According to the article “Managing Sickle Cell Disease”, sickle cell disease is an inherited disorder affecting the red blood cells. The disease causes the red blood cells to become rigid and half-moon shaped, resulting in obstruction of capillary blood flow. Decreased oxygen to the tissues results in pain. The disease is inherited and has the greatest prevalence among Black Americans and Hispanics in the United States. Inheritance is autosomal recessive and results in production of sickle hemoglobin S instead of hemoglobin A. Two copies of the hemoglobin S gene results in the most prevalent type of sickle cell disease, Hemoglobin SS. This type also has the shortest life span. The second type is Hemoglobin SC in which there is one copy of
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Pain management is crucial. Effective medications include over the counter analgesics, ketorolac, opioids and hydroxyurea. Application of heat is also beneficial during a crisis, but patients should avoid extreme temperatures due to the potential for vasoconstriction. Patients should be taught proper nutrition, the benefits of adequate hydration, signs and symptoms of infection, and when to obtain medical care. Coping mechanisms should be discussed with patients to assist them in dealing with their chronic pain. Patients should be screed for anxiety and depression. Additionally, nurses can assist patients by providing information on support groups and educating patients on appropriate treatment and current …show more content…
The only curative treatment for sickle cell disease is bone marrow transplant. According the article “Current Treatments and Prospective Therapies to Manage Sickle Cell Disease” there are several gene therapies being studied to cure sickle cell disease. One therapy, called Gene Addition Therapy involves the transfer of a normal β-globulin gene into hematopoietic cells. This transfer would prevent the RBCs from sickling by replacing the altered gene with a therapeutic gene. The hematopoietic cells are then transplanted into the patient. An additional gene therapy approach would be insertion of a normal copy of the gene by recombination before transplantation. This repair introduces DNA and RNA into stem cells to correct the mutation. A clinical trial, currently in progress, is the modification of the β-globulin gene with an anti-sickling gene in the laboratory. This gene is then transplanted back into the patient’s bone marrow. If successful the patient’s bone marrow will produce normal hemoglobin. To correct the inherited genetic mutation, there are currently studies utilizing the use of induced pluripotent stem cells (iPSCs) derived from adult somatic cells. Interestingly, pluripotent stem cells originate from skin fibroblasts and can differentiate into most cell types. Once differentiated into iPSCs and the genetic mutation is corrected the cells are
Pain management is crucial. Effective medications include over the counter analgesics, ketorolac, opioids and hydroxyurea. Application of heat is also beneficial during a crisis, but patients should avoid extreme temperatures due to the potential for vasoconstriction. Patients should be taught proper nutrition, the benefits of adequate hydration, signs and symptoms of infection, and when to obtain medical care. Coping mechanisms should be discussed with patients to assist them in dealing with their chronic pain. Patients should be screed for anxiety and depression. Additionally, nurses can assist patients by providing information on support groups and educating patients on appropriate treatment and current …show more content…
The only curative treatment for sickle cell disease is bone marrow transplant. According the article “Current Treatments and Prospective Therapies to Manage Sickle Cell Disease” there are several gene therapies being studied to cure sickle cell disease. One therapy, called Gene Addition Therapy involves the transfer of a normal β-globulin gene into hematopoietic cells. This transfer would prevent the RBCs from sickling by replacing the altered gene with a therapeutic gene. The hematopoietic cells are then transplanted into the patient. An additional gene therapy approach would be insertion of a normal copy of the gene by recombination before transplantation. This repair introduces DNA and RNA into stem cells to correct the mutation. A clinical trial, currently in progress, is the modification of the β-globulin gene with an anti-sickling gene in the laboratory. This gene is then transplanted back into the patient’s bone marrow. If successful the patient’s bone marrow will produce normal hemoglobin. To correct the inherited genetic mutation, there are currently studies utilizing the use of induced pluripotent stem cells (iPSCs) derived from adult somatic cells. Interestingly, pluripotent stem cells originate from skin fibroblasts and can differentiate into most cell types. Once differentiated into iPSCs and the genetic mutation is corrected the cells are