you the true meaning of resilience and strength.  Use your resources.  Your child’s hematologist, pediatricians, nurses and other caregivers are all there to help your child and your family battle this disease.  Learn as much as you can. Document all that you learn and ask questions.

Finally, never, I mean never, be afraid or hesitant to be the main advocate for your child.  Through you being your child’s advocate you will teach your child to be able to advocate for themselves as they get older.

For more information on DHEC’s Children with Special Health Care Needs division, visit www.scdhec.gov/Health/ChildTeenHealth/ServicesforChildrenwithSpecialHealthCareNeeds/