C/O 3 Swn yr Afon
Llangefni
Anglesey
LL77 7FD
United Kingom

NB: this is a forwarding address for the Pal, we do NOT disclose home addresses

Email Pal

Tie D's Parents/Guardians

Peter

Tie D's Siblings

Jessica 11/2/1998 likes High School Musical, Jade 12/08/1999 likes Hannah Montana, Jamie-Ann 16/12/01 likes Dora the Explorer, Harriet 15/11/2003 likes Ben 10, Adelle 29/08/2005 likes baby dolls. All the girls like playing with sand, playing outside, Hannah Montana, reading books, going out for trips eg. to the zoo and theme parks, playing with nintendo wii and DS lite, playing with ponies, bratz dolls, painting, craft, singing, karaoke, watching films. Mercedes 13/11/07 likes the WotWots.

Tie D's Interests

Going to places like the zoo, Roary the Racing car, Mr Tumble/Something Special, In the Night Garden, Thomas the Tank Engine, the WotWots, Ben 10.

Tie D's Favourite Colour

Red

Is Tie D able to read?

No

Is Tie D able to use hands?

Yes

Is Tie D visually/hearing impaired?

No

Does Tie D suffer from any developmental delay?

No

Story written 2009

Tie was born with skin missing from his lower legs and feet. Within hours his tiny body had become covered in blisters and skin had torn off his left hand. Even his mouth was blistered inside after nurses tried to feed him a bottle of infant milk. No one knew what was wrong with Tie until a week or so later. A diagnosis of severe EB was made whilst at Alder Hey Hospital in Liverpool. Tie was transferred back to our local hospital in Gwynedd where it was found he had worsened to the point where we were told he would likely die. We chose to bring him home to at least be amongst his family.

After round the clock nursing the plucky little fighter turned a corner and his temperature dropped and he recovered. It still wasn't known how long Tie had to live as only a basic diagnosis of Severe EB had been made and a skin biopsy result was needed to tell how severe. The worst form of EB gives only a life expectancy of 1 year. After a painful wait we were finally told that we would have Tie a little longer! He had the second worse form. All of his bodies’ linings are missing the hooks that hold them in place (skin, eyes, mouth, oesophagus etc). Any friction against a lining causes it to detach in part or wholly, leaving either blisters that have to be drained as they appear, or an open wound. When eyes blister, Tie has to spend his time in a darkened environment until they have healed. His prognosis is one of constant wound dressing that we do each day, his fingers and toes will web and fuse together as a result of repeat damage, and contractures will also add to his disability. He is (and will always be) administered morphine, paracetamol and iron until an effective treatment is found. Fluid loss and wound infections pose a constant threat and he always has to be handled extremely carefully so as not to de-skin him. Because of scarring and narrowing of the oesophagus due to repeated damage, he will eventually find eating difficult, which often leads to a gastric tube being fitted in infancy to aid nutrition and administration of painkillers and medicines. Eventually aggressive cancer will set in as his skin loses its self healing power and beings to breakdown. This is likely to happen in his late teens, but children often pass younger from other complications. Tie’s life expectancy is 30 years.

So we do what we can to make his life as happy as possible and also everything we can do to help Debra charity fund the research that we pray will one day save Tie and end his pain.

Every day he lifts our hearts and amazes us with his smiles and determination.

Update 10th July 2010

Tie has had a bit of a rough month with stubborn infections on his legs, which we now hope are finally healing. He also had some nasty damage to his left hand one day when out walking with his dad - he tripped over and sadly as his dad was holding his hand at the time, the skin of his hand was traumatised severely as his hand slipped leaving his skin behind. After many brave minutes, his dad had replaced as much of the original skin as possible and wrapped up his hand in dressings to protect it. I am happy to report that Tie's hand is now almost completely healed and has retained full function – in fact, his specialist nurse commented this month that his hands appear to have less webbing between the fingers than before - which is good news.

Of course there is always some bad to balance out life, and sadly Tie has continued to choke on his food more and more frequently. This really upsets his throat and causes him to bring up blood. Due to the worry that his throat may have narrowed due to damage, his nurse will now arrange for him have a barium test performed to see if his throat has narrowed. If it turns out that it has been narrowed, then we have been reassured that there is an operation they can do to expand his throat, by basically inflating a balloon in it! With no great surprise, this news of his increased choking also comes with news of a weight loss of 3lbs. So, we are once again focused on increasing his nutrition levels and food intake.

