Have you ever heard of Russell Silver Syndrome? Perhaps its abbreviation, RSS, is more familiar? And, no – not as in an “RSS feed” like you can get on your computer! Neither of the above terms rang a bell with me to begin with, so I did a little investigating and here’s what I found out . . .
Have you ever heard of Russell Silver Syndrome? Perhaps its abbreviation, RSS, is more familiar? And, no – not as in an “RSS feed” like you can get on your computer!
Neither of the above terms rang a bell with me to begin with, so I did a little investigating. I didn’t feel so bad when I found out that it is an extremely rare medical condition – I would have felt a little silly if it was more common, like the flu or a cold!
Anyway, here’s what I found out . . .
Russell Silver Syndrome Defined
Russell Silver Syndrome, or RSS, is an extremely rare condition affecting children at birth. First classified in the early to mid 1950’s, the syndrome was little understood, and even today RSS has only recently come to better understanding.
RSS is generally associated with a less developed body at a particular gestational age: poor growth, low birth weight, and shorter than average height.
Additionally, RSS is characterized by a difference in symmetry of the two body sides. Other physical characteristics include a triangle shaped head with a broad forehead, a wide thin mouth, a small pointy chin and the pinky finger of one side will typically curve inwards towards the other fingers rather than having the normal, straight out protrusion as found in other digits of the hand.
RSS is also known as Silver Syndrome.
Who is affected?
In confirmed RSS cases which have been studied, there has been no indication that the syndrome favors either male or female. RSS is a syndrome that is known worldwide, with diagnoses in all ethnic groups and races; however, estimates of exactly how many RSS cases vary widely with the numbers reported as between 1 of 3000 births to 1 of 100,000 births showing signs of having RSS. The RSS diagnosis usually occurs by early childhood.
What causes RSS?
Prior to a broad base science in genetics, RSS was commonly termed as a “failure to thrive” condition with no known contributing factors. As the study of genetics has improved our understanding of syndromes and diseases, RSS has recently been classified as a genetic disorder, which contradicts many earlier classifications.
What happens to someone with RSS?
As children grow into adulthood, some of the physical characteristics of RSS diminish although they do not completely fade away. It is suspected that the intelligence level of people with RSS is normal at birth, and remains normal throughout life, although some indications are that there are inherit learning disabilities associated with RSS.
Treatment Options and Obstacles
Information relating to affiliated conditions of RSS point to hypoglycemia as being a predominate factor to overcome in the treatment of RSS. Children with RSS are found to suffer higher than normal instances of hypoglycemia and research concludes that making sure children with RSS eat properly (and perhaps more than those without RSS) can significantly contribute to the “catch up” of the body’s growth.
While RSS may also be treated with growth hormones - a therapy that has been gaining acceptance - attention to the nutritional needs of a child with RSS can be of great benefit.
If you have a child with RSS, it is recommended you treat your child according to age, not size. Provide age-appropriate toys and expect age-appropriate behavior. Be aware that some of the effects of RSS affect the self esteem of your child as they relate to others who do not have RSS. It’s important to provide a nurturing, expectant atmosphere for a child with RSS just as you would for any other.
So Now You Know!
Even though RSS is a rare condition, it’s important to be aware of the possibility that your child or grandchild (or future child) could be affected by it. Simply having the knowledge of the disease and its complications will better prepare you for any RSS encounters in the future.