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Sickle_Cell_Ulcers.pptx

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Sickle_Cell_Ulcers.pptx

  1. 1. SICKLE CELL ULCERS
  2. 2. What is Sickle Cell?  People who have Sickle Cell have sickle shaped red blood cells, which causes complications because the blood cells are not able to reach certain parts of the body.
  3. 3. Red blood cells Going through Vessels
  4. 4. SymptomsofSickleCell
  5. 5. Diseases and Conditions people with Sickle Cell are likely to develop:  Acute chest syndrome  Dactylitis – swelling of the hands and feet  Painful crises: really painful episodes when blood cells are blocked from going to certain parts of the body – pain can occur anywhere, but it is usually in the chest, arms, and legs  Enlarged spleen – sickle cells pool in the spleen, and in some cases there is no spleen in the body.  Stroke  Hematuria An x-ray of a hand swollen from dactylitis An enlarged and unhealthy spleen from someone with Sickle Cell
  6. 6. Dactylitis
  7. 7. TheHistory of SickleCell  Many people of African tribes were the first to develop sickle cell disease  Most of the names they had for it had multiple syllables – maybe to account for the many painful episodes  The tribes thought that there were evil spirits and the babies that died from sickle cell were sacrificing their lives for the family
  8. 8. MORE HISTORY… •1846: In the US, a scientist wrote a paper called “Case of Absence of the Spleen,” which talked about a runaway slave whose body was autopsied and they found he did not have a spleen. •1904: A hospital intern discovered the sickle shaped cells in a patient at a hospital. •1910: The first formal report came out that gave details about the strange disease •1922: The disease was finally named “Sickle Cell”
  9. 9. Prevalence o It is estimated the up to 80,000 people inAmerica have Sickle Cell Disease o 1/500 AfricanAmericans have Sickle Cell Disease o 1/1000 -4000 Hispanics have Sickle Cell o 1/12 African Americans are carriers for Sickle Cell o 1/50 Asians are carriers for Sickle Cell o 1/100 Greeks are carriers for Sickle Cell
  10. 10. People can live a relatively normal life with Sickle Cell if they have the proper treatment. The average life expectancy for males is 42. The average life expectancy for females is 48. In 2003 the oldest patient to have Sickle cell was an 85 year old Jamaican woman.
  11. 11. Sickle cell disease (SCD)  Sickle cell disease (SCD) is an inherited blood disorder that causes the bone marrow to produce red blood cells with defective hemoglobin, hemoglobin S (sickled hemoglobin)
  12. 12. Sickle Cell ulcers  Leg ulcers are the most common cutaneous manifestation of SCD.  1These ulcers are characterized by an indolent, intractable course, typically healing up to 16 times slower than venous ulcers.  2 A patient who experiences his or her first sickle cell ulcer is likely to ulcerate again: Approximately 97% of healed sickle cell ulcers will recur in less than 1 year.  3 Due to the recalcitrant nature of these ulcers, patients may experience significant disfigurement, social isolation, and loss of income.
  13. 13. incidence  The incidence of leg ulcers in patients with SCD ranges from 25.7% to 75%.
  14. 14. Risk factors  Risk factors for the development of ulcers include being older than 20 years,  having a lower level of fetal hemoglobin and a hemoglobin level less than 6 g/dL,  having antithrombin III deficiency,  possessing certain human leukocyte antigens (HLA),  having thrombocytosis,  living in certain geographic areas.  History of a sickle cell leg ulcer
  15. 15. Pathogenesis  Although the pathogenesis of leg ulcers in SCD has not been completely elucidated, several theories have been offered to explain this phenomenon. In sickle cell disease, the amino acid valine is substituted for glutamic acid in the beta-hemoglobin chain of hemoglobin, leading to the polymerization of hemoglobin when hemoglobin is deoxygenated. This results in the sickling, or misshaping, of red blood cells Sickled red blood cells are rigid and inflexible, which allows them to become lodged within smaller blood vessels and causes vascular obstruction. Once obstruction occurs, ischemia ensues and tissue necrosis results.
  16. 16. Diagnosis  Sickle cell ulcers normally occur in areas with less subcutaneous fat,  thin skin, and  decreased blood flow.  Common sites include the anterior tibial area, dorsum of the foot, Achilles tendon area, and ankles, with the medial malleolus being more affected than the lateral malleolus .  Sickle cell ulcers classically appear as round, punchcd-out ulcers with raised margins, deep bases, and necrotic slough .Surrounding brown hyper- pigmentation and scaling.
  17. 17. Diagnosis Cont,,,,,,,,,  Multiple ulcers or scarring from healed ulcers may be found in the vicinity.  Patients usually complain of extreme tenderness or pain at the site of the ulcer.  This is because sickled cells lead to tissue death, which is painful.  In essence, sickle cell ulcers represent a type of ischemie ulcer.
