Anemia - sickle cell; Hemoglobin SS disease (Hb SS) ( Part 2)
Ehab Ali Maher
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Anemia - sickle cell; Hemoglobin SS disease
(Hb SS)
Support Groups - Expectations (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:
Strenuous
physical activity, especially if the spleen is enlarged
Emotional
stress
Environments
with low oxygen content (high altitudes, non-pressurized airplane flights)
Known
sources of infection
To promote
proper hydration:
Recognize
signs of dehydration
Avoid
excess exposure to the sun
Provide
access to fluids, both at home and away
To avoid sources
of infection:
Keep
child properly immunized as recommended by the health care provider
Consider
having the child wear a Medic Alert Bracelet
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