On the 23rd of June, coinciding with our 12th wedding anniversary, Tie's baby brother, Calvin, was born. Calvin has EB too, but thanks to the lessons Tie has taught us along with the co-operation of the midwife team at the hospital, Calvin was born with damage limited to his left foot and of a good weight. Since birth, he has continued to do well with little damage occurring - he has certainly had the best start to life that we could have prayed for. We had always said that we would have no further children with EB, due to the extensive affect this severe form of the condition has on them, but as diagnosis was not made until very late in the pregnancy, after much heartache and tears over what may be, we chose to make plans on how to manage the situation to the best of our abilities and feel we have been truly blessed with our second brave soldier.

Tie had a very busy month with also visiting the school that he will be attending for two hours a day come September. He found it a little overwhelming with so many other children about and tired quickly. However, we feel that due to the interest the school has taken in how to provide the best support for him and the fact that he will have his own carer watching over him whilst he is in school, that he will be ok and settle in nicely come September.

Many thanks to everyone for the gifts received this month, Tie and the girls loved playing with all the craft items and toys.

Post Pals provides such a boost for our son, even on days when he cannot open his eyes, he still has a big smile on his face when the postman brings a letter for him. He absolutely loves opening his letters and cards and looking at the pictures. He can’t read yet, but loves us to read them out to him. Thank you all for caring so very much.

Update 11th June 2010

Tie has had a good month on the whole. He only had a couple of days with eye troubles. He has suffered a bit with a sore throat, but it hasn't impacted on his diet too badly and it hasn't developed into anything too dire for him. He has had more blistering with the heat and a couple of weeks of colds made his skin more prone to damage - but the air conditioning stopped the heat from affecting him too badly.

Many thanks to everyone who sent a special gift for Tie or one of his siblings this month, especially to Betty and also to all the children at Letham Nursery.

Update 19th May 2010

Tie has really enjoyed receiving his special post this month. With suffering from a stomach bug throughout, he has been very tired and often very sore - even on days when he couldn't open his eyes he was very happy to hold his post and have them read to him.

A big thank you to everyone who has sent a card to Tie, he really loves receiving them, especially when he is feeling low. Many thanks to everyone, especially Megan from Horndale County Infant & Nursery School, for her letter and picture - Tie really liked your picture.

Update 8th April 2010

Tie has had a reasonable month, with no major injury. He has suffered a few days in the dark with his eyes closed and sore though and also had a few tummy troubles which did reduce his diet a little at the time. He is currently fighting off a couple of infected wounds on his legs which have been hindering their healing, but they seem to be getting better slightly, so we hope they will continue to improve and heal soon.

A big thank you to everyone who has sent a card to Tie, he really loves receiving them - especially when he is feeling low. A very big thank you especially to Jessica and Jade for all the books we received last month. Everyone really loved their books.

Update 12th March 2010

Tie has been fairly ok in the last month. He has had a damaged throat on occasion making swallowing anything an impossibility which caused him great distress, but each occasion settled down within a couple of days. He suffered a minor accident which left him with no skin on two toes and damaged/blistered skin around the area on his foot. The incident really shook him up and he was shaking with the shock of it, but once we had dealt with the damage as best we could, he calmed down and made some craft pictures with us, which really took his mind off his foot.

A recent day trip to hospital for review has showed that he is still gaining weight and currently doing well - we are waiting for blood tests to come back to see if any increase in iron or any other medication is needed.

We would like to thank everyone for the gifts they have sent. Everyone has been thrilled with the books they received in February and Jessica and Jade in particular have enjoyed their gifts and Tie has had a lot of new stories read to him each night. Thank you all very much for letting Tie and his sisters into your hearts.

Update 10th February 2010

Tie has been having a little trouble eating lately, his appetite has been a little shaky, not of great concern, but he has hurt his throat swallowing food a few times and it has led him to eat less as his throat healed each time. It is a worry to us as most kids with Tie’s form of EB have gastric tubes fitted in infancy, as throats tend to narrow with blistering and scar tissue making swallowing difficult. We hope that Tie won’t need one but only time will tell. His weight is still quite reasonable and we hope he will regain his appetite soon.

