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July 14, 2008

The baby no-one wanted

080710emmagrace3hmed11ahmedium Thanks to Heather for sending me this story, which she thinks is the first one in the US mainstream media about a child with DiGeorge. A little baby called Emma Grace was given up both by her blood mother and by her adoptive mother because of her "condition", which happens to be exactly the same as my daughter's: truncus arteriosus and DiGeorge syndrome. The poor baby lay there in hospital waiting for bypass surgery, and nobody came to visit her (though the kind nurses tried to atone for this); when she had the surgery, no-one was pacing up and down outside.

It's completely heart-breaking (and reminds me of the pregnant woman I once spoke to on the phone at the request of our joint cardiologist - her baby also has DiGeorge. Despite my best endeavours, she chose to have an abortion, which at the time made me catatonically depressed for about two weeks). This desperately sad story has a happy ending, you'll be glad to hear - you can read it in full here.

Thank you very much to everyone who wrote about Nell - I REALLY appreciate your messages.

Continue reading "The baby no-one wanted" »

Posted by India Knight on July 14, 2008 at 12:46 PM in 22q11 Deletion | Permalink | Comments (1) | TrackBack (0) | Email this post

July 07, 2008

We're home

Tood_eats ... and it went brilliantly. We'd always been told that Nell's speech problems resulted from some non-specific, mysterious, super-weird  DiGeorge palate anomaly that wasn't, alas, one of the known and correctible DiGeorge palate anomalies, such a a sub-mucous cleft (where the cleft is hidden by the roof of the mouth).

Ergo, we all believed that she was really pretty unlikely to ever speak intelligibly. As I wrote below, we were told that this was an exploratory sort of surgery, and wouldn't produce any tangible result.

Anyway - they went in, the genius surgeon found an occult (i.e. cunningly concealed, even during X-rays and videofluoroscopies) sub-mucous cleft, AND FIXED IT. It honestly feels like a miracle (and like someone's listening when I pray for things that don't sound possible). We are now cautiously optimistic that, with further corrective surgery, Nell may actually speak "properly" and clearly. I am literally delirious with joy.

Other completely amazing thing: she was very poorly and on a morphine drip until Friday lunchtime. It is now Monday morning and she is back at school. Her mouth is sore, obviously - they repositioned the muscles and the whole shape of the roof of her mouth is altered - and she has to keep off crunchy food for a bit, but that's it: she's on nothing stronger than Calpol. Children are amazing. Doctors are amazing. Surgeons are amazing. The world is beautiful.

The picture is of Nell on Friday afternoon, dripless and morphineless, eating, incredibly, not ice cream but a (lightly) toasted cheese sandwich.

Posted by India Knight on July 07, 2008 at 01:50 PM | Permalink | Comments (15) | TrackBack (0) | Email this post

July 02, 2008

Hospital league tables

Heartsurgeonimage There's been lots recently about how marvellous it is that the NHS is to let you
choose your own surgeon. This comes on the back of those terrible, misleading NHS "death rate" league tables, which are now to be published online - basically, you can look up your surgeon and see how many people have died while being operated on by him or her. This all kicked off some time ago with the publication of heart surgery mortality rates in various hospitals.

I expect I'm preaching to the converted and that you've all worked this out already, but there is a really massive point about all of this which many commentators seem to have completely overlooked. I meant to write about it when the heart/mortality figures were first published but I never got round to it.

The point is this: the more complex the surgery, the more likely it is to result in death.  If your stupid league tables show you that nobody died following heart surgery at X hospital, it doesn't mean X hospital has magical powers - it means they probably do very ordinary small repairs, of the kind that are routine (for the surgeons, if not for the parents).

If the table shows that 4 people died at Y hospital, on the other hand, it doesn't mean Y hospital is crap at operations -- it can mean it's so good at them that it contains particularly highly skilled surgeons who are willing (and able) to undertake extremely complex, difficult procedures, even if they are of the high-risk, last-ditch kind.  Sometimes they don't get the outcome they hoped for. That's all.

I do think it's important that people understand this, instead of treating these actually very unhelpful league tables as though they were a surgical version of the Good Food Guide. The thing about giving people these kinds of choices is that it assumes everyone is properly informed and reasonably bright. Ain't necessarily so. You can just see what might happen: someone who needs really complicated surgery picks the surgeon who's not had many people die on him, thinking he's the safest of bets, whereas actually the opposite might be true.

I'll blog again when we're home safe and sound after our own surgery.

Posted by India Knight on July 02, 2008 at 02:12 AM in Current Affairs | Permalink | Comments (6) | TrackBack (0) | Email this post

Ketogenic diet and drug resistant epilepsy

2231597894_3f3d51f4a7_m1 I was just on the Great Ormond Street website and came across this press release. It dates back to May, so apologies if those of you to whom it is relevant are familiar with it, but I do think it's worth reprinting. It's about  a randomised controlled trial about drug-resistant epilepsy and the ketogenic diet, which is high-fat and low-carb. 

