Spleen-related complications in children with Hemoglobin SC
are uncommon, but they do occur and can be life-threatening. The condition of
most concern is acute splenic sequestration crisis (ASSC) which is more common
in children with sickle cell disease (HgbSS), but can occur in those with Hgb
SC. ASSC is when red blood cells become trapped in the spleen, causing severe
spleen enlargement and progressive destruction of more red blood cells
resulting in life-threatening anemia. Doctors can't predict which patients will
have ASSC, but they do look for warning signs that suggest a person is at
higher risk than usual. Doctors may recommend prophylactic splenectomy to avoid
the risks of developing ASSC, but more often for patients with Hgb SS than for
those with Hgb SC. Warning signs of ASSC include fever,
respiratory tract infection, fatigue, thirst, acute abdominal pain, low blood
pressure, rapid heart rate, and rapid breathing.
Children without a spleen do not necessarily get
more infections than those with a spleen, but infections that occur can be much
more serious than in the average individual. Most serious infections occur
within the first two years of surgery, and those with an underlying immune
problem before splenectomy are at higher risk of complications compared to
those with intact immune systems. Children undergoing splenectomy will need
additional vaccinations and should strictly adhere to the standard immunization
schedule. They will also need to take antibiotics regularly to prevent
infection (at least the first 2 years after surgery, but possibly until age
21). Patients and their parents must be especially alert to signs of infection,
and children without a spleen will be treated more cautiously than others
during common childhood illnesses.
Whether you decide to proceed with the surgery is a
personal decision you should discuss at length with your physician.