Just recently, we have had a 19-year-old patient who gave birth through caesarian section to a baby boy. The baby is her first born. But instead of being happy just like any other new parent, this patient felt the other way around. Her firstborn is diagnosed with Pentalogy of Cantrell.
What is Pentalogy of Cantrell? Pentalogy of Cantrell is a rare congenital anomaly characterized by defects in the closing of the supraumbilical abdominal wall, in the anterior portion of the diaphragm, in the diaphragmatic pericardium, ectopia cordis, and intracardiac defects. It associates diaphragmatic and ventral hernias, hypoplastic lung, and cardiac anomalies such as transposition of the great vessels and patent ductus arteriosus. It may be associated to other anomalies determining the variants of this syndrome. (www.humpath.com)
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| This photo closely resembles that of our patient, plus a heart beating normally over the chest area. |
I would just describe the baby’s condition in layman’s term. He is pinkish in color, with good cry, and with a good activity, that he got a perfect APGAR score. But you will easily notice his heart which is located outside his chest and a sac enclosing his other organs outside his belly (omphalocele). Omphalocele is a type of abdominal wall defect in which the intestines, liver, and occasionally other organs remain outside of the abdomen enclosed in a sac. That was the first time I have seen a true heart beating.
After the baby was born, routine newborn care was done, and was maintained on oxygen. The baby’s attending physician referred the baby to one of the country’s biggest and highly modernized hospital, but after a few hours, the baby was brought back to our hospital. The relatives of the baby said that the hospital where the baby was referred to told them that the baby won’t last even if surgery will be made. Surgery might just shorten the baby’s life. The attending physician did not lose hope. Another referral was made to another specialized hospital, but the parents were asked Php 200,000.00 deposit. With no assurance that the baby will survive after the surgery and no idea where to get that amount of money, they made a very hard decision: to just wait for the baby’s life to end. Painful as it is, but they think that they have no other choice.
The hospital made its way in order to help them, including explaining thoroughly the baby’s condition, but they have made their decision. So, comfort measures was the only option left for us. On the baby’s second day, he started showing symptoms of stress. He became slightly cyanotic, and vomited many times. His activities and cries also lessened. And during my shift, on his 3rd day, 20 minutes after my last vital signs to him, which was within normal range, the father reported to us that the baby became bluish and the heart which was covered with sterile OS soaked in normal saline no longer beats. The baby aspirated his vomitus after vomiting just before I left him. The vomiting might be due to another congenital anomaly on his intestines.
No further medical attention was done due to the signed Do Not Resuscitate waiver.
While doing my post mortem care, I made this thoughts: If only money is not a problem, he could have been saved. If only his parents were optimistic, ways in order to raise money should have been made. Who knows, the child might have been the next NASA Administrator or the UN Secretary General in the future.
The baby lived for three days. Those days could have been enough for the parents to change their minds into not giving up the life of their child.
Sigh…
N.B.: This is a reblog from my previous blogsite. This happened December 2010.
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