Epidemiology: Sickle cell anemia (SCA) is the most common form of SCD. Worldwide, it is estimated that there are over 400,000 newborns with sickle cell anemia. The birth prevalence varies according to region, with prevalence greatest in regions affected by holoendemic malaria. In Europe, the pooled birth prevalence is 1/2,300, although this varies between countries. Clinical Description: The disease does not manifest during fetal life or up to the first three months of life due to the presence of high levels of fetal hemoglobin. Clinical manifestations evolve with age and are extremely variable between individuals and at different times. In addition to anemia and bacterial infections, VOAs cause hyperalgic focal ischemia (and sometimes infarction) when they occur in the abdomen, chest or skeleton. Over the course of time, VOAs may compromise the integrity of tissues or organs.   Abstract source

Sickle cell disease (SCD) is a chronic hemoglobinopathy of clinical relevance because of its significant morbidity and mortality, particularly in people of African and Mediterranean ancestry. SCD results from a mutated form of hemoglobin, hemoglobin S (HBS), and vascular obstruction and ischemia result in a wide range of clinical problems. Effects on the musculoskeletal system include extramedullary hematopoiesis, osteonecrosis, dactylitis (hand-foot syndrome), myonecrosis, and osteomyelitis,with bone disease being one of the most common presentations in patients with SCD. Abstract source

Sickle cell disease (SCD) is a group of inherited, multisystemic conditions with episodes of acute illness and progressive organ damage. It is characterized by the occurrence of the sickle mutation (1,2). -mutation in the beta globin gene results in substitution of valine for glutamine at the sixth position in the beta-subunit of a Hb molecule and leads to the production of abnormal beta-globin chain. This alpha2-S2 tetramer is called sickle haemoglobin molecule (HbS) (1) -the disease shows an autosomal recessive inheritance (3), all SCD genotypes contain at least one sickle gene in which HbS comprises at least half of the haemoglobin present (4) -in sickle cell anaemia (homozygous SCD) - 80-99% of the haemoglobin is HbS, with most of the remainder being fetal haemoglobin (HbF) The term sickle cell disease (SCD) represents all of the different genotypes which presents with the characteristic clinical syndrome as a result of the presence of haemoglobin S while the term sickle cell anaemia (commonest type of SCD) is used specifically for patients with the homozygos disease (2).   Abstract source

Priapism in Sickle Cell Anemia: II. Epidemiology Incidence of Priapism in Sickle Cell Anemia: 89% by age 20 years old III. Management Duration: Analgesics IV Fluids Do not transfuse for Priapism 4 hours Administer above measures if not already attempted Exchange transfusion (Risk of CVA symptoms) Epidural Anesthesia Abstract source

Septic Arthritis in Sickle Cell Anemia: II. Causes Staphylococcus aureus (most common cause) Streptococcus Pneumoniae Salmonella III. Labs Synovial Fluid (obtain in all suspected cases) 1. Normal Synovial Fluid cell count in >50% of Septic Arthritis cases 2. Gram Stain Test Sensitivity is only 50% (high False Negative Rate) 3. Synovial Fluid is the gold standard for diagnosis IV. Management A. Obtain Synovial Fluid culture and start empiric antibiotics B. Total antibiotic course: 6 weeks C. Initial empiric management 1. Cephaloporin sensitive a. Ceftriaxone 50-75 mg/kg up to 2 g/dose OR b. Ceftriaxone 75-100 mg/kg in high S. Pneumoniae resistance regions 2. Cephalosporin resistance a. Clindamycin 10-15 mg/kg/dose (preferred) OR b. Vancomycin 15 mg/kg/dose (if systemic Sepsis or Clindamycin reactions) Abstract source

Acute Vaso-Occlusive Episode in Sickle Cell Anemia: II. Epidemiology Affects almost all Sickle Cell Anemia patients May occur as early as age 6 months III. Pathophysiology Deoxygenated Hemoglobin polymerizes in triple helix chains Results in Red Blood Cells assuming sickled shape Results in Occlusion of small vessels and capillaries IV. Precipitating Factors A. Preceded by infection 25% of the time 1. Human Parvovirus B19 is a common viral cause B. Cold Weather due to reflex vasospasm C. Dehydration in warm weather Abstract source

Sickle cell disease (SCD) is a group of inherited, multisystemic conditions with episodes of acute illness and progressive organ damage. It is characterized by the occurrence of the sickle mutation (1,2). -mutation in the beta globin gene results in substitution of valine for glutamine at the sixth position in the beta-subunit of a Hb molecule and leads to the production of abnormal beta-globin chain. This alpha2-S2 tetramer is called sickle haemoglobin molecule (HbS) (1) -the disease shows an autosomal recessive inheritance (3), all SCD genotypes contain at least one sickle gene in which HbS comprises at least half of the haemoglobin present (4) -in sickle cell anaemia (homozygous SCD) - 80-99% of the haemoglobin is HbS, with most of the remainder being fetal haemoglobin (HbF) The term sickle cell disease (SCD) represents all of the different genotypes which presents with the characteristic clinical syndrome as a result of the presence of haemoglobin S while the term sickle cell anaemia (commonest type of SCD) is used specifically for patients with the homozygos disease (2). When exposed to low oxygen tensions or acidaemia non-covalent polymerisation of HbS takes place, resulting in distortion and sickling of red cells. Abstract source

Sickle cell disease (SCD) and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin, hemoglobin S (HbS) [1] (see the image below). The most common form of SCD found in North America is homozygous HbS disease (HbSS), an autosomal recessive disorder first described by Herrick in 1910. SCD causes significant morbidity and mortality, particularly in people of African and Mediterranean ancestry (see Pathophysiology). Morbidity, frequency of crisis, degree of anemia, and the organ systems involved vary considerably from individual to individual. Abstract source

Shakir Cannon was a Health Information Advocate and Co-Founder of the Minority Coalition for Precision Medicine (MCPM), an emerging organization dedicated to the disbursement of health information literacy through a parallel education and awareness strategy performed within minority populated regions as determined by US Census data. Shakir was also an internationally recognized Sickle Cell Disease (SCD) patient and advocate who helped educate and spread awareness for SCD both as a public speaker and social media thought leader. Resulting from his lifelong battle with an often debilitating and at times devastating disease, Shakir lived by the motto "Any Day Without Pain Is a Good Day!"   Shakir Cannon's Obituary: Shakir Cannon, advocate for minority health, dies