I have a bit of a bee in my bonnet about this anyway - I wrote a diet book about losing tons of weight by restricting carbs and eating more "good" fats (butter and olive oil), and today I was just writing a thing about the acne diet (Google it if you're a sufferer), which also involves low-carbing and which can have dramatic results skin-wise. Plus I was reading Felicity Lawrence's new book late last night, which kicks off with a piece about the utter pointlessness of breakfast cereals.

Does anybody actually need useless carbs? It's 1.30 in the morning, so I shan't go into a rant about childhood obesity and the desperately obvious (to me) link between obese children and the amount of nutritionally barren processed stodge they consume, but anyway. Here's the press release. If your child has drug-resistant epilepsy, dump the dough.

Randomised trial confirms efficacy of ketogenic diet

Lancet Neurology press release

A randomised controlled trial has confirmed the efficacy of the ketogenic diet in helping control and prevent epileptic seizures in children with drug-resistant epilepsy. These are the conclusions of authors of an Article published early Online and in the June edition of The Lancet Neurology.

The ketogenic diet has been used widely and successfully to treat children with drug-resistant epilepsy since the 1920s. It is a diet very high in fat, low in carbohydrate and with controlled protein. Although the exact mechanism of action is still unclear, the high fat and restricted carbohydrate content of the diet is thought to mimic the biochemical response to starvation, when ketone bodies*, rather than sugars, become the main fuel for the brain’s energy demands. While there have been many observational studies of this diet, Professor J Helen Cross, Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, University College London, UK, and colleagues have done the first randomised controlled trial to test its efficacy.

The trial assessed 145 children aged between 2 and 16 years who had at least daily seizures (or more than seven seizures per week), had failed to respond to at least to antiepileptic drugs, and had not been treated previously with the ketogenic diet. Enrolment for the study took place between 2001 and 2006, and children were seen at one of two hospital centres or a residential centre for young people with epilepsy. All children had their baseline seizure levels recorded over a four week period. Following this, 73 were assigned to the ketogenic diet immediately, and 72 were assigned to the diet after a delay of three months – both groups had no other changes to treatment. The delay group acted as the control group during the three month delay.

The researchers obtained complete data for 54 children in the diet group and 49 in the control group. Using the baseline figures as 100%, they found that the number of seizures the diet group dropped by more than a third (62% of seizures recorded compared with baseline), while the control group saw their seizures rise by more than a third (136.9% of seizures compared with baseline). 28 of the 54 children who completed the three months in the diet group had greater than 50% seizure reduction compared with just four of 49 children in the control group. Five children in the diet group saw a seizure reduction of above 90%, compared to none in the control group. The most frequent side-effects reported at three months were constipation, vomiting, lack of energy, and hunger.

The authors say: “We have shown that the diet has efficacy and should be included in the management of children who have drug-resistant epilepsy. However, the diet is not without possible side-effects, which should be considered alongside the risk benefit of other treatments when planning the management of such children.”

They add: “In view of this we believe that the diet should be more widely available as a treatment on the NHS, for children with epilepsy, who have failed to respond to anticonvulsant medication.  We stress this is a diet which should only be undertaken on medical advice and under medical and dietetic supervision.”**

In an accompanying Reflection and Reaction comment, Dr Max Wiznitzer, Rainbow Babies and Children’s Hospital, Cleveland, OH, USA, says that more information is needed about the long-term effects of the ketogenic diet, including changes in blood fat concentrations and persistent ketosis. He adds: “Better identification of epilepsies that benefit from starting early on the ketogenic diet and comparisons between the choices of ketogenic diet are needed.”

Notes to editors: *Ketone bodies are water soluble compounds that are produced as by products when fatty acids are broken down for energy in the liver and kidneys. They are used as a source of energy in the heart and brain. In the brain, they are a vital source of energy during fasting.

**This second quote from the authors is their opinion and cannot be found within the Article

Posted by India Knight on July 02, 2008 at 01:34 AM | Permalink | Comments (3) | TrackBack (0) | Email this post

India Knight


  • India Knight was born in 1965. She lives in London with her three children, writes a weekly column for The Sunday Times and has written two fiction books, My life on a plate and Don't you want me? and a non-fiction book, The Shops. After writing an article in The Sunday Times about her daughter's special needs (Nell has a cardiac condition called truncus arteriosus, and DiGeorge Syndrome, aka 22q11 deletion) she was so inundated with e-mails that she has launched this weblog as a forum for parents in a similar position to keep in touch, compare notes and help each other. You can read about India and her daughter here.

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