Precocious Puberty in RSS – Part II
Precocious Puberty in Russell-Silver Syndrome Children
GUEST BLOGGER – Leslie Leach
Mom to Christopher, Russell-Silver Syndrome and Eosinophilic Esophagitis
What is Precocious Puberty?
According to the National Institute of Child Health and Human Development:
“Precocious Puberty is defined as puberty that begins before the age of 8 years for girls and before the age of 9 years for boys. The word ‘precocious’ means developing unusually early.
The signs of precocious puberty are the same as those for regular puberty. The difference is that they start to occur at a younger age than normal.
For females, signs include development of breasts, pubic hair, and underarm hair; increased growth rate; and menstrual bleeding.
In boys, signs include growth of the penis and testicles, development of pubic and underarm hair, muscle growth, voice changes, and increased growth rate.”
According to the MAGIC Foundation and Child Growth Foundation, children with Russell Silver Syndrome can have the tendency to develop puberty early. This can not only affect adult height, but can also have emotional and social implications.
We began to first notice changes in Chris in September of 2010 when he was 9 years old. Possibly earlier than that. The first symptom was body odor and we started buying him deodorant. The next change was acne. It was quite subtle and we actually thought it was from his lacrosse helmet. It then dawned on me when his voice started to get lower that we were dealing with early puberty. I spoke with his endocrinologist who wanted him seen immediately.
At the visit, Chris was examined and the endocrinologist agreed that he was exhibiting signs of early puberty. He discussed the possibility of a pituitary tumor but that was unlikely in light of his Russell Silver syndrome diagnosis. Before discussing treatment options, he ordered the following:
~A blood test for FSH (follicle stimulating hormone), LH (luteinizing hormone) and sex hormone (estradiol or testosterone) levels
~Bone age X-ray (an X-ray that measures the developmental age or maturation of bone)
The results of these blood tests showed an extremely high testosterone level comparable to a 15 to 17 year old. His bone age was that of a 13 to 14 year old. These tests solidified the diagnosis.
We then discussed our treatment options which included Lupron monthly injections, Supprelin implant or Synarel Nasal spray. We were told that Lupron had a high incidence of weight gain side effects, behavioral changes and could also cause some discomfort as it would be a monthly injection. Our doctor did not have much knowledge about the nasal spray, which seemed like the least invasive option to us. In his opinion, the efficacy was not well researched. He encouraged us to consider the Supprelin implant as it was less invasive than the monthly shots and we only needed to replace it yearly. The side effects were minimal according to the physician.
After about a month of insurance red tape, we were able to schedule the implant placement for January 2011. It was a fairly easy (yet costly) procedure, and we were in and out of hospital within a few hours. Throughout the months, his physical characteristics did not go away, but after about 4 months his testosterone level normalized. That was a huge relief as we had proof the implant slowed the process.
One of the main side effects that Chris experienced was a 10-12 pound weight gain within the year. This was difficult not only because of the lack of gaining inches in height, but because he was placed on a restrictive diet for his new diagnosed Eosinophilic Esophagitis. The amount of caloric intake didn’t match up to the weight gain.
We did not notice major behavior changes that appeared to correlate with the medication. It’s possible he became a bit more moody and a tad more stubborn..but that could be chalked up to normal personality development.
Our decision to treat his early puberty had to be made quickly because of the rate his body was changing. Do I regret our decision to treat with the implant? A little. Only because of the cost, surgery and weight gain. But I feel we made a decision we thought was right at the time, not knowing what the future held.
Supprelin Implant (http://www.supprelinla.com)
TAP Pharmaceuticals, maker of Lupron (www.toosoon.com)
Synarel nasal spray (http://www.rxlist.com/synarel-drug.htm)
The MAGIC Foundation (Major Aspects of Growth in Children) (www.magic foundation.org)
Child Growth Foundation (http://www.childgrowthfoundation.org/iugr_rss.htm)
National Institute of Child Health and Human Development (http://www.nichd.nih.gov/health/topics/precocious_puberty.cfm)