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Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
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Appendix N

Glossary

Acidosis: A condition in which there is too much acid in the body fluids. It is the opposite of alkalosis (a condition in which there is too much base in the body fluids).

Acute Hypoxemia: Severe arterial hypoxemia that is refractory to supplemental oxygen. It is caused by intrapulmonary shunting of blood resulting from airspace filling or collapse.

Adenotonsillectomy: The surgical removal of the adenoids and tonsils, a combination of adenoidectomy and tonsillectomy.

Allodynia: Pain sensitization (increased response of neurons) following normally non-painful, often repetitive stimulation. Can lead to triggering of a pain response from stimuli that do not normally provoke pain.

Amniocentesis: A test conducted at 15–20 weeks of pregnancy that tests the amniotic fluid during pregnancy for sickle cell disease and sickle cell trait.

Amygdala: A roughly almond-shaped mass of gray matter inside each cerebral hemisphere, involved with the experiencing of emotions.

Avascular Necrosis: A condition in which poor blood supply to an area of bone leads to bone death. Also known as aseptic necrosis and osteonecrosis.

Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×

Benzodiazepines: Any of a class of heterocyclic organic compounds used as tranquilizers and are commonly used in the treatment of anxiety, such as librium and valium.

Central Sensitization: An increase in sensitivity to pain and in the responsiveness of neurons.

Cerebral Ischemia: A condition that occurs when there is not enough blood flow to the brain to meet metabolic demand. This leads to limited oxygen supply, or cerebral hypoxia, and leads to the death of brain tissue, cerebral infarction, or ischemic stroke. It is a subtype of stroke along with subarachnoid hemorrhage and intracerebral hemorrhage.

Cerebral Vasculopathy: This is vasculitis (inflammation of the blood vessel wall) involving the brain and occasionally the spinal cord. It affects all of the vessels: very small blood vessels (capillaries), medium-size blood vessels (arterioles and venules), or large blood vessels (arteries and veins). If blood flow in a vessel with vasculitis is reduced or stopped, the parts of the body that receive blood from that vessel begin to die. It may produce a wide range of neurological symptoms, such as headache, skin rashes, feeling very tired, joint pains, difficulty moving or coordinating part of the body, changes in sensation, and alterations in perception, thought, or behavior, as well as the phenomena of a mass lesion in the brain leading to coma and herniation. Some of its signs and symptoms may resemble multiple sclerosis.

Chorionic Villus Sampling: A testing process that is conducted between 10–13 weeks of pregnancy. A needle is inserted into the cervix or the abdomen to retrieve samples of the chorionic villi. The samples can then be tested for hemoglobin S and sickle cell trait.

Chronic Care Model: An organizational approach to caring for people with chronic disease in a primary care setting. The system is population-based and creates practical, supportive, evidence-based interactions between an informed, activated patient and a prepared, proactive practice team.

Chronic Splenomegaly: An abnormal enlargement of the spleen.

Cirrhosis of the Liver: A late stage of scarring (fibrosis) of the liver caused by many forms of liver diseases and conditions, such as hepatitis and chronic alcoholism.

Comorbidities: The simultaneous presence of two or more chronic diseases or conditions in a patient.

Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×

Crizanlizumab: A monoclonal antibody developed by Novartis targeted toward P-selectin. It was announced by the company as an effective drug to prevent vaso-occlusive crisis in patients with sickle cell anemia.

Dyspepsia: Also known as indigestion. A condition characterized by upper abdominal symptoms that may include pain or discomfort, bloating, feeling of fullness with very little intake of food, feeling of unusual fullness following meals, nausea, loss of appetite, heartburn, regurgitation of food or acid, and belching.

Erythrocytes: A red blood cell that (in humans) is typically a biconcave disc without a nucleus. Erythrocytes contain the pigment hemoglobin, which imparts the red color to blood, and transport oxygen and carbon dioxide to and from the tissues.

Functional Asplenia: Occurs when splenic tissue is present but does not work well (e.g., sickle cell disease, polysplenia). Such patients are managed as if asplenic, while in anatomic asplenia, the spleen itself is absent.

HbS: Sickle hemoglobin that results from a genetic mutation in the red blood cells.

HbSC: A moderate clinical severity of sickle cell disease (SCD). People with this form of SCD inherit a sickle cell gene HbS from one parent and a gene for an abnormal hemoglobin called HbC from another parent.

HbSS: Also known as sickle cell anemia, HbSS is a severe or moderately severe genotype of sickle cell disease. This term is also used to refer to sickled hemoglobin.

Hematopoietic Stem Cell Transplantation: The intravenous infusion of hematopoietic stem and progenitor cells designed to establish marrow and immune function in patients with a variety of acquired and inherited malignant and nonmalignant disorders.

Hematuria: The presence of blood in a person’s urine. The two types of hematuria are gross hematuria, when a person can see the blood in his or her urine, or microscopic hematuria, when a person cannot see the blood in his or her urine yet it is seen under a microscope.

Hemoglobin: An iron-containing respiratory pigment of vertebrate red blood cells that consists of a globin composed of four subunits, each of which is linked to a heme molecule that functions in oxygen transport to

Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×

the tissues after conversion to oxygenated form in the gills or lungs, and that assists in carbon dioxide transport back to the gills or lungs after surrender of its oxygen.

Hemolysis: The rupture or destruction of red blood cells.

Hemophilia: A medical condition in which the ability of the blood to clot is severely reduced, causing the sufferer to bleed severely from even a slight injury. The condition is typically caused by a hereditary lack of a coagulation factor, most often factor VIII.

Hyperalgesia: Excessive sensitivity and a raised threshold to painful stimuli.

Hyperviscosity: A group of symptoms triggered by an increase in the viscosity of the blood. Symptoms of high blood viscosity include spontaneous bleeding from mucous membranes, visual disturbances due to retinopathy, and neurologic symptoms ranging from headache and vertigo to seizures and coma.

Ischemia Reperfusion: Reperfusion injury, sometimes called ischemia-reperfusion injury or reoxygenation injury, is the tissue damage caused when blood supply returns to tissue (re- + perfusion) after a period of ischemia or lack of oxygen (anoxia or hypoxia).

Ketorolac: Sold under the brand name Toradol, among others, is a non-steroidal anti-inflammatory drug used to treat pain. Specifically, it is recommended for moderate to severe pain. Common side effects include sleepiness, dizziness, abdominal pain, swelling, and nausea.

Methylprednisolone: Sold under the brand names Depo-Medrol and Solu-Medrol, among others, is a corticosteroid medication used to suppress the immune system and decrease inflammation.

Mindfulness-Based Stress Reduction: An 8-week evidence-based program that offers secular, intensive mindfulness training to assist people with stress, anxiety, depression, and pain.

Myelosuppression: A condition in which bone marrow activity is decreased, resulting in fewer red blood cells, white blood cells, and platelets. Myelosuppression is a side effect of some cancer treatments. When myelosuppression is severe, it is called myeloablation.

Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×

Obstructive Sleep Apnea: A sleep-related breathing disorder that involves a decrease or complete halt in airflow despite an ongoing effort to breathe. It occurs when the muscles relax during sleep, causing soft tissue in the back of the throat to collapse and block the upper airway.

Oophorectomy: A surgical procedure to remove one or both ovaries. When an oophorectomy involves removing both ovaries, it is called bilateral oophorectomy.

Pharmacogenomics: The study of the role of the genome in drug response. Its name reflects the combination of pharmacology and genomics. Pharmacogenomics analyzes how the genetic makeup of an individual affects his or her response to drugs.

Pica: A craving for something that is not normally regarded as nutritive, such as dirt, clay, paper, or chalk. Pica is a classic clue to iron deficiency in children, and it may also occur with zinc deficiency. Pica is also seen as a symptom in several neurobiological disorders, including autism and Tourette’s syndrome, and it is sometimes seen during pregnancy.

