Epidermolysis Bullosa Home Care Programme Treatment nutritional status

A good nutritional status can be defined as the situation in which the need for and intake of nutrients is balanced; the intake of nutrients meets the daily demand of these nutrients.

Patients with more severe types of EB need extra nutrients to supplement the abnormal losses and to satisfy the increased requirement of nutrients because of open wounds and for a quick healing process and a good immunity to resist wound infections.

The next section describes the parameters that are used to determine the nutritional status. Subsequently, the causes and effects of a malnutritional status for an EB-patient are dis-cussed. Then, the measures that can be taken to obtain and maintain a good nutritional status are reviewed. In the second to last section, the infant's food is discussed and at the end treatment with tube feeding is discussed.

Parameters for the nutritional status

Parameters are used to determine the nutritional status. The parameters mentioned below can give a clear picture of the nutritional status of EB-patients. They are usually simple to determine:

• relationship length - weight;

• loss of weight;

• albumin.

Relationship between length and weight

The relationship between length and weight provides information about the nutritional status. The Quetelet Index (also called BMI, body-mass-index) is a good way to express this relationship (Quetelet Index = weight/length²).

In a Quetelet Index (QI) smaller than 18 there is a underweight.

When the QI lies between 18 and 20, there is no underweight but nutrition should be watched carefully.

Loss of weight

Loss of weight is an indicator for a deteriorating nutritional status. In order to get a clear picture of the seriousness of the weight loss, it is recommended to assess the weight pattern over a period of three months. In a loss of weight of 10% or more during a period of three months the nutritional status is considered to be poor. In the assessment the presence of oedema should be taken into account. This can obscure the weight loss.

Albumin

The level of albumin can only be used as parameter in a `stable' patient. Because albumin levels in the blood may easily drop as the result of for example, an infection. Blood albumin levels below 30 g/l indicate the presence of mild to severe malnutrition. To check whether the nutritional treat-ment has been a success, it should be taken into consideration that it takes more than 20 days before the albumin levels change.

It cannot be determined on the basis of one parameter whether there is a poor nutritional status and/or malnutrition. It is a mixture of the factors mentioned above which, of course, must be assessed along with the overall picture of the patient. To be able to gain a clear insight as to the patient's consuming pattern, a nutritional assessment may be taken (see chapter Logbook: Nutritional Assessment Form).

Causes of malnutritional status

Patients with EB often present an imbalance between the intake and consumption of nutrients because of:

• Small intake of nutrients;

• Unbalanced diet;

• Increased use and loss of nutrients.

Small intake of nutrients

Especially patients with dystrophic and junctional EB have serious problems with the intake of (solid) food and the digestion of it in the mouth because of several causes such as:

• contracted mouth opening (microstomia);

• sensitive oral mucous membrane;

• decreased saliva production;

• fixation of the tongue to the floor of the mouth;

• poorly and incompletely developed teeth;

• restriction of the oesophagus.

See also chapter Disease: Symptoms.

Besides the above mentioned causes the following factors may play a role in the nutritional intake:

• fatigue: the patient might be so tired (for example, because of anaemia or poor nutritional status) that it is almost or completely impossible to eat;

• chronic constipation: which causes loss of appetite and gives the patient a bloated feeling;

• damage to the upper part of the tongue: this may lead to loss of taste buds. This may cause food to pall on the patient because it has no taste.

Other aspects that might be of influence are:

• functional loss of arms due to contractures. If the arms cannot be moved properly it may be hard to bring the food to the mouth;

• functional loss of hand(s) due to fusion (by webbing or acquired syndactyly). This may cause problems in holding the cutlery. Dressing material may also restrict the use of the hands;

• the patient is afraid to eat (afraid to choke, afraid of pain) or is ashamed to eat (embarrassment about visibility of poor teeth, embarrassment about not being able to eat normally because of microstomia, fixed tongue etcetera);

• the patient is not given the opportunity to eat in an adapted way or adapted time. This may be, for example, the case at school or at work.

Unbalanced diet

The intake of solid food is difficult. Therefore, the nutri-tional pattern shifts towards ground, puréed or liquidized food.

The intake of this food is easier because it does not have to be chewed and it is less difficult to swallow. The disadvan-tage of liquidized food in particular is that it does not always contain sufficient nutrients. Besides, the (total or partial) absence of fibre in liquidized food may result in constipation. The same problem arises when ground or puréed food is sieved.

Increased use and loss of nutrients.

