Introducing

[Guest guest]

Hi everyone,

My name is Ray and I’ve just joined your group. I’m originally from the UK but

I’m now living with my Swiss wife, Carine, near Lausanne, Switzerland. We have

two boys : (born Dec 1999) and (born May 2001). was

diagnosed as having earlier this year.

I was so pleased to come across this discussion group the other day and I

welcome the opportunity it gives us to share our experiences and information

together.

Here is ’s story ….

1) A Summary of Events

May 2001:

born (50 cm, 3.57 kg) - an apparently healthy baby

To summer 2002:

Various minor illnesses (colds) – but nothing out of the ordinary

Autumn 2002 – Spring 2003:

Seemed to always be getting fevers – a month never went by without a visit to

doctor. Originally this series was put down to otitis & other childhood

illnesses picked up at the nursery. He was frequently put on anti-biotics. With

hindsight, there probably were a few cases of genuine otitis during this period,

but sometimes only a slight infection was seen in his ears.

Feb 2003:

Had his first (known) febrile convulsion. By Nov 2003 he’d had 6 of these.

June 2003:

Adenoids removed in attempt to reduce incidence of otitis.

Summer 2003 to present:

Continues to be fevered regularly, at least once a month. A struggle each time

to fend off febrile convulsions (see later). Continued to receive anti-biotics

frequently until diagnosed.

Aug-Sept 2003:

Had a particularly bad spell with 4 separate illnesses within 6 weeks – doctors

could only clearly identify the cause of 1, maybe 2, of them.

admitted to hospital for 4 days for an extensive series of tests

including : blood test to rule out leukaemia, EEG and MRI scan to rule out brain

tumour, ultrasound scans and X-rays.

During this stay it was discovered (somewhat fortuitously by ultrasound scan)

that he had an atrophied kidney that contributed only 15% to the overall

functioning of the pair.

Dec 2003:

Examination of kidney problem revealed that was retaining urine in the

bladder. Tests ruled out hypothesis of kidney damage due to reflux from bladder.

However, it was postulated that urine retention and resulting urinary infections

could have been a cause of the recurring fevers.

Feb-March 2004:

Two small operations to solve the problem of urine retention.

April 2004:

Our pediatrician diagnosed as having . Started treatment with

Prednisolone on a case-by-case basis.

2) Our Experience of

is a sturdy, lively little boy who doesn’t look at all “delicate”. He is

big for his age - at 42 months he now measures 102 cm (3ft 4in) and weighs 17 kg

(37 lb). Between episodes he is full of energy, eats well and behaves like any

other kid of his age.

The first signs of the onset of an episode are: listless behaviour, loss of

appetite, a general grumpiness. You can then be sure that within a few hours his

temperature will have risen – typically up to 39.0°C (102.2°F) if unchecked. He

becomes very listless and sleepy at this temperature.

2.1) Febrile Convulsions – An Additional Complication

Because is prone to febrile convulsions we have to be vigilant. This

means giving a suppository or syrup every 4 – 6 hours (day and night) to keep

his temperature in check. Our “alarm level” is set quite low at 37.5°C (99.5°F)

and we do all we can to keep his temperature below this.

Medications used to date:

Syrups:

Dafalgan (contains Paracetamol) 8 ml dose ;

Ponstan (contains Mefenamic acid) 7.5 ml dose ;

Brufen (contains Ibuprofen)

Suppositories:

Dafalgan 300 mg ;

Mefenacide (generic of Ponstan, contains Mefenamic acid) 125 mg ;

Mephadolor (similar to Mefenacide) 125 mg

Drops:

Novalgin (an NSAID containing Metamizol) (dose of 10 drops)

When his temperature falls back below 37°C under the effects of the medication,

we find our lively little boy again for a few hours. He eats well and plays with

his brother just like normal.

We have found that from episode to episode he does not always respond in the

same way to a particular type of medication. So we have to adapt the type on a

case-by-case basis.

Sometimes, even this is ineffective and we have to put him in a bath for 10-15

minutes. Water temperature has to be 2°C lower than his body temperature, so

about 39°C – 2°C = 37°C (98.4°F).

On 6 occasions so far these measures have failed and he’s gone on to have

convulsions. The very first time this happened, I was fortunate enough to be in

the waiting room of our pediatrician’s surgery. It nearly scared the life out of

me. Luckily, help was on hand within a minute and the doctor gave him a 10 mg

dose of Stesolid (a liquid, epam-based drug administered rectally). This

stops the convulsions within 1-2 minutes and temperature falls.

generally sleeps for an hour or so after this. We find he is usually “tottery”

on his feet for a day after. The doctor told us (and it seems to be the case

from our experience) that it is very rare for a second febrile convulsion to

occur within the same episode of fever. We have a packet of these Stesolid doses

on hand in the house and at the nursery in case of an emergency. Note that

giving Stesolid does not end the fever and it usually runs its usual course (4-5

days in total).

2.2) Effects of Prednisolone

Before was diagnosed as having , his fever episodes lasted

typically 4-5 days. They came every 3-4 weeks or so.

Since the diagnosis was pronounced, we are able to give him a single dose of

Prednisolone (60 drops). This remains under medical supervision (i.e. we cannot

do it on our own initiative) as the doctor always wants to rule out a

“non-” cause. This means 2-3 days spent waiting for the results of

urine/blood tests.

The dose of Prednisolone is very effective: within 4-6 hours the episode is

over.

However, it is our experience that using Prednisolone shortens the interval to

the appearance of the next episode. It can be reduced from the usual 3-4

weeks to only 9-15 days. Then you’re back to square one…

2.3) Further Observations

During ’s episodes we have noticed:

- Some small mouth ulcers, but these are not always easy to see/find.

He sometimes complains of pain when we brush his teeth.

- Lymph glands generally swollen

- Sometimes complains that his knees are sore.

- No sign of urinary infection (particularly since the operation in

March 2004 that I mentioned earlier)

- Sed rate high, but no apparent cause of infection

- No evidence of otitis

- No coughing or “runny nose”

- Doesn’t complain of sore stomach

- Bowel movements normal

- Tends to sleep reasonably well

3) Discussion Points

I am interested to know if anyone knows of an alternative treatment to

Prednisolone – if possible something not based on cortisone. I’m just a bit

worried about the long-term effects of this solution. But maybe my reservations

are unfounded (?)

And has anyone any remarks to make about the long-term effects of on a

child’s development (apart from the consequences of periodically missing

school)?

Thanks for bearing with me to the end of this message, which I realize was far

too long ...

Best regards,

Ray

Daddy to (aged 3 ½) in Fribourg, Switzerland

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