Rett syndrome is a rare neurodevelopment disorder that primarily affects girls. The disease causes symptoms such as motor problems, growth defects, and cognitive disability. Many patients also experience seizures.
It is difficult to predict the lifespan of Rett syndrome patients because the disease is very rare. There are women with Rett syndrome in their 40s and 50s, but not much is known about the prognosis and lifespan of patients past the age of 40. However, early diagnosis is improving, and most Rett syndrome patients are now identified before the age of 25. Early diagnosis and treatment are expected to increase survival rates as well as improve patients’ quality of life.
Current statistics indicate that almost all Rett syndrome patients reach the age of 10. Patients have a nearly 90 percent chance of reaching age 20, and a more than 50 percent chance of reaching age 50. (The general female population in the U.S. has a 50 percent chance of living to age 80.)
As treatments and overall care improve, these statistics are also predicted to improve.
There is very little data to make predictions about life expectancy in atypical forms of Rett syndrome, which are even rarer than typical or classic Rett syndrome.
Causes of death
The cause of death of patients with Rett syndrome is usually related to seizures, pneumonia, malnutrition, and accidents.
The frequency and severity of seizures are closely linked to life expectancy with seizures increasing the risk of death. Anti-epileptic drugs can be used to control seizures and reduce this risk.
Pneumonia can be a risk factor for Rett syndrome patients, especially for those with scoliosis (abnormal curvature of the spine), as this can affect lung function. Swallowing difficulties or problems with the esophagus are also thought to increase the risk for aspiration pneumonia.
Malnutrition is another factor decreasing life expectancy in Rett syndrome. A healthy diet high in calories can improve growth and promote weight gain, reducing the risk of death caused by malnutrition. Tube feeding may be necessary for some patients.
Patients with difficulty moving or problems walking are at increased risk of accident and falls, which can have an impact on mortality rates.
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