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Sickle Cell Anemia Treatment
Patients with sickle cell disease need certain treatment and
follow-up even when not having a painful crisis. Supplementation
with Folic acid, an essential element in producing cells,
is required because of the rapid red blood cell turnover.
Bacterial infections in children are common
and antibiotics and vaccines are given to prevent this complication.
Eye examinations by an ophthalmologist are important because
of the risk of damage to the retina.
Treatment for sickle cell disease usually
focuses on symptoms. While bone marrow transplant can be curative,
this therapy is indicated in only a minority of patients,
predominantly because of the high risk of the procedure and
difficulty in finding suitable donors. Therefore, the purpose
of therapy is to manage and control symptoms resulting from
crises and to try to limit the frequency of crises.
During a sickle crisis, certain therapies
may be necessary. Acute painful episodes are treated with
analgesics and adequate liquid intake. Treatment of pain with
adequate analgesics is critical.
Non-narcotic medications may be effective,
but some patients will require narcotics. Despite common belief
to the contrary, narcotic addiction is not more frequent in
patients with sickle cell disease than in other chronic pain
patients.
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Additional treatments include: antibiotics
for infection; partial exchange transfusion for acute chest
syndrome; potentially partial exchange transfusions or surgery
for neurological events, such as strokes, dialysis, or kidney
transplant for kidney disease, irrigation or surgery for priapism,
surgery for eye problems; hip replacement for avascular necrosis
of the hip (death of the joint); gallbladder removal (if there
is significant gallstone disease); wound care, zinc oxide,
or surgery for leg ulcers; drug rehabilitation and counseling
for the psychosocial complications.
Hydroxyurea (Hydrea) was found to help
some patients by reducing the frequency of painful crises
and episodes of acute chest syndrome and decreasing the need
for blood transfusions. There has been some concern about
the possibility of this drug causing leukemia, but as yet
there are no definitive data that Hydrea causes leukemia in
sickle cell patients.
Newer drugs are being developed to manage
sickle cell anemia. Some of these agents work by trying to
induce the body to produce more fetal hemoglobin (therefore
decreasing the amount of sickling) or by increasing the binding
of oxygen to sickle cells. But as yet, there are no other
widely used drugs that are available for treatment.
Bone marrow transplants are currently the
only potential cure for sickle cell anemia. In this treatment
the patient's bone marrow (which makes the sickled red blood
cells) is replaced with bone marrow from another individual
without sickle cell disease. However, it is difficult to find
the right bone marrow donor, and the drugs needed to make
the transplant possible are highly toxic.
Also, bone marrow transplants are much
more expensive than other treatments. Gene therapy (replacing
the Hemoglobin S with normal Hemoglobin A) may be the ideal
treatment, but it has proven to be very difficult in humans.
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 deoxygenating,
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
4. Be aware of the effects that chronic,
life-threatening illnesses can have on siblings, marital relationships,
parents, and the child
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