Sickle cell anemia, also known as Sickle cell disease (SCD) is a hereditary disorder, affecting the red blood cells (RBCs). RBCs contain hemoglobin, which is an iron-loaded pigment of blood that carries oxygen to various cells, tissues and organs.
\nNormally RBCs are disc-shaped and flexible, thus easily passing through blood vessels, ensuring adequate blood supply to all the organs of the body. Red blood cells undergo constant breakdown and production. Healthy RBCs have a life-span of 120 days, i.e., they rupture every 120 days.<\/p>\n
In sickle cell anemia, inheritance of hemoglobin S gene from both the parent, makes the RBCs attain a sickle or crescent shape. Sickle-shaped RBCs are stiff and inflexible. Therefore, these deformed RBCs do not pass easily through blood vessels and hence, the blood supply of organs are compromised. Reduced blood supply to organs can lead to complications like stroke<\/strong><\/a> , gallstones, blindness, and even organ damage.<\/p>\n Also, these abnormal RBCs have a very short life-span of just 10-20 days, thus rupturing very frequently. While these RBCs rupture, the body is not able to compensate for the blood loss, thus creating a constant state of RBC deprivation, medically known as anemia. A reduction in number of RBCs and an inability of blood to reach organs adequately, lead to various symptoms. These symptoms are described below.<\/p>\n However, a notable fact is that SCD is not similar to sickle cell trait. Sickle cell trait occurs when a person inherits a single defective gene, from either parent. Such individuals are healthy and have no symptoms.<\/p>\n Sickle cell disease has only one cure, i.e., bone marrow transplant, which is also known as stem cell transplant. This process involves infusing stem cells from a related healthy donor to the affected individual. Stem cells have a unique ability to transform into any type of body cell. Infusing these cells produce newer healthy RBCs, thus offering complete remission from SCD. However, this procedure has several fatal complications, including death. Therefore, this is usually not indicated.<\/p>\n Hence, the aim of treatment of SCD is to reduce the frequency of sickle cell crisis and to prolong the life of affected people by preventing the complications of SCD. Medications and blood transfusion form the mainstay of treatment of SCD.<\/p>\n Chief medicines for sickle cell anemia include non-steroidal and opioid pain-killers to treat painful episodes and hydroxyurea to stimulate production of normal hemoglobin. Severe anemia in SCD patients may require blood transfusion. Blood transfusion involves transfusing healthy blood in the body of affected individual. This reduces the amount of sickled red blood cells, thus preventing some fatal complications.<\/p>\n Chief drugs used in the treatment of sickle cell disease are as follows.<\/p>\n Pain and inflammation in our body is caused by a compound, known as prostaglandin. This compound is produced by an enzyme, called cyclo-oxygenase (COX) enzyme. NSAIDs block the COX enzyme, thus blocking the synthesis of prostaglandins. Available as:<\/strong> Oral preparations<\/p>\n I. Aspirin<\/strong><\/p>\n US-FDA Status:<\/strong> Approved for sickle cell disease II. Acetaminophen<\/strong><\/p>\n US-FDA Status:<\/strong> Approved for sickle cell disease III. Ibuprofen<\/strong><\/p>\n US-FDA Status:<\/strong> Approved for sickle cell disease IV. Diclofenac<\/strong><\/p>\n US-FDA Status:<\/strong> Approved for sickle cell disease However, due to the potential side-effects of NSAIDs including kidney damage, liver damage, etc., lowest effective dose should be given along with constant monitoring of patients. [2]<\/strong><\/a><\/sup> Opioid analgesics form the cornerstone of treatment of moderate to severe pain. Pain is experienced when the brain receives signals to perceive pain. Certain receptors namely, mu, kappa and delta, present on the brain, spinal cord and other nervous tissue facilitate this process of pain perception. Available as:<\/strong> Oral preparations, Injectable preparations and Transdermal patch<\/p>\n I. Morphine<\/strong><\/p>\n US-FDA Status:<\/strong> Approved for sickle cell disease II. Codeine<\/strong><\/p>\n US-FDA Status:<\/strong> Approved for sickle cell disease III. Oxycodone<\/strong><\/p>\n US-FDA Status:<\/strong> Approved for sickle cell diseaseSickle-Cell-Anemia-and-Its-Most-Common-Drug-List IV. Methadone<\/strong><\/p>\n US-FDA Status:<\/strong> Approved for sickle cell disease V. Fentanyl<\/strong><\/p>\n US-FDA Status:<\/strong> Approved for sickle cell disease It belongs to a drug class, known as \u201cantimetabolite\u201d. This drug activates an enzyme, called guanylyl cyclase, which stimulates the production of fetal hemoglobin (HbF). Treatment of SCD with hydroxyurea is aimed at reproducing healthy fetal hemoglobin and reduce the production of abnormal hemoglobin S, thus reducing the frequency of sickle cell crisis.