November 1996 to March 1997. Initiation of the ketogenic diet, gradual move from MCT oil to classical diet, difficulties with feeding, use of ng (nasal gastric tube), status at one year.

It was intended that the diet should start on Monday 18 November, but no bed was available. The starting date was revised to Wednesday and then the following Monday. During this period, Marchant’s fits increased in frequency and severity, until he was having up to 90 a day of pronounced salaam attacks with 3 to 5 oscillations. His hands seemed to be less used, he arched his back more, and his tongue movements increased again. On the Tuesday Sue insisted that he has admitted; this was intended to be to ward 31, but, by popular request, he returned to ward 37.

Originally the dietician had been supposed to talk to us at the beginning of November, while Marchant was in hospital. She never turned up for the agreed meetings, and never communicated subsequently, in spite of Sue’s attempts to contact her. By the weekend before Marchant was admitted again, we were becoming concerned, because we had received no instructions on preparation before admission, nor advice on what would be required to implement the diet. We decided therefore to follow the admission instructions for the Packard Hospital at Stanford, and starved Marchant for the day before admission; Sue also weaned him off breast milk. Fortunately that was the right thing to do, although we had not recorded Marchant’s meals for the preceding three days, which was information the dietician requested when we finally contacted her the day before admission.

Sue was given a diet sheet for the evening. This included 300ml of aptamil, one scoop potato, 100g packet food, and half a banana - enough carbohydrate to sink a ship. It was also hopelessly imprecise; the amount of carbohydrate in a packet food varies enormously etc. Finally, Sue had to leave the hospital to buy the ingredients, because they were not provided. Fortunately, Marchant was not prepared to eat, and just had a little of the aptamil.

The following day, after repeated requests, a diet sheet was provided. This was based on the Radcliffe diet, which is the least demanding of the four regimes, having 19% carbohydrate in a diet providing 100% of the recommended daily allowance in calories, as compared with 5% carbohydrate in a diet providing 75% of the recommended daily allowance for the classical diet. The food was now provided by the hospital - however, this had its own disadvantage, because the food was provided at times determined by the hospital, and did not correspond to Marchant’s inclination to eat, Also, the hospital insisted on throwing the food away if it was not eaten within about 20 minutes. As a result, Marchant did not eat much of the food. By the Thursday, it was clear that if we wanted Marchant to operate to a diet, it would be far better to take him home and do it in an environment which was better controlled. This we did, with the agreement of the hospital.

Because of the difficulty of feeding Marchant, Sue decided to make a standard milk and a standard feed, both of which were balanced, so that it did not matter if some were wasted, he would still get the correct ratios at all time. Sue also decided to make excess food, and then to measure the food actually eaten; again, so that any wastage could be adjusted for.

The target number of calories was 650 each day (ie 100% of recommended daily allowance - 100 Kcal per Kg body weight), made up from:

The milk was high in liquigen, because it was the night feed, when the ketones are depleted; for the same reason, a small amount of liquigen was reserved as a top up before bed. The food was made in a jelly, so that the proportion feed at any time was properly controlled.

Marchant’s feeding pattern had been erratic. Prior to going to hospital in October, he had refused to accept a bottle, but had been very happy eating solid foods. In the hospital, he completely refused to eat solid food and, because there was insufficient milk, it was necessary to resort to the bottle, which he gradually accepted after a lot of effort by the nurses (refusing the bottle from Sue). On return from hospital, he started to eat some solids again, and would accept a bottle with great difficulty. For the ketogenic diet, it was necessary to wean him off breast milk. This was done in the week before the diet started, but Marchant largely went onto a self imposed fast, refusing solids, and being very difficult to feed on a bottle. During the period he was in hospital for the ketogenic diet, he was very difficult to feed, taking little except a bottle, and that with difficulty.

It was difficult to measure whether Marchant was in ketosis, because the nappy absorbed the urine too well so it was hard to get a sample. The indication over the first week was that he was slightly in ketosis, but went in and out. Subsequently (February 1997), we established that when the test was done directly with fresh urine, the result was a much higher ketosis level than measured off the nappy. It would appear that the ketone level declines with time, and that testing through the nappy is likely to give an underestimate of the ketone level.

Marchant’s fit level had increased to some 5 or 6 fits an hour before he went onto the ketogenic diet; each fit was a salaam attack with 3 to 5 oscillations. He condition did not changed markedly for the first few days he was on the diet. When he first came home, the level stabilised at some 60 observed fits a day.

On return home, Marchant refused to eat solids, even the jellied food, and took a bottle with difficulty for the first week. He was also sick a number of times, but this may have been the effect of a bug, which both Sue and her mother had, which made them both extremely sick and ill for 24 hours. Towards the end of the week he started to experience pain, which appeared to be both a nagging pain and an acute pain. Our best guess is that this was reflux plus stomach cramps.

Because Marchant refused to eat the solid part of his diet, he was low on protein, also on total calories. Sue also discovered, belatedly, that he should not have started immediately on the full dose of liquigen, but this should have been built up over a period, in order to build up a tolerance. This probably caused or at least contributed to his pains.

Marchant was intermittently in ketosis through the week. Over the week it appeared that there was some improvement in the observed fit level.

The health visitor saw Marchant on Monday. His weight had again dropped to below 14lbs (his weight had only increased by 1lb in 5 months). He was also seen by the physiotherapist, who recommended various exercises to improve his posture. He was also seen by the special needs health visitor from the Tyndall’s Park Centre, Barbara Butcher, but it does not appear that they can help in any significant way - no nursery places, and she said that he needed too much care to attend an ordinary nursery.

On Wednesday, Marchant was seen by Dr Jardine, who did not seem very convinced by his progress on the ketogenic diet, but agreed that it should be continued for another week.

On Thursday 12 December, Marchant went to London to St Mary’s Hospital, to see Dr Ruby Schwartz, and the dietician, Samantha Sargant. They were extremely helpful. Samantha Sargant provided a copy of the chapter of the dietician’s book on ketogenic diets, and suggested vitamin and mineral additives

Ruby Schwartz made a variety of comments:

• There should be a feed just before Marchant went to bed to boost his calorie level through the might, and compensate for low ketones early in the morning.

• He would get constipated if the liquid intake was too low, and this was a risk. Need to ensure there is fibre in the diet (ie wheatabix)

• Starch is good to provide blood glucose

• Eggs are good because they provide the best selection of amino acids - ie eggs are a source of "quality" protein

• Food intake should be based on target weight, not actual weight. At this age, growth is controlled by calorie intake rather than genes.

• Children that respond to the diet seem to fall into two groups. The first are ketone sensitive, and have fits if the ketone level is too low, or too high. The second group take longer to respond to the diet, but are less dependent on the ketone level. She thought this might indicate that two mechanisms were at work in the ketogenic diet.

• The reason for introducing the MCT version, and the Radcliffe variant appeared to be social and financial rather then scientific. People in the UK were brainwashed to think that a fatty diet was bad, and also though that it would be expensive. MCT oil on prescription was free.

• The reason for the increase in carbohydrate in the UK diets was pragmatic (?).

• Her experience was that children would accept the MCT diet, but would gradually start to object over a period of time.

• She thought MCT oil was tolerated better when it was cold.

• She thought that the results were better with the classical than with the MCT diet; although she was responsible for the use of MCT oil in the UK, she clearly now thought only the classical diet should be used.

• The short term problems with the diet were: dehydration, uric acid level increase, gastric problems, rejection.

• The long term problems with the diet were: kidney stones (dehydration), growth (supply of adequate calories). She had one child who was on the diet for 16 years.

• In her experience, hypoglycaemia was no problem. The fatty diet was no problem; fats only caused difficulty if combined with excess calories.

• There is no significant use of the ketogenic diet in UK (except RS) The MCT diet was originally developed by Huttenlocher in the US.

• She had no views on the mechanism of the ketogenic diet. Experiments with animals showed that fat levels were increased in the brain, also beta hydroxybutyrate. The latter had been tried intravenously, but was largely equivalent to oral does of sodium valproate.

Because of Marchant’s pains, his diet was revised to reduce the amount of liquigen (down to 30mls). He still refused to eat solids, but accepted more milk (and so more calories). The diet was eventually modified to a liquid diet of 770 calories a day, made up as:

He still went in and out of ketosis, but there appeared to be an improvement in fit level, with a reduction in observed fits from about 60 a day to 30 a day. The fits were mild salaam fits, with 2 or 3 cycles. He had an increasing number of fits during his sleep.

In subsequent weeks, the observed fit rate went down to as low as 4 on one day, but then started to increase, typically being about 20 per day. The fits seemed to increase because Marchant developed a very low ketone level early in the morning, with the ketone level increasing through the day. Most of the fits were early in the day, Marchant being almost fit free in the evenings. The fits also changed in intensity. Most fits became so subtle that they were difficult to observe. Also, they started to occur more selectively, most on his left leg, then on his left arm. He also had some "startle" fits, when he would throw his arms out wide. These fits would frighten him and he would cry.

Marchant was seen by Dr Jardine on December 11 and 18 and January 8 at St Michael’s Hospital in ward 37. This arrangement was adopted because of the difficulty in seeing him, but it meant that Sue had to wait all afternoon at the hospital. Dr Jardine was kept abreast of developments, and did not have much comment. He agreed with the suggestion that Marchant should be given a carnitine additive. (L-carnitine promotes the transfer of fatty acids across the cell boundary to make them accessible to the mitochondria. There is no research to establish its value for the ketogenic diet, but in principle it should improve the effectiveness of the diet. It is used as a treatment for Alzheimer’s disease, so is known to be safe.) He again undertook to arrange a 24 hour reference EEG, as agreed previously in the hospital.

Marchant was seen regularly (once a week or fortnight) by the physiotherapist, who advised on various exercises that he should be given.

Marchant was also seen regularly (once a week or fortnight) by the cranial osteopath, Kate Burns. She thought that Marchant had problems with his breathing and chest muscles, and that this might be connected in some way to the infantile spasms.

The phantom tooth: In December, life was complicated because Marchant appeared to be teething. He dribbled, his cheeks were red, and he wanted to bite. A very small tooth duly appeared (right lower incisor) - and then disappeared. Sue found a very small white rind on the floor, about the size and depth of the apparent tooth.

January 1997

During the period of January, the ketogenic diet was varied in various ways, because Marchant appeared not to like the food. The amount of liquigen was reduced, and the ratios moved closer to the "classical" diet.

Meanwhile there was great difficulty in arranging for Marchant to be assessed at Tyndall’s clinic by Dr Emond. Marchant was referred to them in October, but the earliest appointment they could offer was 19 March! The health visitor managed to bring this forward to 12 March; the physiotherapist then managed to bring this forward to 27 February. Dr Jardine agreed that this was unacceptable and made an appointment for 20 January, however Dr Emond was not available.

Marchant was now ill again, with a bug, and he started to be sick when fed, and to refuse all food.

After a great deal of pressure, Marchant was seen on Wednesday 22 January by Dr Imelda Bennett, who is the community paediatrician at Tyndall’s Park. (By coincidence, she was at Johns Hopkins, and so was aware of the ketogenic diet.) She had little to say of medical consequence, but did say that we would have to be prepared to spend our time fighting the system if anything was to be done for Marchant. Dr Bennett said that it was important that we started to give Marchant solid food.

Marchant continued to be sick and refuse food. At first we thought this might be due to the diet, but then we found out that Griff Miles (aged 2) from whom Marchant had caught the bug was also being very sick, so it was probably the effect of the bug. The only possible way to feed Marchant was using an oral syringe.

As a result of the sickness and lack of food, Marchant was effectively starved, and his fits disappeared completely.

Marchant’s physical symptoms - arching back, abnormal posture of head, retraction and twisting of arms etc clearly indicate cerebral palsy, although none of the doctors we have seen have commented in any way on this. On Monday 3 February, Marchant was taken to the BIBIC centre at Bridgwater (British Institute for Brain Injured Children). Although it was obviously well funded, we had reservations with the medical approach to the training regime. We also had contact with the Bobath centres in Cardiff and London.

Sue was concerned that Marchant continued to refuse to feed and wrote to Dr Bennett, saying she had reached crisis point and needed help. Dr Bennett rang the next day and said that Marchant might need a g tube, but that she needed to talk to the dietician and speech therapist. On Monday 10 February, Julia Brown, the health visitor, rang Dr Bennett because she was concerned about Marchant. Dr Bennett said that Marchant should have the diet altered or come off it altogether. Sue thought the underlying problem was not the diet itself (although it might be a contributory cause), but that Marchant had always exhibited a basic feeding problem, which was exacerbated by his colds and infections. Sue saw Dr Bennett and the dietician on the Wednesday who said she would refer Marchant to Dr Sandhu; she told Sue that she must co-operate with the dietician, Alison Connell, and let her have the details of Marchant’s diet. The dietician had originally been supposed to talk to us at the beginning of November, prior to the start of the diet. She did not turn up for the agreed meetings, and never communicated, in spite of repeated telephone calls. She made no contact before admission, provided grossly incorrect advice for the first night. Sue meet her for the first time on the first day of the diet, when she provided hand written instructions on the diet, based on the Radcliffe regime. Subsequently, the dietician made no contact with Sue, in spite of repeated telephone calls. In particular, she had been asked by Dr Jardine to put Marchant onto a carnitine supplement, but nothing happened. The meeting on Wednesday was only the second time Sue had seen the dietician.

