A woman who was born with hydranencephalya rare neurological condition in which the cerebral hemispheres are not formed, celebrated its 20th anniversary earlier this month. The milestone contradicted medical expectations that she would not live beyond four years. The information comes from the American newspaper The Mirror US.
Alex Simpson was born in Omaha, Nebraska, and was diagnosed with the rare condition when she was two months old. According to , Alex had only “half the size of a pinky finger” of cerebellar tissue at the back of his brain.
As SJ, Alex’s 14-year-old brother, reports, the young girl needs 24-hour care and constant supervision. In addition to support from her parents and sibling, other family members adjust their daily routines to meet her needs, forming connections through physical contact.
The family tells The Mirror US they believe Alex survived beyond medical expectation thanks to the nurturing environment they created for her.
What is hydranencephaly?
Hydranencephaly is a congenital anomaly (that is, it occurs from birth) that affects the central nervous system. According to , a baby born with this condition does not have the cerebral hemispheres, which are the two halves of the brain, the front and largest part of the brain.
Despite similar terms, hydranencephaly is a different condition from hydrocephalus, characterized by the accumulation of fluid in the brain and increased intracranial pressure. The disease may be caused by a congenital anomaly or be a symptom of injury or disease, and may even be associated with hydranencephaly.
However, in the case of hydranencephaly, the brain itself forms abnormally during .
Scientists still do not know what causes hydranencephaly, but they believe it may be related to genetic issues or exposure to toxins during pregnancy. The condition can be diagnosed by prenatal ultrasound or .
In addition to the absence of parts of the brain, the condition leads to symptoms such as: lack of development, increased or decreased muscle tone, increased head size, vision and hearing problems, stiff arms and legs and difficulty breathing.
Hydranencephaly has no cure. According to the Cleveland Clinic, treatments include surgery to implant a shunt valve (tube) to drain fluid from the brain and reduce intracranial pressure and the use of medications such as anticonvulsants, as well as physical therapy and nutritional support.
Generally, the prognosis for children with hydranencephaly is poor: most babies die before they are born or before they are one year old, according to the Cleveland Clinic.
