What secondary problems can affect the quality of life of a child with HPE
From Holoprosencephaly
What secondary problems can affect the quality of life of a child with HPE?
Kids with HPE often have cerebral palsy, movement problems, and global developmental problems. These are effects of the brain malformation and affect their independence and ability to learn, communicate and take care of themselves.
Pain can be an issue for them. For example, GERD can lead to pain and cause problems with weight gain, and constipation can cause a lot of discomfort. Autonomic instability can also be a problem. The autonomic nervous system is the part of the nervous system that you have no control over at the level of consciousness, like the heart. The sympathetic and parasympathetic nervous systems, which are competing aspects of the autonomic nervous system, control heart rate, breathing, gastrointestinal function, blood flow, and sweating, among others.
Some kids with HPE have disturbance in the control of Autonomic Nervous System so they may overheat, not sweat enough, they can’t keep body temperature so they get too cold. The hypothalamus and the brain stem control temperature, so if the hypothalamus is abnormal they have temperature regulation problems. For many of these problems, no preventive measures can be taken.
Nissen fundoplications may be done to prevent recurring aspiration pneumonia caused by GERD. For most other problems, It’s important to watch for the symptoms. For example, if there are symptoms of constipation, then make sure you are giving the child enough fibers, fruits and vegetables, etc. If they back up, treat early to prevent it from getting worse. Seizures should be treated when they begin so they don’t get more difficult to treat. However, it’s not recommended to prescribe seizure medication to prevent from developing epilepsy if they’ve never had a seizure.
Dr. Eric Levey, Kennedy Krieger Institute
