Ellena's Gallery

Ellena's Diary from June 2012

Ellena's Diary from June 2012



We got Ellena home on the Thursday, she was stable on 1 litre of oxygen. Granny Margaret came round for cuddles and told us that she has got Ellena a tree planted at Normanton Le Heath, Leicestershire forest in celebration of Her Majesty The Queens Diamond Jubilee, the tree is named Ellena May Page "Our beautiful Shining Light".

It was wonderful to get Ellena home. She enjoyed sitting in the sun in the garden although she did make us laugh because she has been indoors for six weeks so not used to a breeze and gave out a little moan every time there was one.

On the Monday we went to Naomi House and on the Tuesday, Ellena's cousins came to visit. They are aged five and six so they absolutely loved playing there and spending the day with their cousin. Ellena enjoyed the ball pool at Naomi House. Her cousins used the fancy dress and the art room. We all played instruments in the music room to Ellena and she loved it.

Ellena's oxygen was now on 2 litres so on the Wednesday we were admitted back to hospital. Ellena was given another strong dose of hydrocortisone (steroids) through her Hickman Line but unfortunately she did not respond to them this time. By Monday she was on 15 litres of oxygen and was getting worse each hour. We got back to Naomi House that evening and she died 12th June 2012 early morning. Naomi House doctor gave her valium to keep her calm and she died peacefully after receiving lots of cuddles throughout the night. Naomi House and the doctor and nurses at the hospital were a great support to us, everyone had fallen in love with Ellena and were completely heart broken.

Whilst we were in hospital, we found a new charity that has been set up especially for Zellweger Syndrome Spectrum's and joined their support group. 

The link to their website is www.thegfpd.org. 

Talking to other parents was extremely helpful and supportive. Ellena's doctor was able to ask questions about their experiences with lung problems and correspond with another doctor with previous experience of Ellena's condition. We also had another genetic meeting with the final results of her skin biopsy. The gene that is faulty is her PEX1 which falls within the most common and severe end of the spectrum. The genetic team said they would be surprised if Ellena beat the lung disease. They said that with blood tests from mum and dad, that they could test us for future pregnancies.

Ellena May's funeral was on the 20th June 2012, 90 people attended. The service was made up of tributes from close family and friends, poems and music. At then end, everyone was given a sparkler to write their own personal goodbye message to Ellena.  We will miss this very special girl so very much and love her forever.

Ellena May.

The name Ellena means shining light; that was part of the reason we chose it for her. Because from the moment Ellena entered our world she shone.

When Ellena was born, she made us the happiest parents in the world.

Ellena was such a content and happy baby; we could not believe what a joy it was to look after her.

Ellena loved dancing in her Dad’s arms to Fleetwood Mac.

Ellena loved blowing raspberries at always just the right time to make everyone laugh.

Ellena loved her little pieces of space blanket, and her muslin blanky.

Ellena loved her cuddles and received plenty of them from everyone who loved her.

Ellena took us on a journey and showed us tolerance, joy and beauty through adversity.

Ellena taught us to make the most of every day, and not to take life for granted.

Ellena, you are, and always will be amazing.

We are so very proud of you, and will love you for ever.

Mum and Dad.

Ellena's Diary from May 2012

May has been an emotional roller coaster. After the first week in hospital, Ellena's breathing improved and her doctor believed she had aspiration pneumonia possibly caused by the NG tube. She then caught a cold so this turned into viral pneumonia and ended up in high dependency unit (HDU) on a CPAP ventilator as in the picture above. She was given antibiotics and Hydrocortisone and Prednisolone (steroids) and slowly improved. At first the CPAP was doing 90% oxygen, it inflates her lungs for her as they were sticky so not inflating properly. She was in HDU for 10 days as they weaned the oxygen down. Then she was transferred back to the ward G3 where we were made very comfortable again by our familiar bunch of wonderful nurses and taken off the CPAP ventilator and put back in nasal prongs on 2 litres of oxygen with an additional face mask wafting more oxygen as and when she needed it.

Ellena's heart rate was between 45 and 60, the lab picked up that she had low potassium levels, she was given some potassium and it picked up almost immediately. It was horrible watching her swell up from the CPAP, steroids and antibiotics, seeing her connected to loads of cables and monitors with alarms. It was awful not being able to pick her up for a cuddle. Ellena would often get frustrated with the constant flow of air blowing up her nose so would try and pull the prongs out when no-one was looking.