<\/p>\n Available as:<\/strong> Oral preparations<\/p>\n US-FDA Status:<\/strong> Approved for sickle cell disease OTC<\/strong> <\/p>\n Sickle cell disease is a life-threatening condition. Also, the medications described above have some serious side-effects. Therefore, self-medication is strictly prohibited. Consult a medical professional for effective treatment of the condition.<\/p>\n","protected":false},"excerpt":{"rendered":" Sickle cell anemia, also known as Sickle cell disease (SCD) is a hereditary disorder, affecting the red blood cells (RBCs). RBCs contain hemoglobin, which is an iron-loaded pigment of blood that carries oxygen to various cells, tissues and organs. Normally RBCs are disc-shaped and flexible, thus easily passing through blood vessels, ensuring adequate blood supply […]<\/p>\n","protected":false},"author":18,"featured_media":33815,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"tdm_status":"","tdm_grid_status":"","footnotes":""},"categories":[18941],"tags":[28277],"class_list":{"0":"post-26389","1":"post","2":"type-post","3":"status-publish","4":"format-standard","5":"has-post-thumbnail","7":"category-drug-by-condition","8":"tag-sickle-cell-anemia"},"_links":{"self":[{"href":"https:\/\/zovon.com\/index.php?rest_route=\/wp\/v2\/posts\/26389","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/zovon.com\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/zovon.com\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/zovon.com\/index.php?rest_route=\/wp\/v2\/users\/18"}],"replies":[{"embeddable":true,"href":"https:\/\/zovon.com\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=26389"}],"version-history":[{"count":0,"href":"https:\/\/zovon.com\/index.php?rest_route=\/wp\/v2\/posts\/26389\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/zovon.com\/index.php?rest_route=\/wp\/v2\/media\/33815"}],"wp:attachment":[{"href":"https:\/\/zovon.com\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=26389"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/zovon.com\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=26389"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/zovon.com\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=26389"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}
\nSickle cell anemia is more common in blacks and in areas with endemic malaria. It is marked by painful episodes, known as \u201csickle cell crisis\u201d.
\nAlthough it is a fatal condition, the quality of life can be improved with the help of medical interventions, including medications and blood transfusion.<\/p>\nSymptoms<\/h2>\n
\n
Treatment<\/h2>\n
\nNutritional supplements, including folate and glutamate may also be given to treat anemia. As per a research paper, first sign of SCD is reduced functioning of spleen, which plays a vital role in fighting against infection. Therefore, it is recommended that children below 5 years of age should be vaccinated for infections, like pneumococci, flu, etc. [1]<\/strong><\/a><\/sup><\/p>\n1. Non- Steroidal Anti-inflammatory Drugs (NSAIDs)<\/h2>\n
\nBy doing so, NSAIDs provide significant reduction in inflammation and pain during sickle cell crisis.<\/p>\n
\nAvailability:<\/strong> Over-the-counter
\nGeneric name:<\/strong> aspirin\/ acetaminophen\/ caffeine
\nBrand name:<\/strong> Excedrin tablet
\nSide-effects:<\/strong> Hives, itching, rash, upset stomach, coughing up blood, black,red or tarry stools, blood in urine
\nIndicated in pregnancy:<\/strong> Absolutely contraindicated in third trimester as it may affect the fetal cardiovascular system
\nAlcohol content:<\/strong> Absent
\nCaution:<\/strong> Do not use in children and teens below 20 years of age as it may cause Reye syndrome (brain and liver damage)<\/p>\n
\nAvailability:<\/strong> Over-the-counter
\nGeneric name:<\/strong> acetaminophen
\nBrand name:<\/strong> Tylenol tablet
\nSide-effects:<\/strong> Bloody or cloudy urine, black or tarry stools, hives, skin rash, sore fatigue
\nIndicated in pregnancy:<\/strong> Only if absolutely indicated
\nAlcohol content:<\/strong> Absent<\/p>\n
\nAvailability:<\/strong> Over-the-counter
\nGeneric name:<\/strong> ibuprofen
\nBrand name:<\/strong> Motrin IB tablet
\nSide-effects:<\/strong> Bloating, belching, acidity, cloudy urine, diarrhea, heartburn, decreased amount of urine
\nIndicated in pregnancy:<\/strong> Absolutely contraindicated in third trimester as it may affect the fetal cardiovascular system
\nAlcohol content:<\/strong> Absent<\/p>\n
\nAvailability<\/strong>: Prescription only
\nGeneric name:<\/strong> diclofenac
\nBrand name:<\/strong> Zorvolex capsule
\nSide-effects:<\/strong> Bloating, cloudy urine, belching, constipation, dizziness
\nIndicated in pregnancy:<\/strong> Absolutely contraindicated in third trimester as it may affect the fetal cardiovascular system
