Newquay toddler Joe Way’s website has helped a young boy from Pakistan after the late toddlers family where contacted for help.

 

Steven Johnsons Syndrome which eventually led to Joe loosing his battle for life was also the cause of young Ali Ghazi’s terrible eye damage.

 

Following a group effort from a family run SJS charity in Canada, The way family assisted by Newquay Lions, Patricia Roberts who has suffered from SJS and Linda whose son suffers from SJS along with Crawley Lions Ali’s family where all flown to London for the young boy to have surgery.

 

After several operations in August, the family decided to stay on in the UK for the month of Ramadan, in order to start fund raising for the next stage of treatment.

 

Patricia Roberts from Yorkshire who co-ordinated the UK help said “The prognosis was not as optimistic as we had hoped. Unfortunately, Ali’s eyes are too dry to enable stem cell treatment to be carried out; therefore the family have returned home for a while, to think about the next stage of treatment”.

 

It may be possible for Ali to be fitted with artificial corneae in the future, but at the moment, he is too young for it to have any chance of success, and the surgeons have advised against any further intervention at this stage.

 

This operation would probably be carried out in Boston where pioneering work is being done on patients not suitable for stem cell treatment, but the operation is also carried out at the Centre for Sight in East Grinstead where Ali had his treatment.

 

He had several operations to open his eyes after they had been stitched shut, this had lead to his eye lashes growing inwards causing an amazing amount pain on a daily basis.

 

To ensure that the benefits of the surgery are maintained, it is vital that Ali has an adequate supply of eye drops and ointment to use everyday.

This is most important, as the eye must be kept as moist as possible if future surgery is to succeed. The family have made many friends within the local Muslim community in Crawley who will continue to raise the huge amount of money which will be needed in future years.

 

Matt Way from Newquay said “We have been very humbled by Ali’s story and privileged to of been of some assistance. I had an email from his father this week very excited about a place at a special school to help Ali and continued treatment in Pakistan of which neither was offered to the family before his UK visit so the real success of this story has been the doors opened up and the continuation of his long term care”.

 

Matt continued “we where so taken with Ali’s plight as he was the same age as Joe when he suffered his reaction, you cannot explain what someone goes through when they suffer from SJS which basically is an adverse reaction to any drug. It made us grateful that Joe lived in a country with such great medical staff as he had another 18 months of life for us to all enjoy”

 

To read Ali’s story in full, Joe Way’s story as well as the rare conditions he battled go to www.joeway.co.uk  

 

Latest Update...17th June 2009

Ali is a little boy from Karachi, Pakistan, who is in excruciating pain, and who is close to having life saving treatment in the UK.isation, and the air tickets for the entire family have been donated by First Hand Foundation.
The accommodation at Ramada Hotel Gatwick has been paid for by and the management at the hotel have been very co-operative with regards to future press coverage.

The family will be in the UK from July 5th until July 24th 2009.
This allows them time to be available for interviews and plan for future procedures, once a full surgical plan with costs and anticipated time frame has been established with Dr Daya.

Patricia Roberts and Linda Harcourt Smith will be travelling to the hospital to meet Ali and his family, and discuss what further help will be needed. Patricia will be selling greetings cards to raise funds in a few weeks time.

How can we help Ali Ghazi Zaidi

Ali is a survivor of Stevens Johnson Syndrome - SJS and as a result of the long term damage to his eyes, lives with severe ocular surface disease, a combination of numerous painful and debilitating eye conditions.  

Ali had the most severe form of Stevens Johnson Syndrome known as Toxic Epidermal Necrolysis.  All of the mucus membranes are involved and in addition to the external involvement shown in the photos, the same process was happening internally to the mouth, esophagus / trachea, lungs, gastrointestinal tract and even the genitals.  

During the reaction itself, supportive treatment is all that can be offered medically and the fatality rate is over 40%.  While a lot of the skin involvement has now resolved (Ali has substantial hyper pigmentation over 90% of his body) the most severe long term damage has been to the eyes.  Stevens Johnson Syndrome patients are not candidates for cornea transplant and this young boy must travel to England, for intensive occuplastic surgery to reconstruct his entire ocular surface so that he can have cornea stem cell graft using corneal epithelial cells grown from the mucus membranes in his mouth.  I would like to describe the different conditions that young Ali lives with every day. It is worth noting that the current ocular diagnosis is standard in SJS survivors and daily life is excruciating for this young boy. 

Request for your help:  

Liaquat National Hospital, Pakistan Feb 2009:  Further to previous examinations, Dr. Imran Ghayoor notes deterioration in Ali's eyes with additional entropian and symblepharon which will require surgical repair, and has referred him to the Queen Victoria Hospital in East Grinstead just outside of London for extensive surgeries unavailable in Pakistan or India.   The Queen Victoria Hospital is recognized as a world leader in the type of ocular surface disease (cornea / conjunctiva) and occuplastic (lid) deformities caused by SJS.  Both Dr. Daya and Dr. Malhotra of the Queen Victoria Hospital have spoken to the doctors in Pakistan and India.  Both Dr. Daya and Dr. Malhotra will perform an examination under anaesthetic to determine a surgical plan for complete ocular surface reconstruction. Based on conversations with Ali's current ophthalmologist we know that Ali will, at minimum, require: 

1. Surgical removal of symblepharon with complete fornix reconstruction.
2. Mucus membrane graft to upper and lower lids OD / OS (both eyes)
3. Surgical repair of previous failed Tarsorrophy and Entropian.
4. Permanent removal of eyelashes to prevent reoccurrence of Trichiasis

Upon completion of the reconstructive procedures Dr. Daya will then establish Ali's candidacy for stem cell transplantation using the latest techniques of tissue engineered corneal epithelial cells grown from autologous oral mucosal cells (patients own mouth). This method of stem cell growth is not available in India where they use donor cornea stem cells for which Ali would not be a candidate due to the dry eye and required immunosuppressive therapies. Upon completion of the reconstructive surgery, Ali will hopefully be fitted with a Boston Ocular Surface Prosthesis, see http://www.bostonsight.org/. 

