Promoting Positive Feeding Behaviors in Children with Kabuki Syndrome
Due to advances in medical technology, more children are surviving severe prematurity, rare genetic syndromes and complex medical conditions. One of the first milestones that children must meet is the ability to take in nourishment safely and efficiently. For feeding to be safe and efficient, multiple physical systems must work together in an effortless, coordinated fashion. Children born with multiple complications are at greater risk for oral functional/feeding problems for a multitude of reasons. Just as with this larger population, children with Kabuki Syndrome also have multiple issues that may interfere with safe, efficient and/or pleasurable eating in the short run. These issues include structural issues such as cleft palatei, hypotonia, poor growth, dental or orthodontic issues, as well as sensory and cognitive issues, just to name a few. As overwhelming as this list may appear, there are many children with Kabuki Syndrome who eat safely, efficiently and happily. The key words here are efficiently and happy. The development of a positive relationship with foods must occur to promote long term functional eating skills.
The first step to treating feeding difficulties is early recognition of elements that impact eating and their potential secondary impact. When evaluating any child for feeding difficulties, the physical and/or structural issues that may interfere with safe and efficient eating must be identified and treated first. As reported by Dr's. Iida,Park and Iida in the 2008 Fall edition a medical journal, the incidence of cleft lip/palate in children with Kabuki Syndrome is higher than average. Children with cleft lip/palatal issues tend to have difficulty with efficiency of oral intake as they cannot build up enough oral pressure to expel the formula from the nipple and may require a non-traditional nipple or feeding method. This may result in lengthy feeding times or total inability to orally feed until their palates/lips are repaired. Children with cleft lip and palate need to be seen by a Speech-Language Pathologist or Feeding Expert as soon as possible to determine which type of nipple may result in the best quality and quantity of intake. Refluxi should also be recognized and treated quickly and efficiently before children can associate the sight of food/bottle with emesisi or pain.
Children with Kabuki Syndrome also are at higher risk for issues with the cardiac system. Children with cardiac issues tend to have limited stamina and fatigue easily. Bottle drinking takes quite a bit of stamina, strength and coordination and can be quite fatiguing for a child. Pair the cardiac issues with any structural issues such as cleft lip/palate and you have a very inefficient system. Compounding the structural and cardiac factors is the issue of hypotonicity frequently seen in children with Kabuki Syndrome. Adequate muscle tone throughout the entire body is imperative for proper strength to expel formula via the nipple and for good trunk support that is needed for spoon and finger feeding down the road.
Children with Kabuki Syndrome have also been reported to have sensory integration issues. Eating is a completely sensory activity; smelling, touching and tasting foods are all sensory activities and can be overwhelming to a child who has a system that is unable to effectively process the input. It is important to recognize how significantly sensory issues can impact a child, especially one with low tone and potential structural issues that can already interfere with eating. Sensory issues can be transitory and change over time as a child matures and is exposed to more of the world.
Developmental delays are common in Children with Kabuki Syndrome. A good in-depth cognitive evaluation is imperative in order to meet the children where they are most successful. Although a child might be 18 months old chronologically, developmentally they may have language and motor skills at a lower level. Requiring behaviors at the 18 month level will only set them up for failure, where as presenting tasks at their current developmental level will result in accomplishment and success.
As brain development is so important for the first few years of life, growth should be monitored closely and stagnant growth should be treated quickly. Children with Kabuki Syndrome frequently have poor growth trends. These children may need a supplemental feeding tube; either a Nasogastric tube or a surgically placed Gastrostomy tube. If a supplemental feeding tube is necessary for longer than 3 to 4 months, it may be better to have a G-tube or PEG placed.
Lengthy use of nasogastric tubes can result in increased oral aversion as they need to be changed frequently, require tape to hold them in place on the face and may impact swallow sensation. To promote appropriate growth, formula can be given as a supplement to what the child is able to eat orally and may not need to be a total replacement for oral intake.
Although the thought of having a gastrostomy tube placed is very scary, being able to support good growth while your child is obtaining the cognitive, motor, and sensory therapy milestones that are necessary for positive and healthy eating habits is important.
So, the bad news is that feeding problems in children with Kabuki Syndrome seems inevitable and overwhelming. The good news is that almost any feeding problem can be overcome if it is approached and handled in the right manner and at the right time. Promoting quality over quantity is imperative. Pushing an infant with cardiac and structural issues to take all calories orally by increasing feeding times, using fast flow nipples or ignoring cues may result in feeding aversion. Sometimes the use of a supplemental feeding tube in the short term can be beneficial for the long run. Allowing a child to eat a small amount with good quality is always better than pushing that child to eat a large amount unhappily or unsafely.
Because children with hypotonia tend to meet motor milestones at a slower rate, it is important to match our feeding expectations with the child 's current motor and cognitive level, not necessarily their chronological age. This may result in a slower transition from bottle or breast-to-spoon feeding and finger feeding, but improved quality of transition. Pushing a child to transition to textures that they are physically not ready for can result in poor quality of intake and feeding aversion. Some children with sensory issues and low tone never do well with purees and this texture may be better skipped.
Exposure to solids usually occurs once a child has good head control, good trunk support to support use of hands for reaching and pincher grasp for picking up small items. This time is really meant for sensory and motor exploration and not really as a method of getting in many calories. This is a time to help your child develop a positive relationship with textures, smells and tastes even if the quantity of oral intake is low. Children who enjoy eating, even small quantities, will go on to be oral eaters in the long run if we can help them become internally motivated to eat once the timing is right. Internal motivation comes from a sense of hunger and satiety.
Helping children with feeding tubes recognize and react to hunger cues is an important first step in building this internal drive. Children on lengthy tube feedings or overnight drips may have a difficult time understanding the full/empty signs. Some children require continuous drips, but others can be transitioned to age appropriate bolus feedings during the day.
It may seem at times that your child is not moving forward in the process of learning to eat. Children without medical complications begin to learn to eat at birth and are not fully competent eaters until at least 15 to 18 months. Children with complications need to be given extra time to accomplish the same skills. Children demonstrate functional improvement in feeding only if it is comfortable, pleasant and internally motivating.
So the bottom line is; take it slow and steady, ensure that it is pleasurable at all times and recognize your child’s cognitive, sensory and motor strengths and weaknesses. Building a positive and safe relationship with foods will result in a child who is motivated to become an oral eater when the timing is right.
Please feel free to contact me with questions regarding the specifics of how to build a positive relationship with foods at MCV2W@hscmail.mcc.virginia.edu.