Longevity in WAGR Syndrome
When a child is diagnosed with WAGR syndrome, parents are usually very concerned about the medical conditions that are associated with the disorder, particularly those that are potentially life-threatening. Many parents want to know if life expectancy is decreased for people with WAGR syndrome.
Longevity in WAGR syndrome has not been formally studied. However, the International WAGR Syndrome Association includes approximately 200 individuals with this disorder. The great majority of these are children, teens, and young adults. There are a handful of individuals in the group who are in their 30s, and a few who are currently in their 40s.
There are several possible reasons for what appears to be a lack of older adults with WAGR syndrome. The first is that effective treatment for Wilms tumor did not exist until the late 1970s. Since children with WAGR syndrome have a 50 percent chance of developing Wilms tumor, only those who did not have Wilms tumor before that time would have survived past early childhood.
A second possible reason is that the term “WAGR syndrome” did not come into use until the mid 1980s. Before that time this disorder was variously referred to as “Aniridia-Wilms tumor syndrome,” or “AGR syndrome,” or “11p- syndrome.” Very often it was simply diagnosed as “sporadic aniridia.” It is possible that there are adults who were originally given a diagnosis of one of these older terms, and that their parents/physicians are unaware of the change in terminology.
Finally, another factor to consider is the age of the parents of adults with WAGR syndrome. Older parents may be much less likely than younger parents to go online looking for information about their child’s disorder. A 2012 Pew Research Center study found that only 53% of American adults ages 65 and older use the internet. In addition, until the disability rights movement of the 1970s-80s, societal attitudes about disability were often negative and little support for parents/families existed. As a result, older parents may be less likely to seek out or join an organization like the International WAGR Syndrome Association.
While life expectancy for people with WAGR syndrome is currently unknown, this much is certain: medical diagnosis and treatment of the conditions associated with WAGR syndrome has been steadily improving since the 1980s. These improvements include:
- better genetic testing, allowing diagnosis of WAGR syndrome prenatally or soon after birth
- monitoring for Wilms tumor beginning at birth
- treatment for Wilms tumor that is not only more effective but shorter and with fewer long-term side effects
- early diagnosis and treatment of chronic kidney disease, which can dramatically prolong kidney function
- dialysis and/or kidney transplants are possible in people with WAGR syndrome and have proven to be successful
Today, the outlook for children diagnosed with WAGR syndrome is brighter than ever before. And with continued research, their lives will be healthier and longer too.
Last updated: April, 2015
Kelly Trout, BSN, RN
International WAGR Syndrome Association