On Tuesday 9th September 2008, Ally Cadence Humphries was born. The following weeks were filled with the joy that our new arrival brought to our family, but at just nine weeks old Ally was rushed in to hospital in an ambulance with breathing problems. She had stopped breathing at home for a short time and although the paramedics and hospital staff had her stabilised quite quickly, they decided to monitor her for a couple of days as she had a bit of a chest infection.
Two days later Ally began to struggle with her breathing again, the infection was getting worse and we were told the shocking news that the doctors wanted her transferred to a Childrens Intensive Care Unit. This meant moving her to a different hospital as the one in Lincoln doesn't have an Intensive Care Unit for children. After some ringing around, the doctors found a bed available for her at the Queens Medical Centre, Nottingham. It was decided that this was where she would be transfered to and in the mean time they closed off one of the recovery sections in the operating theatres and turned it into a kind of temporary intensive care room for her. It was there that we were introduced to a large team of people all dedicated to looking after Ally until the "retrieval team" from Nottingham could get to us. We were instantly made to feel at ease, the people looking after her were so professional, calm and reassuring and we knew that although she was quite seriously ill there was no way the staff in charge were going to let anything happen to our daughter that night.
As midnight arrived, we were all expecting the retrieval team to walk through the door any minute. A short time later the phone rang and we were informed there had been a delay, the ambulance would not be able to get to us until at least 6am. We sat by the bedside blury eyed until around 6am when we got another update letting us know it would be after 9am before the ambulance arrived. With this in mind, together with the fact we had been awake for almost 24 hours, we decided to try and get some sleep on the cold hard floor of the childrens waiting room. After around an hour of sleep we decided that it was actually much warmer and comfier sat on a chair in recovery at Ally's bedside so we headed back in to find the staff still working hard to keep her comfortable. All through the night she had been having Apneas and going Bradycardic. This basically means her breathing was stopping for over 20 seconds at a time and her heart rate was dropping to a rate that could be considered dangerously low. The staff knew exactly how to handle these events and were giving her caffeine via a drip and gently shaking her every few minutes as the apnea alarm went off. In addition to this she was also on a Continuous Positive Airway Pressure (CPAP) machine, which ensured that every breath taken was a good one.
Around 9:30am we saw the doors swing open and in walked two intensive care nurses and two ambulance crew with a very scary looking trolley. Scary is perhaps the wrong word for a trolley full of such a large amount of life supporting equipment, obviously this meant she was in good hands. The scary part comes from the fact that we realised the seriousness of the situation. One hour later and Ally had been carefully moved from the bed to the trolley and we began the walk through the hospital corridors to the main entrance where the ambulance was waiting. Nine of us walked with her as she was wheeled by all the staring people who could tell she was really poorly.
A couple of hours later we arrived in Nottingham and found the way to the Paediatric Intensive Care Unit (PICU). We waited in the seating area just inside the ward for a few minutes and were then shown to Ally's bed. The staff explained that she was very stable after her transfer from Lincoln and that she was responding well to the CPAP machine that she had been on since the previous evening. By this time we were exhausted and as Ally was stable, a member of staff showed us to one of the parents rooms. We had a nice comfy bed, showers, a kitchen and a toilet. We slept for a few hours and went to see how Ally was doing mid afternoon. She was doing well and we were told how they planned to put her in a head box full of oxygen instead of the CPAP as they felt she could manage that way. They tried the head box while we went to get some food and we returned to the PICU later to discover she hadn't managed as well they hoped and was back on the CPAP. During the evening she showed good progress and they tried the head box again, this time she tolerated it which meant she no longer required intensive care and she was moved in the middle of the night to the High Dependancy Unit next door. It was here that we were told Ally had tested negative for the RSV virus that is usually the cause of Bronchiolitis. That said she was still classed as having Bronchiolitis although they didn't know which virus had caused it. Two days later and Ally was well enough to move to a childrens ward. Just one day after that, she was allowed home and although she had a bit of a cough, we thought our nightmare was over.
Over the next few weeks things improved. Ally still had her cough but the doctors had explained that there is always a long lasting cough after having Bronchiolitis so this was nothing to worry about really. The family prepared for Christmas and on the 20th December 2008 we went to the Lake District with some good friends of ours. Christmas in the lakes was lovely, the week went very smoothly and we all enjoyed our much needed holiday.
2009 started well and other than several trips to the GP about the persistant cough, Ally got through January very well. As we entered February however, she seemed to struggle a little with her breathing and feeding again. On the 5th of February she had a bit of a rough day and although she was smiley as ever, she was off her food and working very hard with her breathing. It was that evening that we decided she needed to be seen by the hospital so we rang and explained to the childrens ward that she had started to struggle again but was very happy in herself. They said that as we were 17 miles away from the hospital they felt that Ally should be taken to the ward in an ambulance incase she went downhill during the journey. The ambulance seemed over the top as she was so happy and alert but we did as they asked and dialed 999. The lives responders arrived shortly after and gave her some oxygen, followed by a single paramedic in a car and a paramedic team in an ambulance. They all seemed shocked at her heart rate and oxygen saturation levels considering how bright and happy she looked, and a few minutes later she was rushed into Lincoln on blue lights.
Within an hour of calling the ambulance, our fears were confirmed, Ally had Bronchiolitis again and needed to be put on a ventilator in intensive care. We couldn't believe we were sat in the same room again, wondering how long it would be until the ambulance came to transfer her. We were also wondering which hopsital we would be transfered to, and a few hours later the phone in recovery rang to let us know they had found a spare bed in the Childrens Intensive Care Unit (CICU) at Leicester Royal Infirmary. This meant that our hopes of her going onto a CPAP machine like before, and therefore not needing to be ventilated, were ruled out. The team from Leicester were only happy to make such a long transfer if Ally was under anaesthetic and on a ventilator. We were told of the risks involved in doing so, but it was the only option we had. We left the room while they put her under anaesthetic and put the tube down her throat and about 10 minutes later we were told we could go back in and see her. She looked so pale yet her breathing, now being done by machine, was the smoothest we had seen it in ages. This was clearly what she needed to get back to 100% health as she hadn't ever really recovered from the previous infection.
Once again we sat with her through the night, and once again the ambulance came to collect her during the early hours of the morning. We arrived in Leicester around 4:30am and went straight to the CICU. After waiting for a few minutes we were allowed in to see her and the doctors explained that she had done well on the journey and was very stable. We sat with her while x-rays and other tests were done and we were then told that all the figures on the tests were very positive, but one of her lungs had collapsed. A few hours later we were shown to a sofa bed where we could sleep for 3 or 4 hours, there was no parents suite like the one in Nottingham, just a small room with a sofa bed. After our sleep we returned to CICU to find that nothing had changed, she stayed the same all day and in the evening we were told we could sleep on a camp bed in one of the playrooms. We went to bed around midnight, and returned at 7:30 the next morning. She was a little more alert as they had brought her round slightly but we could tell she was affected by all the drugs too much to really know what was going on around her. As the day went on she became more and more alert to a point where she managed to give us one of her trademark smiles, even with the ventilation tube in her mouth. This made us so happy, it meant she knew that we were there with her, and hopefully it made her feel a bit safer having seen us at her bedside.
A couple more days passed and good progress was made, until suddenly while being turned over, Ally decided she didn't want to be on the ventilator anymore and the tube popped out of place slightly. This meant the machine was no longer breathing for her, a point the doctors hadn't planned on reaching for at least two more days. All of the doctors and nurses on the ward were called over to Ally's bedside and all parents and visitors, including us, were calmly but quickly asked to leave the ward. As we were hurried out the doors we heard them shouting for the "crash trolley" and our hearts sank, all we could do was sit outside and listen to the flurry of activity on the ward, machines were alarming and people were clearly rushing around to get things done as quickly as possible. We sat for about fifteen minutes and then suddenly the doors opened, a nurse told us that everything was fine and Ally was now off the Ventilator and breathing well on her own. Relief flooded over us as we rushed back in to see her, and there she was still smiling back at us as if nothing had happened.
Just as things were starting to look better, we found ourselves being quizzed by the nurse who was looking after Ally about several little things that weren't quite "normal" with regards to her development and her day to day life. The main thing they were interested in was the fact that she was now five months old and was still unable to hold her head up, as well as this she rarely moved her legs and was generally quite floppy. Before we knew it several members of staff were asking us the same questions, and the more people we answered, the higher up the chain we went. Later that day we found ourselves telling one of the wards top doctors the same things we had told everyone else that day, and he expressed severe concern for Ally. He said he wanted us to talk with the neurologist as he felt there was an underlying condition that no one had picked up on before now. He refused to tell us the condition he had in mind, but he said there are several different types and he would be wrong to try and predict it at this stage. We were left to go to bed in our designated playroom where we found ourselves sat googling anything that could be considered the slightest of symptoms to try and work out what the doctors were thinking. This was a scary thing to have done, google suggested many conditions including Cerebral Palsy and Cystic Fibrosis. We were so upset and worried that it could be something like this, Cystic Fibrosis in particular stood out as a likely outcome.
We sat in shock at how things had progressed so quickly, from worrying about the first intensive care visit only a few weeks before to having to go through it all a second time, and now having both of those admissions overshadowed by a possible neurological condition. We kept looking on the internet for other possibilities and suddenly stumbled across a condition called Spinal Muscular Atrophy (SMA). The more we read about it, the more we scared ourselves. One of the symptoms is being floppy, another is a history of lung infections, another is having sweaty hands, the list went on and the more of them we read, the more we realised Ally had. We then looked at it from another angle and began going down the path of convincing ourselves it wasn't any of the conditions we had read about that night. The next day we met the neurologist and he explained his concerns about Ally, he said that he wanted to do several blood tests, an MRI scan, a Nerve Conduction Study, Muscle Biopsy and much more. This sounded quite frightening but the staff all insisted that it was simply to "rule out" a long list of conditions and that we shouldn't be too alarmed at this stage.
A few days later Ally was well enough to move back to Lincoln, but because the ward in Lincoln was full she had to be moved to the childrens ward in Leicester. Just a few hours after moving wards, she had a problem with her breathing and needed a nebuliser. The call was made to move her back into the CICU next door and as we walked in, the nursing staff looked as shocked and dissapointed as we were to see her back in intensive care. The following 24 hours were stable and once again Ally was classed as not needing intensive care, although she didn't get moved out as they were sending us back to Lincoln the next morning. Unfortunately that same night Ally had the same breathing problem she suffered with the night before which meant we had to cancel the transfer to Lincoln and stay in CICU another night.
One day later we got the go ahead to head back to Lincoln childrens ward, the ambulance arrived around 5pm and we all set off for Lincoln. Upon our arival we were quizzed once again by more and more doctors. They were all concerned about how floppy she was and the way she couldn't support her own head, we had simply put this down to the fact that she had been in intensive care at such a young age and therefore was set back with her development slightly. We had mentioned our concerns regarding this to health visitors and doctors just a week before she went into hospital but we were told not to worry for a few months as not all children develop at the same rate.
During the days that followed we were told that Ally's lung had inflated again and that she was doing well, she started bottle feeding again and was allowed home. Finally things could begin to return to normality, we were still worried about all the tests she needed but at least she was at home. We had to return to the hospital the day after she came home to begin the tests. This time it was only for a day visit and after some confusion over the appointment times with the staff, Ally eventually had 16 bottles of blood taken and we headed back home. It was during that day at the hospital that we began to get some honesty from the staff. After we expressed our concerns once again about what the hospital thought may be wrong with Ally, we were told there were several things they were testing for and one of them was a genetic blood test for SMA. Once we were back at home we tried to get on with things as normal, the blood tests would be weeks before the results were ready so we did our best to forget them. We did a good job of this for a few days and then suddenly, 999 was dialed again and Ally headed into hospital for a third time. We couldn't help but worry about what the tests were going to show, we knew something serious was wrong but no one could tell us what.
The doctors at Lincoln did an x-ray and although her lung had started to collapse again, we had caught the infection much earlier this time and there wasn't any need for intensive care. The days passed by and although Ally showed a lot of improvement, the doctors didn't want to start bottle feeding her again as they felt that a problem during swallowing may have been allowing a small amount of milk into her lungs therefore causing infection. Over a week later, on the 6th March 2009, we arrived at the hospital hoping to talk the doctors into letting Ally home on the condition we tube fed her with support from the community nursing team. We waited all morning after being told early in the day that the doctors wanted to chat about Ally with us while we were there, and shortly after lunch time they called us into one of the offices on the ward. We knew this wasn't a good sign as usually all thing were discussed at Ally's bedside. We sat down and waited as three or four members of staff arrived to talk with us. The consultant then explained that the blood test results from two weeks before had arrived back that day. He went on to explain that the test had shown that Ally had Spinal Muscular Atrophy and that it was Type 1.
