Parent booklet - page 2

Q:  How does this affect my healthy children?

A:  Your healthy children are not affected at all by Costello syndrome.  Their children will not be affected by Costello syndrome either.  The only way your healthy children or their children could have a child with Costello syndrome is if situation (2) or (3) listed in the previous question was to happen. – The same chance as anyone else.With what we guess to be about 250 cases identified WORLDWIDE, our children are the information from which the scientists and doctors are making theories!  If we use the numbers known in the United Kingdom to generalize, the prevalence is 1:500,000.

The Importance of Parent-to-Parent Communication

In addition to doctors and researchers observing our children for patterns, we parents have been important participants in developing a more detailed picture of what Costello syndrome is.  Our direct communication with each other speeds up the identification of a pattern, and starts an immediate database for a doctor interested in following the research. Here are some shared observations many parents notice but are still mysteries:

Our children are “hot-blooded.”  Whether it’s the metabolism or the heart issue (both can cause a person to be hot all the time) or something else, or all of the above, many of our children sweat a lot - even to be a bit “ripe smelling.”

ƒSome children experience unexplainable fevers.

ƒMany parents notice their child is very sensitive to sunlight and touch, particularly the hands and feet.

ƒ Most parents talk about difficulty sleeping through the night.  Some grow out of it, but many don’t.

We are still looking for a gastroenterology researcher! This system is just about always involved, and some kind of intervention is needed.  This is the most chronically difficult issue for our families.  Families of older children outside the USA and Canada tell of spending time in the hospital every couple of months or so, with NG (nasogastric) tube feedings and i.v.’s for dehydration, or feeding their children via NG tube at home. 

In the US particularly, most children have g-tubes (gastrostomy tubes), which go directly through the stomach wall for feeding either by “bolus” (pouring in the formula or meal in one sitting) or timed drip-feeding (requiring a pump, often scheduled for feeding overnight).  Some children have a surgical procedure, a Nissen’s fundoplication (fundo), performed, where a surgeon puts an extra fold in the esophagus just above the stomach to help reduce reflux.  But for some children, the “fundo” is not appropriate.

The rate of feeding has been a serious issue for many families.  Some need to start slower than 60cc/hr.  These children also have a hard time ramping up after “NPO” (nothing by mouth) for surgery.  Keep an eye on this and document it because you may need to provide evidence to your child’s doctor.

Good news, the feeding issues usually resolve themselves somewhere between the ages of 2 - 8 years old. There are a few cases where the child will clearly need the g-tube for life.  There are also a few children had mild feeding issues that resolved around when they were a year old!

Many children have difficulty with constipation.  All do better with more fluids, but it's hard to get a child –any child- to drink enough!  If your child is having trouble with constipation even with what the doctor can prescribe, you may find some useful suggestions on our website's Frequently Asked Questions (FAQ) section  on our website at  Low muscle tone (hypotonia) affects smooth muscles particularly, and the gut is one long straight muscle from lips to butt.