After a week on the ward, Ellena caught para flu so we were given our own room for isolation. She managed to breathe without any oxygen for a day before catching para flu! She took another turn for the worse and after struggling to breathe again, ended up back in HDU. Ellena's Hickman Line has come in very useful. She had a blood transfusion which helped her oxygen levels and she picked up, it was also used for administrating steroids and the many blood tests. It saved her a lot of distress. This time, her doctor gave her a stronger dose of steroids and decided to keep on her a low dose long term. The doctor arranged for us to have oxygen installed at home. After 5 days in HDU, she improved again so was transferred back to the ward G3 where our nurses (Ellena's many aunties) were very happy to see us. We were warned to expect the worse the second time she went back to HDU so everyone was extremely worried and then extremely over joyed when she bounced back. Ellena is a true battler. Although it was lovely to be back on G3, Mum and Dad were worried Ellena might catch something else as the ward was busy. We wanted to get her home safe so pushed the doctor for a decision to let her home for a weekend. As she had been stable on 1 litre of oxygen for a few days, her doctor decided this was a good idea and we got her home. It was so wonderful to have her home as this was after six weeks admission exactly, which was the longest by far.

Ellena's Diary from April 2012

Ellena's Diary from April 2012

Ellena between NG tube change.

Ellena is doing very well. Her biliruben blood count has gone down to 16 which shows her liver is functioning as normal as anyone's. Her jaundice has completely gone. She is continuing to put on weight, presently 10lb 3oz or 4.62kg.

We are making weekly visits to the local hospital as she is getting uncomfortable with her NG tube. They are meant to be temporary after all and she has had it for months. She seems to choke on it sometimes and she tends to hook it out when the home support nurses are off duty. Her Hickman Line has been stiff to flush through so we had to visit the hospital for this as well. We are looking forward to having the PEG put into her belly so we can remove the ng tube and be rid of the Hickman Line as she does get a little distressed with her weekly visits from the nurse to maintain it.

Another picture with Ellie NG tubeless.

Physio has told us that when Ellena,s body weight catches up, she will then be able to hold up her head, presently it is too heavy for her. Portage is going well with lots of sensory play and stimulation ideas. Baby massage has got a little difficult, Ellena does not like being on her front, we think it is because of the Hickman Line so it has limited what we can do. She does love having her legs massaged. Music is the still the best stimulation, she loves it, she sings along and waves her arms back and forth. It is good for mum and dad too.

Some more of the skin biopsy results have come back. They believe she has Classic Zellwegger. We will be told more in the next appointment but we are wondering if we really want to know if it is bad news. Her liver enzymes are improving so the doctor said hand on heart, he honestly can not tell us how Ellena will be in two years time. From the skin biopsy, they can now make the test at ten weeks pregnant, if we were to decide to have anymore children. Having the worry is not attractive to us in anyway. We want to concentrate on our beautiful little Ellie.

18th April 2012

Ellena has developed a cough so her community peaditrician came round the house the following day. She was concerned so asked our nurse to return on the friday with a sats monitor to see how much oxygen Ellena is inhaling. Ellena's oxygen level was in the 80s which is low so we were admitted to hospital. They think she has aspiration pneumonia.

The wonderful nurses on G3 put Ellena in an oxygen tank for a few days and on nil by mouth to give her a rest. Then she had oxygen prongs up her nostrils. They reduced the amount of oxygen a little each day. After a course of anti-biotics she improved well.

28th April 2012

Ellena took a turn for the worse. She struggled to breathe more and turned a shade blue. We all had caught a cold. Her oxygen level on the machine was getting turned up. They xrayed her lungs and compared the xray to when she was first admitted. Her lungs looked a bit clearer but not much.

1st May 2012

Ellena was struggling more. She was constantly panting as if she was running a marathon. She has been transferred to high dependency unit and put on a CPAP oxygen machine. At first, the machine was doing 90% oxygen on a high flow but this has now been reduced to 25%. She has been in there for a week, slowly improving with her breathing and taking breaks from the CPAP (still on oxygen when taking the break but a lesser amount through nostril prongs.) They are trying to wean her off oxygen. Her heart beat has dropped to readings between 50 and 60 over night whilst she is asleep, which is causing concern. They wonder if she has a naturally low heart rate, they have repeated the ECG and her heart seems fine. They are keeping a close eye on this and will repeat the ECG.

Ellena's Diary from Mar. 2012

Ellena's Diary from Mar. 2012

8th March 2012.

Ellena has been weighed today, and unbelievably she has gained a further 12oz. in two weeks.
She now weighs 9lb 6oz or 4.270 Kg. We are really pleased with her dietician.
As you can see from the picture above she is still looking really well. She had a blood test today also. We should have the results tomorrow at clinic at Southampton General.

Below is a little video of just how chipper she is at present.

She has also rolled onto her side a couple of times recently, and generally seems to be gaining energy a little more each day.

9th March 2012

Ellena's appointment at the hospital. Her blood clotting result is still good but we are waiting for her latest bilirubin. We are still waiting for the skin biopsy results, it has been 4 months now. They did have some of it back, we can rule out the mildest form of Zellweger, Ellena does not have Infantile Refsum Disease (some sufferers we found on the net lived until 20 years old.) This is a shame. Time buys hope.

Ellena is doing really well at 6 months and looking like she will see her first birthday. Her doctor said as all the results are not back from her skin biopsy yet, they may want to do another one but he is waiting to hear. We will refuse this as feel Ellena has had enough poking and prodding really.

The doctor wants to put Ellena through another operation to remove the Hickman Line that Evelina's hospital put in as he is taking fortnightly blood tests through it and wants to go to monthly. It is a risk of infection. Evelina's hospital prediction of Ellena needing regular infusions was wrong as her liver is functioning well and confusing the doctors. Whilst she is under, they want to put a PEG in which is a tube straight into her belly for feeding. We could then remove the NG tube from her face that goes up her nose. This will be more comfortable for her and easier to use for us.

Mum and Dad are extremely nervous about Ellena going back into hospital. It should not be for a month or so. There is no rush so she can get stronger in the meantime.

13th March 2012

Mum's oldest friend Lisa has had Ellena's name tattooed on her wrist. Lisa has decided to do a sponsored slim to raise awareness of Zellwegers and to raise money for Naomi House. She weighs 18 stone 9 and wants to slim for three months. 

Her Justgiving page is www.justgiving.com/Lisa-Barnes3. 

We are at Naomi House for two nights respite. Ellena has her first cold (which is a worry that it does not get more serious) and just had her 2nd set of jabs so is very grumpy. It is handy the staff can look after her whilst mum and dad catch up on sleep (after popping down the local for a night cap). Ellena is on three hour feeds as little and often is better for her liver than larger meals and is being drip fed at night which keeps mum and dad awake.

The swelling of her liver and spleen seems to have gone down a lot. The staff are impressed how well she is looking even with a cold. 

22nd March 2012

Back at home and the nurse came round today for weigh in, blood tests, flushing Hickman line and changing dressings. Weight now 4.34kg so put on a couple of ounces only this time and her biliruben count is down to 39 (30 is normal). We have stopped the drip feed overnight as the machine beeps and it was causing us to have little sleep. We have changed her feeding times slightly to try to get Ellena to sleep through the night. 

Her breathing has been erratic on occasion and her cold has turned into a cough. Ellena is struggling to swallow. We are trying to wean her on fruit juices such as melon, grape, cucumber and baby food but she holds the juice in her mouth and hardly swallows. The speech therapist is trying to help with this, it is a concern that she is loosing muscle control. We are however, getting a lot more chuckles and smiles. She likes to feel the texture of people's clothing when they cuddle her. She likes bubble wrap and feathers, we have made her a sensory treasure box full of different things to feel.

26th March 2012

Another milestone reached, 7 months old today and doing really well. If Ellena could hold her head up and the ng tube was taken away, you would never know that she is poorly in anyway.

We would like to say thank you to everyone who is praying for Ellena.

Ellena's Diary from Feb. 2012

Ellena February 2012

Week Beginning Monday 20 02 12

Monday and Tuesday nights were spent at the fantastic Naomi House, an absolute pleasure again. Ellena really seems to love it there, all the staff adore her, we struggle to get a look in with all the nurses queuing up for cuddles.

Ellena at Naomi House looking a very healthy colour.

Ellena does appear to be thriving at the moment, and on Wednesday she was weighed. We were quietly confident that she had gained some weight but when we saw she had gained 11oz or 310 grams in just over two weeks, we were elated to say the least.
We decided to start her herbal medication on Wednesday night. Only half the dose was given with Ellena's milk through her NG tube as a first try. She did not seem to mind the addition to her diet, although she was a little sick in the night, this was six or seven hours later so we don't feel the herbal medication was the cause.
Ellena has also now got earing aids. She did look very shocked when we first put one in.