The EB-patient uses extra nutrients for wound healing and loses nutrients through tissue fluid and blood through blis-ters and wounds. The blood and tissue fluid are absorbed by the dressing material and this fluid is also lost by evapora-tion.

There is a great loss of protein and the requirements for protein and energy is considerably increased. The energy requirement is approximately 20 - 50% higher than usual. The protein requirement is increased to 1.5 - 2 grams of protein per kilogram bodyweight while the protein requirement normally amounts to 0.5 - 1 gram protein per kilogram of bodyweight. After a possible operation, the effects on the metabolism are also considerable (catabolic stress). The nutritional require-ments largely increase.

Effects of malnutritional status

The lack of nutrients and fluids delays the development and the healing process of wounds, causing a deterioration of the general condition and a lack of iron (anaemia). The patient displays a serious delay in growth and feels tired. Due to the deteriorated healing process of the wounds, infections are easily developed which last long and which heal with difficulty. The patient finally ends up in a downward spiral.

Measures to obtain and maintain a good nutritional status

In order to obtain and maintain a good nutritional status

attention should be paid to the nutritional intake. The use and loss of nutrients and fluid can be of influence.

The daily menu is based on the nutritional assessment. This assessment indicates what the patient eats, how much and when

(see chapter Logbook: Nutritional Assessment Form).

The nutritional intake is stimulated by:

• paying extra attention to meals;

• adjusting mealtime;

• adjusting the composition of the food:

- ground or puréed food;

- liquidized food;

- enriched energy intakes;

- supplementation of nutrients;

• adding nutritional supplements;

• tube feeding.

Do not let the administration of food become a `fight'. When children are forced to eat and/or to eat more this may result in stress. This stress may in turn cause itch and anxiety.

Pay extra attention to meals

Apart from the specific measures that are mentioned in the following sections, the nutritional intake may be stimulated by measures that improve the appetite such as exercise and fresh air and by paying extra attention and care to the meal. The following tips might be useful for this:

• keep the food warm: it takes EB-patients a long time to eat and cooled off food often palls on people;

• do not dish up large portions: it is better to clean one's plate first and perhaps dish up again;

• vary by alternating for example, green vegetables (green beans, spinach) with coloured vegetables (carrots, beetroot, etcetera);

• try to alternate sweet, savoury, sour and bitter flavours. Bear the patient's preference in mind with this;

• avoid hot food in the case of a painful mouth or throat; the food might possibly be served cold, lukewarm or at room temperature;

• smooth food is easier to chew and swallow than dry food. A sip of a drink with every mouthful or rinsing the mouth with water may also simplify the process of chewing and swallowing.

Adaptation of mealtime

To eat a small quantity of food several times is less tiring

than a lot of food at once. Advise patients to eat five or six smaller meals instead of three large ones. If the patient often complains of nausea (lack of fluid worsens nausea) try to determine the times at which the nausea is less severe.

For children who do not have the energy left for eating dinner at the end of the day it might be a better solution if you give them dinner at noon.

The EB-patient often takes longer to finish off a meal. Bear this in mind when determining mealtimes: do not have a meal served when this means the patient has to eat under pressure. In such a case, supply a nourishing snack such as buttered gingerbread or possibly Fortimel®.

Adaptation of composition of the food

If the intake of solid food is not possible the food may be processed. Possibilities are:

• Ground or puréed food;

• Liquidized food;

• Enriched energy intakes;

• Supplementing nutrients.

Ground or puréed food

If there are difficulties with chewing or swallowing, ground or puréed food generally presents fewer problems than solid food. This kind of food may be prepared with a hand mixer or a food processor.

Dinner may consist of:

ground meat with gravy, ground vegetables and mashed potatoes prepared with a pat of butter or margarine;

small jar of baby food, toddler food or pre-school children's food prepared with a pat of butter or margarine;

The consequences of ground or puréed food are:

• the food volume enlarges. Use smaller portions spread over the day;

• less food is consumed (because of the larger volume), which in particular may cause a vitamin and energy defi-ciency. This deficiency may be supplemented by vitamin-rich vegetables and fruit juice (or consult a doctor in attendance for the use of a multivitamin preparation) and high-energy foods such as whole milk, a pat of butter, extra sugar and full-cream cheese;

• dilute food with broth, milk or cream instead of water to increase the nutritional value;

• the taste becomes worse. Variation and careful use of herbs (for example, parsley) may add more taste to the food;

• the food looks monotonous. Do not grind the meat, pota-toes and vegetables together. By grinding and serving them separately the whole meal does not have the same colour and taste;

• if the food is sieved it is poor in fibre. This increases the risk of constipation. Avoid sieved, ground or puréed food as much as possible.

