Anemia - sickle cell; Hemoglobin SS disease (Hb SS) ( Part 2)

table of contents
  1. Anemia - sickle cell; Hemoglobin SS disease (Hb SS)
  2. Support Groups -  Expectations (prognosis)  - Complications -  Calling your health care provider - Prevention . 
  3.  
  4. Support Groups    
  5. Sickle cell anemia, like other chronic, life-threatening diseases, can cause great stress to the patient and family members. Joining a support group, where members share common experiences and problems, can relieve this stress. See sickle cell anemia - support group.
  6. Expectations (prognosis)    
  7. In the past, death from organ failure often occurred between the ages of 20 and 40 in most sickle-cell patients. More recently, because of better understanding and management of the disease, patients live into their forties and fifties.
  8. Causes of death include organ failure and infection. Some people with the disease experience minor, brief, and infrequent episodes. Others experience severe, prolonged, and frequent episodes resulting in many complications.
  9. Complications    
  10. recurrent aplastic and hemolytic crises resulting in anemia and gallstones
  11. multisystem disease (kidney, liver, lung)
  12. narcotic abuse
  13. splenic sequestration syndrome
  14. acute chest syndrome
  15. erectile dysfunction (as a result of priapism)
  16. blindness/visual impairment
  17. neurologic symptoms and stroke
  18. joint destruction
  19. gallstones
  20. infection, including pneumonia, cholecystitis (gallbladder), osteomyelitis (bone), and urinary tract infection
  21. parvovirus B19 infection resulting in aplastic crisis
  22. tissue death of the kidney
  23. loss of function of the spleen
  24. leg ulcers
  25. death
  26. Calling your health care provider    
  27. Call your health care provider if acute painful crises occur or at the first sign of any infection.
  28. Prevention    
  29. Sickle cell anemia can only result when two carriers with sickle cell trait have a child together. Therefore, genetic counseling is recommended for all carriers of sickle cell trait (about 1 in 12 African Americans has sickle cell trait ).
  30. Prenatal diagnosis of sickle cell anemia is also available. Prompt treatment of infections, adequate oxygenation, and preventing dehydration may prevent sickling of red blood cells. Antibiotics and vaccinations may prevent infections.
  31. General health visits with a physician are recommended to ensure the patient is getting adequate nutrition, maintaining proper activity levels, and receiving proper vaccinations.
  32. PREVENTING CRISES
  33. Parents whose children have sickle cell should encourage their children to lead normal lives. However, in order to decrease the occurrence of sickle cell crises, consider the following precautions:
  34. To prevent tissue deoxygenation, avoid the following:
  35. Strenuous physical activity, especially if the spleen is enlarged
  36. Emotional stress
  37. Environments with low oxygen content (high altitudes, non-pressurized airplane flights)
  38. Known sources of infection
  39. To promote proper hydration:
  40. Recognize signs of dehydration
  41. Avoid excess exposure to the sun
  42. Provide access to fluids, both at home and away
  43. To avoid sources of infection:
  44. Keep child properly immunized as recommended by the health care provider
  45. Consider having the child wear a Medic Alert Bracelet
  46. Share above information with teachers and other caretakers as appropriate
  47. Be aware of the effects that chronic, life-threatening illnesses can have on siblings, marital relationships, parents, and the child
  48.  
  49.   Read more :Anemia - iron deficiency

Anemia - sickle cell; Hemoglobin SS disease (Hb SS)


Support GroupsExpectations (prognosis)  - Complications -  Calling your health care provider - Prevention . 

 

Support Groups    

Sickle cell anemia, like other chronic, life-threatening diseases, can cause great stress to the patient and family members. Joining a support group, where members share common experiences and problems, can relieve this stress. See sickle cell anemia - support group.

Expectations (prognosis)    

In the past, death from organ failure often occurred between the ages of 20 and 40 in most sickle-cell patients. More recently, because of better understanding and management of the disease, patients live into their forties and fifties.

Causes of death include organ failure and infection. Some people with the disease experience minor, brief, and infrequent episodes. Others experience severe, prolonged, and frequent episodes resulting in many complications.

Complications    

  • recurrent aplastic and hemolytic crises resulting in anemia and gallstones

  • multisystem disease (kidney, liver, lung)

  • narcotic abuse

  • splenic sequestration syndrome

  • acute chest syndrome

  • erectile dysfunction (as a result of priapism)

  • blindness/visual impairment

  • neurologic symptoms and stroke

  • joint destruction

  • gallstones

  • infection, including pneumonia, cholecystitis (gallbladder), osteomyelitis (bone), and urinary tract infection

  • parvovirus B19 infection resulting in aplastic crisis

  • tissue death of the kidney

  • loss of function of the spleen

  • leg ulcers

  • death

Calling your health care provider    

Call your health care provider if acute painful crises occur or at the first sign of any infection.

Prevention    

Sickle cell anemia can only result when two carriers with sickle cell trait have a child together. Therefore, genetic counseling is recommended for all carriers of sickle cell trait (about 1 in 12 African Americans has sickle cell trait ).

Prenatal diagnosis of sickle cell anemia is also available. Prompt treatment of infections, adequate oxygenation, and preventing dehydration may prevent sickling of red blood cells. Antibiotics and vaccinations may prevent infections.

General health visits with a physician are recommended to ensure the patient is getting adequate nutrition, maintaining proper activity levels, and receiving proper vaccinations.

PREVENTING CRISES

Parents whose children have sickle cell should encourage their children to lead normal lives. However, in order to decrease the occurrence of sickle cell crises, consider the following precautions:

To prevent tissue deoxygenation, avoid the following:

  1. Strenuous physical activity, especially if the spleen is enlarged

  2. Emotional stress

  3. Environments with low oxygen content (high altitudes, non-pressurized airplane flights)

  4. Known sources of infection

To promote proper hydration:

  1. Recognize signs of dehydration

  2. Avoid excess exposure to the sun

  3. Provide access to fluids, both at home and away

To avoid sources of infection:

  1. Keep child properly immunized as recommended by the health care provider

  2. Consider having the child wear a Medic Alert Bracelet

  3. Share above information with teachers and other caretakers as appropriate

Be aware of the effects that chronic, life-threatening illnesses can have on siblings, marital relationships, parents, and the child

 

 




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