Re: Digest Number 3363

[Guest guest]

Hi Judy,

I have a 12 year old RSS son who has been through this. My son has

kyphosis and scoliosis, and wears a back brace for this, but his

kyphosis is worse than his scoliosis. As far as the puberty goes,

are you referring to physical signs or has this been proved through

labwork? The reason I am asking is that there is something called

adrenarche that has signs that resemble puberty. In adrenarche, the

adrenal glands in the kidneys are active in premature production of

increased amounts of dehydroepiandosterone. Physically, a child

develops any of the following secondary sex characteristics: body

odor, axillary and/or pubic hair, and acne. This is also a period in

which the bone age can advance rapidly, which is bad for RSS children

or children with any type of growth disorder. Adrenarche is a risk

if your child's weight for height ratio is too high. Typically, Dr.

Harbison, who you may have read about in some of the posts here (she

is a pediatric endocrinologist who specializes in RSS) like for RSS

kids to be somewhere between the 10 - 25%ile weight for height. In

puberty, you will see the same signs that are exhibited in adrenarche

in addition to penis & testicle enlargement. Since your son is 10,

he is somewhat within the normal range of going into puberty, but

nevertheless, your doctor should be able to do some labwork that will

determine if it is true puberty or if it is adrenarche. An ACTH test

is used to determine if a child is in adrenarche and an LHRH test is

used for puberty. Regardless of which of these your child is

experiencing, the doctor will likely want to prevent the development

of either one of these, so that the bone age does not advance and you

have more time to treat with GH for maximal growth. Many patients of

Dr. Harbison use Aromidix or Aromasin who are in or at risk for

entering early adrenarche to stop the bone age from advancing. To

delay puberty, Lupron or Zoladex is used. My son is on Aromasin (a

pill taken daily) and Lupron Depot (an intramuscular injection given

every 28 days).

Which doctor do you see in London? Dr. Stanhope practices

there at the Great Ormond Street Children's Hospital, and he too is

an expert in RSS & would be very familiar with these protocols.

Kim C.

>

> Subject: Scoliosis/early signs of puberty

>

> I have just joined the Magic Website( I Live in England) and my son

> who is 10 in March has Silver. We have just found out that he

> has scoliosis and he has been showing signs of early puberty. He has

> been on Growth Hormone since he was 2. I would like very much to hear

> from anyone who has a son of similar age or alittle older who has had

> these problems.

> Thanks Judy