  18. 18. Diagnosis Cont,,,,,,,,,  A biopsy of the ulcer is nonspecific; however, sickled red blood cells may be visualized within the dermal blood vessels.  Laboratory analysis for patients with suspected sickle cell ulcers includes peripheral smear to visualize the pathognomonic sickled red blood cells,  a complete blood count to determine the level of anemia, and hemoglobin electrophoresis to measure the levels of hemoglobinA, S, and F .Several types of hemoglobin exist, with hemoglobinA being the normal variant. Hemoglobin F is the fetal hemoglobin, which is fairly resistant to sickling.
  19. 19. Treatment  Therapy for sickle cell leg ulcers encompasses 3 broad areas:  prevention,  treatment of existing ulcers,  and treatment of infection.  Because trauma is an inciting factor in these ulcers, simple measures-such as wearing properly fitting shoes, using insect repellent, and promptly treating any minor trauma-are essential components of prevention.
  20. 20. Treatment cont  Given the risk of concurrent venous or lymphatic disease, the use of support stockings, leg elevation, and sodium restriction may aid in decreasing edema and preventing ulcer formation. Other important prevention strategies include proper hygiene and liberal use of emollients to reduce drying and cracking of the skin.
  21. 21.  Treatment options for sickle cell leg ulcers can be divided into 4 categories:  topical treatments,  dressings,  surgical interventions,  and systemic medications
  22. 22. topical  topical triple antibiotic ointments have been shown to heal sickle cell ulcers and treat concurrent infections
  23. 23. Dressings  A variety of dressings have been used to heal sickle cell ulcers.The use of an Unna's boot, a zinc-oxide impregnated dressing, controlled edema in patients with sickle cell ulcers and led to subsequently faster healing.5'12 Dressings may be used in conjunction with elastic compression bandages for controlling edema and have the advantage of requiring weekly or twice weekly dressing changes, minimizing cutaneous trauma
  24. 24.  a hydrocolloid dressing that protects newly forming epithelium while preventing infection, has also been used alone and in conjunction with an Unna's boot or compression stockings to aid in the healing of leg ulcers in SCD
  25. 25. Surgical interventions  Surgical interventions for sickle cell ulcers include debridement and the use of myocutaneous flaps and split-thickness skin grafts. Debridement with dressings or debriding ointments enables the removal of necrotic slough.
  26. 26. Systemic medications  Pentoxifylline is believed to work by decreasing the sickling of red blood cells, increasing erythrocyte deformability, increasing leukocyte flexibility, inhibiting platelet aggregation, reducing blood viscosity, and decreasing plasma fibrinogen levels.
  27. 27. Penicillin Persons with sickle cell anemia have an increased susceptibility to infection d Penicillin  Persons with sickle cell anemia have an increased susceptibility to infection due to damage to one of our major defenses against infection, the spleen, due to restricted blood flow.  Penicillin is an antibiotic which would attack these infections helping in quick healing and protection.  Folic acid, which aids in the formation of healthy blood cells could also be taken daily in large quantities.
  28. 28. Hydroxyurea  Hydroxyurea was the first effective drug treatment for adults with severe sickle cell anemia.  A study showed that daily doses of the anticancer drug hydroxyurea reduced the frequency of painful crises and acute chest syndrome in sickle cell anemia. In addition, patients taking the drug needed fewer blood transfusions.
  29. 29. Blood Transfusions Effectiveness Blood transfusion is currently the single most effective and proven treatment for severe complications of sickle cell disease. It also significantly reduces strokes. Age Blood transfusions are mostly administered to children who have severe cases of sickle cell anemia. However, persons of all age can receive this treatment.
  30. 30. Blood and Marrow Stem Cell Transplantation Bone marrow transplants are the closest things possible to a cure for sickle cell anemia. Process  In bone marrow transplantation, the affected person’s bone marrow is replaced with cells containing genes for the non-sickle cell hemoglobin.  Production of healthy red blood cells starts and the sickle shaped cells are prevented. If the transplant is performed when the affected person is still young then success rates can be as high as 90-95%.(Sickle Cell Society 2005)
  31. 31. Gene Therapy Definition Gene therapy is the relatively new idea of inserting genes into the cells of an individual’s tissues and cells in order to treat a hereditary disease, such as sickle cell anemia, in which a defective mutant allele is replaced with a functional one. Gene Therapy would be the best cure for sickle cell anemia, but work in this method of treatment is still very new.
  32. 32. Thanks

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