Marchant’s fits changed in nature. Instead of random movements of one limb, they were concentrated in his right arm, which would jerk slowly, and several times. Marchant was now aware of these fits, and would look at his arm. Also, his two index fingers would curl up while the rest of his hands were relaxed and open. This was not associated with the fits, and would happen for considerable periods of time (10 mins).

By Sunday 16 February, Marchant had become impossible to feed. He was admitted to hospital again on Wednesday 19 February (Ward 36, Bristol Children's’ Hospital, St Michael’s). Marchant was put on a saline drip (30ml per hour). Sue had difficulty in stopping Marchant from being put onto a feed drip (which contained glucose) and eventually had to call Dr Schwartz at St Mary’s Hospital to have this stopped. By the weekend, Marchant was taking about one quarter of his expected food. He was to be given cisapride for the reflux, but the drug was in a sucrose suspension, in spite of being specified as sugar free. Sue managed to stop the drug from being administered, and a sugar free version was made up.

A 24 hour reflux test was scheduled for Tuesday 25 February with Dr Sandhu, but she was on holiday for the week. The test was done by her registrar (Dr Amir). This showed mild reflux (acidity for less than 10% of the time). Marchant’s blood and stools were tested, and a swallow test scheduled for the Monday. Dr Curran, Dr Jardine’s senior registrar, agreed for Marchant to be discharged on the Thursday, because there was no point in continuing to stay - the drip had been removed, there was no medical attention, Marchant could be looked after better at home, and Sue could attend to him at night. Dr Curran agreed to make a follow up appointment with Dr Sandhu.

The speech therapist at Bristol Children’s Hospital saw Marchant three times, and was helpful. On the first two occasions, Marchant was too sick to feed after the third session, she advised that:

• Marchant should not be force fed, but only be fed when he wanted.

• To use different textured foods to encourage Marchant to eat

• To try chilled food

Marchant attended the hospital on Monday 3 March for the video fluoroscopy "swallow" test. This showed that Marchant had an infantile feeding response. He could swallow, but he did not aspirate and had difficulty in pushing food from the front of his mouth to the back. The speech therapist said that, on this evidence, she was not surprised that Marchant did not want to feed and only fed when he was tired. He needed to be relaxed to feed, and would be tired by the feeding process.

For a few days after returning from hospital, Marchant fed quite well and put on weight. Then the cold/flu infection worsened again with worse mucus and a cough, and Marchant refused to feed, or was sick after feeding.

Marchant had been seen by a physiotherapist on a weekly basis. The physiotherapist changed, and the new one, Sally Jary wrote an assessment:

• . . . He presents with mildly increased tone at rest in all four limbs arms > legs. However postural tone rises to moderately severe with effort (such as vocalising, attempts to use hands, balance up against gravity, during eating and drinking). His movement patterns are predominantly into flexion, especially in his thoracic spine and around his shoulders, but with effort can push back into extension at hips and head and a tongue thrust is evident. There is also some asymmetry with more flexion and retraction of left side and more trunk side flexion on the right with flexion and aDduction of right shoulder evident in positions of more extension (e.g. prone Iying, supported standing). The right hip can also flex and aBduct quite strongly, pulling the left leg into more relative aDduction. These patterns place him at risk of spinal scoliosis, convex to the left and hip dislocation, left > right.

• Marchant's mum (Sue) has been given a series of suggestions for home aimed at:

Inhibiting flexor hypertonus, mainly in thoracic spine and shoulders

Facilitating more normal extensor activity, particularly of thoracic spine to

reduce the effect of associated reactions and improve

- head control to enable more selective oral movement during eating and drinking and use of eyes for communication

- freedom of arms to enable play

- sitting posture and ability to be held in supported standing

Reducing risk of contracture and deformity

• Marchant responds well to treatment and his mother has noted improvements in head control and that his arms are looser and he is able to use them more. He currently sits in a tumbleform chair with a small table. He is to be assessed for a supportive standing frame in the near future.

