Sickle-cell disease (SCD) is an inherited blood disorder in which red blood cells are abnormally shaped like a sickle or crescent, which decreases their ability to move through blood vessels smoothly. This abnormal blood flow can result in a variety of debilitating complications, the most common of which are painful events that result from blocked blood flow and oxygen to organs and extremities, known as the “sickle pain crisis.”
Signs and symptoms of SCD usually begin in early childhood, but not all patients have the same or a constant course through their lifetime. Some patients may have only mild symptoms, while others may be hospitalized frequently for more serious complications. Importantly, chronic lung disease is often associated with SCD can start early in life for many patients. SCD can cause recurrent pneumonias, asthma symptoms, sleep problems, and decrease oxygen levels causing strain to the heart. Luckily, many of these problems can be treated or prevented with careful monitoring.
While awareness about SCD is increasing in the United States, overall resources and awareness of SCD treatments remain low, especially for the most life-limiting problems associated with the disorder. SCD is the most common inherited blood disorder in the United States and affects millions of people around the globe. Among the hardest-hit areas is Africa, where close to 1,000 babies are born with SCD each day. Most die before the age of 5 due to a lack of medical resources.
While in the United States we are fortunate to have great role models with SCD who have successfully managed careers and advocacy efforts, such as singer Tionne ‘T-Boz’ Watkins of TLC, other areas of the world aren’t as fortunate. So what can do about this problem?
- Take the time to ask or tell someone about what SCD is like. A little knowledge goes a long way.
- Use social media to advocate for your cause: Twitter, Facebook and Instagram are great ways to raise awareness.
- Encourage local and national government agencies to increase funding and resources for SCD. At the heart of every great advocacy campaign are passionate patients and family members. Encourage members of Congress to join the Congressional Caucus for SCD today! This legislative movement is focused on increasing funding and opportunities for SCD research, care access, and public awareness. It is led by Representatives, Charles Rangel (D-NY), Danny Davis (D-IL), and Senator Tim Scott (R-SC).
- Talk to your medical team if you have ideas to improve symptom monitoring or care.
Recognize breathing symptoms that have long-term effects:
- Poor sleep or snoring in children with SCD and can lead to breathing difficulties and heart strain.
- Forms of asthma can complicate and contribute to life-threatening pneumonias.
- Abnormal oxygen levels, whether asleep or awake should not be ignored.
- Deterioration in exercise or sports abilities is not normal.
Get regular follow-ups:
- NCH has a multi-disciplinary sickle cell clinic for patients to receive many services in one setting.
- We’ve started a pulmonary/sickle cell clinic to follow breathing problems at the same time as regular sickle cell care.
Fewer hospital visits and improved disease management means more time to enjoy life and advocate. With a concentrated effort, we can all work together to improve outcomes for patients with sickle cell.
Learn about the most common form of sickle cell disease with Dr. Mike Patrick in PediaCast.