This year we have awarded the “Eve’s Grant” funds to a remarkable brave boy by the name of Lewis Jeynes. His story is an incredible one, both brave and sad.
It was a honour to get involved.
Eve’s Grant is our way to continue to help other Doncaster people, not necessarily just the unfortunate people suffering with Cancer but Doncaster people that still need that little bit of support or assistance.
Please read below the full story or Lewis told by his mother:
My son Lewis was born on 28 February 2004 after a normal pregnancy with no problems. The labour was quick and uneventful.
Lewis was beautiful, perfect, and he reached all usual milestones up to the age of 2½ years. He learned to hold his head up, to roll, to crawl, to stand and to walk; to talk and to eat. He was brilliant at sports, even at that age!
Not long after his 2nd birthday, February 2006, Lewis started limping. We sought medical advice and were told he probably had Irritable Hip Syndrome (something quite common in toddler boys). The doctor said we should rest Lewis for a couple of weeks which we did, although it was difficult to keep a 2½ year old from walking and running about! His limp went and we put that behind us. Unfortunately, around a month later the limp came back and one morning Lewis could not stand – his legs gave way.
We went back to the doctors and were referred to the hospital for tests. This started six months of watching Lewis deteriorate before our eyes. During this six months, the epileptic seizures started. We were so scared.
Lewis had so many tests, non-invasive to start with and then more invasive as the negative and normal results kept coming back. Every test or scan had come back as completely normal. Lewis was a mystery.
By Christmas 2006, Lewis could not stand, crawl, sit, use his left or right hand, hold his head up or speak or eat. He had to be fed via a tube in his nose. This was soon replaced with a tube into his stomach as his weight continued to drop. Still we had no idea what was happening to our son.
Lewis continued to be tested as new tests or new suggestions for his condition arose. As we quickly ran out of options, we agreed to very invasive brain surgery to see if an answer could be found there. The only thing they could discover was that Lewis had dark pigment in his brain cells which was highly suggestive of Batten Disease. He had been tested for this in 2006 with a negative result so we were shocked to hear these words being spoken again, but again the results came back negative. Around this time, Lewis was put on the DDD study which is where Mum and Dad and child give a saliva sample which is then used to micro analyse DNA to find any mutations or deletions in the chromosomes.
Lewis physical condition continued to deteriorate and his seizures continued to cause huge problems for Lewis. He also suffered from positional problems and developed dislocated hips and a severe kyphoscoliosis. His bones became very weak from lack of weight bearing and so he suffered several fractures.
In 2013 he caught a tummy bug and got severe diarrhoea. At this time his blood levels and physical condition deteriorated rapidly so he was admitted to hospital as an emergency to try and find out what was wrong. Lewis’ bloods were so poor that they thought he might have bone marrow failure. Before carrying out very painful bone marrow aspirate surgery, the doctors decided to stop giving Lewis his main anti-epileptic medication as this particular AED can cause bone marrow to work less effectively. Lewis had lost so much weight, he was having severe diarrhoea several times a day, and he was also vomiting what looked and smelled like diarrhoea. This liquid was also oozing and sometimes gushing out of his PEG stoma which was becoming badly infected.
Less than a week after Lewis stopped his AED, he went into status epilepticus and had to be sedated, intubated and transferred by Embrace to intensive care at Sheffield Children’s Hospital. While in ICU, the doctors discovered through doing a fluoroscopy that Lewis abdomen, bowel and stomach were all joined together. This had happened when he had his PEG replaced in 2010. The surgeon had accidentally put the peg completely through Lewis’ bowel on its way to his stomach and a tunnel had grown connecting them all together. For a period of time, Lewis bowel was completely bypassed by the tube which was going straight through it, but his bowel was also openly connected to his abdomen which explained why Lewis’ PEG stoma site was always infected. We can only guess that the severe diarrhoea bug forced the end of the PEG tube to migrate backwards through the tunnel and so that it was sat in Lewis bowel, so that when we were feeding him, the food was going directly into his bowel and the contents of his bowel were also being pushed back up through into his stomach, and out of his PEG stoma, causing infection. So basically, Lewis had a feeding tube going into his bowel, he was receiving very little nutrition and his stomach was being traumatised by faecal matter which he was then vomiting. Lewis weight dropped from around 26kg to around 15kg during this time. He was very ill.
Because Lewis was too weak to have surgery to correct the PEG problem, it was agreed he would have an interim surgery to shut off the old PEG hole, and to fit a new tube into his abdomen which would feed down through his stomach into jejunal area (top of intestines). This would allow Lewis to be fed bypassing his stomach and the surgeons hoped that the stomach and bowel would heal themselves as they would be rested.
