Its only skin

I talked a little bit about what ichthyosis was quite early on in the blog but I wanted to delve in a little further.  Ichthyosis is a group of skin conditions and it is very easy to assume that it just involves the skin.  This couldn’t be further from the truth.  When I use examples in this post I am talking from experience of being a mum to a child with EI (Alfies type of ichthyosis) but they are not exclusive to this type.  All types of ichthyosis come with their own set of issues but many are common across the different types.

Dry, flaky skin:  This is a staple characteristic of ichthyosis.  When I say dry, this is not the dry skin that people with (and I hate this word but can’t think of another more suitable one at 11pm) ‘normal’ skin get that can be resolved by a little bit of lotion; this is chronic dry skin that can absorb even the thickest of greasy ointments.  Alfie once described the puff pastry pie lid he was eating as looking like his skin and he was right!  The skin requires moisturising multiple times a day to stop it from drying out completely.  The skin flakes a lot and we often find large bits of skin around the house; this does not bother us in the slightest, we can always tell where Alfie has been and I like that he leaves a little trail.

Itching:  Mostly this comes from the skin drying.  Anyone with a skin condition knows that the dryer it gets, the more itchy it gets.  The itch can drive you crazy.  Alfie does get itchy, especially at night and as we have had to share a room with him recently due to building work at home we have noticed it more; he is constantly fidgeting in bed and rubs his feet together! He will itch his scalp when he is tired or hot and he will itch at other parts of his body when he needs cream on.

Blisters: This characteristic is (I think) exclusive to Alfies type of ichthyosis.  These are not like blisters that you or I get.  They can be caused by heat, friction, infection or trauma to the skin and they can also appear randomly out of nowhere.  These are also unlike regular blisters in that we cannot leave them to go by themselves because they are very painful and they just get bigger.  We have to burst them with a sterile needle and dress them if they are infected.

Fragile skin: Alfies faulty keratin gene means that the layers of skin do not hold together properly.  If he knocks himself or falls over he will take the top layers of skin off leaving red raw, sore skin that is easily prone to infection.

Thickened skin: Ichthyosis skin grows much faster than regular skin but it does not shed at the same rate.  The skin builds up into thick plates, scales or in lines.  For Alfie this is mostly around his joints.  The thick skin can crack easily which is sore and can lead to infection.  We aim to keep the skin exfoliated as much as possible to reduce the cracking without making the skin so soft and thin that it becomes very fragile.

Infections: Ichthyosis skin is prone to infection.  Over Alfies 4 years he has needed quite a few courses of anti-biotics for skin infections.  The first time that Alfie got an infection was very scary.  He had a small 10p size blister on the base of his back that became infected.  Over night the infection had spread to half of his back.  We now know what to look for and make sur we catch infections early before they have chance to spread.  We can usually catch the infection before we need anti-biotics and can treat it with hydrogen peroxide cream and specialist dressings.  If left untreated, in certain circumstances, skin infections can be life threatening.

Mobility:  Alfies mobility has been affected by his skin.  When he was a baby he held hi body in positons that were comfortable for him.  Over time his muscles and joints became tight and he needed physiotherapy.  He never rolled over or crawled as a baby and was late walking because of this.  He still walks slightly differently to his peers and his balance is not as good as his peers.  He find his own way of keeping up with his friends though and never lets his mobility get in the way of having fun.  Alfie has daily physiotherapy to help him with the joint and muscle stiffness and this has improved a lot.  Alfie cannot always walk as far as other children and we currently have a board that attaches to Renes pushchair and if he gets tired, he hops on and hitches a lift with her.  When Rene outgrows the pushchair we will have to consider the possible use of a wheelchair if his feet are sore or if he cannot walk far.

Overheating:  Many people with ichthyosis cannot sweat and therefore cannot regulate their body temperature.  Alfie does not struggle with the cold weather but the warmer weather is becoming an issue.  He seems to be ok in anything up to around 18 degrees C but anything over that, especially in direct sunlight, seems to be problematic.  Overheating and heat stroke can happen very quickly for people with ichthyosis so we have to be able to recognise the early warning signs of overheating and we act quickly to cool him down.  Early signs include scratching the back of his head, neck or ears, red face, irritability.  Alfie will sometimes tell us that he is hot as he is starting to recognise signs himself.

Ears:  Many people with ichthyosis have issues with their ears.  Ears can become blocked with a build-up of cream, skin and wax.  Alfie has his ears checked every 6 months when we take him to his dermatology appointments.  He has them cleaned out as needed but luckily because we have them checked regularly and we clear away any lose skin we can see, he has never had any problems with his ears. 

Diet:  Due to the high regeneration of skin cells, people with ichthyosis use many more calories than usual.  Alfie struggles to put weight on and can easily lose weight if his skin is sore or is healing from an infection.  As a baby Alfie was prescribed a high calorie formula to enable him to put any weight on.  He eats like a horse now but still struggles to put weight on so he also has 2 high calorie supplements every day which add up to an extra 350 calories extra per day.  Even with the supplements, he is lighter and shorter than most of his peers.

There are other things associated with ichthyosis that I haven’t mentioned, some things run across the board whilst others are very type specific.  The point of todays post was to highlight that ichthyosis is so much more than just dry skin.  It affects many aspects of life, not only the skin that you can see.