Information and guidance

Treatment of IS with steroids/ hormonal treatment

 

  • In the UK the treatment you are likely to be offered is PREDNISOLONE, which is available as dissolvable tablets or liquid. It is a corticosteroid, which is a similar to the hormone cortisol normally produced by the adrenal glands.
  • In the USA, the hormonal treatment most often prescribed is ACTH (adrenocorticotropic hormone) injection. It forces the adrenal gland to produce high levels of cortisol, so the two treatments act on the same pathway and have similar side effects.
  • In some cases ACTH may work where prednisolone has failed, and vice versa.
  • Often parents will be given the option of hormonal treatment or vigabatrin (usually to start one with a plan of introducing the other in a few weeks if spasms have not stopped). Recent research published in December 2016 showed that the combination is effective in about 20% more cases, so it is worth discussing whether to start both straight away.
  • Prednisolone needs to be given at very high doses (>4mg/kg/day) to be effective against IS. Older studies used 2mg/kg and this is not very effective.
  • Most centres use the same protocol as the UKISS study which is 40mg/day irrespective of the weight of the baby, increasing to 60mg/day if this is not effective in the first 7 days.
  • The aim of treatment is cessation of all spasms and clearing the hypsarrhythmia within 2 weeks of high dose treatment, after which the steroids will be weaned, typically over 2-3 weeks.
  • Stopping steroids abruptly is very dangerous because the high doses given suppress the adrenal gland and stopping can lead to adrenal crisis. Adrenal suppression is sometimes a problem even after a slow wean and parents need to be aware of the symptoms to look out for;

Cold, clammy skin.
Fast heart rate and breathing.
Weakness.
Confusion.
Irritability.
Poor feeding.
Dehydration: dry tongue, increased thirst, fewer wet diapers, dark circles under the eyes.

The main potentially serious side effects of steroids are immunosuppression, diabetes and high blood pressure. Your doctor should be monitoring blood pressure and blood or urine glucose throughout the steroid treatment. Your baby will be considered immunosuppressed from the third day of steroid treatment and for up to 6 months afterwards. You need to take precautions to reduce risk of infection, and should be given information on delaying vaccination after steroids (not recommended to vaccinate for at least 3 months) and what to do if your child is exposed to a serious viral infection like chicken pox or measles.

  • The side effects that parents are often not warned about are how hard it is to care for a baby on steroids. They are likely to be ravenously hungry/thirsty all the time and gain weight rapidly. They are often grumpy and irritable, sleep poorly and are hard to soothe. They will develop a “cushingoid’ appearance – big cheeks, thin skin leading to reddening of cheeks, acne, excessive hair growth, and weak muscles. Most babies stop smiling and appear spaced out, parents often worry that the smiles have gone for good.
  • It is important to remember that however hard this period is, the majority of these effects will disappear as the steroids are weaned. Try to call in some help to get a break for yourselves during a trying time!
  • It is not unusual to get all sorts of weird movements in a baby on steroids or during/just after the wean, for example head shaking, head rubbing, eye rolling, twitchy movements. Many of these are just steroid side effects but it is best to video anything suspicious and send footage to your neurologist as some of these types of movements could be seizure related.

Pictured here Faith and Jorgie with what is commonly known as ‘steroid cheeks’ these symptoms do disappear.