Tag Archives: sickle cell

Raising the Awareness Bar on Sickle Cell Trait and Sickle Cell Disease

September is National Sickle Cell Awareness Month, let’s raise the awareness bar to highlight sickle cell trait and sickle cell disease.

What is Sickle Cell Disease?

 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. Persons 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.

What is Sickle Cell Trait?

 SCT results when 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 are SCT and SCD related?

 An individual who has SCD has a family history of SCT – meaning the person’s parent(s) have sickle cell trait or sickle cell disease. SCD is inherited when a child receives two sickle cell genes from each parent. For someone who 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 the child may have SCD, and a 25 percent chance the child will not have SCD or SCT.

SickleCellAwareness 2018

The urge to increase awareness on sickle cell trait and disease is apparent across several organizations.  The Centers for Disease Control and Prevention released new informational materials and videos of individuals’ personal experiences living with sickle cell disease or sickle cell trait. To view these videos, visit cdc.gov/ncbddd/sicklecell/materials/video.html.

What is South Carolina’s response?

The South Carolina Sickle Cell Disease Advocacy Team (SCSCDAT) was established in 2017 with one common goal – to improve the treatment and care received by individuals and their families who have sickle cell disease. A multidisciplinary team comprised of physicians, hematologists, government agencies, non-profit organizations, healthcare management organizations, and individuals living with SCD and their family members, has been working on a statewide sickle cell disease plan to address the lack of resources to proficiently care and treat individuals of all ages living with sickle cell disease. The plan will help coordinate and improve collaboration in the areas of education, outreach, treatment, and funding.

Community-based organizations at work

Currently, DHEC maintains partnerships with four sickle cell community-based organizations — the James R. Clark Memorial Sickle Cell Foundation, the Louvenia D. Barksdale Sickle Cell Anemia Foundation, the Orangeburg Area Sickle Cell Foundation and the COBRA Human Services Agency Sickle Cell Program. Through these partnerships, more people with sickle cell are able to obtain services and support. These organizations work to provide education, counseling, testing for sickle cell trait, and family support.

Although September is National Sickle Cell Awareness Month, increasing public and community knowledge about sickle cell is a 365-day initiative. Raise the awareness bar on sickle cell trait and sickle cell disease.

If you have questions about testing for you or your family, you can visit one of the four sickle cell community-based organizations. For more general information about sickle cell, visit cdc.gov/sicklecell or 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.

Help is available for those challenged by sickle cell disease

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

When a child inherits sickle cell disease it can cause emotional, financial and other strain on a family.

It also raises serious questions, such as “How will our child cope with a disease that can cause sudden extreme, pain-filled episodes?” or “How will our family handle the challenge of caring for a child with a chronic, untreatable illness?”

While families bear much of the load, there are services in place to help, including those available through the S.C. Department of Health and Environmental Control’s Children with Special Health Care Needs program.

What kind of help does Children with Special Health Care Needs offer?

 Under the Bureau of Maternal and Child Health, Division of Children’s Health and the DHEC Lab, the Newborn Screening Program includes tests for SCD, sickle C disease, sickle B thalassemia, and variant hemoglobinopathy disorders and traits, including sickle cell trait. Based off DHEC Lab Newborn Screening data, there are approximately 70 babies born with SCD annually and over 2,400 babies identified to have SCT.

After a diagnosis is confirmed, the Children with Special Health Care Needs Program assist South Carolinians by covering:

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

Additionally, Care Coordinators in DHEC Regional offices provide information and/or referral, and support services.

Every summer, children with sickle cell disease spend a week at Camp Burnt Gin in Wedgefield, South Carolina, where they enjoy a fun camping experience while learning about blood disorders, treatment and ways to manage the disease.  The week at Camp Burnt Gin is a partnership between DHEC’s CSHCN program and Palmetto Health, which conducts educational activities during the week that foster disease management, positive self-esteem, confidence and independence, while giving campers a positive childhood experience.