\nAlcohol content:<\/strong> Absent<\/p>\n
\nNSAIDs are also used in the treatment of other painful conditions, like tendinitis<\/strong><\/a>, abdominal migraine<\/strong><\/a> , etc.<\/p>\n2. Opioid Analgesics<\/h2>\n
\nOpioid analgesics bind to these receptors and block the pain perception, thus relieving the pain in sickle cell crisis.<\/p>\n
\nAvailability:<\/strong> Prescription only
\nGeneric name:<\/strong> morphine sulfate
\nBrand name:<\/strong> Kadian capsule; Duramorph PF injection
\nSide-effects:<\/strong> Nausea, vomiting, dizziness, abdominal pain, blurred vision, cough, confusion
\nIndicated in pregnancy:<\/strong> Only if benefit outweighs fetal risk
\nAlcohol content:<\/strong> Absent<\/p>\n
\nAvailability:<\/strong> Prescription only
\nGeneric name<\/strong>: acetaminophen and codeine phosphate
\nBrand name:<\/strong> Tylenol w\/Codeine No.3 tablet
\nSide-effects:<\/strong> Drowsiness, dizziness, lightheadedness, nausea, shortness of breath, vomiting, abdominal pain
\nIndicated in pregnancy:<\/strong> Only if benefit outweighs fetal risk
\nAlcohol content:<\/strong> Absent<\/p>\n
\nAvailability:<\/strong> Prescription only
\nGeneric name:<\/strong> oxycodone hydrochloride and acetaminophen
\nBrand name:<\/strong> Percocet tablet
\nSide-effects:<\/strong> Abdominal pain, dark urine, chills, black tarry stools, itching, headache, nausea
\nIndicated in pregnancy:<\/strong> Only if benefit outweighs fetal risk
\nAlcohol content:<\/strong> Absent<\/p>\n
\nAvailability:<\/strong> Prescription only
\nGeneric name:<\/strong> methadone hydrochloride
\nBrand name:<\/strong> Methadose tablet
\nSide-effects:<\/strong> Bleeding gums, black tarry stools, blurred vision, blood in urine or stools, headache
\nIndicated in pregnancy:<\/strong> Only if benefit outweighs fetal risk
\nAlcohol content:<\/strong> Absent<\/p>\n
\nAvailability:<\/strong> Prescription only
\nGeneric name:<\/strong> fentanyl
\nBrand name:<\/strong> Duragesic transdermal patch
\nSide-effects:<\/strong> Chest pain, black tarry stools, blurred vision, cough, confusion, convulsions, dizziness
\nIndicated in pregnancy:<\/strong> Only if benefit outweighs fetal risk
\nAlcohol content:<\/strong> Absent<\/p>\n3. Hydroxyurea<\/h2>\n
\nNormally, fetal hemoglobin is produced in the developing baby and is replaced by adult hemoglobin within first 6 months of the infant. However, in children with sickle cell disease, the adult hemoglobin is defective hemoglobin S, which causes the RBCs to become sickle-shaped.<\/p>\n
\nAvailability:<\/strong> Prescription only
\nGeneric name:<\/strong> hydroxyurea
\nBrand name:<\/strong> Droxia capsule
\nSide-effects:<\/strong> Chills, cough, fever, back pain, hoarseness, blood in urine, black tarry stools, mouth sores
\nIndicated in pregnancy:<\/strong> Only if absolutely indicated
\nAlcohol content:<\/strong> Absent<\/p>\n
\nOf the medications listed above, some pain-killers are available as over-the-counter drugs in certain countries. These are summarized below.<\/p>\n\n\n
\n Name of the Country<\/strong><\/td>\n OTC Drug(s) Available<\/strong><\/td>\n<\/tr>\n \n Argentina<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Australia<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Austria<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n Canada<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n China<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Croatia<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n Czech Republic<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Denmark<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n France<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n Germany<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Greece<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Hungary<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n India<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n Ireland<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n Italy<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Japan<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n Mexico<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Philippines<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n Russia<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Singapore<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n South Africa<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n South Korea<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Spain<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n Switzerland<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n The Netherlands<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)
\nDiclofenac (oral)<\/td>\n<\/tr>\n\n UK<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n\n USA<\/td>\n Acetylsalicylic acid (oral)
\nAcetaminophen (oral)
\nIbuprofen (oral)<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n