After being discharge from hospital 2 months passed fairly uneventfully. We still struggled with Bethany's feeding, she would cry and choke on the milk, and we were always told she was fine and it was normal. We were once told she was a 'fussy' baby if you can believe it. Still we struggled on with the feeding and Bee struggled on with the weight gain. She cried most of the time and life got very stressful. By the time our second hospital stay rolled around in May Bee still only weighed 12lbs at 7 months old.
Bethany started with a cough, high temperature and vomiting which meant she was getting down very little food. For a baby of Bethany's weight that was worrying so I made an emergency appointment at the GP's surgery. I took her down and she was examined. After listening to her chest the Doctor made a few notes on the records in front of him. Out of concern of looking like a paranoid parent, which was how I'd so often been treated, I broke the silence with: "I just wanted to get her checked out, I was worried about the cough and her throwing up. She's so little to begin with. Last time this happened we spent a week in hospital". To my relief the Doctor replied: "that's where I'm sending you now." Bethany's chest had sounded 'crackly' and with the constant vomiting and her low weight it was his opinion that she should be admitted to the children's ward for possible Bronchiolitis. As worried as I was I was also really pleased that the Doctor had taken me seriously and we were being seen immediately. Luckily the hospital is only a 5 minute walk from my GP's surgery.
And so we were admitted back on to B11 Cubicles for the second time on Thursday 7th May 2009. For the first few days Bethany was extremely poorly. Her temperature was sky high and she couldn't keep any food down. She coughed so much and so severely that her throat was hoarse and when she cried hardly any sound came out. She needed quite a bit of oxygen during the course of the illness as her breathing was very laboured and she would desaturate, meaning the percentage of oxygen in her blood would drop. It was heartbreaking. A chest x-ray had confirmed bronchiolitis but unfortunately the only treatment was to wait it out. We fell back into the old routine. I would stay there 24/7, sleeping on a camp bed on the floor and Foz would visit in the evenings and on his days off. Thankfully the bronchiolitis eased after 4 days, although Bethany's cough remained. Unfortunately she lost a lot of weight, 8ozs in total, which is a lot for such a tiny baby. She went from 12lbs 4oz to 11lbs 8oz.
After 6 days we were transferred to a larger room with a cot for Bee and a bed for me! Joy! Normally Bee would probably have been allowed home once her bronchiolitis symptoms has eased. But with her weight loss and reluctance to feed normally again we had to stay. Of course being in-patients had it benefits: we were seen every day by a Doctor, sometimes more than one. Bethany had more blood tests and examinations, trying to diagnose a cause for her failure to thrive. Bethany has also always had a rattle when she breathes. It sounds like she needs a really good cough and it's been there since she was born. One doctor once told us that it was milk in her throat. What 24 hours a day, 7 days a week?? I don't think so. After complaining to the umpteenth doctor about Bethany's difficulties with feeding and her rattle one finally suggested that Bee be checked out by ENT, or the Ears, Nose and Throat department. Hooray! Progress! Someone who listens!
