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Feeding difficulties in children with PHHI (Persistent Hyperinsulenimic Hypoglycemia of Infancy).

Maria Ramsay, Ph. D., Clinical psychologist, Director of the Failure to thrive and Feeding Disorders Clinic, The Montreal Children's Hospital.
When it comes to mealtimes, children with PHHI and their parents experience similar difficulties as children with other medical disorders and their parents. The children are disinterested in eating or trying new foods and the parents are disappointed and frustrated with trying to change their children's mealtime habits. In the end, mealtimes become prolonged battlefields, where there is no winner but cause distress for the whole family.
To understand feeding difficulties in children with PHHI, first I will outline the normal feeding process and what can go wrong with it. Then, aspects of PHHI that specifically contribute to feeding difficulties, and their treatment will be described. The article will conclude with several suggestions for parents to try.
The normal feeding
1. Feeding is a developmental skill
Feeding, like other developmental skills or abilities in infants (eg: walking and talking), is a highly complex sensorimotor skill which matures over time (see table below). However, because of its survival value, feeding, unlike other sensorimotor skills, is highly charged emotionally from early on in life. When the feeding ability is intact, mealtime is a pleasant social act and results in adequate nutrient intake and good growth.
Feeding and other developmental skills in infants
Age in months
Skills
0 -2/3
3-6/7
7-10/11
11-15/16
16-24+
Feeding
(oral motor)
Suckles
Sucks/bites
Munches
Chews
Chews (mature)
Food texture
oral sensory)
Liquid
Purees
Lumpy
Chopped
Table foods
Speech
Cooing
Babbling
Speech sounds
Single words
Joint words
Fine motor
Plays with fingers
Reaches/holds
Transfers
Releases
Scribbles
Gross motor
Lifts head
Turns over
Sits alone
Stands/walks
Runs/jumps
2. Feeding is a physiological process

Feeding is a physiological process that depends on
(A) an incentive or appetite to initiate ingestion and
(B) the ability to ingest food.
(A) Appetite
Appetite is controlled by hunger and satiety centers in the hypothalamus. Factors that affect appetite is poorly understood, but it is suspected that several sensory input (taste and smell) cortical input (I am tired) and visceral feedback (gastric volume, hormones) influence appetite regulation.
(B) Ability to ingest food
To ingest food, the child has to be able to smell the food, to taste and tolerate different kinds of food textures and consistencies. These are sensory abilities in the oral and pharyngeal areas. The child also needs motor skills for sucking, chewing and swallowing, which cannot happen without sensory input. These sensory and motor skills are coordinated for the act of ingestion and cannot function well without the integrity of the cardio-respiratory, gastrointestinal and all other organ systems, as well as intact metabolic and genetic components.
Feeding difficulties: What can go wrong?
1. Poor or erratic appetite/hunger
Feeling of hunger is not uniform in infants. Some infants feel hunger intensely, others not at all, and still others have erratic appetite. Infants with poor appetite do not wake up for feedings as newborns, or later, do not reach or ask for food. In extreme cases, very young infants with such a problem will indeed become dehydrated if left to their own internal cues.