Eating and drinking skills:

• Marchant was seen to gag and then vomit when being spoon fed mashed food due to a combination of oral hypersensitivity, poor alignment of head, difficulty controlling food in his mouth and so difficulty co-ordinating an effective swallow. His ability to control food in his mouth was significantly improved by careful positioning combined with sternal pressure to elongate the back of his neck and use of the spoon to facilitate downward movement of his top lip to take food from the spoon. Recommendations were also made to address his oral hypersensitivity and facilitate chewing away from mealtimes. . .

On Friday 7 March Marchant had an eye test at Tyndall’s Park. Contrary to the previous test, the optometrist said that Marchant was using both eyes, but that his squint had deteriorated.

There was a consultation with Dr Jardine on Monday 10 March 1997. Dr Imelda Bennett was present, also the speech therapist, Moira McKinnon, for part of the time.

• Dr Jardine said that in the first year, nutrition was more important than genes as a determinant of weight. He was concerned about Marchant’s weight, and thought that perhaps Marchant should have a g tube inserted:

• He arranged an appointment to see Dr Sandhu about feeding problems on Monday 17 March.

• He said he was concerned that Marchant was still having fits, and thought that the ketogenic diet should be supplemented by a low dose of lamotrigine - however no decision was made. He agreed that nothing should be done until Marchant had a 24 hour reference EEG, and undertook to arrange this (again).

• He proposed that Sue should talk to the social worker Dominique about respite.

• He provided a prescription for sugar free cisapride to be made up at the hospital.

• Dr Bennett took a blood sample to check whether Marchant needed a carnitine additive.

• The speech therapist arranged to call on Friday 14 March.

She had first been asked to contact Sue on 22 January. Subsequently she had received further requests from Drs Jardine and Bennett, Barbara Butcher, the health visitor and others, but had never made any contact.

Merchant was sick and hardly fed at all on the Tuesday or Wednesday; he lost a lot of muscle tone. On Wednesday 19 March, he was very weak and disoriented. In the afternoon he appeared hypoglycaemic, and Sue gave him orange juice to drink, which perked him up considerably. He was taken to the doctor (Dr Ring) on Tuesday, who said he had a cold, but was not very helpful. It was agreed that Marchant should be taken to Bristol Children's Hospital, and admitted, so that he could be seen by Dr Sandhu immediately. This was not done, because Dr Sandhu was not available that day. The same arrangement was proposed for Wednesday, but again Dr Sandhu was not available. Marchant continued to refuse to eat, and his weight fell.

Sue called Dr Freeman (Johns Hopkins) he said:

• In his experience the diet did not make children sick, and it was far more likely that the cause was the infection.

• That the amount of protein used for young babies should be increased from 1 gm per kilo body weight to 1.5 gm, and that the amount of carbohydrate in the diet could be minimised.

• That the right ratio for a baby was 3:1.

• That the diet operated at 75% of nominal body weight would provide sufficient calories for a baby to grow normally.

Dr Sandhu was seen at the appointment time on Monday 17 March. She said that Marchant should start by having a nasal gastric tube (ng tube) with overnight feeding. If that did not resolve the problem, a gastrostomy and g tube would be needed.

Marchant was admitted to Ward 37 at Bristol Children’s Hospital again on Tuesday 18 March. An ng tube was fitted in the evening and he was fed overnight at 20 and then 25 ml per hour. The feed was a brew provided by the dietician, Alison Connell, as follows:

[Recipe of brew. …. To be inserted]

The cisapride was changed to ???

Marchant did not feed during the day on Wednesday. He was again fed over night via the ng tube, but he dislodged this, and the food was pumped into the bed. In the morning, he was found in a sodden pool of food. Marchant remained in hospital because it was required that Sue be taught how to use the pump. This was supposed to happen on Wednesday, but did not. It was again supposed to happen on the Thursday, but was delayed until the evening. Again, it did not happen, this time because the dietician had the supply of pump tubes locked in her office, and failed to provide one before leaving. The tuition was finally provided on the Friday morning for one minute. Sue eventually left at about five pm.

Status at one year

During the second six months the following measurements were made:

[Status at one year

Weight:

Height:

Head circumference . . . to be added]

Marchant’s fits were largely controlled by the ketogenic diet. He was having about 5 - 20 fits a day, which were slow jerks of his right arm. He was aware of these fits.

{Physical status

Eyes

Teeth

Mental status . . . to be added]

[still no access to medical records - letter to Dr Barrington]

NEED RESULTS OF MRI scan

continue to   Progress with the ketogenic diet
return to   Medical notes