So I wrapped Bethany in a blanket, the Children's Ward is always stifling but the corridors off the ward are cold, and headed off to the ENT department. We were accompanied by a nurse, as if I couldn't be trust not to run away with her. She dropped us in the waiting room, informed the receptionist that we were there and told me she'd be back to collect us. I tried to reassure her I was very familiar with the hospital by that point and the hospital wasn't that big to begin with but she insisted. So there we sat, waiting, amongst the eldery men and women. Eventually Bethany's name was called and we followed the nurse into the room. This was mine and Bethany's first experience of a throat examination and I was quite prepared for how unpleasant it would be. I had to sit Bee on my knee, facing me, then lie her down so her head was resting upside down on the Doctor's knee. He then used a large wooden stick, like a giant lollipop stick, to push down Bethany's tongue and see right down her throat. My poor girl gagged and retched and I honestly thought she was going to throw up but she was a trooper and persevered. It only took 20 seconds and it was all over. I lifted her up and she sat on my knee, blinking and taking us all in. I murmured some reassuring words to her and she gave me a big smile. That's my girl. I turned to the Doctor for the verdict and it was here we got our first clue on the way to getting Bethany's final diagnosis. Our little Bee has a bifid uvula. My first reaction was probably pretty much what yours is: "A what??". Well, according to Wikipedia a uvula is "a conic projection from the posterior edge of the middle of the soft palate." In laymans terms it's a piece of tissue that hangs down like the tonsils. Bifid means split. So a bifid uvula means it is split. I asked about the ramifications of that and was told that although it can cause trouble swallowing, it's not a huge problem in and of itself and many people live with them with no effects whatsoever. He referred us to the ENT department at Alder Hey Children's Hospital with the recommendation Bethany have an endoscopy (camera) down her throat. Of course it's never pleasing that a Doctor does find a problem with your child but if there is something to be found we are always relieved when a Doctor does finally find it. We were pleased with the Alder Hey referral. They have a great reputation and are the nearest children's hospital to our home. It is also a regional centre for so many specialist units we knew we'd be in safe hands.
As a result of the ENT diagnosis and because of Bethany's feeding difficulty we also had a Speech and Language Therapist come and see us. We were pretty confused when we were told this as Bethany wasn't yet old enough to be speaking and she'd only just started baby babbling. When Claire the Speech Therapist arrived we felt immediately at ease with her. She was one of the nicest and approachable health professionals we'd met with so far and explained everything very clearly, instead of just assuming we understood the long medical terms. She explained that a speech therapist didn't just do the obvious of encouraging and developing speech. It was her job to look at the function of the mouth and throat, see how the mechanics were working and if there were any problems. We'd had to starve Bethany before the appointment to make sure she would eat while Claire was examining her. Consequently she was irritable and moaning so we started with the examination pretty quickly. Whilst Bethany had some solid food and then her bottle Claire listened to her neck with a stethoscope. She let Bethany finish eating then had a quick examination of her throat as the ENT Doctor had done, I then settled her in the cot with some toys so we could have a chat. She asked me lots of questions about Bethany's feeding, breathing and general health and finally gave me the verdict. Claire confirmed what the ENT Doctor had told us about the bifid uvula and gave us some rather bigger news. She suspected Bethany had a sub-mucous cleft of the soft palate. A cleft palate?? But she was fine, there was no split in her lip or the roof of her mouth. How could she have a cleft palate? I just didn't understand. Claire explained that the mucousal layer is the layer of skin and tissue that forms the roof of your mouth and cleft means split. So basically the sub (underneath) of Bethany's mucousal layer is split. That's why we couldn't see a split, it's underneath the roof of the mouth. The physical evidence was a high arch and a faint silver line running down the center of her roof. What this means is the muscles haven't come together and fused after the tongue descended during development. When a regular person swallows those muscles lift up the soft palate at the back of the mouth, to close off the passageway to the nose. As this hasn't happened in Bee's mouth it means when she swallows she gets fluid coming back up into her nose. This means effectively she has to swallow twice for each mouthful of food, doing twice the work, and she's got a constant amount of liquid sitting in the back of her nose, making her rattle as it doesn't drain properly. The second major effect that a sub-mucous cleft can have is difficulty sucking. To get a decent suck a baby needs to create a vacuum with it's mouth. If the soft palate isn't closing off the nose passage then air can still circulate and sucking becomes difficult. Suddenly all those hours struggling to breastfeed popped into my head and things started falling into place. Of course the little we knew about cleft palates involved surgery, speech therapy and all kinds of drastic procedures. Claire reassured us that in a lot of instances no surgery was required. As long as special bottles were used and weaning encouraged Bethany shouldn't have a problem. Usually surgery was only performed if the child developed nasal-sounding speech as they got older. That was reassuring. Bethany just seemed to tiny to go through surgery and it was the last thing we wanted to put her through. Claire gave us some special squeezy bottles so Bethany wouldn't have to struggle sucking, we could help push the milk into her mouth and promised to inform Alder Hey Cleft Palate department, who would send a specialist nurse to come and see us.