2. Oral/pharyngeal sensorial aberrations
Some children are very sensitive (hypersensitive) in their mouth, others are not sensitive enough (hyposensitive),
(A) Infants with hypersensitivity in the oral area tend to gag when an object (nipple, toys, finger or food) is put in the mouth. Infants with hypersensitivity in the pharyngeal area tend to gag as they try to swallow. These infants may accept food, chew it, but instead of swallowing, they spit it out. Gagging triggers vomiting in many infants. Children may be hypersensitive to food consistency, food temperature or specific food textures and food chunks. These children are
often called finicky or picky eaters with the assumption that they wilfully do not eat certain food types. Such negative terms are not helpful and may only contribute to problems in the feeding relationship.
(B) Although less likely to occur, children can be hyposensitive to foods. That is, they have diminished sensorial reaction to taste, volume and consistency of food. For them the food in the mouth does not automatically trigger sucking or chewing and swallowing. These children react to food by holding the formula or solids in their mouth for several minutes or hours (yes, hours!!!) without chewing. The taste of new foods tend to trigger faster chewing or swallowing for a short while, but these children quickly habituate to the new taste and become slow feeders again.
To complicate mealtimes and confuse parents, some children may be hypersensitive to some textures and hyposensitive to others.
3. Oral/pharyngeal motor difficulties
Some children have inefficient sucking or chewing abilities secondary to poor muscle tone or poor motor coordination. These children may also be delayed in other sensorimotor skills.
Behavioural concomitants
When feeding problems are chronic, they give rise to disruptive feeding behaviours between the children and their caretakers.
1. Food refusal by children
Children have a large repertoire of behaviours they can use to hinder adequate nutrient intake or avoid specific food types (eg. Head turning, throwing or playing with food). Initially, food refusal should be seen as a sign of persistent difficulties (poor appetite, sensorial aberrations or oral motor delays) ingesting food. However, it is important to recognise that these disruptive feeding behaviours also serve to maintain previously established feeding patterns and mealtime interactions and prevent the acquiring of higher level feeding skills.
2. Maladaptive parental feeding behaviours
Maladaptive parental feeding behaviours develop from earlier compensatory feeding practices. When infants are young, compensatory feedings (eg. Very frequent feedings or extensively long feeding times) are necessary to ensure adequate nutrient intake for the survival of infants. When feeding problems persist for years, earlier compensatory feeding are replaced by cajoling, pleading, threatening or allowing unstructured mealtimes in an attempt to ensure increased food intake. Young children quickly learn how to "side-step" these parental attempts at offering food and the "battle of the spoon" is on.
In my experience, the reason such battles develop is two-fold. One, the parents are usually not aware of the complex physiological nature of feeding difficulties and thus, they think that the child is being purposefully negative towards them. Second, the professionals also do not understand the physiological nature of feeding difficulties and thus, they tend to blame the parents for inadequate intake and mealtime difficulties. Thus, the parents are caught in the middle and feel frustrated.

Feeding difficulties in children with PHHI
By now I am certain that you recognised your child and yourself in the above descriptions. However, there are certain aspects of feeding difficulties that are specific to the condition and treatment of PHHI.
The very problem that causes PHHI may itself influence appetite. That is appetite regulation may be effected by pancreatic malfunction, though no research has been conducted to establish this relationship.
In terms of treatment there are several factors that interfere with normal feeding pattern :
(1) The need to feed frequently, and a specified amount to keep up the blood sugar level diminishes appetite.
(2) The necessity of long-term tube feeding, often via gastrostomy, also diminishes appetite, and deprives the mouth of the normal process of ingesting food. That is, the oral-pharyngeal sensory and motor skills are not used, therefore they do not have the opportunity to develop.
(3) The hormonal secretion in the oral cavity, associated with oral-motor activity, and chemo receptors in the back of the mouth, which triggers swallowing of food bolus do not have the opportunity to function in the absence of food ingestion. The physiological implications of the absence of feeding activity can only be surmised, as to my knowledge, they have not been researched.
(4) The necessity of regular and rigid mealtimes that is typical of PHHI treatment puts particular stress on parent-child relationship during mealtimes. Because the inevitability of having to give ' "the treatment" through the tube, any attempt by the parent to regulate intake in a child who is not hungry becomes an impossible and frustrating task.
The wish by parents that their child would cooperate and follow parental request about eating is natural. However, no child listens to adult reasoning. No child has the adult's perspective of the world. Besides, the child's physiology tells him that he feels full or that he gagged the last time he tried this new food, so he is not about to try again. He may feel awkward about what to do with the food once it is in the mouth. Thus, internal cues do not motivate the child to eat. As well, the child learned long time ago, since a tiny baby, that there will be food placed in his stomach, no matter what the parents say. If all this sounds discouraging, be assured that things do get better over time, even if they get better over a long period of time!

