Moving house is sucking all my blogergy, so here’s a tale for you.
Trish and Bobby. [Image source]
Background to this story:
Bobby was born with hypoplastic left heart syndrome. This is a congenital defect in which the left side of the heart – the side that pumps blood to the body – is severely underdeveloped. With this disorder, as blood returns from the lungs, it must be pumped by the right ventricle instead of the left, flowing through two remnants of the fetal circulation (atrial septal defect and patent ductus arteriosus) in order to circulate to the body. Essentially, the two circulations (lung and peripheral) are connected in parallel instead of in series, and the right ventricle must do the work of two.
Babies with HLHS become pale and very ill within days of birth. Left untreated, the condition is universally fatal. A series of staged operations are needed, over the first few years of life, to limit the effects, but there is no definitive cure. Medication must also be given to keep the ductus arteriosus open. The period after the first operation can be particularly difficult. Complications and infections are common. Before 1979, there were no survivors, so the long-term effects even with successful treatment are unknown.
Bobby’s Dairy was first published in 2003. Born with HLHS, Bobby suffered severe complications after his second operation at three months of age. He developed septicaemia and a chylothorax. A chylothorax develops when there is damage to the part of the lymphatic system that transported ingested fat from the gastrointestinal circulation back up to the blood system. With a chylothorax, the fat-laden lymph (chyle) leaks out and accumulates in the chest cavity.
Babies with chylothorax are typically placed first on total intravenous nutrition, then on a low-fat formula – or were, before Trish, John and Bobby came along. Trish, a Le Leche leader, knew that Bobby needed her milk to fight his life-threatening infection. Mother’s milk contains a multitude of infection-fighting substances that cannot be duplicated, and is more readily absorbed than artificial milks. Artificially fed babies are far more likely to succumb from infection, and heal more poorly after surgery.
Snippets from the Leaven (La Leche League publication) article (but read the whole thing!):
“Your baby cannot have your milk.” The words shot through me like a bolt of lightning. I looked into the doctor’s eyes and choked out in utter shock, “What?” He repeated softly, “The baby cannot tolerate the fat in your milk. He cannot have your milk.”
Etched in my mind forever will be the day I was at my son’s bedside during a routine dressing change of one of his four chest tubes. When the nurse unveiled the area, I was appalled to see that a 2 inch (5 centimeter) crater had formed around the half inch (2 centimeter) chest tube, a sure sign that the nutrients he was absorbing were not adequate. Skin health is considered one of the most accurate indicators of nutritional status. Though I knew physical touch was vital in this situation, I was discouraged from holding my child in my arms because the sutures, which held the chest tubes in place, often tore through his fragile skin as if they had been sewn into butter. The breathing tube, which barely rested against his lips and chin, had caused a large bruise underneath. I thought of my other, healthy robust breastfed babies, and here was my son, my beautiful breastfed baby boy, literally starving to death. It seemed as if his life was draining out the tubes.
Ideally we needed a fat-free form of food for Bobby’s stomach, but there was nothing available.
During that time I continued to pump and freeze my milk, praying for the day he would be able to receive it. Pump, freeze, and wait was my daily routine. After weeks of drainage and no food by mouth, Bobby’s condition was deteriorating rapidly. John and I decided that if we wanted our son to come home alive, we had to take the matter into our own hands.
While the medical team was trying these interventions, my husband, John, and I had already begun tapping into our LLL network for possible solutions.
The milk needed to be spun in a centrifuge at a specific force of gravity over a specific amount of time. The only problem was that we did not own a centrifuge, and we could not use the centrifuges at Yale New Haven Hospital. Centrifuges were normally used to spin “blood and body fluids,” so whatever human milk we would spin in these would not be fit for human consumption. John would not be deterred. If necessity is the mother of invention, then desperation is the father of creativity. John spun it in our top-loading automatic washing machine.
You can also read “Bobby’s Dairy: An Update” here. In 2005, Bobby had what should be his final heart surgery for a long time. He continued to receive his mother’s milk until over the age of five, including a period on centrifuged defatted feeds after developing another postoperative chylothorax and infections. He is now seven years old and thriving, though he still requires some therapy to catch up, and he has a hearing loss from medication.
The case of Bobby, Trish, and Bobby’s Dairy has been written up in the Annals of Thoracic Surgery, in the hope that others may benefit from their determination and creativity.
Resources for information on Hypoplastic Left Heart Syndrome: