Some Info

Very true - but you just get 30 minutes with her at the convention
and you'd get a whole lot more time at her office....
jenny
--- In RSS-Support@y..., Jeanne Marie Mirabella

Katy Badt has a letter that was written for her specifically.
However, the MAGIC RSS brochure has some specific directions written
in it (e.g., hospitalized the night before with IV dextrose 10% if
NPO is needed, and continued IV until child can eat food.)
Jenny

No Melissa, it is not inevitable for a child to have a feeding tube.
Some very small infants have them placed simply because their health
demands it. When toddlers/ preschoolers and older have G-tubes
placed, it is usually because the parents have tried everything else
(e.g., caloric increases, diet changes, and Periactin to try and
increase the appetite). When all else fails, then the G-tube
discussion begins.
Some physicians may rush to put a G-tube in earlier than others.
Our daughter lindsay gained ZERO weight from age 3.3 to age 4.3yrs
old. Despite getting more than enough calories (according to them).
Two physicians wanted to put in a G-tube. It was right at that time
we found Dr. H. She saw Lindsay the first time and said whoa
nellie. If this child can eat so much, we need to try and get even
MOREEE calories into her (g-tubes are most often prescribed for the
child who simply has no appetite). She prescribed a diet switch to
high in complex carbohydrates, and Lindsay gained 3 pounds in the
first 3 months. Then came Periactin, and Lindsay's weight continued
to climb on its own. No G-tube.
So now, it is not inevitable. But for those children who need it, it
is a lifesaver. Jennifer

LOL, maybe I'll fly out to the conference... round trip air fare would
be less than I am going to have to pay for my office visit to see Dr.
Harsison in NYC!!!

Melissa - Jodi said it all. Ditto! Remember that the ketone
spilling/hypoglycemia risk is from being so underweight -- part of
any SGA (small for gestational age) child, not just RSS.
jennifer

Well. Let's see. I guess one thing I would try at your next appt is
to bring with you the MAGIC Foundation blue RSS brochure. Second of
all, call MAGIC at 800-3MAGIC3 (are you a member, I can't remember)
and ask them to mail you a copy of the current MAGIC magazine - tell
them you want to bring it to a physician. Inside this four-color
magazine is an entire section on RSS - which basically copies the RSS
brochure BUT ALSO adds an entire page of graphs showing treatment
results for different children on Periactin, Lupron (for delaying
puberty) and GHT. And it is all written by a physician with years of
RSS experience and who has more than 120 current RSS patients and
consults on hundreds more each year. This should bypass your
endo's "internet anecdotal" comments.
And lastly, if you are already going to CHOLA, why not simply make
your next appointment with Dr. Mitch Geffner? Remember, your son
would be a PRIME candidate for the new SGA study!!!! FREEE growth
hormone!!!! I have more info on the study if you want it. But
basically they want SGA or RSS children who have NOT been on any type
of medication (Periactin or GH yet), they will run all the standard
tests, and then you get the GH for free, and come in every 3 months
for a checkup and they see how he is growing. And it is all FREE!!!!
Let me know if you want more info or contact info. I also have Dr.
Geffner's private email address (for anyone who lives on the west
coast and is interested in having your child on growth hormone, and
would like it free.... ha!!! Dr. Geffner was the Chief of Pediatric
Endocrinology at UCLA Children's Hospital and just moved over the
Children's Hospital of Los Angeles. He is an amazing physician, and
does lots of free work for MAGIC, just like Dr. Harbison. Not that
much experience with RSS kids, but lots of experience with other kids
that are small.
Email me!!!).
Jennifer
Jennifer

Can someone please send me a copy of Dr. H.'s letter on RSS
and anesthesia, or tell me where I can get it? Joshua is having
ear surgery on Friday. He hasn't had any problems with
anesthesia in his previous surgeries (he's had about a half
dozen: G-tube placement, two sets of PE tubes, and several for
chordee repair), but this may be the longest procedure he's had,
so I wan't to make sure the anesthesiologist is aware of any
possible RSS-related issues, such as the risk of hypoglycemia.
- Joe

We went to Dr. Rothenberg in Denver - I found him on the web, and has done
papers around the world. He actually put in both a Microvasive Mini-button and
a Nissen fundoplacation (where the top of the stomach is wrapped around the
esophogus to prevent reflux) endoscopically. He only had 4 1/4 inch
incisions. Many people can only endoscopically do either just the g-tube or
the other kind of fundo, where the top of the stomach is puckered or something
and it can come out easier.
Inga

Can anyone tell me the process your doctor's used in putting in your
child's G-tube? And the brand/type... Thanks!
Jeanne

Jodi
When is Katy going to visit her parents (Or Katy, when are you going to
visit your parents?!) My moving doesn't really matter, because I am
only 1/2 hour from you right now... not far at all! Who all would be
interested in meeting?
I have three kids, my older one is Sam, 7 and he is in school. Josh, 3
is with me all the time as is Rosemarie. There are many great parks to
meet in, we could meet at the great swamp for a hike or something or a
picnic at one of the area parks... Let's plan to really do it once we
hear who else is interested.
Jeanne

THANKS for this info... I just went and got some ketosticks today. I'm
hoping we can cut off any future maladies by knowing sooner than waiting
for the blood tests to scream at us like last time. THANKS Jodi

Hello all
As you've heard me discuss in the past, it takes forever for us to have
actual appointments with the specialists. There are waiting lists that
go for months sometimes. I have found that it is helpful to show up on
the day of the first appointment with the basic tests for that
specialist already done, and they are usually good about ordering them
in advance.
I'm wondering what tests an endocrinologist would be ordering. That is
the appointment that is farthest away (mid-august) and since some test
results take weeks to get back, I'd rather have the basic tests early
and then be able to show up with her already having the results rather
than having to order and then try for a follow-up appointment in
september.
Any suggestions?
Jeanne

[RSS-Support] Clothes help in the US please...Hi Steph,
Try www.naturino.com for some beautiful Italian shoes, that seem to be
narrower than AMerican shoes.
If you go to http://www.naturino.com/eng/dove.htm
you can select "Naturino sales outlets", to find out a store near you.
Katy

Melissa,
In my experience, it is the endocrinologist who should be taking
charge of Maddison's care and making sure she gets what she needs.
It is this doctor who is the one to check her growth, development
and metabolism. I can't remember if you have one on your team, so
please fill me in on that.
The hypoglycemia/ketone issue is controversial among doctors. While
Dr. Harbison has documented that RSS kids do have issues with
spilling ketones, hypoglycemia and becoming acidotic, there are
those medical professionals who do not believe it is a component of
RSS. She, and I and many others, believe that all children at least
until the age of three should be monitored for this. It is easy,
painless and of very little cost. Some children, I should tell you,
have to be monitored until they are older, like my son Max. Every
time he gets sick, he spills ketones and must be monitored.
Sometimes it happens even if he is well. I am good at detecting
them, though, because to me his breath smells like nail polish
remover.
Anyway, spilling ketones is one of the first signs of your child
becoming hypoglycemic/acidotic. It is the body's signal that there
is not enough fuel for what it needs. The brain will take what it
needs first and then start using the reserves to get what it needs.
Our children have little body fat, so once that is used up as the
extra fuel, the system goes a little haywire.
Checking is as easy as can be. Just purchase a package of
ketosticks at your local pharmacy. They are usually where they
store the diabetic supplies. Collect a bit of urine from Maddison,
even if you have to put a cotton ball in her diaper to do it. Drop
the urine onto the pad on the ketostick, wait 15 seconds and
compare the color of the pad to the colors on the chart on the
bottle. If she has ketones, don't fret. Feed her something,
preferably liquid, and try to flush them out. Also, try protein and
complex carbohydrates. Sugary things will quickly eliminate the
ketones, but they will not stay away because they are burned up too
quickly. You need more complex foods to help.
IF the ketones do not go down, then she will probably need IV fluids
with 10% dextrose. You would need to contact your doctor who would
tell you to take her to the ER and then proceed from there. Email
Jennifer Salem at magicrss@ mindspring.com and she will have a
letter, I believe, that Dr. H. has written for us to take to the ER
with our RSS children.
I hope I have helped you a little bit. If you have more questions,
just ask!
Jodi

I wanted to update everyone on Max's continued recovery since I have
had many private emails asking about him.
As most of you know, Max had surgery two months ago to remediate an
ongoing problem he has had with pronated feet. (That means they
were flat and turned out and floppy.) He also had his achilles
tendons lengthened. While the surgery was fairly simple, the
recovery has been more than we expected, but we are over the hurdles
now. Max was casted, in a wheelchair, used a walker and now uses
crutches. I'm really happy to say that this weekend he started
walking without crutches and also walking up the steps. He still
needs the crutches when his feet get tired and sore, but he is
trying.
Max has also started PT. The therapist who is working with him is
excellent and has pushed him just the right amount. He looks
forward to going.
Not everything has been easy, however. Max went through a period
where he had terrible muscle spasms and could not keep his legs
still. It was quite upsetting. Fortunately, he is over that and
back to being his more independent self. He will be 14 one month
from today!!!
For those who are going to the convention in Chicago, Max and I will
be there. He is more excited than I am!
Thanks for all the support you guys have given ME.
Jodi

Steve - WOW! You can join our group anytime, you sound as organized
as the rest of us!!! let's see, my name is Jennifer Salem and I am
the parent of a 6-yr-old girl with RSS, considered mild RSS.
None of us are physicians, so we truly can not tell you if your child
is RSS or not. But his picture is still so cute! Honestly, there
are only a handful of geneticists and endocrinologists in the country
who are good enough at diagnosing the more milder RSS kids (your son,
if he has RSS, would be considered a more mild form).
Mild? Basically, the genetics thought right now is that some kids
with RSS may have either fewer cells involved with markers, or less
of the chromosome, or something.
Yes, your son does resemble what we call "the stereotypical RSS
look." My question would be as follows:
what were the birth weight and lengths of his siblings?
and what is your height and what is his mom's height?
That will help me determine a lot more.
Do you live anywhere near Chicago? Have you checked out the website
www.magicfoundation.org? MAGIC has an RSS Division (the only one in
the U.S., may I say!!!). I am the volunteer director and many of us
are members. We have a huge convention in Chicago July 18-21, with a
full day dedicated just to medical presentations on RSS and various
issues involved. Most importantly, for you, is that Dr. Madeleine
Harbison, a peds endo who now pretty much specializes in RSS 9has
currently 120+ RSS patients and consults on hundreds more) will set
up an evaluation consultation with you at the convention - she will
read your reports, examine, measure, weight blah blah your son, and
give you lots of good info, including a diagnosis if she knows it.
Anyway. let me know the answers to those questions I asked about
height, etc. Jennifer

Gail, welcome to the RSS listserve. I loved reading everyone's
helpful hints to Tikva, but yours was so amazing!!! You are so right
on that you just have to keep plugging away, every little thing you
can keep trying. Thank you for joining and writing! jennifer

Sandy - I was in the exact same place you are when Lindsay was 3 yrs
old. She was 24lb 7oz and 35 inches tall. Not on the weight charts,
3%tile for height (supposed to be at 80%'tile or so). Our local endo
at the time kept saying, well she is growing OK, she is just petite,
and RSS kids don't grow that well, so let's keep watching her another
6 months. We wasted another 15 months before finding new help.
Lindsay is now in the 50 percentile for height, still skinny but
slowly gaining.
I would recommend that at your son's next peds endo appt, you ask the
endo the following questions: Why is it "acceptable" to your
endocrinologist for your son to possibly be a full 12 inches shorter
than he should be? I would also ask your peds endo if he or she is
aware of the large SGA study which found serious long-term side
effects of being an UNTREATED SGA child? Ask him what he knows about
RSS kids and early puberty? Is he aware that bone age x-rays should
be done every year? Is he aware that a delayed bone age for an RSS
child quickly accelerates just prior and during early puberty? Does
he have a copy of the RSS growth chart - just because an RSS child is
at the 10 percentile of the NORMAL growth chart does NOT mean that
the RSS child will end up at the 10th percentile as an adult.
Usually it is far less, because if left untreated, early puberty
radically accelerates the bone age and leaves the RSS child with even
less growing time than the average "normal" boy.
Yes, there are parents of RSS boys who even WITH growth hormone
treatment would be ecstatic at having their son at the 10 percentile
in height. But as you said, your son SHOULD be at the 80
percentile. So his genetic makeup is going to be very different.
I encourage ALL OF YOU that if your own pediatric endocrinologist is
not aware of all of the above information, that you either find a new
peds endo, or that you find one that is willing to converse with Dr.
Harbison or another good peds endo across the country who is aware of
the RSS specifics. There are lots of good peds endos in this country
who are aware of the RSS specifics, or who are learning about them by
talking with Dr. Harbison.
Do not be afraid of questioning your physician. RSS is very rare,
and we can not assume that every endocrinologist is going to know
everything about RSS. This is why we as parents have to do the
research, and then either find a physician who is willing to become
educated, or find that rare doctor who specializes in RSS.
OK, I will step off the podium now. Sorry! But as you can tell, I
feel very passionate about this!
Jennifer

We were wondering if our son has RSS.
First, a picture: www.infotrail.com/family (age 5.5 months)
We've seen a ped. geneticist, ped. neurologist, and ped.
endocrinologist.
The geneticist mentioned Russell Silvers as a possibility.
I have a lot of info and two questions:
1)on two blood tests his glucose has been normal - I thought RSS
were hypoglycemic? Or did the tests need to be done after fasting?
2)He doesn't look as short as the RSS kids photos I've seen
born 5 lbs 5oz 19" (39 weeks gestation)
6 months 11 lbs 3oz 25.5"
*Pectus excavatum
*Triangular face
*Broad forehead
*High arched roof of mouth
*Storks bite on the forehead
*Big head for length/weight (~15% head, ~5% length, ~1% weight at
six months)
*Very little body fat
*Active - seems to burn a lot of calories moving
*Spits up moderatetly - we don't think excessively
*3 older siblings, all normal - no family history of this.
*No asymmetry (one pupil is slightly larger than the other)
*Nothing odd with fingers or toes or different limb lengths.
now for the tests:
*MRI (six months) some atrophy - brain not filling the skull
completely, ventricles a bit large (not hydrocephalitic, though).
Good myelination pattern, small, presumably benign cyst in sub-
arachnoidal space (left temporal fossa)
*Spinal Muscular Atrophy - negative
*Congenital Hypomyelination - negative
*Bone growth (6 month actual age - bones looked 4 months). Big
whoop, at 11 pounds, he probably does look like a 4 month old).
*Free T4 - negative
*TSH - negative
*Serum Amino Acids - normal
*Urine organic acids - normal
*Aldolase - normal
*Creatine - low, is this a confirmation that he doesn't have a lot
of muscle?
*CO2 has been low several times - not by much - about 1 to 2 points
below normal range - indicative of starvation? - inadequate
nourishment?
*Albumin a smidge high - indicative of mild dehydration?
He had significant muscle stiffness that improved with physical
therapy, but he is still delayed - at 7 months, like a 5 month
baby. A lot of muscle weakness - tiny muscles.
Breastfed exclusively 4 months, then pumped and bottle fed. Now
eating about 20 oz a day - 4 oz max at a time. Using Enfamil 24 cal
premie formula (probably 14oz the rest breastmilk)
Any thoughts?
thanks,
Steve

What a cutie! I guess he does look like he could be RSS
others help with that.I'm pretty new to this:)
My daughter has been diagnosed with RSS recently .She is almost 6 but really
doesn't have the look, so I'm not convinced.
Jennie
Lindsey's site
http://lindsey03.homestead.com/mygirl.html

Hi Tik,
Our son, Matt, does not have RSS, but he is GHD. He had feeding
problems when he was a baby. He did a bit of vomiting, but not
enough to be of real concern. However, he was like your baby in that
he needed to be occupied when eating. Two things we tried that
worked very well for him (and this is going to sound very silly, but
it worked) were this: (1) We used to turn the faucet on (cold water)
in the kitchen and sit Matt on the counter so he could see and hear
the water. Then we would feed him while he watched. I don't know
why, but it worked. (2) As he got bigger, I would sit him on the
bathroom floor in his rocka-roo (I don't know what you call those
things now) and turn the shower on (cold water). Then, I would
proceed to feed him in his rock-a-roo in the bathroom (of all places!)
as he watched the water flow and heard it also. He has also always
liked the sound of rain. Eventually, we bought one of
those "soother" machines that imitates the sound of rain, a stream,
etc., etc., and this helped also. It also helped with his sleep. I
know this probably sounds pretty crazy, but all these things helped
with Matt. Another sound we found that he found soothing was the
sound of a hair dryer. He is 11-1/2-years-old now and I can still
remember those days. Once Matt got onto whole milk and could feed
himself (i.e., hold the bottle on his own) his feeding increased
dramatically. I can't remember how old he was when he started doing
this, but it was older than five months and before one year. Once he
was on "baby food", he really started to eat. However, he is still
quite thin, but other than the GDH, is a healthy boy. Never, never
give up! I remember those days trying to feed him and how
discouraged and worried I would get. One night when he was about
three or four months old, after having seen every pediatrician in the
city over a period of a few months and getting no answers to our
problems, we took him to the emergency department of our local
hospital. The doctor there said he had "never seen anything like
it". (This was not helpful! :) We tried every nipple, every bottle,
every formula, you name it. We just keep on plugging away, and
eventually things got better. Keep in touch with your doctor, and
hopefully things will get better. Us your "mother" instincts, you
will probably be right! I don't know if your baby has other medical
problems that I am not aware of, so I don't want to give advice, just
let you know the little "tricks" that seemd to work for us to help
get some food in. Let us know how it goes. Thinking of you . . .
Gail P. in Canada. P.S. - Matt still likes the sound of rain, and
still uses his "soothing machine" sometimes at night! Guess he would
probably like to live in the rainforest :)

Hi Tik,
My son, Matthew, ate exactly the same way when he was a baby. I could never
hold him. Every bite was a game. It was very frustrating and scary. He
also vomited most of what he took in. We got great relief when the doctor
placed a gastric feeding tube. I didn't have to fight with him anymore.
Matthew is now 6 1/2 and eats like a champ, but it took many, many years.
He still has his feeding tube because he spills ketones if he does not eat
overnight.
Keep your chin up.
Judy

Tikva,
Has your daughter been seen by a gastro-enterologist? I know you
are in Israel, but I don't know which doctors she has been seen by.
Have you kept a record of how many calories she takes in each day?
That is important, too.
My guess is that Mai has some kind of sensory issues going on.
That means she does not like the feeling of being touched -
especially while she is eating. Are there any early intervention
programs in Israel? Here, in the US, there are programs for infants
to age 3 where the babies are evaluated and then receive physical
therapy, occupational therapy, speech therapy, etc. at no charge to
the parents. The purpose is to get to these kids while they are
young and try to prevent and treat problems before they become more
complicated when they are older.
I know you have a friend in New York. Have you or do you ever come
over here to New York? If you do, then you could see Dr. Harbison.
If not, then perhaps I can get you to email her and see what she has
to say. She can be very helpful with these things.
As for the hypoglycemia part, you can check for ketones if you can
get ketosticks in your local pharmacy or have your friend mail them
to you. You would just put a cotton ball in Mai's diaper, collect a
bit of her urine and drop it onto the pad on the stick. If the pad
turns a certain color, it would let you know that she has ketones
and is on the way to hypoglycemia. You would know that she must eat
at that time. In any case, you should try to get her to eat even
small amounts every two to three hours during the day. Even just a
few sips from the bottle would be better than none.
Let me know if I can help you any more, okay?
Shabbat Shalom!
Jodi

Sandy,
Great news about Matthew. I'm glad he is doing well and hovering in
that 10%ile range. Don't worry that you are not doing something or
everything that you can for him. You have plenty of time and know
from reading posts what is ahead for him. Remember to use your gut
instincts, too. If something does not feel right, then keep on
trucking along until you get what you need/want.
Jodi

Jody,
Max had delayed stomach emptying and it was a problem for us, too.
The treatment for him was to give him medication that helps speed up
the emptying and to put him on 24 hour feeds so that he got the
needed calories, just in lower doses. He was able to only tolerate
30cc/hour at best at age 2. Don't worry. There is hope and help
available.
Jodi

hello everyone,
i would like to describe you how my baby eats,and please if any one had the
same situation- i do need help.
my daughter,Mai,almost 5 months old.she has a very poor appetite and i give
her a special formula with many calories as i can.BUT to make her eating i
have to make her busy- or to look at her mobileor to make her a little-bit
sleepy...by the way,never eats in my hand when she is awake-she has to
lay(?) in her bad.
during the nights she eats good(every 4 hs.) but in the morning she eats
after 7-8 hs.{smiling,plying,not crying) and thats make me a little afraid
because of the hypoglycemia....i do not want to force her,because if i do
she vommits.
any ideas,please
thank you...tik

hi,
i asked my friend who had been in NY to buy me ela-max in a pharmacy(i read
it is OTC) but she could not find. the pharmacits did not know what is this
medicine for.
can you please tell me where can it be reached in NY.
thanks..tik

Hi All,
Matthew had his 3 year check up today. He is 24 lbs 10 oz, and 36".
I had really hoped we would be breaking the 25 lb mark, but maybe
soon. He is right on track with everything, just little. The doc
said he is doing great. We saw our 2nd ped. endo in March, and she
just wanted to see Matt again in 6 mos.
I get the feeling everyone (including my husband sometimes) thinks I
should just relax and let him be, since he is doing fine. But I
think about where he could be (above 80% in height based on parental
height), and read about how much Periactin and GHT have helped some
of the kids here, and it is tough. Sometimes I wonder if I am the
one off track, or everyone else is!
So, I guess we'll see what happens in Sept. when we go back to the
endo. She seemed inclined to do nothing since he is hovering around
the 10% for height, which is "really good for an RSS child." Maybe
we will have to move on to endo #3.
I don't post often, but read everything every day, and appreciate all
the info and support.
Sandy - Mom to Matthew - 3 yrs old, 24lb 10 oz and 36"

Jeanne, her direct number is 212-746-3462.
I am trying to remember my bills, I know that although they say her
hospital on them, she is the provider. Because she dropped using
CIGNA as a "preferred." So I have to pay up to my deductible, and
then CIGNA covers her at 70%. HOWEVER, CIGNA does pay 90-100% of all
my New York Cornell Weill lab tests. So she is separate, I believe.
Jennifer
--- In RSS-Support@y..., Jeanne Marie Mirabella

Hello all
I've been trying to call Dr. H's office in NYC, but have been unable to
move beyond the message and hold at the switchboard. If I had an
extension, I would be able to move a bit further!
Can anyone forward me the contact information for her office?
Also, does she bill as a private doctor or does she bill through the
hospital?
THANKS
Jeanne
--
squirrels in the attic... any advice?

Marla - I don't have your last name so I couldn't check. But I know
in my MAGIC RSS database, there are a whole slew of RSS families who
live in the Bay Area. Jennifer

Jeanne - I will bet you that if you call Dr. H's office you will be
able to see her long before Aug. 13th. Jennifer
--- In RSS-Support@y..., Jeanne Marie Mirabella

Katy - this is great. So in actuality, it is possible for Steve to
go into the city and get his blood drawn while he is working in CT.
And then later when I fly out with Lindsay for her Oct appt, we could
do ours? I will confirm with Dr. H in July at the convention. It is
too hard to bring both kids to the appt, and too hard for us to leave
Tyler by himself with a babysitter for a weekend. Jennifer
--- In RSS-Support@y..., "Badt-Frissora, Katherine S (Katy)"

blood drawn locally. Dr. H. wrote out a prescription for him to get
the blood drawn and the # of vials she needs.

Hi just curious if anyone here lives near me. I live in San Jose
California. Thanks, Marla

Hi Everyone,
I need advice.
Liam has had his g tube for 3 weeks, in the last 2 weeks he has been
unsettled at night(on pump feeds) and leaking around the tube, we
think this is a pressure build up, and he is going to have tests on
stomach emptying and bowel movement??What are the treatment options
if he does have slow emptying etc.
How will we ever get enough calories into him if his gut wont
tolerate what he is having now(less than 700mls a day)
Any advice appreciated
Jody (mum to Liam 1year next Wednesday, 5.9kg 65cm-sorry cant
convert to lb and inches lost my chart)

Jeanne,
Dr. Harbison (Dr. H.) is an endocrinologist at New York Hospital.
It is on 68th and York in Manhattan. She is one of very few doctors
in the world who know a lot about RSS. In fact, I would venture to
say that RSS has become her specialty. I have been taking Max to see
her since he was a year old and she had maybe 10 RSS patients. She
used to try to schedule us all on the same day so we could network
and she could compare kids. It was a long day, but I met a lot of
really nice people that way.
Those who see Dr. H. know that she is to the point and almost
abrupt. She used to be worse, but has worked hard on her bedside
manner. She is truly dedicated to these kids and their parents.
Did you know that she donates her time and efforts each summer for
the MAGIC conference? Jennifer and I spend hours and hours with her
while she examines and confers on many RSS kids and those who may or
may not have RSS. It is all at no charge to the parents. She does
not stop working until she has seen all those who want to be seen.
I don't think I went to bed before 1am each night last year.
The study that Dr. H. is, I believe, related to this "new" info
about that hormone or whatever it is that is responsible for weight
gain and appetite in people. Ghsomething or other? She wants to
test families to see and compare levels of the stuff in their
blood. I have an email from her that explains it better. I just
did not realize that she wanted all of us who see her to participate
in the study. I don't care. My body won't miss the blood and maybe
it will help someone in the future.
I know that your daughter is not yet confirmed with RSS, but I would
really, really recommend that you take her to see Dr. H. and get a
firm opinion. If you are seeing Dr. Starkman at MMH, then you may
never have a confirmed diagnosis. He is a bit soft about giving out
diagnoses. And our experience with the GI doctors at MMH has not
been good, but I know that there are different ones there now, so
that is not fair for you to use as judgment. It's just a shame
that you are so close to Dr. H. and you have not seen her. All you
need is the one time visit to confirm or not whether your daughter
has RSS. You can always follow up closer to home. It's just that
we tried that and it did not work.
Anyway, I hope I have helped. You can email me privately if you
would like or we can continue here. It's your choice.
Jodi

I know you all have been talking about this long before I got here, but
what exactly is the Dr. H study, and what exactly is her practice? Is
she a GI doc, a neurologist, a genetecist, an endocrinologist? Im
guessing, have no idea. If you want to email me off the list to answer
because it's been talked about here too much, that's ok too. Just
curious. Like I said, Rosemarie doesn't even have RSS Dx, yet, but I
like to keep up!
Jeanne

Katy
When you see Dr H. (or anyone going before then) ask her if it's feasible
and reasonable for those of us further away than New York to participate.
She may appreciate the larger data base. If so, I would also wonder at the
end of her study will she share the results with her patients?
When Adam and I and his dad were part of the chromosome 7 study group that
eventually discovered that connection to RSS, we were given a little verbal
explanation afterwards. I learned the whole explanation and that Adam did
not have that marker. If you participate in a study it's always good to
"learn something" from it for yourself too.
Debby

Gee, thanks, Katy. This means that when I take Max to Dr. H in
June, she will want MY blood, too. I guess I'll make things easier
and make Michael come with us so that he does not have to do what
your husband is doing. I hate having blood drawn. Then again,
maybe Michael will buy me a present like we do for Max when he gets
a needle!
Jodi

Judith,
Is it Dr. Laquaglia who will be doing the surgery? If so, he is
wonderful. He has operated on Max twice and I just love him.
As for getting to speak to Dr. H in detail while Jason is in the
hospital, don't count on it happening too often. I have found that
I actually get less out of her in the hospital than I have over the
phone or in person at a regular visit. She tends to leave a lot of
the visiting and checking up to her fellows. If you can get Dr.
Marshall involved, I think he is the best one there.
As for food in and around the hospital, it is easy to find. The
nurse's station will have many take-out menus so you can order in
at any time. McDonald's even delivers! If it is a nice day, there
will be a lot of vendors around the outside, too, mostly in the area
between 69th and 70th Streets. There is a deli on the corner of
York and 70th and I believe it is open 24 hours. There are also a
lot of restaurants in the area. I'm bad when Max is in the
hospital. I don't leave him for very long. I usually just settle
for a sandwich or soup from the coffee shop at the front entrance
and leave it at that. Most people order in, though.
I really wish you the best of luck. Things will really be okay. I
hope that you are able to have a private room in the section that
Dr. H. uses. If you are down the hall, you will have to share a
room. At least there are full bathrooms in each room now. That
makes things easier.
If I can answer any more questions, please let me know.
Jodi

Hi Alison,
Here is how Dr. H . told me to handle the blood tests (since I typically go with
Paul to NY without my husband).
- We will be back in August. At that time, both my blood and Paul's will be
drawn.
- Before we go, no more than 24 hours, my husband will get his blood drawn
locally. Dr. H. wrote out a prescription for him to get the blood drawn and the
# of vials she needs.
- We will bring up his blood vials in an envelope she provided.
- Then, she submits all of our vials to the study protocol.
When I get home tonight I'll find the write up on the study and share it with
all of you,
Katy

Jeanne,
I have little to no internet fear. This may change when we move. But.
for now I am so far removed from the world that it doesn't bother me.
Right now we are in Anchorage, AK. But, we will be moving to MD soon.
For right now N. NJ seems close. We look at the map of the states and
every thing seems close...for now. We have just started down this
path as well. The only solid answer we have so far is that Joshua is
RSS, but no test have been done to prove this. Just his small size,
and his looks. We have on going doctor problems. My husband is in the
Air Force and our medical needs are "met" at the military clinic.
Worse then dealing with an HMO. We see a GI that is military, and our
ped. is military, but the Endo we saw last week was an outside
doctor...what a mess. We needed tests done, blood can only be drawn
at the military clinic. The endo wanted another CF test done...but
those CAN'T be done at the clinic. Like I said its a mess.
good luck
Cami

Hi,
We live in Fair Haven, NJ.
Our son sees Dr. Harbison in NYC
My parents live in MOrristown, and in fact, Paul is staying with them there
today.
Jodi (Max's Mom) lives in Morris County too!
Katy

Hello all
I understand that there are a few other folks here from New Jersey. Is
it acceptable practice to ask where you are? I'll start (have little
remaining internet paranoia!!!)
I'm in in the middle of the northern part of NJ. Most of my doctors are
affiliated with Morristown Memorial Hospital (which now has a Children's
Hospital). The docs we see (or have appointments this century to see!)
are her priamary care pediatrician, a ped neurologist, a GI doc, a
genetecist, an endocrinologist and we have an appointment for a swallow
study and/or full eval at St. Joe's in Paterson. We are also in
Somerset County's EI program.
How about you all?
Jeanne

Jenniffer, My 4 year old will be 5 next saturday. When she was born she only
weighed 5 pounds and the doc said she is perfect right now. I'm not sure how
tall she is but i'll have to figure that out cause kelsey is only 50 inches.
My husband and i both knew that the youngest was going to blow by kelsey just
by the way she was growing! The school finaly called me and they are in the
process of doing that testing on kelsey so things are finaly moving forward.
I still don't have her medical files yet, i gave them untill june 6th so
hopefully i will get them soon! Thank Jackie

Hi Chris,
Can you email me privately. I have a few questions (mainly puberty)
that I hope you can help with. My private address is
abarrie@....
Adrianne
:

Hi all,
Thanks for all of the support. Jason is scheduled to go into the
hospital on Monday with testing on Tuesday and surgery on Wednesday.
Although we still can't figure out why continuous feeds is a good
idea, we are going to order the pump that Dr H wants so we will have
it ready. We decided that it would be easier to talk to her about it
next week while Jason is in the hospital rather than over the phone.
We are currently in the midst of creating a schedule of people to
watch our daughter who is going to freak out when she doesn't see
mommy for most of the time that Jason is in the hospital.
BTW, does anyone know what food is around the hospital (other than
the cafeteria)? Although Jason will be taken care of, Steve and I
will need to eat also.
We will try to let everyone know how we are doing.
Judith, Steve, Jason (RSS) and Rebecca (non RSS) 27 mo twins

Irene
THANK YOU. I'm going to go tonight to get that newletter...
good advice...
Jeanne

Melissa
When did madison start to babble? This is one of the docs main concerns
with Rosemarie, that in addition to not having an appetite and not
taking in enough calories through the bottle AND not being able to eat
any solids, she isn't babbling at all yet. No consonents at all, except
the rare "p" when she is blowing. She just started the open vowels at
about 10-11 months. She is now 13 months.
THANKS and welcome to the group
Jeanne
She walks,

Hello Jeanne,
Regarding your question of which type of tube to go with, a few
thoughts....
First, most of us, RSS or not, who have had tubes placed in our
children believed (or WANTED to believe) that the tube was just a
short-term thing to get us 'over the hump'. I can't tell you how
many families I have met with tubefed children that felt this way
when making their decision. If that is your feeling, then you may
be thinking the NG tube is the way to go. Less invasive, easily
undone. Unfortunately, most children who need tubes need them for
longer than 3-4 months. I never would have believed that my daughter
would still have her tube at 5. She uses it much less now, but she
still needs it without a doubt.
The maintenance on an NG tube is far greater than on a G-tube. It
has to be replaced regularly. Replacing it is a very unpleasant
experience for the child. Our daughter's NG tube had to be
replaced weekly. I don't know what the current protocol is. But
she used to scream bloody murder while it was being threaded through
her nostril and down her throat. When she screamed, the tube would
inadvertently get threaded through her nose and out her mouth. It
was horrible.
The NG tube is also an irritant in the child's throat. It does
impact swallowing to a some degree. If the child has any oral
aversions, this doesn't help matters.
Also, (and this is ultimately what pushed us to go with a G-tube), a
toddler is learning mostly via his mouth. Mouthing his fingers and
toys is a critical step in their development at that age. Anything
that makes the mouth unpleasant detracts from this important
phase.
I am involved in a support group in New England called Kids with
Tubes. We have a newsletter that you will probably find very
helpful given where you are with your daughter. The issue is
called "The Decision to Place a Feeding Tube" It includes several
personal stories from families (including one with RSS), and it
includes a terrific glossary and articles from the medical
community. It is accessible at www.kidswithtubes.org
No one ever dreams of having a child with a tube. On the other hand,
when it is necessary, it is a true lifeline.
Good luck to you,
Irene
Mom to Emily, 5

Jeanne,
First you have to consider the results of all the tests that are
going to be done. You have to have to GI testing to see if there is
a delay in your daughter's stomach emptying, to see if she has
reflux, and to see if there is anything else causing her to not gain
weight and have an appetite to eat what she should for her age.
Now, the question of ng tube vs. g-tube or doing one before the
other. Well, you need to have proof that the feedings will work no
matter which one you try. If the GI testing is okay and there are
no problems there, then my advice would be to go straight for the g-
tube. My son is now almost 14 and had an ng tube for 4 years. G-
tubes at his younger age were just not done for kids like Max, who
looked healthy and were intelligent and alert. He seemed to be the
one who was breaking ground for all the others. Now g-tubes are
used all the time.
I guess what I want to say to you is that it is much easier with the
g-tube. While you don't want your child to have surgery, at least
it is really a simple procedure with little discomfort for her, but
some managing and learning for you - whether it is an ng tube or g-
tube, you still have to learn and manage, though. The advantages
far outweigh the disadvantages - no one will know it is there!!!
Max used to get stares or looks of pity when people saw him. I had
so many questions to answer! Once he had the g-tube, it became a
private thing that most people never knew about. We were so happy.
So, that is my advice. If you have to choose, then go for the g-
tube. There are certainly enough people on this listserve to help
you with questions, frustrations, etc. My favorite "hint" is to
layer the bed/crib/mattress with a waterproof mattress protector,
sheet, waterproof protector, sheet, etc. That way if the pump leaks
in the middle of the night, you don't have to remake the bed. You
just peel off the two layers and you have a bed ready for the next
shot.
Jodi

hello all...
What do your nutritionists and docs recommend in terms of calories per
pound (or kilo) per day.
I've heard a few things, but the most consistent seems to be that for a
1 year old, for maintaining current rate of growth, it is 100 calories
per kilo per day. To gain the extra weight needed if growth is lagging,
I haven't been able to find a consistent answer.
Rosemarie is 13 months old, and weighs 16 lps, 4 oz (approxiamtely 7.4
Kilo) This would mean about 750 calories per day to maintain, and more
to grow to catch up. but how much more? Any ideas? Will be talking
more to the GI doc about this at appointment next week, and there is no
rush on the answer because the 650 she gets is the max we can do on a
regular basis at this point anyway...
THANKS
Jeanne
THANKS
--
squirrels in the attic... any advice?

Katy,
Thanks for the explanation!
Inga

Hi all!
I am very new here, and am in the process of reading everything I can
get my eyes on about RSS... Most recently, we have seen a GI doctor and
a neurologist... we have appointments (WAY out there in terms of dates)
for a swallow study, an endocrinologist and a geneticist... I know
you've all been down there roads before.
This question is specific...
I'm wondering what you all have to say about the different feeding
tubes. Rosemarie is 1 now, and we are STILL testing her, looking for
causes for her many difficulties, but don't seem to be finding any
causes. (her doctor remains convinced that this is the result of the
drug I took for my clot while pregnant and nursing; ot perhaps the thing
I have that caused my clot... the new doctors are looking for even more
and more things, including but not limited to RSS and IUGR).
It looks like we are headed for a feeding tube. I am getting different
opinions from the different doctors about whether to start with a nose
tube and then move on to the stomach tube, or just go straight to the
surgery.
What are your thoughts? What questions would YOU be asking the doctors?
What experiences have you had with feeding tubes? Please don't hold
back thinking "well, this is too basic, too obvious, she MUST already
know this..." I am looking for the kind of info you can only get from
folks who've been there!
THANKS THANKS THANKS in advance...
Jeanne

Hi Steph,
I found www.oldnavy.com that site usually has "Big Kid's" clothes but
in small sizes. Shoes, I wouldn't be able to help you there, the only
place I still buy from is www.payless.com. Hope this helps.
ali

Judith,
The only experience I can draw on is Jake's. We only do continous
feeds at night. During the day we rely on whatever food he eats by
mouth, plus some bottles in between. The only time we use the tube
during the day is when he is sick, because he refuses to eat anything
by mouth. Jake was gaining nothing for months before the tube was
placed and once we started continous overnight feeds and kept his day
schedule the same, he has gained wonderfully every month!
I wonder if Dr. H would consider a trial run of just using the tube
at night. I know how hard you and Steve have worked on getting Jason
to eat by mouth and it seems to me that you might lose your momentum
if he was continously fed by the tube. I know there are others on
this list that have used continuous day and night tube feeds, maybe
they can weigh in and give their experience.
Hope you don't mind my two cents worth. I could be totally off base
here.
Alison

Jennifer,
Thanks for checking. I look forward to your next post.
Leslie

I have learned that the "normal gene checks" done at birth are very
superficial and do not get at detailed genes.
Paul's doctor said the chromosomal checks are like being in a plane 30000
feet high and that specific gene checks are done at a very close level, and
are not typically done.
For example, chromosomal checks can quickly find things like Downs Syndrome
or extra chromosomes.
Even unidisomy of Chromosome #7 isn't done during typical gene checks. It
is only done at special request.
Katy

Tim had a 'normal' gene check when born - is this something special, or just
something they didn't know to check for 7 years ago?
Inga

One thing that has helped us it seems is adding the Udos's oil (135 cal/tbs and
very digestible and easy on his liver) just before Tim's bolus feeds -- the GI
in LA said we could add 2 tbs at night and 1/2 tbs before each of his 4 daytime
boluses then check triglicerides. We have been doing better w/the night pump
with learning to take the crimp off the connector tubing to prevent it
shutting off pump by momentarily pinching shut in the night and keeping
relatively new tubing. Thus I haven't checked triglycerides yet, but I may do
it anyway soon, since we have added oil in the day. He seems to be gaining
very well (over 5 pounds since January, and at 20% few cal per kg - doesn't
waste so much energy fighing us about food, perhaps? Or maybe its the GH...).
He needed a continuous pump for maybe the first week or so, I can't remember...
Inga

To the RSS Listserve, from Jennifer Salem:
As per your request, I forwarded the question regarding the recent APP newspaper
article on GHRELIN and whether or not there might be a link between it and the
lack of appetite in RSS kids. For those of you planning on attending the MAGIC
convention, put your seatbelts on!!!!! Read below! Jennifer
Dr. Harbison Replies:
I have been thinking about a possible link between GHRELIN and the appetite
defect in RSS for over a year. At the meetings I shall be discussing a protocol
that a colleague and I are working on to look at affected genes in SGA and RSS
children. To participate in the study, the child and all family members need to
sign our consent and have blood drawn for DNA. GHRELIN is one of the genes we
will be looking at. The problem is that the gene for GHRELIN is not on a
chromosome that has ever been implicated in RSS. It is possible that GHRELIN
function or control is affected in RSS children, but this is much harder to
measure. We will also be measuring GHRELIN and leptin levels on the patients in
the future.
Dr. "H"

Wow, do I feel sheepish! Paul (and Tony and I) are signed up for Dr H.'s
study! I guess I never read the fine print..she asked if we would
participate; I said sure, I signed consent, and in August the 3 of us will
get our blood drawn. I never asked her what the specific genetic study was.
I'll find the text of the protocol and type it in for you so you can see the
actual research question.
Katy

Eileen,
I live in ohio. I don't know anything about any of these programs, this is
all knew to me, half the time i'm not even sure what i'm doing but my gut
tells me to just keep pushing! Any info is great. Thanks Jackie

Hi all,
Jason's one month grace period from Dr. H was up yesterday. The good
news is he gained 1/2 pound in 1 month up from 1/2 pound in the two
months before. The bad (and expected) news is that the gain wasn't
enough and a gtube is in our immediate future.
We had been looking to do the surgery elsewhere even though we are
close to Dr. H. It would have been much more convenient with Jason's
twin sister at home and our families much more able to help. Also,
the parking situation is abysmal in the city.
Anyway, Dr. H convienced us that we really should do the surgery at
her hospital since she will be right there.
The biggest "confusion" to me right now is the fact that she wants
Jason on a 24 hour/day pump. She said that he needs 120/cal/kg/day
which for Jason would mean about 900 cal/day. He currently consumes
625 cal/day in liquid alone plus he does eat. So, I don't understand
why he would need a 24 hr/day pump.
I will be calling my insurance company today to straighten out a few
things like the pump and supplies.
Also, Dr H wanted me to speak with Leslie Lacey since her son Jordan
was the last one she did. Leslie, please email me.
Thanks all,
Judith, Steve, Jason (RSS) and Rebecca (non RSS) 27 mo twins.

I can't wait to see you guys at the convention again! Will Jon tell
Lindsay about his tooth, please? I keep telling Lindsay that I was
almost 7 before I lost my first tooth and so was daddy. Genetically,
early tooth losing is not in her genes!!! Jen

Donna - what a great idea. My daughter's class read a book on a
little boy with diabetes (her classmate has diabetes).
Any writers and illustrators out there? Jennifer

there anyone on this list or know someone who could possibly create a
story line of a child/children with RSS and dealing with real life
issues? It could be published and sold through MAGIC.
purchased several books written for children explaining food
allergies and what they do to be a part of the group and not feel
left out by their peers. The series of books I have are written
about an elephant named Alexander and he is allergic to peanuts. I
also found a movie about Alexander and his peanut allergy. At the
end of the movie there are several children who talk about their
allergy. These books have been a great learning resource for the
kids at Katherine's preschool. She really enjoys reading the books
because she can relate to Alexander, no peanuts, medic alert
bracelet, only foods approved by mom, etc.

Jessie's endo is correct, and Dr. H agrees, that she does not believe
in doing stim testing prior to doing GH unless insurance requires
it. She told us that all it can do is create one more reason why an
insurance company would reject GH (when the majority of RSS kids test
normal for GH). But I know that there are insulin levels and other
things that are tested regularly. I am going to call her office and
ask for copies of all Lindsay's test results and I will let you
know. HA! Jennifer

Hi everyone one of the mums from the IUGR group is having trouble
buying age-appropriate clothing for her little 9-month old daughter -
she has only been able to find pastels. Shoes too - Katy what was
that website??
Thanks in advance to all you wonderful helpful people
{{HUGS}}
Steph

Hi Jeanne,
Welcome to the list. GHD stands for Growth Hormone Deficiency. Our
son does not have RSS, but he does have GHD. This basically means
that his body does not produce enough Growth Hormone on its own, and
he has to receive growth hormone therapy, which in our son's case
involves injections six times a week. Other people on this list will
be able to fill you in on the use of growth hormone therapy in
children with RSS. From what I have learned from them, children with
RSS produce enough growth hormone, but apparently are not able to
utilize it like they "should". However, I will stay out of this
topic because it is not my "area" - I will let the parents who have
children with RSS answer with their expertise. I am on the list for
growth hormone deficiency as well. The reason I have joined the RSS
group also is because our son is very tiny (thin), and a lot of
people here who have children with RSS have been helpful in providing
answers to questions I have re weight gain, and many more things.
You will find many wonderful people here! Gail P. in Canada (not to
be confused with Gayle Duca in Florida, who is the division consultnt
for GHD for MAGIC :-)

Gail
what is GHD?
Jeanne (also new, and even further south in US!)

I love that book, and many others by Max Lucado... He has another called
"you are loved"...
My nieces and nephews did a stage production of "you are special" a few
years back where the things just kept sticking or falling off. It was
great!
Thanks for the reminder...
Jeanne

Hi everyone,
Just wanted to let you know I will be on a brief business trip tomorrow.
So if anyone has any questions for me, I will respond on Thursday,
when I return.
Ken M
:)

Hi Jackie,
Start with the evaluations just like you are planning and inquire
about getting the evaluation done in fairly short order. Your daughter
may not be able to do it all at once. My only concern would be if they
did part now and then another part in the fall. If you are dealing
with a phyciatrist, they should be able to answer all your questions
about how long the testing takes and how long to get the results etc.
My experience has been that the evaluating only takes a couple of
hours, maybe a little longer. However, like some doctors, it is
sometimes hard to get the results in a timely fashion.
You might also want to inquire about how much experience the
phyciatrist has with testing small children etc. especially with
whatever you are concerned about with your daughter. It can't hurt to
ask a few questions. They worst they can say is that they are too busy
to answer now.
Like I mentioned before, many, many schools and teachers do an
excellent job. My experience has been otherwise, that is why I advise
some vigilance once she starts receiving services.
Given the concern you have shown here about your children, I am 100%
confident you will do ( and are doing )an excellent job of advocating
for your daughter.
I hope this helps.
Ken M
:)

Boy Ken i don't even know where to start! Do i wait untill i here from the
school again? They said they could let me know whats going on i think by the
end of this week. I'm not real sure how long it actualy takes but the
phyciatrist was going to do her best to get in as much as she can! I know her
teacher had alot of input on how she was doing in school, so that might make
it go faster! Thanks Jackie

in the last two weeks we have seen Ben's pead and his genetist(sp!),
first the pead - "Ben will eat when he is hungry" is the best i could
get.
the genetist still could not give us a definate dx of anything,
suggesting that he has characteristcs of about 5 different syndromes,
but he is presenting Ben's case to a review board to see what they
think. one thing that he did say, which i have often suggested to a
friend of mine, "he is perfect in size and development - then you
look at his date of birth, he was born 12 months too early!"
we are to see an endo for the 1st time in july, i am not holding out
much hope.
enough wallowing, planning for a party on saturday for his 3rd
birthday, hoping for decent weather so we can hold it outside, i did
want to hold it on sunday, however apparently there is a football
match that day!(uk joke!)
Justina

Hi Melissa and Jeanne,
Welcome to our group. I hope we can answer all your questions.
I apologize for the delay in approving your first messages. I have
this list set so that new memebrs are moderated until I can be sure
that they do not have an alteria motive. It has happened in the past.
So I try and keep those distractions away from this list.
Since this was a hoilday weekend, I, of course, planned more things
than I had time for and keeping up with this group was not one of the
items on my list.
Again, I apologize for the delay.
Ken M
:)

Hello... I'm new and tried twice to post my introduction. I did receive
emails from the group, but mine is not going through so I'm trying this.
Please do not reply to this, I am only checking to see if it is going
through. I know I have the address correct, I checked it many times.