There is lots of potential for this young boy's vision and a pain free life.  The only problem so far has been accessing the necessary technologies and the funds to proceed with treatment.  As such we would like to ask for your support in our fundraising efforts

  during SJS            

How can you help

  After SJS             

Send donations to the trust below

Please make cheques payable to “Centre for Sight Trust” write Ali fund on the back and send to:                               

Corneoplastic Unit & Eye Bank                

Queen Victoria Hospital                               

East Grinstead W. Sussex RH19 3DZ
Tel   07000 288288 or +44 1342 321 201    

http://sdaya@centreforsight.com/          

http://www.centreforsight.com/

We need a desperate push for some spending money for food etc.   Does anyone have any ideas? email info@joeway.co.uk

     

National newspaper stories about Joe

Join Joe's Facebook Group

YouTube - joeway.co.uk's Channel

joe way - Tags - mirror.co.uk

Source: www.mirror.co.uk

A boy who featured in a national campaign for hospital cleanliness after he battled 14 superbugs has died - aged four....

Boy, 3, who fought off ten superbugs | Mail Online

Source: www.dailymail.co.uk

They say that cats have nine lives but three-year old Joe Way has already gone one better. The little boy has shown astonishing resilience by fighting off ten superbugs – any one of which could have killed him

Four-year-old who battled 14 superbugs and became the face of a national campaign for hospital clean

Source: www.dailymail.co.uk

A toddler who became the face of a national campaign for hospital cleanliness after he battled FOURTEEN superbugs has died aged just four

    

Steven-Johnsons Syndrome

We do not know why Joe had the reaction know as SJS it could of been triggered by a Drug or virus.

Although the first bad reaction was thought to be Drug related as he started a new drug it was probably that over the two years and his final reaction was viral triggered with his body fighting against other problems.

Most patients survive SJS but do not underestimate the effort or the medical care required.

SJS and lung failure due to on going issues after Joe had complete organ failure with serious damage to his lungs & kidneys was the start of what two years later became too much for him.

See our medical facts page and other links www.joeway.co.uk/info/stevens-johnson.aspx 

Blog your daily thoughts to others and pass on information

Be inspired….

 

April 2009 up dated news

NeLM news service

Drugs associated with Stevens-Johnson syndrome in children,Reference: Pediatrics 2009; 123: e297-e30, Source: Pediatrics, Date published: 09/02/2009 15:54

by: Jim Glare 

Analysis of two previous case-control studies confirms the association between anti-infective sulphonamides, phenobarbital, carbamazepine, and lamotrigine, and an increased risk of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in children. The data also suggest a possible associate with paracetamol. 

The mucocutaneous reactions SJS and TEN are variants on a spectrum; they are rare (estimated incidence 1 to 2 cases per million population per year) but serious: mortality in TEN may be over 40%, and many survivors will have serious sequelae. By definition, skin detachment over <10% of the body surface area define SJS, whereas >30% defines TEN. Between 10% and 30% is termed SJS/TEN-overlap. In adults it is estimated that around 70% of cases are adverse drug reactions, although other non-medication factors may also be involved. While a number of drugs have been implicated in adult cases, there is limited information on those most likely to be associated with the condition in children. The authors of this study extracted data relating to children (age <15) from the two largest available case-control studies of SJS/TEN (SCAR, n=368, and EuroSCAR, n=383) to identify those drugs most likely to be implicated in childhood cases. 

They selected children from the pooled studies, and matched each case with up to three controls by age, gender, and country. Controls were selected from patients hospitalised for acute conditions not expected to be drug-induced (e.g. infection, trauma, etc.). Drug exposure was assessed for the 7 days (3 weeks for phenobarbital) preceding the probably day of disease onset. A possible spurious association with drugs given to treat the early symptoms was controlled for by repeating the analysis for suspect drugs with an onset day three days earlier. 

The pooled data provided 80 cases aged <15, and 216 controls; 26 were classified as having SJS, 34% TEN, and 40% SJS/TEN-overlap. Median duration of hospitalisation was 17 days, and six cases died. Cases were much more likely than controls to have taken drugs in the period before onset: only 7.5% of cases took no drug in the week before onset compared to 25% of controls, and mean numbers of drugs to which they were exposure were 2.4 for cases and 0.75 for controls. In a univariate analysis, anti-infective sulphonamides, phenobarbital, lamotrigine, and carbamazepine were strongly associated with SJS/TEN.  Statistically significant associations were also found for valproate (remaining statistically significant when patients taking other anticonvulsants were excluded), NSAID as a group, and paracetamol. The association for paracetamol remained statistically significant when the analysis was adjusted to minimise cause and effect confusion. 

The authors conclude that their data support the hypothesis that medication use is the major risk factor for SJS/TEN in children, and confirm an association between four previously suspected drugs / drug groups (anti-infective sulphonamides, phenobarbital, carbamazepine, and lamotrigine) and significantly increased risk of SJS/TEN in children. They also identified an increased risk associated with valproate, paracetamol, and NSAID as a group: this association could not be completely explained by bias due to their use as symptomatic treatment for a condition precipitated by one of the highly suspected drugs. There were insufficient data to confirm or refute associations with other drugs suggested of being causative: 30% of cases were not exposed to a highly suspected drug, and 7.5% were not exposed to any drug within the preceding week, suggesting a contribution from other non-drug risk factors. 

© National electronic Library for Medicines 2007 Guy's and St Thomas & UKMI