 

Ellena was sick a further two times and rather uncomfortable on Friday. We decided to stop the herbal tea, and e-mail our findings to the doctor concerned.  We will continue the treatment but at a much reduced dosage to half gram once every three days.


On Sunday 26th, Ellena was six months old. We felt it only fitting for her to have a Birthday celebration.
We invited a few close family and friends round for a buffet on Sunday afternoon. Ellena's cousins enjoyed playing and a good time was had by all.

Diet and medications

We have no medical experience or training and can not give any medical advise. This blog is Ellena's story. If you are a parent of a child suffering under the Zellweger Spectrum, please remember all Zellwegers children are different so what might be good for Ellena may not be good for your child.

However, if you are a parent or a doctor or medical professional reading this blog and do have some comments, please feel free to post them as we are open to suggestions. Before we try anything for Ellena, we always communicate with her doctors and never self medicate her.

When we were first diagnosed, we were told there is no cure, no treatment and no hope. We have spent days on the Internet searching for answers. We found that medical trials have been going on in Spain with DHA. It seems to improve some children as without it they could go completely blind and from MRI scans, their brain myelin seemed to improve. Our paedritian started Ellena on Key Omega. Evelina's hospital didn't think much of the success of this treatment but have prescribed Ellena Doc Omega as we told them we definitely saw an improvement. She started to hold up her head for up to 10 seconds a time, fixed and followed her eyes on lights and seems more alert.

She is on Vitamin E, Vitamin K, Ursodeoxycholic Acid and Delavit multi vitamins. She is on prescribed MCT pepdite milk with polycal.

We discussed weaning her as she is now getting towards six months old. Also, since her stay at Evelina's, she will not take her bottle at all so now all milk and medication is going down the ng tube (apparently this is common after long nil by mouth sessions.) We are concerned she will loose her ability to swallow so we are keen to start her on solids so to exercise this motion.

Mum is doing research to ensure we only introduce foods that are kind to her liver and nothing that will make her liver work hard. There are lots of helpful websites for liver support. Her jaundice was really bad before going to Evelina's but it has been much better after our stay there. Her bilirubin blood count went up to 180 but has decreased which is brilliant news.

We discussed herbal remedies with the doctors. This is outside their area of expertise so they asked us to find some professionals. We have found a gentleman who did a PHD in China on Western and Traditional Chinese Medicines. He moved to England and opened a clinic in Norwich and lectures Traditional Chinese Medicines in London. Amazingly, he has heard of Ellena's condition and told us what it is called in China and the effects it has. He is also keen to help (we asked others before him who declined as Ellena's condition is so serious and unknown.)

He asked all the right questions similar to a qualified doctor and got photos. We corresponded several times before he sent some tea with 14 different natural Chinese herbal ingredients. We have researched the ingredients to ensure they do not clash with Ellena's current meds and do not have side effects. The doctors have also checked it out and although we are taking them outside their comfort zone with this as this has never been tried before (as far as we are aware), they are happy to monitor Ellena's progress with regular blood tests and check ups.

This should start at the end of February.

End of March 2012

Ellena has been improving, her jaudice is fading fast and she has been putting on weight.

Her biliruben blood count was decreasing back to a normal functioning liver level. At 73 we introduced the Chinese Herbal Medicines. Over two weeks it reduced to 59.
The next two weeks it decreased to 39, this is without the tea. It seems she did better without it. It has made her uncomfortable, we think because the ingredients are alien to such a young patient, not that they are harmful in anyway.

We will save the tea for when she starts getting poorly again as at the moment, she is doing just fine without it.

After Diagnosis

14th November 2011 was the day Ellena was diagnosed. The doctor said because her long chain fatty acid test came back abnormal this means she is unable to process fat. We need fat to feed our brains so she will slowly become brain damaged. This also affects the nervous system and all her organs. Her skeleton will grow but she will struggle to put on any weight. Life expectancy in the region of 6 months to 3 years maximum. It is called Zellweger Syndrome. It affects 1 in 50,000 to 100,000 babies and the doctor had never come across it before and neither had the nurses. He explained about Peroxisomal Bio Genesis Disorder, cell structures and missing enzymes.


Words can not explain how crushed and heart broken we felt. It took us weeks to really digest the news, a roller coaster of high emotions. The thing is we were grieving for our baby but Ellena is still alive. It would be a real shame to spend the precious time we have with her being upset. That is no good for her. She needs us more now than ever before and she is showing us the way. It is so wonderful to have her back at home, it is easier to stimulate her.


We had genetic counselling. We were told that we had done nothing wrong to cause this and could not had done anything to change it. It is a mix of both mum and dad's genes. There is a 25% chance this would happen again if we tried for another baby, 50% chance the baby would be a carrier and 25% chance that it would be perfectly OK.  They started talking about making a test we could use  at 10 weeks pregnant if we were to try again. We felt like they were saying Ellie is broken, try again but we want Ellie, we want them to fix her! They persuaded us to allow them to take a skin biopsy so they can categorise her and work out exactly which strain of Zellweger she has. There are 3 strains but they suspect that it is not looking good. They said it may show which enzyme or enzymes are missing.


The doctors had taken so many blood tests by then that they were started to damage her tiny veins. They started taking them from her head. This time she haemorrhaged and her head was bruised and had the size of a golf ball swelling to the side. She was most upset. We decided enough was enough and from now on, its about quality of life. Try and keep her out of hospital as much as possible.

The paediatrician arranged for us to have superb home support. We have a social paediatrician and nurses that visit home every week. We got a new dietician (this one is really good). We started baby massage course which Ellie loves. We had visits from a visual impairment teacher, speech therapists which is about communication and more hearing tests. The calendar soon started to fill up with all Ellena's appointments. She started physio and portage (which is for helping development for disabled preschool children). Portage has been great as she has really start to play.

Ellena aged 5 months.

We started doing spread sheets to log of her milk intake and medicines each day. It is a good way to monitor her progress. It also helps when talking with the dietician and the doctors. Ellena was doing really well with her feeds, so much so we could take out the ng feeding tube and feed her like any other baby. This lasted a few weeks before she started drinking less and less. The tube had to go back in but we are trained on how to use it and now have actually got used to it. 

We have some true friends who visit Ellena. Al bought Ellena some formula one booties when he went to the grand prix in Monaco. Tanya, Andy, Lukie, Lisa, Angie, Les, Julie, Jackie, Dave and Sara to name a few have been amazing friends spending time to get to know Ellena. 

Ellena with her best friend Lukie.

Our families and colleagues have also been amazing, thank you to you all for your love and support. Ellena is a very content baby. She coos and gurgles a lot. She still does not cry much even though she has gone through so much. She feels the love.

Before Diagnosis

30^th August 2011 late evening we got Ellena home. There was a bottle of bubbly waiting from next door with a gift for Ellie and our other neighbour had knitted her some cardies. A beautiful cot bumper set was also waiting from Paul and Kris. Over the next few weeks the house filled with balloons, flowers, cards, teddy bears, baby clothes and visitors. Everyone was queuing for a cuddle. Ellena was acting like every other baby, she is very dainty and hardly cried. She is a happy baby. We soon started to forget the worries from when she was born. We had the happiest times of our lives, thanks to Ellena. Our home feels complete. We want to thank everyone that was part of that for their generosity and making such a warm welcome to Ellena into our world.

We played her lots of music as she loves being danced around the front room. She likes Stevie Nicks, Fleetwood Mac, Massive Attack, David Bowie, Reggae, Blues, Adele, Pink Floyd, Black Eyed Peas, Dance Music but she did not like Led Zep, Rock or Jimmy Hendrix unfortunately. Maybe she will grow into them? She also loves Dad playing his guitar to her.

6 Weeks Later, the health visitor said she was very concerned that Ellena was not putting on much weight. We had appointments coming up as follow up from the hospital so her GP arranged to attach to one of these, an appointment with the paediatrician to discuss her milk. We went in on an outpatient appointment for a hip scan as she had clicky hips due to being breach. They put her on high calorie milk and she loved it but they asked us to stay overnight. The following day they said her bilirubin blood count was high. It should be in the 30’s but it was in the 60’s,70s. This indicated a problem with her liver.  They took her off high calorie milk and put her on pre-digested milk which is better for her liver and they wanted to run more tests. The tests seemed never ending,  every day was filled with tests and scans. They changed her milk 7 times, formulas and concentration. She went in as a healthy baby but within weeks she turned very jaundice and got weaker and weaker. She started losing weight despite all their efforts.

We went to Kings College Hospital for a day with a nurse and ambulance car from Southampton General. They are the liver specialist. We had a lump in our throats the whole day. They took load of blood tests and scanned her liver. They did not seem concerned about her liver, they said her bile ducts were slightly inflamed and it may even settle down in a few months. They were more concerned about her being floppy and her eyes occasionally flickering. We returned to our local hospital, they did some eye tests and head tests.