A "What to think and what to do" list for parents
(1) Put feeding difficulties into their proper place. They are not your fault, nor your child's.They may be part of the condition of PHHI and are part of the " side-effects" of the treatment for PHHI.
(2) Remember that any illness can set your child back several months in terms of eating. Do not get discouraged, it happens to all children with feeding difficulties. The good news is that when you restart with the same method you got the first few bites going, this time, the learning curve will be much faster.
(3) Think of food refusal as a sign of poor appetite or hypersensitivity in the mouth, and not something the child made up to annoy you or get back at you.
(4) To diminish stress, do not feel that your child at age one (for example) should be eating pieces. The feeding stages in PHHI children take much longer than in the table shown. Children stay longer on the bottle and longer on purees. (Some reasons were given above.)
(5) Remember that infants tend to drink from the bottle while asleep better because they are not aware that they are not hungry while asleep.
(6) To diminish stress during mealtimes: Have your child at the table, with an empty bowl or plate, so they can "pretend feed", if they wish. The goal is that mealtimes are pleasant social events for everyone. Remember he is more likely to want to feed you than himself, as he is not hungry.
(7) It is difficult to give specific suggestions, as each child is in a different stage of feeding ability. But, here is an example of how to introduce a new food over several weeks to a child who is capable of eating at least purees and had a few spoonfuls of some favourite purees. If you want your child to try something new, put a 1/2 teaspoonful on the plate and tell him that if he wants he can just look at it. Do not remind him every few minutes to look at it. Ignore the plate and have a nice mealtime. At the end of the meal, just remove the plate. If your child actually looked at the food, you may comment: "You decided to look at the food. Good boy." After several or even weeks, the next step may be to touch the food on the spoon, then to touch the lips with the food and then touch the tongue with the food and then let the food stay in mouth without swallowing and then with swallowing.
(8) Food refusal should not be the topic of discussion for the family at the table or on the telephone.
(9) If the hypersensitivity is so strong that you cannot get food near the child's mouth, consult an occupational therapist who can give exercises for oral sensorimotor development.
(10) Remember that your child does not have an internal motivator to eat even if he can tolerate food in the mouth. Consult a behavioural therapist to help you use external motivators systematically to get your child to eat.
(11) Medications have been used to increase appetite in children over 2 years of age, when there is a total absence of appetite. Consult your doctor about it.

Good luck!
Bibliography
Evans TJ, Davies DP (1977). Failure to thrive at the breast: an old problem revisited. Archives of Disorders in Childhood 52: 974-975.
Lindberg L, Bohlin G, Hagekull B (1991). Early feeding problems in normal population. International Journal of Eating Disorders 10: 395-405.
Ramsay M. (1999). Problèmes alimentaires chez les bébés et jeunes enfants. : La revue PRISME 30:10-27.
Ramsay M, Gisel EG (1996). Neonatal sucking and maternal feeding practices. Developmental Medicine and Child Neurology 38: 34-47.
Ramsay M (1995). Feeding disorder and failure to thrive. Child and Adolescent Psychiatric Clinics in North America 4: 605-616.
Ramsay M. Gisel EG, Boutry M (1993) Nonorganic failure to thrive: A growth failure secondary to feeding skills disorder. Developmental Medicine and Child Neurology 35: 285-297.
Reilly SM, Skuse DH, Wolke D, Stevenson J. (1999). Oral-motor dysfunction in children who fail to thrive: organic or non-organic. Developmental Medicine and Child Neurology 41:115-122.
Stevenson RD, Allaire JH (1991). The development of normal feeding and swallowing. Development and Behavior: The very young Child. (1991). Pediatr Clin North Am, 38:1439- 1453.
Tolia V (1995). Very early onset nonorganic failure to thrive in infants. Journal of Pediatric Gastroenterology and Nutrition. 20: 73-80.


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Cornstarch and hyperinsulinemia

January 2002
Nathalie Beland,
Clinical dietitian,
Montreal Children's Hospital

Cornstarch, this white powder that everyone has in his or her kitchen can efficiently help treat recurrent hypoglycaemia. Raw cornstarch is commonly used to prevent hypoglycemia in children with glycogen storage disease as well as in children with hyperinsulinaemia. Hyperinsulinaemia is characterized by an abnormally high production of insulin which constantly "burns" the sugar circulating in the blood.
The use of raw cornstarch has been found to extend time between meals. The raw starch is digested slowly because it is a very long and complex chain of carbohydrates. Like pasta, it enters the bloodstream little by little over a longer period than cooked starch, which is absorbed more or less in 45 minutes, or 15 minutes for simple sugars like fruit juice. The effect of various starches on the blood sugar differs according to their physical state (size of granules, presence of fibre). The starch of the corn grain constitutes the main part in the center of the grain; cornstarch is widely used in America.
Cornstarch feeding can be started around 1 to 2 years of age. Younger children do not have what it takes to digest raw starch. The necessary enzyme produced by the pancreas, called amylase, matures around 2 years of age. Before the age of 2, cornstarch may be used with the administration of the amylase enzyme.
Children who eat by mouth will take the cornstarch raw, mixed with water or milk. Children who are fed with tube can take it added to the milk or formula being used. The cornstarch has to be prepared just before it is needed. Cooking or shaking vigorously the cornstarch and liquid mix will denature the long starch molecules and it will loose its effect of helping maintain normal blood sugar.
The amount of cornstarch needed per day varies for each child depending on his age, his weight and his metabolism. The suggested dose of raw cornstarch is 1.75 to 2.5 gm/kg of body weight (1 table spoon of cornstarch weighs 8gr). For example a child of 2 years old weighing 12 kg, might need 3 ½ to 4 tablespoons of cornstarch 4 times per day. An older child of 25 kg may need 4 to 4½ tablespoons 2 times per day only.
If the child eats well, 2 to 3 doses of cornstarch per day (after meals or between meals) may be enough to maintain blood sugar in the normal range. If the child's eating is variable (like it is for many children!) it is probably best to give smaller doses more often between two meals, with a higher dose at bedtime for nighttime support which is the longest period without eating or drinking.
The amount of cornstarch needed generally reduces with age. Some children can go up to 4 hours between each feeding, when older they can extend to 6 hours between feedings or meals. Some adolescents may even control their glycaemia with a single dose of cornstarch per day! However some patients may not respond as well as others to the cornstarch therapy, and may continue to need overnight infusion of milk or glucose.
A few side effects may be observed with the use of cornstarch, they include: smaller appetite for food (children may feel full by taking the cornstarch), occasional diarrhea (especially with too much cornstarch) or constipation and interference in iron absorption.
Introduction of cornstarch for children with recurrent hypoglycaemia should always be under medical supervision, as each child is different.

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Diabetes mellitus following pancreatectomy for Hyperinsulinism.

