Ichthyosis Awareness Month: Jayden and Jane's story. "there are people in this world that are born a little bit different and that it's ok,"

Little Jayden has Harlequin Ichthyosis - his mum Jane writes of the challenges of giving birth to a baby with a very rare and severe skin condition. As she writes, Ichthyosis can affect so much of a baby's development - from feeding and weight gain to movement. She cites the stares Jayden receives as being the hardest thing to deal with. The assumptions that strangers make about her parenting are utterly intrusive and accusatory. I really hope that by sharing these stories people will think twice about making comments about a stranger's appearance - especially that of a child.

It's a small world because Julie, mother of Jack (who has Netherton's Syndrome) told me she and Jane are friends.

Meet Jayden and Jane.

"It all started in 2009, my partner had just left his job on the front line in the British Army and I was in my last year at college, training to be a beauty therapist, my dream was to travel around the world whilst doing the job I love on cruise ships. I had each step of my life planned out, until I found out i was pregnant, all of my careful planning had to be put on hold. As the weeks passed by we were ecstatic to announce we were expecting a perfectly healthy baby boy! 9 days after completing my course and at 38 weeks pregnant, my waters broke and I coulnt contain my excitement that my baby woud be here so much sooner than expected! 5 days after and after 15 hours of labour I was finally rushed down to theatre for an emergency cezarian. Jayden was born 9 days early weighing 6lbs 4oz.

At birth it was clear to see that Jayden’s skin did not resemble that of a newborn or of anyone else for that matter. I didn’t see Jayden right away due to complications during the cezarian, I had to be put under general anesthetic. When he was born he was rushed straight to SCBU (Special Care Baby Unit) he was put in an incubator and on a gel bed to protect him from the outside world. When I finally woke up my partner could only describe Jayden as looking like The Rock Man out of the film The Fantastic Four! Seeing Jayden for the first time was hard, he was so tiny and his skin looked so painful and sore, he was hooked up to all types of monitiors and he was being tube fed as he couldn’t suckle. A team of specialists had gathered in Jayden’s room to see if they could obtain information so we could have a rough idea of what condition we were dealing with. That was the first time that we heard the word Ichthyosis.

Jayden’s condition was severe and we were told to spend as much time with him as possible because he may not survive past a few weeks or even days. In the following days we were told all about ichthyosis, we were told things such as there is a high risk of fatal infection, respiratory failure and dehydration, being just some of the things we were faced with in our newborn babies future. Jayden was tube fed a high calorie milk called Infitrini, this was to help him gain weight, his weight was dropping rapidly due to all his energy and calories bring used up by the rate of his skin growth. The hospiatal chaplain came to visit us to pray for our little baby and the rest of our family.

At 2 days old Jayden was transfered to a childrens hospital. That night he had his first operation to remove the colodion membrane from his hands and feet. As I was still in hospital all I could do was wait and hope for good news. After that I didn’t see Jayden for 5 days as we were in different hospitals, so I made the decision to discharge myself so i could travel to see him. Jayden baffled hospital staff because at first he looked like he had a condition called Harlequin Ichthyosis but on closer inspection showed he didn’t have the typical characteristics of a harlequin baby. Later on, genetic testing proved he didn’t have the typical mutation on the ABCA12 gene as other harlequin sufferers, he has new mutations on this gene that have never been described before but are still inkeeping with Harlequin Ichthyosis. Jayden was oficially diagnosed with Harlequin Ichthyosis just before he turned 2 in summer 2012.

When Jayden was eventually allowed home life was difficult for a while, we had endless hospital appointments to attend and it was hard for us to get used to all the new names we came across such as a dietitian, peadatrician, ENT, opthamologist and dermatologist! People stared at Jayden everywhere we went, above all this was the hardest thing to deal with, it broke my heart knowing that strangers were looking at my beautiful boy in horror and disgust.

In September 2011, when Jayden was around 15 months old he was referred to a physiotherapist to help improve his moblilty, I longed for Jayden to be able to walk and even run around with his friends and I could see it when I looked at him that he was so desperate to get up and play with them too. It took him a long time to walk but at about 17 months old he took his first few steps alone! He was still very unsteady and fell over a lot in the following 6-7 months, it was so difficult to winess each fall because every time he would cut himself which would cause him to bleed. Although he was doing very well he still struggled to do things such as clumb up and down stairs and get down onto the floor and stand up by himself. It wasn’t until his liver biopsy in 2012 that he began to crawl, by this time he was around 27 months old.

At the start of 2012 we discovered he had abnormal liver function test results, he was admitted to hospital for numerous tests and ultrasound scans. In September 2012 he had to go under anesthetic for a liver biopsy and endoscopy. The results showed he has an enlarged liver and spleen and severe scaring on his liver, the cuase of the scaring it still unknown. If specialists had to guess, they would put the scarring down to the drug Acetretin he was on fom birth, althoughto their knowledge this has never happened to anyone who has taken the drug before. Jayden was taken off this drug months before as his skin was becoming very sore and he had severe photosensitivity, which is a side effect of the drug.

In February 2013 he had operation no3, his plastic surgeon grafted a piece of skin from his arm and put it in his fingers to help straighten them out from the fixed fisted position they were in. He also had his ears deep cleaned to remove any skin build up and 4 front teeth removed due to tooth decay which he got from drinking lots of milk throughout the night to aid rehydration which resulted in the milks natural sugars sticking to his teeth despite me brushing them twice daily. In the weeks after his operation he was put under sedation a few times to change the dressings and clean his wounds. In May 2013 he started seeing a occupational therapist to help with his hand movement. In this same month he also started seeing a speech therapist which he is doing so well in!

The past 3 years have been very up and down for Jayden and the rest of the family. He has been through a lot and come a long way but he still has a long way to go. We are already talking about his next few operations in the coming years, not to mention the constant skin care routine he has to go through and his daily bath and shower to aid the skin shedding process. We have tried many different creams along the way but are currently very happy with our daily routine. Jayden has never had a full nights sleep due to the soreness and itchyness of his skin, he takes a medicine at night calles Alimemazine which helps him sleep and reducs itchyness. Some nights we put him in Dermasilk pyjamas which help to keep him cool and stop him from overheating-which is another struggle especailly in the summer months.

Jayden is a happy little 3 year old just like any other! The only difference is, that because of how unfamiliar most people are to ichthyosis some people can be very cruel to both Jayden and myself, parents have pulled their children away from Jayden so they do not catch anything from him-may I add ichthyosis is not cantagious its genetic!

People say that this is a cruel world we live in but until that cruelness is directed straight towards your family, then you have no idea. I have also been acused of leaving him in the sun to become sunburned and even having spilled hot liquid on his skin for him to become scalded. Some people can be very inconsiderate towards our feelings which is why raising awareness for ichthyosis is so improtant. Jayden started nursery in September 2013 and I am hoping he will settle in well and make lots of new friends while he is there.

I don’t think there will ever be an end to Jayden's struggle but I hope that the more people know about ichthyosis the more people will begin to understand and accept that there are people in this world that are born a little bit different and that it's ok! Most people who meet Jayden become instantly captured by his bright blue eyes, his fun personality and his general love for life!"

 

May is Ichthyosis Awareness Month - I am sharing stories of people who have experienced Ichthyosis. Read all stories in the Ichthyosis Awareness Month Blog Project here.