Hi Deb,
Have you ever read "You are Special" by Max Lucado? The little
Wemmick guy (Punchinello)(they are all little, I guess, at least not
as big as their "maker"), goes through a lot, and in the end things
start to get better, but it doesn't say that all his problems
disappear. It has basically a Christian analogy, but it is also sold
through Scholastic, so you can take it how you want, the underlying
meaning being that we are all "special" no matter what others may
think, and when we start believing in that, in ourselves, it
helps. "The stickers only stick if you let them". Just wondered if
you were familiar with it, which you probably are, working in the
school. Punchinello learns how to cope because he is special! Gail
P.

Hi Deb,
Well, I managed to get signed-on. I am here! To those of you who
don't know me from the GHD List, my name is Gail, and our 11-1/2-year-
old son, Matt, is GHD. I am a member of GHD List. However, I decided
to sign-on to this list as a crossover because we also have issues
with Matt being so underweight, etc., and a lot of you who post to
the RSS list seem to have such good ideas re this, that I thought it
would be good to join you. I will probably be "lurking" a lot as
Matt does not have RSS, but may share from time to time. We live in
Ontario, Canada, not that far from Debby B., about 60 miles down the
road. P.S. Thanks for the endorsement, Deb. We Canucks need all the
help we can get! Hi Ken M.!
:-) Gail P.

Here's a link to an article from Science news on-line:
http://www.sciencenews.org/20020216/bob10.asp
Inga

Katy
I just wanted to thank you for sharing the link to the NY times
article with us - I downloaded the aticle and read it with interest -
I'm particularly interested in the apparant link between ghrelin,
appetite and GH stimulation. It would explain so much about Finlays
condition! I wonder if a Ghrelin deficiency in utero could explain
the SGA/IUGR as well - I have sent the article to our dietician and
endo - I'll be very interested to see what they make of it - I'd also
love to hear Dr H's reaction to it.
Thanks again katy
Julia

Hi Adrianne
Patrick had his appointment today with his paediatrician and we got his bone
age - this was taken last July and was 9 months behind which I think is
normal for rss. He will be due another bone age test done this summer. It's
funny but we have to ask for all these things - no-one seems to know a lot
about rss. It can be very frustrating.
Thanks for your reply.
Maeve

Hi All,
As some of you may remember we have struggled with granulation tissue
since Finlay had his g-tube placed nearly 6 months ago - we had tried
everything...eucalyptus oil, silver nitrate, tea tree oil.....
anyway although we were able to decrease the amount of tissue it
never went away completley...until now!!! The thing is I'm not sure
what has finally made it vanish - the only thing that has changed in
the last few weeks is that mark (husband) has been taking Finlay
swimming at the local pool which is very highly chlorinated and I'm
wondering if it could be the chlorine in the water that has finally
killed off the granulation tissue???
Anyway I wondered if anyone else had noticed a change in granualation
tissue after swimming in a pool?
Obviously I am thrilled that it is no longer a problem for us - his
tummy looks sooo much better now.
Julia

http://www.irsc.org:8080/irsc/irscmain.nsf

Jodi,
That is wonderful! I'm looking for the day that Max can do a victory lap around
the block....pushing his old wheelchair (while you get a ride)! I bet he will
rapidly improve as he gains more confidence. It is great that things are
finally starting to look up.
Chris
....................................
To get your own free faithmail account, click here to join:
http://www.womenoffaith.com

I think it's a great idea - not that I have that type of talent. But one place
that might give someone ideas (as well as address some of our issues, although
they mostly address achondroplasiacs) is the Little People of America resources
link:
http://www.lpaonline.org/resources_library.html
Inga

Jen,
We have talked many times about how much Lindsay and Jonathon are
alike. I just thought I should let you know that he lost his first
tooth at 7 years and 4 months. We were sitting in the waiting room
at Dr. H's office and he pulled it right out! Tooth number 2 came
out two days later. He asked me how much they were worth and I told
him the first tooth would bring in a higher amount because it came
out in New York, and everything is more expensive in New York!
Pattie

Hi all,
I know this may sound like a wild idea, (I'm brainstorming) but is there anyone
on this list or know someone who could possibly create a story line of a
child/children with RSS and dealing with real life issues? It could be published
and sold through MAGIC.
I attended the Food Allergy conference last month and while there I purchased
several books written for children explaining food allergies and what they do to
be a part of the group and not feel left out by their peers. The series of
books I have are written about an elephant named Alexander and he is allergic to
peanuts. I also found a movie about Alexander and his peanut allergy. At the
end of the movie there are several children who talk about their allergy.
These books have been a great learning resource for the kids at Katherine's
preschool. She really enjoys reading the books because she can relate to
Alexander, no peanuts, medic alert bracelet, only foods approved by mom, etc.
Donna (mom to Allison 13yr and Katherine 3yr both RSS)

Leslie,
Jake has been obsessed with the "Franklin and The Toothfairy" video
we borrowed from the library. I think he has watched it 100 times in
just a few short weeks. In it Franklin, who is a turtle with no
teeth, puts a fake tooth under his pillow in hopes of getting a gift
from the toothfairy. No such luck!
Maybe Jessie would like the video too. But, then again the premise
of the video is to show Franklin that he is growing up even though he
doesn't have teeth that fall out. So they go to the doctor to show
Franklin he too is growing up just like his friends are and they
proceed to measure his height as proof........so maybe it isn't the
best for RSS kids.
Alison

Don't you just hate it when you get a show/movie that you think we can use
with our kids and of course by the end all is solved. Like Arthur and his
tooth not falling out, or what Alison said about Franklin, and then he gets
measured. The message is right there.....but why solve it?? Why not end with
"even though it didn't happen for you........you still have wonderful
talents and gifts" It makes me so mad.
There's a new show on tv now that drives me bananas. Don't know if it's in
the States or only up here, it's called "George Shrink". Nice eh? It's about
a boy who is basically the size of a blade of grass but he manages his life
just fine. His parents and baby sibling are all "normal" size. But what
drives me nuts is that instead of George "realistically" overcoming issues
that arise from his small height, he has all these gadgets. Like a little
flying helicopter to get around in. Or he gets stuck in the grass and a
bunch of ants want to have him for lunch, he whips a rope and some tools out
of his sack and manages to escape. As if our kids have all these tools to
help them cope.
OH don't get me started.
Remember our discussion on Shrek? Well I finally saw it. And I am in love
with the movie. All my worry about Adam identifying with Lord Farquart (he's
like a dwarf). Adam just laughs his head off "mom look he's so small they
have to lower the table". Nothing hits him. He totally identifies with Shrek
and feels his pain that because he is ugly (ie. different) Fiona may not
love him. He understand that message much more. Sometimes I guess we worry
too much.
OK enough I gotta go to bed.
Debby

I thought the following information might be of interest to any
families that live in the DC metropolitan area (including Northern
Virginia and parts of Maryland). The Eric Fund (Eric C. Savader) is
a nonprofit group that gives grant money to disabled people to
purchase assistive technology devices. The deadline for applying is
at the beginning of June so if you are interested in please go to
www.ericfund.org
Alison

Hi Cami, we just got our RSS diagnosis too (well as close as the
geneticists are going to get to it without a test anyway lol); SGA
means 'small for gestational age'; IUGR means 'intrauterine growth
retardation'. You can have one without the other; being small for
gestational age means below a certain birth weight (small) at the
time of birth. Some families 'normally' have small babies, some
don't. So a baby born 6lb to a mum whose other kids were 6.5lb may
be considered small for gestational age, but not necessarily IUGR. A
baby born 7lb to a mum whose babies are usually 10lb isn't SGA but
would probably be considered IUGR. Does this make sense? It is
easier when it's obvious - Tessa was 7lb 9 full term, Darcy was 3lb
11 so obviously SGA and IUGR.
I've seen a website explain it really well, I'll go hunt it down.
{{HUGS}}
Steph
ps the doctors and nurses don't always talk to each other ;-)

Adam had lots of problems with the "tooth fairy" thing at school. Lucky
there is one episode of Arthur where Arthur hasn't lost a tooth yet, and he
feels bad. Unfortunately by the end of the episode he does loose his tooth
(why can't they just leave well enough alone) but still that helped Adam in
some of the rough spots. The other thing was that I never made a big deal
about Colette's visits from the tooth fairy.
Last night at Church Adam leaned over and said to me "will the tooth fairy
come when I have the tooth pulled out". And I said "yes the dentist gives
you a little treasure box to put the tooth in and in the morning you get
money." He says "wow!! how much?" I said "oh I don't know 2 or 3 dollars is
what your sister got". And he says in this loud voice "WOW! The tooth fairy
must be RICH.....wish you had that job mom!!!!!"
Kids!!!
Debby

Jodi,
Boy do I feel for you and Max. I had two food surgeries in six
months and it was the most painful thing in the world. And my
procedures were no way as severe as Max's. I remember the
painkillers, they were my best friend. I think you hit it right on
the head when you mentioned it could be the distance he walked which
caused the pain. The whole reason I needed a second surgery was
because I pushed myself and was back to work 3 days after surgery and
my foot never healed correctly. Later I found a new surgeon who
refused to do another surgery unless I could commit to missing work
for six weeks. I did it and I have been pain free for seven years.
I hope you are planning a well deserved big vacation for yourself
after this is all done. You have been through so much and I know it
breaks your heart to see Max in pain. You are in our thoughts.
Alison

Hi,
I had a question about SGA. When Joshua was born he was 5 lbs 14oz. I
remember the nurse saying that he was NOT SGA. But, I was looking
thru his records the other day and saw it noted that he WAS. What
makes a child SGA? I delivered at 41 week, 4 days. My other two full
term babies were 8 lbs 9oz, and 8lbs 12oz. My first baby was pre-term
at 30 weeks, and she was 3lbs 10oz. SO, how do they classify a child
SGA?
Cami
p.s. I think we have a confirmed RSS diagnosis...The Endo took one
look at josh and said "when was he diagnosed RSS?". No one has been
willing to give us a firm RSS diagnosis. The Endo said he was 99.9%
sure. Only one other doctor has ever brought up RSS and that was his
GI when I asked for the referral to the Endo. We will get a full
report from the Endo by the end of the week.

Allison,
You are so incredibly organized and thorough. Thank you for the test
listing. I am going to go over it with Jessica Endo. She did NOT do any
blood testing prior to starting Jessie on GH. This left me feeling a bit
uncomfortable.
Our Endo said Jessie being Classic RSS was probably not GH deficient,
but hopefully would be GH receptive. (She is impressed with Dr. Wollmann's
research and protocol) She said that testing (GH levels) would/could make
insurance coverage more of an issue or fight for us and that Jessie was
covered under the new ruling of being SGA at birth and not having caught up
by 2.
Anyway-thanks to all of you who responded to my inquiry about testing
prior to starting GH. It looks like most Endo's do test. I hope that we
haven't really missed something here. But I have to say it was comforting to
read that Dr. Crawford did not do specific tests prior to starting (Thanks
Irene).
So a big thanks to all of you who responded. And a big prayer that we
haven't missed an important step in our daughters medical care. It certainly
is a struggle to trust the medical professionals sometimes.
Leslie

Debby - that is great news. 10years old. I can not imagine
suffering another 4 years. Lindsay is 6 yrs old and in Kind. here
the make a big deal about losing your first tooth. Most of her class
has lost at least one tooth. Lindsay "believes" that her bottom
front teeth are "wiggling." We are going along with her. But those
teeth are not moving at all!!! I feel for her, as BAD as she wants
to lose a tooth!!!! But I sure feel bad that Adam will have to go
through such dental work! jennifer

that I want something done because at almost 10 years of age he has
all his baby teeth still.
That's not going to be great for Adam and he's going to rebuild his
molars since they are disintegrated from use for so long.
She has had all her baby teeth pulled. The last ones, the eye teeth
have come in way above at the top of her gums near the top of her
mouth (!!) she looks like she has fangs. So to get them in place,
she'll be getting a retainer to pull them in and down.
(they are permanent teeth....I never knew that...I thought they fell
out too). But he's been working on those 4 teeth for a few months now
and having that bottom centre tooth come through (since Tuesday???)
perhaps explains the lack of growth over the past 4 months (all
energy going to the teeth). Well I can kid myself!!!!

In a message dated 5/26/2002 2:42:35 AM Eastern Standard Time,
magicrss@... writes:
talking about an older brother or sister and the toothfairy's charity! Haha

Hi Deb: I am so gald that you finally have some answers with the teeth. Just
stay on top of them until they do everything that needs to be done. There is a
chance that the adult teeth will fall into place still. Keep me posted. Are
you going to Convention????? Marion
"Deb B." <debbbail@...
I had Adam at the endo on Tuesday and I am bitching (basically) that I want
something done because at almost 10 years of age he has all his baby teeth
still.
Today was the annual check up at the dentist and cleaning. Low and behold what
does the dentist find?????
His first adult tooth has broken through behind his bottom front baby teeth!!!
IT'S THERE!!!!!!
I could just scream from the roof tops I'm so thrilled!!
Now the bad news.......he has to pull the baby teeth to make room. That's not
going to be great for Adam and he's going to rebuild his molars since they are
disintegrated from use for so long.
Colette will be having her last baby tooth pulled at the same time. She has had
all her baby teeth pulled. The last ones, the eye teeth have come in way above
at the top of her gums near the top of her mouth (!!) she looks like she has
fangs. So to get them in place, she'll be getting a retainer to pull them in and
down.
Have a feeling since Adam's *NEW* tooth is so far back, he'll end up the same
way.
Guess it runs in the family (the father's!!!!)
But right now I don't care!!! He's starting to get the teeth!! I am
thrilled!!!!!
So perhaps the GHT was instrumental in getting this going. That is making the
jaw slightly bigger.
Oh and now I have been told that all those 6 year molars are in (they are
permanent teeth....I never knew that...I thought they fell out too). But he's
been working on those 4 teeth for a few months now and having that bottom centre
tooth come through (since Tuesday???) perhaps explains the lack of growth over
the past 4 months (all energy going to the teeth). Well I can kid myself!!!!
I'm off to celebrate!!!!
Debby

Go Max GO!! I am happy for you and It's about time things go good for you and
Max!! Hope to hear more good things.
Marla

WELL!!!!!
I had Adam at the endo on Tuesday and I am bitching (basically) that I want
something done because at almost 10 years of age he has all his baby teeth
still.
Today was the annual check up at the dentist and cleaning. Low and behold what
does the dentist find?????
His first adult tooth has broken through behind his bottom front baby teeth!!!
IT'S THERE!!!!!!
I could just scream from the roof tops I'm so thrilled!!
Now the bad news.......he has to pull the baby teeth to make room. That's not
going to be great for Adam and he's going to rebuild his molars since they are
disintegrated from use for so long.
Colette will be having her last baby tooth pulled at the same time. She has had
all her baby teeth pulled. The last ones, the eye teeth have come in way above
at the top of her gums near the top of her mouth (!!) she looks like she has
fangs. So to get them in place, she'll be getting a retainer to pull them in and
down.
Have a feeling since Adam's *NEW* tooth is so far back, he'll end up the same
way.
Guess it runs in the family (the father's!!!!)
But right now I don't care!!! He's starting to get the teeth!! I am
thrilled!!!!!
So perhaps the GHT was instrumental in getting this going. That is making the
jaw slightly bigger.
Oh and now I have been told that all those 6 year molars are in (they are
permanent teeth....I never knew that...I thought they fell out too). But he's
been working on those 4 teeth for a few months now and having that bottom centre
tooth come through (since Tuesday???) perhaps explains the lack of growth over
the past 4 months (all energy going to the teeth). Well I can kid myself!!!!
I'm off to celebrate!!!!
Debby

Hi Leslie,
Believe it or not, we did not have any blood work done before
starting GH. After reading Alison's message, it does make me
concerned....BUT I have so much confidence in our endo. Dr Crawford
from Mass General (Dr. H's mentor).
At one point last year, he did have us give a urine sample for
testing of hormonal surges related to puberty (not sure exactly
what). And I remember he said that the urinalysis is equally as
good as blood work but that it is rarely used anymore and so they
must rely on an outside lab to do it. This test was done because
Emily was having underarm odor.
We do a hand x-ray at least twice a year.
Irene
Mom to Emily, 5

Jody that is great news.
Congratulations to Max!
{{HUGS}}
Steph (Darcy 30 months