Blue Baby Syndrome

Despite ongoing experimentation and research, the illness known as Blue Baby Syndrome (BBS) remains a problem to be solved. Nevertheless, doctors have made great progress in treating BBS, and the discovery of the link between nitrates in drinking water and BBS was great headway in medical prevention. Further studies in underlying genetic causes will hopefully help to piece together this medical puzzle.

Definition

• Blue baby syndrome is an illness resulting in lack of oxygen in the bloodstream. While many babies are born with blue baby syndrome, it can also be acquired after birth.

  According to Bhia.com's helpful online article, BBS can also be caused by high blood levels of methemoglobin. In a normal adult, the enzyme methemoglobin is changed into hemoglobin, which carries oxygen into the bloodstream. However, babies under six months have not yet begun to produce methemoglobin reductase, which is the enzyme that allows methemoglobin to change into hemoglobin. This is dangerous, because methemoglobin carries no oxygen into the bloodstream. In BBS, a large amount of methomoglobin is received in the bloodstream, but the baby's small body cannot yet transform it into oxygen. This causes the baby to suffer from oxygen deprivation, which can often result in a bluish tint in the skin. The syndrome can also affect older children and adults, but since their systems can balance out the large amounts of methemoglobin, it is usually not a serious condition.

Symptoms

• Symptoms of BBS include bluish skin ("cyanosis"), diarrhea, vomiting and shortness of breath. Other symptoms include fatigue, extreme lethargy, poor weight gain, difficulty eating and a heart murmur.

Causes

• Although there are many theories about the cause of BBS, most cases are unexplained.

  As noted at iVillage.com's article on BBS, in the case of congenital defect, BBS is usually caused by a heart defect called "right-to-left shunt." This basically means that oxygen-poor blood is leaked either into the left side of the heart or directly into the bloodstream, causing a lack of oxygen throughout the baby's body. It can also be caused by various defects of the lungs and bloodstream.

  The role of genetics in BBS development is being investigated, as seen at the Howard Hughes Medical Institute's online article, "Homing in On a Cause of Blue Baby Syndrome." As of yet, there is no conclusive link to family history.

  Blue baby syndrome is also commonly linked to ingestion of nitrates. Nitrates react with hemoglobin and produce methomoglobin, and when too many nitrates are ingested, an excessively dangerous amount of methomoglobin is produced. In many rural areas, nitrates are found in drinking water, and methemoglobinemia is often linked to water contamination.

Treatment

• Depending on the severity of the particular case, treatment ranges from medication and careful monitoring to open heart surgery. In the case of congenital disorder, treatment often begins immediately after birth. Some of these surgeries include arterial switch operation, shunting procedure, Damus-Kaye-Stansel procedure and Fontan procedure. All of these procedures are invasive cardiac surgeries, and are often not required.

Prevention

• In the case of nitrate-associated BBS, preventive steps can be taken. As knowledge about blue baby syndrome increases, more safety precautions are being made. In the U.S., drinking water is carefully monitored to make sure that nitrate levels stay in check. Families with well water can also monitor water levels themselves with store-bought water safety kits.