Lewis was moved from ICU onto the medical ward to await surgery. The surgery was a success and we planned to go home with Lewis and return to hospital in 3 months to check if the holes had healed, and if they had not, the surgeon would carry out a surgery to stitch and repair the bowel and stomach when Lewis was more able to cope with the anaesthetic, etc.
The evening before we were due to bring Lewis home, he was again rushed into ICU because his left lung had collapsed. He was sedated and intubated again.
As the doctors had discovered why Lewis bloods were poor (malnutrition), they re-started his anti-epileptic medication. Lewis’ lungs were struggling with profuse secretions, which had caused the original lung collapse, and this continued to happen. Lewis needed suctioning almost constantly (which he had never needed before) and chest physio to try and keep his lungs clear of plugs which would cause them to collapse again. It was a battle but eventually we got Lewis off the ventilator. He was doing ok, and again we looked at bringing him home, but unfortunately he went into status epilepticus again so had to be re-sedated and ventilated and went back into ICU. Once the seizures were under control, Lewis was again extubated and transferred back to HDU and again we thought about bringing him home. Unfortunately, Lewis got a stubborn strain of urine infection and the antibiotic which was prescribed is an antibiotic that completely zeros the levels of AED in his system. Lewis went back into status and again we went into ICU for his longest period of ventilation. Lewis accidentally extubated once which was a very terrifying time as the tube became lodged in his airway and he could not breathe. He was quickly re-intubated, but through his nasal passage to see if that would help him not to cough up his tube again. Unfortunately, with the nasal tube, the medical staff could not suction Lewis (due to the shape of his nasal passages) so he had to be re-sedated and re-intubated through his mouth again. Each time Lewis was intubated, he had to be paralysed. The doctors use an anaesthetic that completely paralyses the body, to the extent that the lungs cannot breath on their own. During this time, a doctor will breath for Lewis with a ‘bag’ until he is back on the ventilator. The most terrifying experience I think was taking Lewis down to the fluoroscopy room while he was intubated – they had to sedate him and paralyse him so his lungs stopped working on their own and a doctor had to ‘bag’ him down in the lift, through the procedure, and then back up in the lift to ICU and back onto the ventilator. Terrifying to think one person is breathing for your child.
As Lewis’ lungs had become accustomed to being assisted with the ventilator, it was difficult to get him off. The first attempt, he was off the ventilator for around 30 minutes before they had to re-intubate him as he could not breathe on his own.
We let him have a couple of days to let the sedative completely leave his system (he had been on enough sedate to put a grown man into a coma, but had remained awake and having seizures). The extubation was tried again and was successful but this time Lewis was put straight on to non-invasive ventilation which is a face mask that blows air into the lungs to keep them open and prevent them from blocking off.
As we again prepared to bring Lewis home, this time on the face mask ventilator, he continued to require oxygen and his suction requirements were so high that he was suffering from severe trauma to the nasal passages, throat and trachea because of the need for deep suction.
The decision was made to give Lewis a tracheostomy so that he would breath through a tube into his neck and we would then come home on a ventilator once we had all been trained. This took 3 months and finally in December 2013 we brought Lewis home – a completely different Lewis – but we were home.
The support I got from SWAN UK was amazing. The parents I met (online and in person) through SWAN have become lifelong friends who helped me to keep going, had amazing advice, and offered incredible support.
We still had no diagnosis but Lewis had been put on the DDD study in and so we were hoping we might hear something soon.
In July 2014 we finally received a diagnosis for Lewis. The dreaded words Batten Disease arose again. The second stage of the DDD study discovered a gene defect which both Lewis’ father and I carry and which we both passed on to Lewis. Batten Disease is a rare neurological degenerative disease also known as neuronal ceroid lipofuscinosis. This is a terminal disease and incredibly life limiting.
We would not have found this out if not for the DDD study. It has helped us immensely because we can now plan what future we have left with Lewis, and do all the things we want to with him while he is still well enough. That is why we have started the GoFundMe for Lewis’ UK Pilgrimage and Wish List. We know Lewis is too ill to travel abroad, hence our UK travels where we intend to visit as many of Lewis’ fans and supporters as we can. Lewis’ supporters and now great friends have come from our links with the charities, Post Pals, SWAN UK, Strongbones Children’s Charitable Trust, and through word of mouth via friends and family.
Without the DDD study, we would have continued to blame ourselves, what did we do wrong? What could we have done differently? Now we know that Lewis had this disease the second he was conceived and that nothing could ever change it.
You can find out more about Lewis and find information related to his story through the following external links: