What Is Sickle Cell Disease?
Sickle cell disease. It's a strange sounding name — but the word "sickle" doesn't come from "sick." The disease got this name because it causes blood cells to be curved or C-shaped, like the shape of an old-fashioned farm tool called a sickle. Normally, blood cells are round and look a bit like donuts. In people with sickle cell disease, some of the red blood cells harden and curve. They also break down faster than healthy red blood cells and can clog a person's small blood vessels. This causes severe pain, called a "pain crisis."
A pain crisis can last for a few moments, a few days, or even longer. Medications can help lessen the pain. People with sickle cell disease can develop anemia. They also may have some jaundice. Sickle cell disease is inherited. People are born with it. It‘s not contagious, so you can't catch it from someone. Sickle cell disease is a lifelong health condition. Right now, there's no cure, but there are things doctors can do to help.
What's My Friend Going Through?
Because of sickle cell disease, your friend may be tired more often than you are or have trouble fighting infections. Most teens with sickle cell disease have to pay extra attention to diet, take vitamins (especially folic acid), exercise, get enough rest, and stay hydrated by drinking enough water. They need to watch how their bodies respond to infections like a cold. Some people take antibiotics to help fight off bacteria.
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Sickle cell disease will be with you throughout your life, in some way or another. As you grow older, you will have questions about how it may affect you in the future. Now is a good time to start looking into "transitioning" from your pediatric specialist to doctors who treat adults.
What Is Transitioning?
When doctors talk about "transitioning," they mean safely shifting a patient's care from a child to an adult medical practice. But who takes care of you is just one part of transitioning. Transitioning also means learning how to take charge of your own health and manage every aspect of your care, from handling insurance forms to understanding and making decisions about treatments.
Your parents have probably played a big part in your medical care, and that's great. But after you turn 18, they may not be able to see your medical records or talk about your health with your doctor even if you want them to.
Your medical team should be able to guide you in the transitioning process, but here are some good things to know.
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This study was conducted to measure the health literacy (HL) and disease-specific knowledge (DSK) of caregivers for children with sickle cell disease (SCD) and relate them to their child's health care utilization. The authors conducted a cross-sectional study of caregiver-child dyads attending an urban pediatric sickle cell clinic. Caregivers were administered the Shortened Test of Functional Health Literacy (S-TOFHLA) and a locally developed DSK questionnaire. Retrospective review of the child's electronic medical record (EMR) was performed to determine annual emergency department (ED) visits and hospitalizations. A total of 142 caregiver-child dyads were recruited for the study. Less than 5% of caregivers had limited HL, with less education (P =.03) and primary language other than English (P =.04) being the only risk factors. Although caregiver HL was not associated with ED visits or hospitalizations, surprisingly DSK was.
Caregivers with higher DSK scores had children with higher annual rates of ED utilization (P =.002) and hospitalizations (P =.001), and these children were also more likely to be classified as high ED utilizers (≥4 visits per year; P =.01). Further, caregiver adherence to medication and clinic visits was associated with their child's age (P =.01). Although HL and DSK are both constructs that measure basic health understanding, they differently affect caregivers' ability to navigate and understand the health care system of children with chronic illnesses. This study suggests that the DSK/health care utilization relationship may be a more important measure than HL for programs following children with sickle cell disease and could also have applications in other pediatric chronic diseases.
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Purpose: To identify successful recruitment strategies, challenges and best practices for researchers to engage African American communities in clinical studies taken into consideration target participants’ culture and context.
Methods: We reviewed 50 studies conducted from 2001 to 2012 at an inner-city research center to determine the type, duration, anticipated enrollments and actual enrollments. Survey was sent to study coordinators to obtain data on recruitment and retention strategies, challenges and dropout rates. We also interviewed 25 study coordinators on challenges and strategies.
Results: Of the 50 studies, 24 had completed recruitment at the time of this report. The completed studies achieved a median recruitment rate of 88% (range: 50–110). Successful recruitment and retention strategies included: fi eld-based strategy and snowballing. Major barriers were: distrust, compensation, education disadvantage, lack of interest, and inability to have study partner. Strategies to reduce barriers included providing informational sessions, disseminating newsletters about study outcomes. Best practices include being culturally sensitive including demonstrating a caring attitude and being responsive to participants needs.
Conclusions: Cultural competence is critical in order to design and implement successful recruitment strategies in this population. Research teams should consist of multiethnic staff, involve the community, demonstrate trust and deliver concise education of the research endeavor.
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This fact sheet has general information about sickle cell disease (SCD). Every child is different and some of this information may not apply to your child specifically. Certain treatments may be recommended for some children but not others. If you have specific questions about SCD and available treatments, you should contact your doctor.
The majority of the management recommendations pertain to hemoglobin SS, which is the most common type of SCD. If your child has hemoglobin SC or hemoglobin beta-thalassemia, ask your doctor for more specific information about that condition.
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Sickle cell disease is common in many regions of the world where mosquito-borne malaria is present. It is believed that people who carry only one sickle cell mutation (they do not have the disease) can tolerate malaria better than people who carry no mutation. This may be why the mutation persists in the population despite the high mortality associated with untreated sickle cell disease.
Sickle cell disease is the most common single gene disorder in Africans, affecting one in every 375. Globally, a quarter of a million children are born with the disease each year, mainly in Africa, the Mediterranean, Arabia, and South Asia.
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The Sickle Cell Program at UI Health is the largest in the Midwest, providing individualized care for pediatric, transitional, and adult patients. Its hematologists and oncologists work to help patients with health maintenance and pain management, including blood infusion and transfusion therapies and stem cell transplantation.
Patients in their Sickle Cell Program have access to a number of supportive services, including:
- Counseling
- Education and Outreach
- Support Groups — pediatric and adult
- National research studies
The mission of the Sickle Cell Program is to improve the quality of life for individuals with sickle cell and reduce the prevalence and mortality of the disease.
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