Bacterial infections in children are common and antibiotics and vaccines are given to prevent this complication. Eye examinations by an opthalmologist 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.

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

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 (10-50% of patients)
• gallstones
• infection, including pneumonia, cholecystitis (gallbladder), osteomylitis (bone), and urinary tract infection
• parvovirus B19 infection resulting in aplastic crisis
• papillary necrosis (tissue death) of the kidney
• loss of function of the spleen
• leg ulcers
• death

Call Your Health Care Provider If:

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

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