Dr. K. Hussain MSc MBChB MRCP MRCPCH
Clinical Lecturer in Paediatric Endocrinology
The London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children NHS Trust WC1N 3JH and the Institute of Child Health, University College London WC1N 1EH United Kingdom.
Pancreatectomy for Hyperinsulinism is only performed as a last resort for the treatment of hypoglycaemia which is unresponsive to medical treatment.
Some children who need a 95% pancreatectomy (or greater) will develop diabetes mellitus. It is difficult to predict which child will develop diabetes mellitus. Following pancreatectomy the blood glucose levels in most cases will tend to be high because of the body's stress response. This does not necessarily mean that the child will have diabetes mellitus. What is important is the glucose levels over the five to seven days following pancreatectomy once the stress response subsides. If these glucose levels are still high then it is possible that the child will have diabetes mellitus. This diabetes mellitus will require treatment with subcutaneous insulin. The diabetes mellitus following pancreatectomy for hyperinsulinism seems to be different to the type 1 diabetes mellitus that is seen in adults and very rarely in children. This may be due to several reasons.
Firstly following post pancreatectomy some children demonstrate extreme sensitivity to small amounts of exogenous insulin whereas others require more insulin. Each child will need his or her own particular insulin regimen to stabilise their blood glucose levels. Some children will need twice daily insulin injections whereas others will need three daily injections. Occasionally a once daily insulin regimen will be sufficient.
Secondly removing a large portion of the pancreas also removes the cells, which produce other hormones such as glucagon. This hormones plays an important role in counter-acting insulin's actions. This hormone is normally produced in response to a falling blood glucose level, hence the lack of this hormone may be associated with hypoglycaemia.
Thirdly it is conceivable that the remaining 5% of the pancreas is some cases is able to generate some endogenous insulin. There is no hard scientific data for this yet, but is based on observations in some children who require a very tiny dose of insulin and yet show severe unpredictable hypoglycaemia.
Last but no least is the important fact that some children with hyperinsulinism also have gut related feeding problems. Following pancreatectomy these problems related to the gut do get better over time. The symptoms of vomiting, diarrhoea, lack of appetite and in some cases inability to absorb food will also affect the control of the diabetes mellitus.
It is important to understand that the decision to perform a pancreatectomy is a major undertaking in the management of a child with hyperinsulinism. This will only be performed if the child shows no response to medical treatment and is still at risk of hypoglycaemia and subsequent brain damage.



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Tube feeding: The importance to maintain primary oral motor skills and sensitivity.

Translated from French by the PHHI Web Committee
French version by Marie-Hélène Laplante, ergothérapeute
Centre de réadaptation l’Émergent
Sept-Îles, Québec.

In children with PHHI tube feeding is sometimes required in order to maintain appropriate blood sugar levels. Children may need nasogastric or gastric tube feeding, occasionally or exclusively, for short or long term treatment.

Although in such cases tube feeding is of the utmost importance to ensure appropriate blood sugar levels, parents are seldom informed of the possible consequences of exclusive tube feeding.

  • Exclusive tube feeding for a prolonged period may result in, amongst other things, oro-facial defences and the loss or non development of basic motor skills needed for feeding.

However, appropriate stimulation, regular exercises and a follow-up by a qualified professional in feeding problems can reduce the consequences of tube feeding. It is thus essential for the child to follow an oral-motor treatment, concurrently with tube feeding in order to diminish the problems that can occur.

  • Parent’s who’s child is tube fed can carry out different exercises at home in order to maintain facial and oral sensitivity in the order of normal limits, stimulate oral motor skills needed for oral feeding, maintain a positive image of oral feeding and if possible continue a basic type of oral feeding. Consult feeding therapist in dysphasia and your child’s physician before applying any type of exercise.

For an infant, the use of a pacifier while tube feeding may be of interest to stimulate oral movements. Mouth-object games must also be encouraged in order to maintain a normal oral sensitivity and to work oral-motor skills.

Different types of techniques exist to normalize tactile sensitivity of the face and mouth. You may consult an occupational or speech therapist in order to ensure that they apply to your child. If possible, you may brush your child’s teeth with a soft tooth brush (Nuk), without water if it is contraindicated for your child.

If possible you can keep contact with foods by asking your child to help you when you do the cooking. This way, he/she will smell the foods. Encourage him/her to touch them and even lick his/her fingers if he has the capacity to do so. You can play games with juice: put his/her finger in it and lick it. If your child retches at the sight of food you may start by using odorant crayons or clay. You can play with him/her to feed a doll or push a toy. You can create meals with plastic foods.

If your child has the capacity, he/she may continue oral feeding with the consistencies of food that are safe for him/her. You must consult his/her physician regarding that matter.

