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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
 

Blood disorders are diseases that affect blood components: 1) red blood cells, 2) white blood cells, and/or 3) platelets.

 Red blood cells are disc-shaped cells that carry oxygen from the lungs to all the cells in the body White blood cells are immune cells that help the body heal, and protect itself from infections and cancerous cells that might grow into tumors or cancers of the blood.  Platelets are blood elements that stick to the lining of blood vessels and help the blood to clot when  bleeding from a wound.

 Some common blood disorders are  anemia, thalassemia, sickle cell anemia,  idiopathic thrombocytopenic purpura (ITP),pernicious anemia,  hemolytic anemia, and aplastic anemia.

 All of these disorders have a single commonality, mainly that individuals with these types of conditions have low numbers of red cells, white blood cells, and/or platelets.

 Inflammation is necessary for our survival. Invasion by pathogens initiates inflammatory processes that attack these organisms. However, too often the “forest fire” gets out of control, and healthy cells, tissues, and organs are damaged.  When the body attacks its own cells, the condition is called an autoimmune, against -oneself, response.

 Thalassemia is an inherited disease in which people have abnormally low numbers of red blood cells and low hemoglobin. The hemoglobulin molecule is faulty and unable to carry its typical complement of oxygen.  [Hemoglobin is a protein that  helps  transport oxygen throughout the body.  Red blood cells also carry waste gases like carbon dioxide  to the lungs where it is released and then exhaled.]

 Individuals with thalassemia often suffer from inflamed blood vessels and slower blood flow in their blood vessels.  Both problems put individuals at greater risk of suffering from thromboembolism.  In this condition, a blood clot, an embolus, partially or totally blocks blood vessels deep in the body (deep vein thrombosis) or a clot is released that suddenly interferes with blood flow within a lung artery (pulmonary embolism), which can be fatal.

As blood clots form, an inflammatory response is triggerred to break up the clots.  More inflammation results in the production of more cytokines, immune messages that affect blood clotting.  Individuals with thalassemia, as with other blood disorders, typically have higher levels of inflammatory cytokines than individuals without such conditions.

It never ceases to amaze me how many health practitioners ignore the contribution of inflammatory process to diseases such as thalassemia.  In blood disorders, as with most other diseases, achieving and maintaining immune inflammatory homeostasis, balance, is essential.

 Being in homeostasis means that there are enough immune factors, pro-inflammatory cytokines to initiate a proper inflammatory response, and corresponding anti-inflammatory factors to limit inflammation and the damage it may cause.  A delicate balance of these messages are essential.

 What does one lose by moderating excessive inflammatory responses?  Control inappropriate levels of inflammation, and improve the quality of life of those with blood disorders, and most other diseases.

 [Please look for future posts on other blood disorders such as sickle cell anemia, pernicious anemia, and idiopathic thrombocytopenic purpura (ITP)].

 There is no cost to readers of these posts to speak with Dr. Hellen.  She can be reached at 1.302-265.3870 [USA] or contacted at:  drhellen@drhellengreenblatt.info .

 

www.nhlbi.nih.gov/health/public/blood/
www.nhlbi.nih.gov/health/health-topics/topics/pe/
www.sciencedirect.com/science/article/pii/S1079979609001387
bloodjournal.hematologylibrary.org/content/87/12/5051.full.pdf

 

 

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