Sickle Cell Disease - Complications

Surfactants. Poloxamer 188 (Flocor, RheothRx) is an investigative synthetic compound known as a surfactant. It coats damaged blood cells, allowing them to slip over one another, thereby improving blood flow and oxygen delivery. Late clinical studies have been promising. A 2001 study reported that it reduced the duration of the crisis from 141 to 133 hours (which is still a long time). It was even more effective in children (reducing it to 21 hours) and in patients taking hydroxyurea (16 hours).

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Cordox. A natural sugar-based compound called fructose-1,6-diphosphate, FDP (Cordox) reduces inflammation and protects cells against the oxygen-depriving effects of sickling. This drug also is investigative. Studies are indicating that it relieves vaso-occlusive pain. In one study, taking only one dose reduced pain scores. It is not addictive and does not appear to have significant adverse effects.

Acute Chest Syndrome

Acute chest syndrome (ACS) occurs when the lungs are deprived of oxygen during a crisis. It can be very painful, dangerous, and even life threatening. It is a leading cause of illness among sickle cell patients and is the most common condition at the time of death. At least one whole segment of a lung is involved and the following symptoms may be present:

Fever of 101.3 F degrees (38.5 C) or above
Rapid or labored breathing
Wheezing or cough
Acute chest pain

Pain often lasts for several days. In about half of patients, severe pain develops about two and a half days before there are any signs of lung or chest abnormalities. Acute chest syndrome is often accompanied by infections in the lungs, which can be caused by viruses, bacteria, or fungi. Pneumonia is often present. A dull, aching pain usually follows, which most often ends after several weeks, although it may persist between crises.

Air is breathed in (inhaled) through the nasal passageways, andtravels through the trachea and bronchi to the lungs.

Causes of Acute Chest Syndrome. The two primary causes of acute chest syndrome are one or a combination of the following:

Infection. Infection from viruses or small atypical organisms (Chlamydia and Mycoplasma) is the most common causes of the oxygen deprivation that leads to acute chest syndrome.
Blockage of blood vessels. Blockage in the blood vessels (called infarction) that cuts off oxygen in the lungs is another important cause of acute chest syndrome. Blockage may be produced by blood clots or fat embolisms. (Fat embolisms are particles formed from fatty tissue in the bone marrow that enter and travel through the blood vessels.)