We were told in the eye test that Ellena is blind. They said she has a syndrome and whatever it is, it is extremely rare. She may be able to see lights, bright bold colours and shapes. Tunnel vision at best but her vision is severely impaired. Normal eyes have veins at the back connecting to the retina but Ellena has a leopard print instead of veins. Very unusual. They did a ECG and her heart was showing a long QT.

She had a few hearing tests before we came into hospital. They were not sure if it was their equipment or Ellena as they kept getting variant results that did not seem to make sense. They thought her hearing was impaired but were not sure so they wanted to repeat them. We felt confident that she can hear because she loves the music and responds to our voices.

Head scans and other blood tests kept coming back that everything was ok. We did make a new friend in hospital, a lady with her poorly little girl. We also got to know the nurses on G3 very well, we have a very good paediatrician, they have been very supportive and really bonded with Ellie.

Eventually after 1 month in hospital the Kings College blood results came back with the news that her long chain fatty acids test was abnormal. At first we thought this could be controlled by a special diet and some medication surely.

Nothing would have prepared us for the real meaning as she was diagnosed with Zellweger Syndrome which is fatal.

Evelina Childrens Hospital

We should start first by saying we were invited and indeed persuaded to visit Evelina Children’s Hospital so the doctors there could have a look at her and the dietitians to see if there was anything missing from her medication and diet. A two night visit was planned. After all these people are supposed to be the metabolic genetic disorder specialists in the country and although we were sceptical about visiting Evelina’s we were also hopeful.

The nurses at Evelina’s Hospital were inspirational people. We are privileged to have met them and their standard of care was fantastic. We learnt a lot from our visit, mostly to listen to Ellena and have faith in her. She is truly a tough cookie and a hardy little soul with a strong will to survive.

Tue. 17^th Jan 2012.

The day before we were due to travel to London we noticed some blood in Ellena’s stool and vomit. We were then worried about her blood clotting level, which is an indicator of liver failure. Ellena was also displaying some blood from the nostril in which the NG feeding tube was in place. We wondered if there was some irritation with the tube and because her clotting was low, that the tube may be causing the bleeding.

Wed. 18^th Jan.

We rang Southampton General Hospital early the next morning, they advised us to pull the NG tube out ourselves and continue as planned to travel to Evelina in London.     

We arrived at Evelina at 2pm as planned and made our way straight to Savannah ward. Ellena had missed some of her feeds and she was getting rather agitated due to the fact that we were struggling to top her up without her NG tube. We were given our own cubicle for the reason that Elllena has still not had her immunisations and is prone to infection.

Ellena of course had to endure another round of blood tests, this time though the doctor fitted a temporary cannula to her hand straight away, which was a first for us. The blood was taken and sent for testing. A new NG tube was fitted. We had a meeting discussing Ellena’s history and condition. We then settled for the evening.

Thur. 19^th Jan.

The hospital addressed the blood clotting with an IV vitamin K, and FFP drip. This worked and her blood clotting returned to normal. This was a short fix as the blood clotting problem would return in time so we discussed having a hickman line inserted into her chest surgically. They were going to try and squeeze Ellie in for the operation the following day. They explained the benefits would be no more pricking her veins for blood tests and we could have regular infusions done at our local hospital to prolong her liver and life. Ellena had another test on her heart and it came back that it is normal. She can now have pain relief and should be safe for an operation.

Fri. 20^th Jan.

There were no slots available for the operation so they booked Ellie in on the Monday morning between 8am and 10am. Ellena was losing a lot of weight so mum bought this up as a concern. The dietitian was repeating steps that had already been tried at our local hospital and that had failed. They explained they were trying to eliminate Ellena loose stools so did not agree with mum to increase her calories in the feed to what she normally has at home.

Sat.21^st Jan.

Ellena continued to lose even more weight. She started to appear very ill. She stopped holding up her head and fixing her eyes and following on lights. By the afternoon mum was having panic attacks because it had taken months to maintain that weight on Ellena and knew it will take months to replace it. The nurses agreed to add Polycal (sugar) to Ellena’s milk to stop her loosing anymore weight. We were getting concerned Ellena would not be well enough for the operation on the Monday.

Sun. 22^nd Jan.

Ellena stopped losing weight. She had gone from 3.74kg  to 3.4kg.  We had a meeting with registrar who assured us that Ellena would only miss one feed over night for 6 hours nil by mouth before the operation.

Mon. 23^rd  Jan.