Because each child is different, you must always consult your child’s physician before applying any techniques or giving food to your child. His/her security depends on it. Many types of professionals touch this area of work and can be of help to you: occupational therapists, speech therapists, psychologists, dieticians. A follow-up with these professionals is highly recommended.

It is of the outmost importance to respect your child’s rhythm in the process of going from tube feeding to oral feeding. The process is items long but can be facilitated if the child has followed an appropriate follow-up at the early stages of tube feeding.

The above advice is of general matter; refer to a health professional in order to ensure that the exercises apply to your child.


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Focal vs diffuse PHHI.
Dr. K.Hussain MD MSc MBChB MRCP MRCPCH
Clinical Lecturer in Paediatric Endocrinology
Institute of Child Health / Great Ormond Street Children's Hospital
Department of BEM
30 Guilford Street, London WC1N 1EH

Histologically Hyperinsulinism in Infancy (HI) is classified into diffuse and focal forms. The diffuse form of the disease affects the whole pancreas whereas the focal form is localised to one single region of the pancreas (either in the head, body, tail or isthmus of the pancreas). The focal lesion is very small (6-12 millimetres in diameter) and in most cases cannot be seen with the naked eye at the time of surgery. In the UK about 40% of children presenting with HI will have focal disease. Children with diffuse and focal forms of HI present in a similar manner with hypoglycaemia and there are no clinical or biochemical markers that allow us to distinguish between focal and diffuse disease.

It is now very important that children with the focal form of the disease are identified as they will require a partial pancreatectomy (limited resection of the focal lesion) to remove the focal lesion leaving behind a normally functioning pancreas. On the other hand children with diffuse disease may require a near total pancreatectomy to control the hypoglycaemia. The focal lesion cannot be seen with imaging techniques such as ultrasound, CT scan or Magnetic Resonance Scans. Currently we here at Great Ormond Street Children's Hospital in London are performing a technique called Intra-Hepatic Pancreatic Portal Vein Sampling (PVS) to try and identify those children who may have the focal form of HI. This is a very complicated procedure, which involves taking multiple blood samples from the tiny veins draining the pancreas and looking for a "hot spot" of insulin secretion.

The focal and diffuse forms of HI are genetically inherited in different ways. The most common cause of diffuse HI is due to abnormalities in the proteins that make up the KATP channel on the pancreatic b-cell membrane. This form of HI is inherited in an autosomal recessive manner. This means that the chances of another child being affected is about 25%. Other rare forms of diffuse HI are due to abnormalities in the enzymes that control energy production in the b-cell. Fortunately these forms respond to medication with diazoxide and do not need surgery. They are inherited in an autosomal dominant manner which means that there is a 50% chance that the next child will be affected.

The focal form of the disease is inherited in a completely different manner compared to the diffuse form. The abnormality in focal disease is present only in the abnormal b-cells localised to the focal lesion. In simple terms the b-cells in the focal lesion contain two copies of the fathers chromosome 11p (short arm of chromosome 11) instead of one copy from each parent. Although the fathers of children with focal disease are clinically well, analysis of their genes on chromosome 11p shows that they are carrying an abnormality in one of the genes controlling the function of the KATP channel (since they also have one normally functioning gene they will not have problems with hypoglycaemia). Since the focal lesion contains two copies of the father's chromosome 11p then there will in effect be two abnormal genes in the focal lesion, which will cause disturbance in the function of the KATP channel leading to uncontrolled insulin secretion. We do not at present understand why this abnormality occurs and the underlying mechanism. The b-cells around the focal lesion are completely normal and do not show this abnormality. This unique abnormality arises during the very early stages of development of the pancreas. Since this abnormality occurs during development of the pancreas the chances of it happening again are about 1%, which is the risk associated with developing any congenital abnormality in the general population. There have been no reported cases of focal disease in siblings.


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