Community based organizations at work

Through partnerships with four sickle cell community based organizations — the James R. Clark Memorial Sickle Cell Foundation, the Louvenia D. Barksdale Sickle Cell Anemia Foundation, the Orangeburg Area Sickle Cell Foundation and the COBRA Human Services Agency Sickle Cell Program — more persons with sickle cell are able to obtain services and support.  These organizations work tirelessly to provide educations and counseling, testing for sickle cell trait, family support and education for hospital staff.

Throughout September, which is Sickle Cell Awareness Month, each community based organization will engage community members in different events to help promote and increase awareness about SCD.

If you have questions about testing for you or your family, you can visit one of the four sickle cell community based organizations.  For more general information about sickle cell, visit cdc.gov/sicklecell or http://www.nhlbi.nih.gov/health/health-topics/topics/sca.

SickleCellSeptemberEvents

National Sickle Cell Awareness Month

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

September is National Sickle Cell Awareness Month.  The purpose of the month is to bring attention to a crippling genetic disease that many people around the world struggle with and manage daily.

Sickle Cell Awareness Month helps to debunk 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 are Key Points to know about SCD & SCT:

  • SCD is a blood disorder that causes sickling of the red blood cells.
  • SCD is the most common hereditary blood disorder.
  • Red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle”.
  • Persons who have SCD may suffer from crises — acute episodes of intense and excruciating pain.
  • Different types of sickle cell disease are caused by different genetic mutations.
  • SCT is where a person inherits one sickle cell gene and one normal gene from either of their parents.
  • Persons with SCT can pass the trait on to their children.

Who is affected?

While sickle cell disease affects millions of people throughout the world, it is more common among African-Americans. It is estimated that SCD affects 100,000 people in the United States.

Sickle cell trait is estimated to affect 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.

While 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 the child may have SCD, and a 25 percent chance the child will have not have SCD or SCT.

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 Center for Disease and Control 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 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.

There are treatments that can help to prevent complications and medicate the acute or chronic pain episodes as they occur.  Treatment options for persons with SCD differ by patient.  Children with SCD can begin taking daily doses of penicillin starting at birth up to 5 years to help prevent bacterial infections.  Blood transfusions are utilized to help reduce the risk of stroke.  Hydroxyurea, a drug that increases levels of fetal hemoglobin that is less likely to become sickle-shaped, has helped reduce pain, hospitalizations and lung damage.

Currently hematopoietic stem cell transplantation (HSCT) is the only cure for SCD.

For more information about sickle cell, visit http://www.cdc.gov/ncbddd/sicklecell/index.html or http://www.nhlbi.nih.gov/health/health-topics/topics/sca.

SickleCellSeptemberEvents

Show the world what a Q-tip can do

By DHEC Division of Cancer Prevention and Control and Be The Match

What impact or difference could a Q-tip have on the world? Answer- FIGHT CANCER!

Every three minutes someone is diagnosed with a blood cancer. By the time the blog post is complete, eight people will have been diagnosed by a doctor they never planned to see with a disease they never thought would impact their life. Each day 480 people are diagnosed with various blood cancers/blood disorders including leukemia, lymphoma, aplastic anemia and sickle cell anemia.

1,500 South Carolinians each year are part of these sobering statistics. Half of these 1,500 patients will need a bone marrow transplant. This transplant is their last and (and in many cases) only hope for survival. Be The Match supports these patients in their search for a marrow donor through the national marrow registry.

You have the chance to partner with the Q-tip to fight cancer. Joining the Be The Match registry is as easy as filling out a form and swabbing your cheek with a  . . . wait for it . . . Q-TIP.

You will join a list of over 12 million people who are willing to help if they are called (which is only about a 1 in 70,000 chance). You are probably asking – “does this hurt?” The most common bone marrow donation is very similar to giving blood. The extent of the pain is a needle stick. There is a less common method where we take marrow from the hip, but you are asleep and not aware of what is going on.

No matter the method, you have the chance to save a life – to give a patient and their family a second chance! Interested in showing the world what a Q-tip can do?  You can join the Be The Match by visiting http://join.bethematch.org/sc

Dr. Suess said it best (as usual) – “To the World you may be one person, but to one person you may be the world.”

For more information on Be The Match in South Carolina, contact:

Ashley Collier
Be The Match South Carolina
803-543-9034
acollier@nmdp.org

Be The Match Save a Life