Ellena started nil by mouth at 1am. The anaesthetist came up in the morning and went to a different baby. We were confused and asked if she was about to see Ellena but she said Ellena was not on the list for an operation that morning, she was down for the afternoon! She finally went down at 2.30pm, she had been nil by mouth for thirteen and half hours by then. She looked extremely weak so they told us that when she recovers, she will go into intensive care on an oxygen machine. They warned us the risks, we were so worried. The Hickman line cannula was inserted, she woke and signed for her milk, we gave her it and she was fine. No need for intensive care and they put her on a lipid drip to help regain some weight and used the hickman line for antibiotics as well.

Tue. 24^th Jan.

Ellena blood results showed she was anaemic. The doctors explained that she has the same amount of blood as a can of pepsi. They were taking roughly 4 bottles a day and then she lost blood in the operation. They gave her a 4 hour blood transfusion over-night. She was sick during this which surprised the doctors as it was meant to make her feel better.

Wed. 25^th Jan.

In the morning they took Ellena for a scan. They could not take any blood tests as she now has someone else’s blood in her system so it would not give accurate readings. Dad went down with her for the scan and the guy doing it was training another so dad could hear their discussion. The guy scanning said Ellena has an Intussusception which is where the lower bowel turns into the upper bowel like a sock turning in on itself and he believed it was self-limiting. The guy explained that it was probably going to rectify itself. 

The doctors and nurses come rushing into Ellena’s room and told us it was critical. They said she was going to die that afternoon. They gave us options;

1.    Give her more morphine and let her die in our arms.

2.    Go for pneumatic reduction (blow air up her bum and hope it pops back in place.) They told us that because Ellena’s liver and spleen are very swollen that there was not a lot of room for the air to go so her bowel would probably rupture which is extremely painful. She would be rushed into surgery.

3.    Surgery, they would have to remove her bowel, fix the rupture and manhandle the intussusception. This would paralyse her bowel for a couple of weeks.

They told us her chances of survival were extremely slim. We asked several times if it could self rectify but the doctors were convinced that this was impossible. They said the rest of her bowel was moving on the scan but this part was not. As a knee jerk reaction, we both agreed to emergency pneumatic reduction and said goodbye to Ellena. We took loads of photos and cried. The doctors waited outside the room and had a chat amongst themselves and were laughing. We could see them through the glass.

Ellena did not seem to be in any distress which we did think was a little weird, we thought it must be the pain relief from earlier. Mum went down with Ellena, the surgeons asked her to sign consent form which stated warn parents of possible death. About 20 minutes later the doctor returned and said the intussusception has gone and Ellena was fine. We were so relieved that we picked the doctor up in a group hug and kissed her.

Ellena was crying on her return so we asked if they had given her any pain relief for the procedure. They said no so we asked if she could have some as she must feel uncomfortable, they gave her morphine.

The nurse came in at the end of her shift and told us her heart had gone out to us that day. She has 2 children herself and she was ever so upset to watch us say goodbye to Ellena. She said she was amazed and relieved when the intussusception self-rectified without Ellena needing any procedure. We were shocked. Why did the doctor not tell us it rectified itself? Was it to save face? Why did they give her morphine when she had nothing done and why did we think she had? Had we misunderstood? Dad complained about the doctors laughing whilst we were saying goodbye to our baby earlier and also for the serious break down of communication.

Later that evening her heart beat started to drop. It went down to 61 beats per minute. They called for the heart surgeon. They told us that because Ellena’s liver does not function as fast as normal, the morphine was stuck in her system. At 60 beats per minute she would go into cardiac arrest. Mum then stimulated Ellena by talking loudly in her ear about everyone at home who loves her and looking forward to seeing her again. Our family, friends and the cats. When mum tired, dad took over with the nurse until 4am. She responded well to our voices, we did not let her sleep.

Thur.26^th Jan.

5am Dad asked if we could be discharged to get her closer to her family back home. We wanted out as we felt it was their fault she nearly went into cardiac arrest and lost trust. They had her on nil by mouth for 28 hours. It took all day to wean her off the drip. Then we had an ambulance with blue flashing lights from Westminster to Southampton. It was cool watching the London traffic part in rush hour for our gorgeous little girl. Ellena loved the flashing blue lights and sirens, she would not sleep.

