Born without a brain, Alex Simpson of Nebraska defied all odds by celebrating her 20th birthday recently; doctors said she wouldn’t live past age four. Alex’s remarkable milestone made the news headlines; her parents Shawn and Lorena Simpson, shared her inspiring story with local news outlet KETV. Hydranencephaly is very rare, affecting an estimated 1 in 5,000 to 1 in 10,000 pregnancies. Alex was born with a condition where most of her brain is missing. As her father, Shawn, explained, "Hydranencephaly means that her brain is not there." He noted that she only has a small portion of her cerebellum, about "half the size of my pinky finger"—in the back of her head, but little else. Tragically, doctors initially told the family that Alex was not expected to live past the age of four. Can A Person Live Without A Brain? Hydranencephaly, according to the Cleveland Clinic, is most often fatal within the first year of life, making Alex’s 20th birthday an exceptional medical outlier and a true testament to her strength and her family's devotion. Hydranencephaly is a very rare birth defect that affects the central nervous system. A baby born with this condition is missing a significant part of the brain called the cerebral hemispheres. Missing Parts The cerebral hemispheres are the two large, front halves of the brain (the cerebrum). These parts are responsible for higher functions like thinking, memory, and movement control. Fluid-Filled Sacs Instead of the actual brain tissue, there are large sacs filled with cerebrospinal fluid (CSF). This is the fluid that normally acts as a cushion for the brain and spinal cord. Outlook Because so much of the brain is missing, the condition is usually fatal either before birth or shortly afterward. Babies who survive often develop an enlarged head and have severe symptoms. What Causes A Baby To Be Born Without A Brain? The Cleveland Clinic explains that abnormal brain formation happens during early pregnancy and it can be caused by a birth defect, injury, or illness. The exact cause is unknown. Scientists suspect it might be inherited, but they don't fully understand the pattern. Some cases have been linked to exposure to harmful substances during pregnancy. Does Hydranencephaly Have Symptoms? A baby born with hydranencephaly might seem normal at first. Symptoms usually appear within the first few weeks or months: Head Size: The head gets larger than normal.Growth Problems: Failure to gain weight or grow well ("failure to thrive").Muscle Issues: Muscles might be too stiff (rigid arms/legs) or have increased or decreased tone and twitching.Basic Senses: Problems with seeing and hearing.Breathing: Difficulty breathing. How is Hydranencephaly Diagnosed and Treated? It can be spotted during a prenatal ultrasound, which uses sound waves to check the baby. If hydranencephaly is suspected, an MRI might be used for clearer pictures of the missing brain tissue. If it wasn't seen before birth, a doctor will make the diagnosis based on the baby's symptoms and a detailed brain MRI. Other tests like a CT scan, angiography, which is an X-ray of blood vessels, or genetic testing may also be used. There is no cure for hydranencephaly. So, the treatment focuses on making the baby as comfortable as possible and managing the symptoms: Fluid Management Surgery to place a shunt, which is a thin tube, can drain excess fluid from the skull to reduce pressure, which helps manage the enlarging head, though its effectiveness is limited compared to hydrocephalus. Medication Antiseizure medications may be given to control twitching or spasms. Supportive Care This includes nutritional support (feeding), physical therapy to help with muscle problems, and sometimes a tracheostomy or ventilator if the baby has trouble breathing.
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