I would like to thank everyone who sent a smile package to my girls, they loved them very much. It seemed like everyone had really taken their time in thinking what our girls would like and got the packages just right, making big smiles appear. Many thanks.

Update 10th January 2010

Tie had a fall from a dining room chair on the Monday before Christmas, so the brave little fella went through Christmas with a sore face and some internal blisters, but he didn't let them slow him down much and enjoyed himself. When he fell he damaged the skin on his forehead, causing blistering across it. He also took the skin off his chin and his nose and blistered his hands, elbows and knees!

He has healed up now and has started 2010 with a good appetite and a reasonable weight. As usual we are hoping for the best for Tie, and even though we know that 2010 will be a busy year for him full of new challenges like starting nursery in September, we know that he will face each challenge with a smile and a determination like no other. We are very proud of him.

We were absolutely amazed by how many people remembered us on their Christmas card list and many thanks to everyone who sent a card. Again, a big thank you to everyone who remembered Tie and his sisters this Christmas by sending post. We have never seen so many gifts come via post before and Tie and his sisters absolutely loved opening them all. Many thanks.

Update 3rd December 2009

Tie has had a good month, with no major injuries and only a few days with his eyes closed.

The kids will absolutely love their gumball machine when they unwrap it at Christmas – thank you!

Post Pals is fantastic, there is nothing that lifts your heart like seeing your child have a smile on their face!

Update 31st October 2009

Tie had another month of eye sores/infections that often left him with closed eyes a lot of the time. He has also had a number of skin rips and blisters all over. At the moment we are waiting for his current bout of blistering to ease off to give him a better chance of healing up.

He absolutely loves his post, many thanks to all his post friends. Tie loved his Iggle Piggle Doodle toy, he won’t put it down and he even doodles on it when his eyes are blistered and he can’t see! Everyone loved the Halloween toys that came through too.

Update 30th August 2009

Tie has had a better month this month as his eyes have been far better, suffering a lot less blistering thankfully. He has suffered a bit more than usual with blistering on his feet, and his lower back has remained raw, but the worst time he had was in mid August when he asked us to lance a blister or,"bubble" as Tie calls them, and when we looked at his belly where he was pointing there was no sign of any damage - the blister was internal in his bowels i guess – and that caused him a lot of pain over a couple of days. At the moment he is fairly ok and thrilled that his 3rd birthday is just around the corner on the 2nd September!

Thank you to everyone, it is nice to hear from people that care. A big thank you to those who remembered Jade’s and Adelle’s birthdays and it was really nice that everyone received a gift this month – they all loved their special lollipops! Thank you.

Update 3rd August 2009

A lot of this past month has seen Tie passing his days with his eyes closed and in a darkened environment for days at a time, whilst blisters on his eyes and the inside of eyelids healed. Touch wood he has had a few days without eye blisters now and it is feeling like he is over them for a while… or until he rubs his eyes again!

He is still eating okay and receiving post has been putting such a smile on his face! Many thanks to everyone, especially for the party goody bag and the Roary book that was sent – he absolutely loves it!

Update 11th July 2009

Sorry we couldn't make the Post Pals party, but Tie has been finding it hard going with the heat and the trip would have proved too much for him. Thank you for sending him a goody bag though.

I would just like to thank all of Tie’s friends for cheering him up this last month. He has had a lot of blisters, injuries, painful insides, and has been stuck in the dark a lot of the time with eye blisters. Even when he can’t see though, he still loves holding and feeling his post. Thank you everyone for helping the postman put a smile on my boy’s face.

Update 30th June 2009

Tie has had a few quite bad days this month. He fell over whilst holding a spoon in his hand and it ripped off the skin from his left hand, and an hour later he fell again and took the skin off his left knee. These wounds have healed now though and he is back on his feet, but he is finding the heat a little much and tiring, so he is spending most of his time in the house in front of the air conditioning unit. He has had a change of antihistamine to Vallegan syrup for relief of his night-time itching and he has just had a stronger painkiller prescribed for his dressing changes (I’m not sure of the name, but I think it is called Midazolam). It is theatre medicine and we are waiting for the prescription to come though.

Many thanks to all of Tie's post pals. Tie especially loved the cars card that he received and the Bob the Builder pop up card. He also loved the cuddly koala bear that turned up one day. Post Pals is fantastic and it brightens Tie’s mood when something nice turns up in the post for him.