Russell-Silver Syndrome – Weight and The Growth Chart

It’s easy to tell a parent that they shouldn’t put too much emphasis on the issue of eating.  Russell-Silver Syndrome parents are continuously told to “not worry”, “don’t make a big deal about it”……and “by the time our children are going through puberty – the weight issue will level off and eventually cease to be an issue”.

Yet, watching a child struggle with the most natural of human functions is disheartening.  We forge memories with eating, we experience different sensations with eating (such as pleasure and distaste) but we also eat to survive.

One of the first indications that confirmed the diagnosis (at 9 mos) of Russell-Silver Syndrome for my son Ian was his lack of catch-up growth immediately following his birth.  As I continued to watch his inability to maintain his weight on the growth curve, I also began to witness symptomatic episodes of hypoglycemia.  He was sleeping through the night as a newborn infant, and he only ate 2 oz of formula at a time – most of it “spitting-up” during feedings.  At the age of 8 months he was still unable to eat baby food.  He became failure to thrive.  What was worse – his physicians ignored my pleas and lacked the knowledge to help him.

The growth chart proved to be one of the single most important factors for determining the health of my son.  It has become an essential tool for understanding how to manage my Russell-Silver Syndrome child.

Weight – Not a Perfect Science for RSS Kids

Weight gain is an incredibly sensitive subject for RSS children.  Believe it or not, the weight-to-height ratio is critical in managing weight.  Our children can not be too heavy – or they become susceptible to early adrenarche and possibly diabetes later – and they can not be too thin.   Finding an equal balance in nutrition and calories is essential.  Maintaining a weight-for-height ratio in the 10 to 25 percentile range is typically a safe range (each child has their “appropriate” range).   I am always careful to emphasize that Russell-Silver children more often than not are healthy when on the “thin” side.

Some of the options for RSS kids and families that need assistance in maintaining a “healthy” weight:

1.  Speak to your Gastroenterologist to determine if your RSS child is experiencing reflux delayed gastric emptying (DGE), etc.  These types of issues could result in your child not wanting to eat.

2.  Speak to your Endocrinologist to determine if your RSS child is prone to hypoglycemia or insulin resistance, etc.  Endocrine issues such as hypoglycemia can also deter your child from eating.  A child with low blood sugars – if too low – can affect your child’s lack of desire for eating.

3.  Speak to your Pediatrician to determine if your RSS child is having difficulties with allergies, food sensitivities, sensory issues, or other medical issues.  In the case of sensory issues – different textures (yogurt vs. applesauce) can be a deterrent factor for eating volitionally.

4.  Speak to your Cranio-Facial team (which will include a dentist – about the palate and other issues with the face.

Once you have determined all aspects of care that need to be addressed in correlation with absence of volitional eating – a proper framework for food intake can be more effectively addressed.

Ian had many issues…reflux, DGE, hypoglycemia, oral sensory issues, crania-facial issues (he has a 100% overbite and cross-bite) and behavioral issues.

Weight Management – The Reality

Let’s face it – it’s EXHAUSTING!  Running around the house with a cream cheese cracker in one hand and M&M’s in the other as a treat for taking a bite – was a constant in our home.  We were encouraged by our nutritional therapist to strap him in his chair during meal-times so he wouldn’t get off his chair.  We were constantly providing nutrition logs to determine whether or not Ian was getting enough calories, protein, carbs AND fat to maintain a healthy weight.  All the while making sure he wasn’t getting FAT.

As you can see from the above growth chart – he is currently between the 7th and 10th % for weight.  He received a g-tube around the age of 2.5 – the result showing a healthy response to the growth chart by following “his” curve.  The recent fall between 8 and 8.5 was the lack of Growth Hormone.  Loss of muscle tone and feeling “hungry” have contributed to the change in “his” curve.

The important thing to remember is that every RSS child – while they may have similar characteristics and symptoms of RSS – are all “different”.  They all have their “own” set of medical circumstances, personalities and Growth Curves.  We need to treat each child according to their own personal needs – but, also have an understanding of what “characteristics” may exist that will affect how each child is treated.

The Constant Challenge

Although I can honestly say that it DOES get easier the older they get; unfortunately, eating can still be quite the challenge.  At least it is for Ian.  Over the last couple of years I have encouraged Ian with the idea that if he learns to manage his eating habits on his own – we will remove his Mikey button (G-tube).  When you have a child who is as strong-willed and determined as Ian (just like his mother <wink>) this can be quite the challenge.

Recently, I asked Ian to finish his dinner.  He flat out refused.  He had one bite and decided he didn’t like it.  He’s 8.5 now – and believes he knows everything there is to know about life.  So, I told him he would get bolused (term for quick g-tube feedings) if he didn’t attempt to eat anything.  He said he didn’t care.  He wouldn’t help me either.   Ian’s newest and most proudest moment is when he is mad and tells me “I’m not eating for the whole day”.

It’s a constant challenge.

When they are babies don’t like what you are feeding, they push the food out of their mouths with their tongues.

When they are toddlers, they are too busy to eat.  They rather be playing with their toys.

When they are starting school, they rather eat their friends food than their own – and since this is not encouraged in a school setting, finding the right choices for their lunch bag becomes a difficult feat.

When they are in grade school they are embarrassed to go to the nurse to show them their lunch boxes.

And, the ultimate challenge?  Down right REFUSAL to eat.

Ahhh….the life of a child who was born not hungry.