Trish, the Cleft Palate Nurse Specialist, came the following day. She confirmed everything Claire had told us and referred us on to the Cleft Palate Surgeon, Mr. Van Eden for a surgical consultation. Trish expressed concern during her examination of Bethany that her fontanelle (soft spot on her skull) appeared to have closed. We already knew Bee's was small but it having closed so early in life was a cause for concern. Trish explained that normally, as babies brains grow their skulls grow with it, closing the gap between the skull plates, known as the fontanelle. As Bethany's has closed so early their was concern that whilst her brain would continue to grow, the skull would not be able to accommodate the growth. This would result in the skull essentially squashing the growing brain and causing inter-cranial pressure leading to brain damage and an abnormally shaped skull. The correct term for this is Craniosynostosis. She told us she'd put a referral through to the Cranio-Facial Unit at Alder Hey and they would contact us in due course. Of course when she left I panicked. I imagined every minute going by was causing more and more brain damage to my beautiful little girl. As you can imagine we were completely freaked.
Bethany was finally discharged, after gaining 6 of the 8ozs she'd lost, on the 13th day. It was the day before my 26th birthday and the best present I could have hoped for, as cliched as that is. This time round we felt like we'd made progress. We had an answer as to why Bethany struggled to feed and I had the comfort of knowing the difficulty with breastfeeding wasn't my fault. But the up-shot of all this was it becoming increasingly obvious the Geneticists had something to find when they started testing. Now not only did Bethany have failure to thrive, developmental delay and hypotonia, she also had a cleft palate and bifid uvula to contend with as well as possible craniosynostosis. Trish actually asked us if we'd be surprised by a genetic diagnosis of something and we told her we wouldn't be. Her reaction was "if I'm brutally honest I wouldn't be surprised either". That just said it all.
We again couldn't have got through this period without the support of some very wonderful people: my Mum and Ken, Foz's parents and our good friends Lynne and Mike, even when it was just a pint of milk for a cuppa in the parent's room! :)
Bethany started with a cough, high temperature and vomiting which meant she was getting down very little food. For a baby of Bethany's weight that was worrying so I made an emergency appointment at the GP's surgery. I took her down and she was examined. After listening to her chest the Doctor made a few notes on the records in front of him. Out of concern of looking like a paranoid parent, which was how I'd so often been treated, I broke the silence with: "I just wanted to get her checked out, I was worried about the cough and her throwing up. She's so little to begin with. Last time this happened we spent a week in hospital". To my relief the Doctor replied: "that's where I'm sending you now." Bethany's chest had sounded 'crackly' and with the constant vomiting and her low weight it was his opinion that she should be admitted to the children's ward for possible Bronchiolitis. As worried as I was I was also really pleased that the Doctor had taken me seriously and we were being seen immediately. Luckily the hospital is only a 5 minute walk from my GP's surgery.
And so we were admitted back on to B11 Cubicles for the second time on Thursday 7th May 2009. For the first few days Bethany was extremely poorly. Her temperature was sky high and she couldn't keep any food down. She coughed so much and so severely that her throat was hoarse and when she cried hardly any sound came out. She needed quite a bit of oxygen during the course of the illness as her breathing was very laboured and she would desaturate, meaning the percentage of oxygen in her blood would drop. It was heartbreaking. A chest x-ray had confirmed bronchiolitis but unfortunately the only treatment was to wait it out. We fell back into the old routine. I would stay there 24/7, sleeping on a camp bed on the floor and Foz would visit in the evenings and on his days off. Thankfully the bronchiolitis eased after 4 days, although Bethany's cough remained. Unfortunately she lost a lot of weight, 8ozs in total, which is a lot for such a tiny baby. She went from 12lbs 4oz to 11lbs 8oz.
