She knew at once

She knew at once

In Brief

When Bloom recalls the death of his infant son in Calypso, he twice thinks that the experienced midwife ("Lots of babies she must have helped into the world") could tell at once that the infant would not survive: "She knew from the first poor little Rudy wouldn't live. Well, God is good, sir. She knew at once." How, one wonders, could she have known that? And can readers share in her omniscience by learning what killed Rudy? Two chapters later, the novel offers a clue to the first question: Rudy's skin looked purple. With no diagnostic testing or autopsy having been performed, the second question cannot be answered definitively. But the addition of another clue—in Ithaca, we learn that Rudy died eleven days after birth—suggests that he may have died of a congenital cardiac defect known as Hypoplastic Left Heart Syndrome (HLHS).

Read More

HLHS is characterized by a severely underdeveloped left ventricle and ascending aorta, and by the presence of a septal defect allowing blood to flow between the two atrial chambers. In a normal heart, blood that has flowed from the right ventricle to the lungs to take in oxygen returns to the left atrium and moves from there to the left ventricle, which pumps it through the aorta to the rest of the body. In the abnormal development of HLHS the left ventricle is too small to pump oxygenated blood to the body (in the worst cases, only a slit), and the ascending aorta is likewise diminutive (in the worst cases, only a thread).

With its normal passage blocked, the oxygenated blood in the left atrium passes through the atrial septal defect always found in cases of HLHS into the right atrium and thence into the right ventricle, where it mixes with the oxygen-poor blood that the veins have brought back to the heart from the body. The pulmonary artery carries some of this mixed blood (shown as purple in the illustrations) to the lungs, but some of it passes to the rest of the body via the ductus arteriosus, a fetal blood vessel connecting the pulmonary artery and the aorta that, in utero, lets most of the blood bypass the fluid-filled, not-yet-functioning lungs of the fetus. In healthy babies the ductus arteriosus closes shortly after birth.

As long as the fetus with a hypoplastic left ventricle remains in the womb it is sustained by the oxygen it receives from the mother's blood. As soon as the umbilical connection is severed, however, the baby must depend solely on the poorly oxygenated blood pumped from its right ventricle. Oxygen saturation plummets to about 85%, resulting in marked cyanosis (purplish skin). The decreased oxygenation delays the closure of the ductus arteriosus, allowing adequate blood flow to the body for a short while, but eventually this vessel does close—typically in about 11 days. Blood flow is greatly reduced, and death follows quickly. Without treatment, HLHS is universally fatal.

The precise birth and death dates detailed in Ithaca provide one indication that Rudy may have died from HLHS or some other "ductal-dependent lesion" revealed by the closing of the ductus: "birth on 29 December 1893 of second (and only male) issue, deceased 9 January 1894, aged 11 days." Another clue comes in Hades when Bloom sees a child's tiny coffin and is reminded of his little boy's cyanotic appearance: "A dwarf's face, mauve and wrinkled like little Rudy's was. Dwarf's body, weak as putty, in a whitelined deal box." Although the illustrations here exaggerate the red color of oxygenated blood, the blue color of venous blood, and the purple color of their mixing, the three kinds of blood do display these distinctive colors. The narrative itself exaggerates the cyanosis of Rudy's skin by describing it as mauve, a rich purple hue. The purple coloration must have been the trigger for Mrs. Thornton's intuitive judgment that the baby was suffering from a fatal condition.

Today, there are three staged surgical procedures for rebuilding these infants' malformed hearts: the multifaceted Norwood procedure, generally performed in the first week of life and fatal more than 10% of the time; the Glenn procedure (Bidirectional Glenn Anastomosis), usually done at about six months of age and carrying a much lower surgical mortality risk of 1-3%; and the Fontan Procedure, performed at 18-24 months with a comparably low risk of death. The three operations are performed successively, each building on the work of the former. They are palliative, not curative, leaving the child with only a single functioning ventricle.

Even with surgical and medical interventions, mortality and morbidity from HLHS remain high. Children with the condition typically suffer from incomplete brain development during gestation and score in the low-normal range on IQ tests; other somatic complications are commonplace. One study showed that, a year after the Norwood Procedure, the mortality rate was 25%. Over time, survival rates increase. For children who survive to the age of 12 months, long-term survival up to 18 years of age is approximately 90%. Even had the Blooms been able to take advantage of these open-heart surgeries, their lives as parents would not have been easy. But the development of the three procedures lay many decades in the future. In 1904, HLHS was a death sentence. 

Surrounded by large families, Joyce may have known of infants who died at about 11 days of age, or his brief stints as a medical student in Dublin and Paris could have familiarized him with the deadly heart condition. Although rare, HLHS is common enough to be known to all doctors: in the U.S. the prevalence is approximately 2-3 cases per 10,000 live births. HLHS accounts for 2-3% percent of all cases of congenital heart disease and afflicts males approximately 1.5 times as often as females.

 Bruce Hardy and John Hunt 2021
Diagrams of a normal heart (left), in which high-oxygen blood (red) is pumped to the body and low-oxygen blood (blue) recovered from the body is pumped to the lungs, and an HLHS heart (right), in which the two kinds of blood mix in the single functioning ventricle. Source: Moss & Adams: Heart Disease in Infants, Children, and Adolescents (Philadelphia, 2022).
Blood flow problems in an HLHS heart, showing blood pumped from the right ventricle to the lungs, with some of it entering the aorta via the ductus arteriosus and going to the rest of the body. Source:
Blood flow after the Norwood procedure has reconstructed the diminutive asending aorta, joined it to the trunk of the pulmonary artery, and installed one of two kinds of shunt to carry blood to the lungs. Source:
Results of the Glenn procedure, in which blood from the upper body is routed directly to the lungs via the pulmonary artery, bypassing the heart and freeing the right ventricle to pump blood only to the body. Source:
Results of the Fontan procedure, in which blood from the lower body is routed directly to the lungs via the pulmonary artery, letting the right ventricle pump only high-oxygen blood to the body. Source: