In a previous post, I discussed the role of excessive inflamation in thalassemia, a blood disorder in which individuals suffer from low numbers of red blood cells and hemoglobulin levels. This post focuses on another blood disorder that results in low hemoglobulin levels, sickle cell anemia.
Abnormal Red Blood Cells
Normally, blood cells are rounded, disc-shaped, and flexible enough to move easily through blood vessels. In contrast, people with sickle cell anemia have crescent, sickle-shaped, red blood cells that are stiff and sticky, and tend to become “stuck” in various tight spots in blood vessels. This blocks blood flow leading to pain and organ damage from inflammation in response to the blockages. Additionally, since their hemoglobin structure is abnormal, the red blood cells are unable to carry their full complement of oxygen throughout the body, resulting in oxygen deficits.
Red blood cells typically live for about 4 months in the blood stream, but sickle cells die after only 10-20 days. Individuals with sickle cell anemia, cannot make fresh red blood cells fast enough to replace the dying red blood cells. The lack of oxygen leads to fatigue, feelings of weakness, shortness of breath, dizziness, headaches, cold hands and feet, pale mucous membranes, and a yellowish tinge to skin or the whites of the eyes.
Pain Crises
Perhaps the most devastating symptom that many individuals with sickle cell anemia experience is excruciating pain that lasts for hours, weeks, or months. These are called “pain crises”. Painful crises are the leading cause of emergency room visits and hospital stays for people who have sickle cell anemia.
The pain results from inflammation and damage to blood vessels by the sickled cells. When the red blood cells block the flow of blood to limbs and organs, immune cells come into the area and release inflammatory cytokines, immune molecules that result in a vicious cycle of more inflammation and pain.
Some individuals experience these crises a few times a year, others may more frequent episodes. Repeated crises can damage the bones, joints, kidneys, lungs, eyes, heart, and liver. Moreover, in children, damage to their spleen, an immune organ, can leave them more susceptible to infection.
Cascade of Inflammation
Inflammation not only plays a major role in damaging blood vessels, but the immune cells release inflammatory cytokines, molecules that trigger inflammation, and biological compounds that cause cells to become “sticky”. The blocked blood flow leads to pain and other health issues.
When compared to those without sickle cell disease, individuals with sickle cell anemia have different profiles of messenger cytokines. For example IL-6, which helps the body return to immune balance, immune homeostasis, is at significantly higher levels in sickle cell anemia patients.
Summary
Inappropriate levels of inflammation pose major challenges for the quality of life of individuals with sickle cell disease. A rational approach to benefiting individuals with sickle cell is to help their bodies achieve inflammatory homeostasis, immune balance.
Help your body return to immune balance, immune homeostasis. Dr. Hellen may be contacted at: 302.265.3870 ET USA, or use the contact form. Thank you.
http://umm.edu/health/medical/reports/articles/sickle-cell-disease
www.ncbi.nlm.nih.gov/pubmed/8746787
http://www.ncbi.nlm.nih.gov/gene/3569
http://www.ncbi.nlm.nih.gov/pubmed/24383847
www.ncbi.nlm.nih.gov/pubmed/24589266
http://arthritis-research.com/content/8/S2/S3