Although we are still awaiting the skin biopsy results to tell us which strain of Zellweger Ellena has, the metabolic genetic specialist believe that she probably does not have the most severe strain. Her liver is the worrying factor. They explained because this is so rare and all zellweger babies are different, that we are the specialists as no-one knows Ellena as well as we do. They confirmed that no-one is doing any research in this country on her condition. No-one can replace missing enzymes in an organ let alone a body as yet and we are decades away from that kind of research.

Ellena learnt to blow raspberries on her last day at Evelina's. We asked her what she thought of her stay there, she blew a big raspberry, her timing could not had been any better. She is such a funny girl.

We spent the night at the local hospital, her weight is now 3.62k after the lipid drip but it was damaging to her liver. The swelling had gone from roughly 3cm to 6cm. Then we were admitted to Naomi House the following day as a family as Ellena was still critical. 

Fri. 27^th Jan.

Naomi House arrival, for end of life……………..?

What a fantastic place. The building is in beautiful grounds and the staff were amazingly supportive to all of us. As parents, we were emotionally and physically drained. They are trained nurses so very qualified to help with Ellena’s feeds and medication. The accommodation is extremely comfortable. We met some inspirational parents both at Evelina’s and at Naomi House who kept telling us that it does not matter what the doctors say about limited life, do not give up hope. Ellena is not giving up. The staff encouraged us to spend quality time with Ellena whilst they looked after us as a family. Ellena loved the sensory room and the music room. She had her first music lesson and we painted her hand and foot print onto a plate as a keepsake. It is run by a charity and is much better atmosphere than hospital, much more comfortable for the family.

Ellena with Grandparents Steve and Helen.

Mum and dad had some quality rest and the grandparents got to visit our little princess. Ellena grew stronger each day. We started to explore any other options that we had not considered before. Dad suggested traditional Chinese homoeopathy medication. We feel that if we can help maintain her liver, we have a good chance to prolong her life. Our own research begins…..

Ellena with Grandad Ian.

We were there for 4 days and when we got home, we got busy turning Ellena’s nursery into a sensory room. Lots of fairy lights, lazer show lights, balls that light up to sound and lamps etc. She loves it. 

 

Uncle Ron has named a star after Ellena May, it is in the constellation of Orion. So for you star gazers, the next time looking up, think of our shining light.

Ellena's Arrival Into Our World

Friday 26^th August 2011 Ellena May Page was born. She was 10 days overdue and weighed 7lb 2oz. After 10 hours labour, the midwife realised something was wrong so whipped us down for a scan which showed that Ellena was breach. We had an emergency caesarean as she was also wrapped up badly in her cord. The cord was wrapped round her neck a couple of times and her foot had been caught up in it. She had been like this for a while in mum's womb as it appeared she had been trying to release her foot and tightened the cord more and more round her neck, strangling her. Her neck had sores where this had rubbed for some time and her head was swollen.

She is our first and only child; we were so very excited, absolutely over the moon.

Our excitement was cut short by the midwife telling us that she believed something was wrong with Ellena as she had a single crease across her palms, a hole at the base of her spine and a funny shaped head. We have never been so insulted as like all typical new parents; Ellena was the most beautiful baby we have ever seen! She took her away for the doctors on Neonatal ward to have a look at her and take some blood tests. They said she may have Downs’s Syndrome. 

A short while later, the doctors returned Ellena and said they thought she was fine. Her head was probably swollen from the cord strangling her. They did not bother to take blood tests as she responds well and seems fine after all. What a relief!

On the second night still in hospital, Ellena became quite restless. The nurse took her back to Neonatal Ward and they did a heal prick blood test. She had a drop in sugar levels. Mum was struggling to breast feed her so they suggested giving Ellena some bottle milk as well as breast. This did the job but they did a further seven lots of blood tests from her the following day to monitor the sugar levels. She cried each time and we could not help but cry with her. It was horrible to watch them keep pricking her.

A few days later mum asked if we could go home. Mum wanted to get home for her birthday and wake up in our own beds as a new family, how exciting! The doctor asked us to stay as they believed something was wrong with Ellena. We were shocked as she seemed perfectly fine. We asked what was wrong and they said they believe she may have a genetic disorder such as Downs. We were confused by this as she does not look Downs. The doctors asked us to stay 1 more day so they can start genetic tests.

After a 4 day stay we were to arrange an appointment as an outpatient instead as the genetic team was busy. We were delighted to be able to leave and take our beautiful baby home for the first time.

This is Ellena at five weeks old.

We all got home and it was wonderful. The following weeks were the best times of our lives, the first time we were a complete family, Jim, Kerry and baby Ellena May.