Russell-Silver Syndrome and Eating

Why won’t my RSS child eat?
My RSS child takes one bite and says she is full. How do I get her to eat?
Why does my RSS child only like sweets and salty crackers?
How will my child gain weight on tomatoes and raspberries?
Is feeding therapy necessary?
Does my child need a Gastrostomy tube (G-tube)?
Where do we go for help?

There are a myriad of these types of questions that run through the minds of Russell-Silver Syndrome caretakers, and none of them are unwarranted. From a personal perspective – it gets easier. At the age of 8, my Russell-Silver Syndrome child actually tells me when he is hungry! But as a toddler, following him around with a cracker covered in cream cheese in my hand was a daily routine. And more often than not, I was following him around with M&M’s!

There is no simple answer. Not for a Russell-Silver Syndrome child. The fact remains – MOST Russell-Silver kids are just plain not hungry. Yes, there are few children – such as Ian who would eat. Yet, due to a multitude of characteristics of an RSS child, eating is quite challenging.  And even if they ARE eating – the question still remains of whether or not they are getting enough caloric intake to sustain the needs of their bodies.

Why won’t my RSS child eat?

Honestly? He/she is not hungry. There are numerous reasons which can be attributable to the syndrome itself. GI problems can be one culprit – such as gut dysmotility, reflux (Gastroesophageal reflux disease or GERD), or delayed gastric emptying. Volitional (oral) feeding issues such as sensory issues and/or oral –motor delays can exacerbate the problem. And an RSS child that is facing fasting hypoglycemia (low blood sugar levels) due to their disproportionate head to body size may exhibit lack of appetite as they have already missed their window of opportunity to “feed” the body. And let’s face it, when a toddler has a choice of whether to eat or play – he/she most assuredly will choose play time. Yet, aside from the above characteristics, there is still debate about why an RSS child is just plain not hungry. I personally believe more research into the genetic components of the syndrome in years to come; will help to establish a better understanding of their lack-of-hunger.

My RSS child takes one bite and says she is full. How do I get her to eat?

If your child is suffering from reflux – you can guarantee she is not interested in putting anything in her stomach that will eventually come right back up again. If she hasn’t had a bowel-movement that day, or in several days – she has no room for more food. And if she has gut dysmotility, the food she ate for breakfast very likely has not been entirely processed through her system – resulting again in a “full feeling” in her stomach. If she just ate 1 hour ago – and did not eat a sufficient meal, her tiny stomach is not ready to handle more food. Although snacking can be very useful (Ian has snacks 3 times per day along with meals) – depending on your RSS child’s GI issues, a strict nutritional regiment is necessary to make sure she is getting the proper nutrition and calories.

Why does my RSS child only like sweets and salty crackers?

I have no idea! But, I do know that my RSS son, Ian, has the same cravings. He loved M&M’s as a toddler and it was a great tool during our nutritional therapy to encourage positive eating habits. He would get M&M’s as a treat after a nutritional meal. And, during his severe hypoglycemic issues (when he was too young for peanut butter) – we offered him M&M’s in an emergency when “food” was not available and we needed to keep his blood sugars up. Please note: I am NOT encouraging this tactic and got the approval from our nutritionist before we proceeded. I was extremely intuitive of my child’s behaviors before, during and after a hypoglycemic episode – and could discern what the best treatment option was for Ian during those times. I always recommend you discuss different options with your physician and/or feeding therapist before trying “home-made” solutions. What works for my child may not work for yours.

How will my child gain weight on tomatoes and raspberries?

Obviously, he won’t. He will get great nutritional benefits from vegetables and fruits – but, it certainly won’t be enough calories to sustain him. Incorporating these into his diet will certainly enhance his immune system and provide him with great vitamins – but, adding the correct levels of protein and carbohydrates is essential to a good nutritional diet. It’s important to note that RSS children have a different caloric intake need that that of a non-RSS child.

Is feeding therapy necessary?

If you are stressed out from the constant worries of getting sufficient calories in your RSS child, then I would highly recommend speaking to your physician about obtaining a feeding therapist. A feeding therapist will help augment current food selections (such as olive oil and butter on pasta / rather than just butter, protein powder mixed with ice cream and milk for a healthy milk shake, etc.) – and rather than you trying to discern the proper amounts – a feeding therapist will know exactly the measurements and daily requirements that will assist in your RSS child’s caloric needs.