Liquidized food

Liquidized food may be given as a snack but also in place of the main meal.

Liquidized food refers to a liquidized dinner, porridge, liquid dairy products (milk, yoghurt, custard, etcetera), broth and soup, coffee and tea, fruit juices, lemonades, etcetera.

Liquidized food is usually easy to consume. If the patient has problems with swallowing, thin liquid food may result in choking. Liquidized food may possibly be condensed with a thickening agent (see chapter Materials & Medication: Nutrients).

Instead of bread or bread products the following may be used:

• porridge such as Brinta, Bambix, oatmeal porridge, bread and milk, rusk and milk, buttermilk porridge;

• yoghurt, custard, cream cheese;

• soup.

Examples of snacks that may be used are:

• milk, drinking chocolate, buttermilk, drinking yoghurt;

• custard, yoghurt;

• lemonade;

• tea, coffee.

The consequences of liquidized food are:

• the food volume enlarges. Use smaller portions as much as possible spread over the day;

• liquidized food is poor in fibre. There is a greater risk of constipation.

Enriched energy intakes

In order to meet the increased requirements of energy and proteins, enriched energy intakes may be used. The following options are available:

• select foods that do not only provide building materials but also plenty of energy such as whole milk, pudding and full-cream cheese;

• eat brown bread if someone can eat more of this than of wholemeal bread, 3 slices of brown bread are preferred to 2 slices of wholemeal bread;

• use plenty of butter, margarine, whipping cream, sour cream or crème fraiche;

• use extra sugar, dextrose, honey or lemon syrup.

In order to meet the increased requirements of iron, iron-rich food may be used. The following options are available:

• (preferably) daily meat;

• as many wholemeal products as possible;

• as snacks: gingerbread, dried fruit;

• relatively rich in iron are the following foods: meat (especially organic meat), green vegetables, dried peas and beans, fresh fruit, dried fruit, wholemeal products, rough grain products, nuts, syrup, soft brown sugar and rosehip syrup `ferro'.

Supplementing nutrients

Liquidized food may additionally be enriched by build-up pre-parations such as Fortify® or Nutrilon Plus® and food sugar such as Fantomalt® (see chapter Materials & Medication: Nutrients).

Addition of nutritional supplements

In consultation with the doctor and/or dietitian supplementary preparations may be supplied to meet the deficiency. Examples of this are:

• Iron preparations

Required for patients with anaemia due to chronic blood loss.

• Fibre preparations

Constipation is a common problem in EB-patients with diets that do not contain enough fibre. Constipation also occurs in EB-patients that use iron preparations. In these cases the problem might be reduced by a high-fibre diet (or fibre preparations such as Metamucil®) and increased fluid intake.

• High-protein preparations

Supplementing high-protein liquid (for example, Fortimel® or Meritene®).

• High-energy preparations

Supplementing (drinking)food (for example, Ensini® or Nutridrink®).

• Vitamin preparations

For example, in the form of liquid multivitamin-preparations.

If a serious delay in growth is impending which cannot be dealt with by dietary measures, tube feeding should be con-sidered.

The consequences of energy-rich food and supplementing food are:

• when eating quickly a full feeling. Use smaller portions spread over the day as often as possible;

• development of caries: especially when extra sugars are added there is a great chance of caries (see chapter Treatment: mouth and dental care).

The infant with EB

The intake of sufficient calories and other nutrients is espe-cially important for infants because part of the nutrients will be used in the wound healing process, whereas the infant requires a lot of nutrients for growth and further develop-ment. In this section specific problems will be discussed that may occur with the nutritional intake by infants. This section subsequently describes:

• breastfeeding;

• bottle-feeding;

• changing to solid food.

Breastfeeding

The following problems may occur in breastfeeding:

• Blistering in the mouth if the baby is given the breast incorrectly

Make sure the baby holds the nipple straight in its mouth

(and not `at an angle').

• Blisters in the mouth

These usually burst when sucking. If they do not burst spontaneously, open them with a needle.

• `Full' breasts

Squeeze a little milk out of the breast so the child does not choke.

• Not taking enough time for feeding

Give the baby the breast regularly and let it suck as

long as it wants. Take plenty of time for feeding so the

mother and child do not feel rushed.

If the mouth of the infant is too painful to suck or if the infant is too tired (or becomes too tired) consider expulsion of milk and feeding the infant with a little spoon or pipette.