SMA is generally found in three types, Type 1 being the most severe with a life expectancy of up to two years, Type 2 typically shows itself around a year later in life and has a few more years life expectancy and Type 3 typically shows itself later still and has life expectancy often as far as 20 years or more. These details are certainly not set in stone as there are cases of SMA Type 1 being diagnosed at a very early age with the person in question living to be over 40.
We had already done a lot of reading on SMA and we knew that this type 1 diagnosis would mean that Ally may not live to be two years old. The doctors confirmed that was the case during the same meeting, and as Ally was already just about six months old, they worded it as "We would expect her to have around twelve to eighteen months". Ally was allowed home later that day and we spent the next few days explaining the diagnosis as best we could to friends and family, and of course spending as much time as we could making Ally smile.
Over the next few days we met several people who were all going to play a regular part in Ally's life, we had oxygen installed at home and were provided with small mobile bottles so we could give oxygen to her while we were out. We continued to tube feed Ally on a 4 hourly basis and we gave her oxygen every now and then when she required a small boost. We had a sats monitor so we could check exactly how well her body was coping with the way she was breathing and although we were very busy, we managed around four hours sleep each night and felt we adjusted very quickly into our new routine. There were several short visits to the hospital over the next week but all of them were for minor queries. These visits stopped once we had the suction machines at home so we could help Ally when she was unable to clear any secretions on her own.
After a couple of days Ally began being sick after her feeds, this was mainly happening in the mornings and before bed time. We knew something was wrong, but we didn't know what. Ally was her usual smiley self and the people we spoke to at the hospital said that if she seemed happy and alert then there was probably nothing to worry about. After being sick one evening, she seemed to go very pale, her sats were quite low even though she was on 5 litres of oxygen per minute so we contacted one of the comunity nurses to express our concerns. We were told that as long as she was alert then we should probably turn the sats monitor off as it wasn't always a reliable way to check on her condition. As soon as we had put the phone down, Ally seemed to begin to drift in and out of conciousness, she became extremely pale and her sats dropped lower than we had ever seen before. Again we dialed 999, the four minutes that it took for help to arrive seemed like forever, but when it did they managed to bring her sats back up to a healthy level within seconds using some high flow oxygen. The ambulance crew arrived a few minutes later and took her into Lincoln, as soon as she arrived she was put into a large room of her own and the doctor came in and closed the door behind him. He began to explain that Ally may not have as much time as we first expected and, although reluctant to give us an idea of how long she may have, after we insisted that we should be given an honest answer we were told that Ally had just a few hours left to live.
We called all family members and friends to tell them the devastating news and within an hour we had around 20 people in the room with us. Ally's christening was planned for the following day but we managed to arrange for an emergency baptism in the hospital ward that night. We sat with her all night and somehow she fought on into the next day, and then it was decided that her oxygen was making her uncomfortable and she would be more settled if it was turned down quite a lot. Quite soon after, her breathing slowed a lot and we were told that she may only have a few minutes left. She fought on for several hours after this, her oxygen was turned down further still and she was given morphine to ensure she was as relaxed as possible.
Later that day, just twelve days after her original twelve month prognosis, at 8:37pm on the 18th March 2009, Ally Cadence Humphries died peacefully in our arms with several family members by her side. The day she passed away was our 9th wedding anniversary, the date that all three of our other children had been christened and the day Ally was due to be christened too. It has always been a special date for us, and now it will always be the date our whole family will celebrate Ally's life and remember her beautiful smile. We had a huge amount of support that day, and in the days and weeks after. We simply cannot find the words to express how grateful we are to those who have been there for us through this difficult time, so many of you helped us cope with the rollercoaster of events that we had to deal with. Thank You.
Rest In Peace Ally Cadence,
Love Mummy, Daddy, Ethan, Jay & Kasey x x x x x