After 6 days we were transferred to a larger room with a cot for Bee and a bed for me! Joy! Normally Bee would probably have been allowed home once her bronchiolitis symptoms has eased. But with her weight loss and reluctance to feed normally again we had to stay. Of course being in-patients had it benefits: we were seen every day by a Doctor, sometimes more than one. Bethany had more blood tests and examinations, trying to diagnose a cause for her failure to thrive. Bethany has also always had a rattle when she breathes. It sounds like she needs a really good cough and it's been there since she was born. One doctor once told us that it was milk in her throat. What 24 hours a day, 7 days a week?? I don't think so. After complaining to the umpteenth doctor about Bethany's difficulties with feeding and her rattle one finally suggested that Bee be checked out by ENT, or the Ears, Nose and Throat department. Hooray! Progress! Someone who listens!
So I wrapped Bethany in a blanket, the Children's Ward is always stifling but the corridors off the ward are cold, and headed off to the ENT department. We were accompanied by a nurse, as if I couldn't be trust not to run away with her. She dropped us in the waiting room, informed the receptionist that we were there and told me she'd be back to collect us. I tried to reassure her I was very familiar with the hospital by that point and the hospital wasn't that big to begin with but she insisted. So there we sat, waiting, amongst the eldery men and women. Eventually Bethany's name was called and we followed the nurse into the room. This was mine and Bethany's first experience of a throat examination and I was quite prepared for how unpleasant it would be. I had to sit Bee on my knee, facing me, then lie her down so her head was resting upside down on the Doctor's knee. He then used a large wooden stick, like a giant lollipop stick, to push down Bethany's tongue and see right down her throat. My poor girl gagged and retched and I honestly thought she was going to throw up but she was a trooper and persevered. It only took 20 seconds and it was all over. I lifted her up and she sat on my knee, blinking and taking us all in. I murmured some reassuring words to her and she gave me a big smile. That's my girl. I turned to the Doctor for the verdict and it was here we got our first clue on the way to getting Bethany's final diagnosis. Our little Bee has a bifid uvula. My first reaction was probably pretty much what yours is: "A what??". Well, according to Wikipedia a uvula is "a conic projection from the posterior edge of the middle of the soft palate." In laymans terms it's a piece of tissue that hangs down like the tonsils. Bifid means split. So a bifid uvula means it is split. I asked about the ramifications of that and was told that although it can cause trouble swallowing, it's not a huge problem in and of itself and many people live with them with no effects whatsoever. He referred us to the ENT department at Alder Hey Children's Hospital with the recommendation Bethany have an endoscopy (camera) down her throat. Of course it's never pleasing that a Doctor does find a problem with your child but if there is something to be found we are always relieved when a Doctor does finally find it. We were pleased with the Alder Hey referral. They have a great reputation and are the nearest children's hospital to our home. It is also a regional centre for so many specialist units we knew we'd be in safe hands.