An important note; however, is that we do NOT want our RSS children to be too heavy. This has always been a source of difficulty to comprehend. But, RSS children are actually susceptible to diabetes if their weight to height ratio is not properly maintained. The fact remains – RSS children are THIN. There is a balance that needs to be met for RSS children – enough calories to grow, but not too much to make them fat.

I always suggest to parents/caregivers, based on my experience, that it is crucial to finding a feeding therapist that will work with you and your RSS child to find the proper nutritional treatment based on the specific needs of the syndrome itself. I always remind them – RSS children are not genetically made like a non-RSS child. Therefore, they should not be treated the same – and we have to be vigilant about their specific nutritional needs.

It’s also an important fact that parents / caregivers understand that your RSS child’s failure to eat is not caused by you. Your child’s syndrome is not caused by you. However, instilling proper behavior during meal-times and snack times greatly increases an RSS child’s success for optimal eating habits and proper nutritional consumption.

Does my RSS child need a Gastrostomy tube (G-tube)?

This is always a hot topic of debate (in my opinion) in the Russell-Silver Syndrome community. Many RSS children (including my own) require the G-tube for nutritional intake. It isn’t necessarily due to the fact the child won’t eat. As in the case of Ian – he did eat. Unfortunately, even after tremendous efforts on my part to get him to take in enough calories to sustain his weight, it just wasn’t enough. To add to the difficulty – Ian had a rather large head to body size (his head was in the 85th percentile and his length was at the 5th percentile and weight at the 0-3rd percentile). We just were not able to “feed his brain” and he developed severe fasting hypoglycemia rather easily. More often than not, every time Ian sustained an illness of some kind (including a minor cold), his body could not manage and we ended up in the hospital.

I opted to get the g-tube for Ian for the following reasons.

1. Due to Ian’s disproportionate head to body ratio – Ian needed to maintain his blood sugar levels. In order to do this, he needed more calories to accumulate fat stores for better processing of sugars.  Once he received the g-tube, we accomplished this with overnight  continuous feeds.  And, occassionally, if he refused to eat a meal or didn’t eat enough – I bolused him. (Bolus is a term for feeding a child through the g-tube in a short period of time.  I used different concoctions approved by his nutritionist; i.e., olive oil, protein powder, Nutren, Jr. w/ Fiber, and pureed fruit.)
2. Ian needed more calories to help him “GROW”. Without proper nutrition and caloric intake – Ian was falling off the growth chart.
3. We were confident that his occasional reflux symptoms were attributing to his intermittent lack of eating. For some reason, I was able to document that Ian would “not be hungry” for about two weeks every month. Then, miraculously – he would eat like a little piggy for the next two weeks. Unfortunately, due to frequent hospital stays and illnesses – depending on where Ian was in his personal cycle of eating, Ian was not getting enough caloric intake to help him stay healthy and gain weight – AND grow.  Once he obtained the G-tube, we could maintain his blood sugars with continuous feeds – and during vomiting illnesses, the G-tube was very helpful in getting him back to “eating” volitionally again.
4. Mild feeding / mouth sensory issues.
5. Ian was termed Failure to Thrive.

The decision of a g-tube is not an easy one. We were given the option at the age of 16 months to proceed with a g-tube or start growth hormone therapy. As his caretaker, I was not ready to make either decision and waited. Ian’s issues surmounted and the decision became easier and much clearer, he needed the G-tube to survive.

But, this is not the case for some RSS children. Some have done remarkably well without the G-tube. I have met several adult RSS children that were not exposed to special treatment options when they were younger and managed without intervention.

Yet, some RSS children due to reflux and gut dysmotility issues, gag reflex and/or sensory issues, are desperate for nutritional intake. These children receive the J-tube which is inserted into the jejunum or small intestine rather than the G-tube that goes directly to the stomach.

It is important to consider all your options, speak with your pediatrician, gastroenterologist and/or endocrinologist and work together to make the decision.

Where do we go for help?

Your Pediatrician is a great resource. If you determine your Pediatrician is unable to comprehend the specific needs of your RSS child, seek another physician. Find a Gastroenterologist and Endocrinologist. Working together as a team is crucial for your RSS child.

Organizations such as The Magic Foundation, Global Genes, and CheckOrphan are great resources for rare disease. They have support groups and access to reliable related medical information beneficial for the RSS child. And finding other families and/or individuals who have a child with RSS is also advantageous – as their experiences can help allay fears and concerns that may or may not be warranted.