Pre-eclampsia: A condition in pregnancy characterized by high blood pressure, sometimes with fluid retention and proteinuria.

Priapism: A prolonged, unwanted erection of the penis. It is usually painful and not related to sexual stimulation or arousal.

Prolonged Exposure: A form of behavior therapy and cognitive behavioral therapy designed to treat posttraumatic stress disorder. It is characterized by two main treatment procedures—imaginal and in vivo exposures.

Renal Medullary Carcinoma: A rare type of cancer that affects the kidney. It tends to be aggressive, difficult to treat, and is often metastatic at the time of diagnosis.

Renal Osteodystrophy: A bone disease that occurs when the kidneys fail to maintain proper levels of calcium and phosphorus in the blood. It is common in people with kidney disease and affects most dialysis patients.

Rhabdomyolysis: A serious syndrome due to a direct or an indirect muscle injury. It results from the death of muscle fibers and release of their contents into the bloodstream. This can lead to serious complications such as renal (kidney) failure.

Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×

Sickle Cell Disease: A group of genetic blood disorders resulting from point mutations on the beta globin gene that produces in hemoglobin variants that alone or in combination cause early death and profound debilitation.

Sickle Cell Trait: Sickle cell trait is not the disease but occurs when an individual has inherited one sickle cell gene and has one unaffected beta globin gene.

Small Gestational Age for Infants: A term used to describe a baby who is smaller than the usual amount for the number of weeks of pregnancy. Small gestational age babies usually have birthweights below the 10th percentile for babies of the same gestational age.

Somatosensory: Relating to or denoting a sensation (e.g., pressure, pain, or warmth) that can occur anywhere in the body, in contrast to one localized at a sense organ (e.g., sight, balance, or taste).

Spirometry: The most common of the pulmonary function tests. It measures lung function, specifically the amount and/or speed of air that can be inhaled and exhaled. Spirometry is helpful in assessing breathing patterns that identify conditions such as asthma, pulmonary fibrosis, cystic fibrosis, and chronic obstructive pulmonary disease.

Splenic Infarction: A condition in which blood flow supply to the spleen is compromised, leading to partial or complete infarction (tissue death due to oxygen shortage) in the organ. Splenic infarction occurs when the splenic artery or one of its branches are occluded, for example, by a blood clot.

Spontaneous Epidural Hematoma: Manifests from blood accumulating in the epidural space, compressing the spinal cord and leading to acute neurological deficits.

Supraspinal: Means above the spine, and may refer to above the spinal cord and vertebral column or the brain.

Transcranial Doppler: A non-invasive ultrasound method used to examine the blood circulation within the brain.

Venous Thromboembolism: A blood clot that starts in a vein.

Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×
Page 491
Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×
Page 492
Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×
Page 493
Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×
Page 494
Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×
Page 495
Suggested Citation:"Appendix N: Glossary." National Academies of Sciences, Engineering, and Medicine. 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. doi: 10.17226/25632.
×
Page 496
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Sickle cell disease (SCD) is a genetic condition that affects approximately 100,000 people in the United States and millions more globally. Individuals with SCD endure the psychological and physiological toll of repetitive pain as well as side effects from the pain treatments they undergo. Some adults with SCD report reluctance to use health care services, unless as a last resort, due to the racism and discrimination they face in the health care system. Additionally, many aspects of SCD are inadequately studied, understood, and addressed.

Addressing Sickle Cell Disease examines the epidemiology, health outcomes, genetic implications, and societal factors associated with SCD and sickle cell trait (SCT). This report explores the current guidelines and best practices for the care of patients with SCD and recommends priorities for programs, policies, and research. It also discusses limitations and opportunities for developing national SCD patient registries and surveillance systems, barriers in the healthcare sector associated with SCD and SCT, and the role of patient advocacy and community engagement groups.

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