Bottle-feeding

In (case of a painful mouth and when sucking the teat causes blisters) bottle-feeding the hole in the teat may be enlarged and a softer, more flat teat may be used. An example of this is the Milupa Orthodontic®.

When bottle-feeding, it is useful to moisturize the teat before feeding with cooled off boiled water to prevent the teat from sticking to the blistered areas.

Up until six months, all infants can be adequately fed by just breastfeeding or bottle-feeding. When the infant is thirsty, boiled water which has been cooled off until it is at room temperature may be given.

Changing to solid food.

Every child slowly changes from liquid food to solid food. Every child goes through this process and a child with EB is no exception to this. However, because the mouth and tongue are painful it takes longer before the child is used to changes in taste and consistency. In order to habituate the child to solid food, the following advice might be useful:

• give solid food with a spoon. Do not add it to the bottle;

• use a special spoon (child cutlery without sharp edges);

• purée or liquidize the food (do not use sieved food since this is poor in fibre and leads to constipation);

• feed the child according to its needs (when it is hungry);

• take the time for feeding;

• add grain products to one of the meals (for example, 1 or 2 teaspoonsful of rice; mixed with milk into porridge)

Tube feeding

If there is a serious delay in growth (especially in children which cannot be dealt with by conventional measures, tube feeding should be considered. See for full information the KITTZ - home care programme Tube Feeding. The formula may be administered via naso / gastric tube or a gastrostomy. The

naso / gastric tube should be soft to prevent the blistering or the damaging of the mucous membranes in the nasal cavity / pharynx and oesophagus as much as possible. This is usually a silicone tube. The tube should not be too thick.

When inserting the tube, vaseline is put on the nostrils to prevent injuries. The tube cannot be secured with adhesive tape as this will cause extensive skin loss on removal. To secure the tape, a small piece of Tubifast® may be placed over the head, to be situated between the nose and upper lip. Wind the tube around this. Even despite this, it is still possible for the tube to move.

Because of this the location of the tube needs to be checked before feeding is started. This is done as follows:

• mark the tube with a small piece of adhesive tape to indicate how far the tube should/can be inserted;

• check before feeding on the basis of the adhesive tape whether the tube is still in the right place;

• first, instill air into tube (2-5ml). The tip of the tube is located properly in the stomach when a gurgling sound is produced when the air is pushed through. For this purpose, a stethoscope is placed on the gastric area.

In order to prevent damage of the nasal cavity / pharynx and oesophagus, a gastrostomy is sometimes considered. A gastro-stomy is surgically placed. For this, the patient is ad-ministered a general anaesthetic.

Choice of formula in tube feeding

The tube feeding policy will be determined by the deficiency and the result desired. This usually means the use of a pro-tein or energy plus formula. To prevent constipation a bottle of energy or protein plus tube feeding is often alternated with a bottle of high-fibre solution. This formula is thicker than normal preparations. Attention must be paid to prevent blockage of the tube and/or infusion system.

Tube feeding is usually supplementary to ordinary food. It is usually admini-stered in the evening or at night. Continuous administration is only desirable when the patient is not able to take any food orally or he/she is at risk of dehydration. It does, however, occur that tube feeding is clearly indicated but impractical. Examples of the causes for this are for instance the inability to bear a naso gastric tube and a contra-indication for the placement of a gastrostomy. In these patients, it is often difficult to preserve the intake of all the similar necessary nutrients within the patient's limited possibilities and the use of several foods and supplementary preparations

Literature used

Haynes L. Nutrition for babies with dystrophic epidermolysis bullosa. DEBRA.

Balans tussen verbruik en inname, 3M TegaMagazine 1996; 1:6-7

Voedingsadvies bij kauw- en/of slikproblemen. Diëtetiek Academisch Ziekenhuis Groningen, 1993.

Mixdranken op basis van Nutrilon Plus poeder. Diëtetiek Academisch Ziekenhuis Groningen, 1994.

Allman S. Diet for Epidermolysis Bullosa. DEBRA, 1989.

Klasen HJ. Enkele voedingsaspecten bij patiënten met ernstige brandwonden. Wondbehandeling: kunst en wetenschap. Amsterdam Excerpta Medica, 1990.

Klinische depletie, bepaling en voedingstherapie. Nutricia, 1991.

Richtlijnen ter verhoging van de ijzerintake. Dienst Diëtetiek Academisch Ziekenhuis Groningen.

KITTZ-thuiszorgprogramma Sondevoeding. Groningen: KITTZ, 1996