As a result of the ENT diagnosis and because of Bethany's feeding difficulty we also had a Speech and Language Therapist come and see us. We were pretty confused when we were told this as Bethany wasn't yet old enough to be speaking and she'd only just started baby babbling. When Claire the Speech Therapist arrived we felt immediately at ease with her. She was one of the nicest and approachable health professionals we'd met with so far and explained everything very clearly, instead of just assuming we understood the long medical terms. She explained that a speech therapist didn't just do the obvious of encouraging and developing speech. It was her job to look at the function of the mouth and throat, see how the mechanics were working and if there were any problems. We'd had to starve Bethany before the appointment to make sure she would eat while Claire was examining her. Consequently she was irritable and moaning so we started with the examination pretty quickly. Whilst Bethany had some solid food and then her bottle Claire listened to her neck with a stethoscope. She let Bethany finish eating then had a quick examination of her throat as the ENT Doctor had done, I then settled her in the cot with some toys so we could have a chat. She asked me lots of questions about Bethany's feeding, breathing and general health and finally gave me the verdict. Claire confirmed what the ENT Doctor had told us about the bifid uvula and gave us some rather bigger news. She suspected Bethany had a sub-mucous cleft of the soft palate. A cleft palate?? But she was fine, there was no split in her lip or the roof of her mouth. How could she have a cleft palate? I just didn't understand. Claire explained that the mucousal layer is the layer of skin and tissue that forms the roof of your mouth and cleft means split. So basically the sub (underneath) of Bethany's mucousal layer is split. That's why we couldn't see a split, it's underneath the roof of the mouth. The physical evidence was a high arch and a faint silver line running down the center of her roof. What this means is the muscles haven't come together and fused after the tongue descended during development. When a regular person swallows those muscles lift up the soft palate at the back of the mouth, to close off the passageway to the nose. As this hasn't happened in Bee's mouth it means when she swallows she gets fluid coming back up into her nose. This means effectively she has to swallow twice for each mouthful of food, doing twice the work, and she's got a constant amount of liquid sitting in the back of her nose, making her rattle as it doesn't drain properly. The second major effect that a sub-mucous cleft can have is difficulty sucking. To get a decent suck a baby needs to create a vacuum with it's mouth. If the soft palate isn't closing off the nose passage then air can still circulate and sucking becomes difficult. Suddenly all those hours struggling to breastfeed popped into my head and things started falling into place. Of course the little we knew about cleft palates involved surgery, speech therapy and all kinds of drastic procedures. Claire reassured us that in a lot of instances no surgery was required. As long as special bottles were used and weaning encouraged Bethany shouldn't have a problem. Usually surgery was only performed if the child developed nasal-sounding speech as they got older. That was reassuring. Bethany just seemed to tiny to go through surgery and it was the last thing we wanted to put her through. Claire gave us some special squeezy bottles so Bethany wouldn't have to struggle sucking, we could help push the milk into her mouth and promised to inform Alder Hey Cleft Palate department, who would send a specialist nurse to come and see us.
Trish, the Cleft Palate Nurse Specialist, came the following day. She confirmed everything Claire had told us and referred us on to the Cleft Palate Surgeon, Mr. Van Eden for a surgical consultation. Trish expressed concern during her examination of Bethany that her fontanelle (soft spot on her skull) appeared to have closed. We already knew Bee's was small but it having closed so early in life was a cause for concern. Trish explained that normally, as babies brains grow their skulls grow with it, closing the gap between the skull plates, known as the fontanelle. As Bethany's has closed so early their was concern that whilst her brain would continue to grow, the skull would not be able to accommodate the growth. This would result in the skull essentially squashing the growing brain and causing inter-cranial pressure leading to brain damage and an abnormally shaped skull. The correct term for this is Craniosynostosis. She told us she'd put a referral through to the Cranio-Facial Unit at Alder Hey and they would contact us in due course. Of course when she left I panicked. I imagined every minute going by was causing more and more brain damage to my beautiful little girl. As you can imagine we were completely freaked.
Bethany was finally discharged, after gaining 6 of the 8ozs she'd lost, on the 13th day. It was the day before my 26th birthday and the best present I could have hoped for, as cliched as that is. This time round we felt like we'd made progress. We had an answer as to why Bethany struggled to feed and I had the comfort of knowing the difficulty with breastfeeding wasn't my fault. But the up-shot of all this was it becoming increasingly obvious the Geneticists had something to find when they started testing. Now not only did Bethany have failure to thrive, developmental delay and hypotonia, she also had a cleft palate and bifid uvula to contend with as well as possible craniosynostosis. Trish actually asked us if we'd be surprised by a genetic diagnosis of something and we told her we wouldn't be. Her reaction was "if I'm brutally honest I wouldn't be surprised either". That just said it all.
We again couldn't have got through this period without the support of some very wonderful people: my Mum and Ken, Foz's parents and our good friends Lynne and Mike, even when it was just a pint of milk for a cuppa in the parent's room! :)
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