Elain and Siôn Llwyd Lewis’ NAIT Story
By Nia Llwyd Lewis, mum to Elain, 5 years old (untreated, platelet count of 4,000) and Siôn, 2 and a half years old (treated, platelet count of 130,000) , North Wales, UK.
2005 was a very eventful and emotional year for me. Aled and I got married in June. I broke a bone at the bottom of my back by falling down stairs at work in October and was off work for a month. Then, at the end of November, my Mum died suddenly aged 58. She had suffered from Ovarian cancer for nearly 3 years and although she was having chemo she was ok…she made Sunday lunch with all the trimmings for me, my sister and my Dad on the Sunday, and then on the following Thursday, she died. I was devastated. Then a month later, I found out I was pregnant with my first child. I was sooo happy and yet so sad at the same time that my mum would never get to see her grandchild.
I had an amazing pregnancy; I didn’t get any kind of morning sickness or any complications at all. Elain, my daughter, made her appearance into the world two and a half weeks early at the end of August 2006, weighing just over 6lb. She was perfect. The doctors mentioned that she was a little bit cold so ordered a hot cot and that her feet were a little bruised but that should clear up (looking back they were more than a ‘little’ bruised – they were both purple! But being the first child I really didn’t think it was anything out of the norm!!). Looking back, it’s a miracle that it was only her feet that were bruised due to the normal delivery…having read all of the other stories and case studies that I’ve come across, we are so lucky. Elain’s been a little fighter since the day she was born!! So off we went to the Labour Ward where she slept for most of the night. At 9.30am the following morning, I changed her nappy and the nurse came over to check her over, she was fine. At 10.30am the doctor came over doing his rounds and when he checked Elain she was covered in these purple, freckle like spots, which was initially thought to be some kind of rash. She was rushed into the Special Care Baby Unit (SCBU) and started on antibiotics as a precaution and a full blood count was done. That came back saying that her platelet level was 4,000…I’d heard of platelets but hadn’t a clue what they were at the time!!! It was then the Doctor explained that a newborn baby’s platelet count should be at least 150,000 – 300,000 and that her count was alarmingly low and she would have to have a platelet transfusion…this would increase her platelet levels and then once they were up they wouldn’t come back down again… so her platelets went up to 7k but went back down to 4k the day after. That second night in hospital was awful. I was a new mum, surrounded by 5 other new mums, but they all had their babies with them….and I had to lie there listening to them all crying etc when I had no idea what was happening to my daughter. I was absolutely terrified. She then had another platelet transfusion, but the same happened again, and were back down to 6,000. We were in absolute shock as we couldn’t understand why they had gone back down again…of course what the first doctor had failed to explain was that they wouldn’t just stay up after two (normal platelets) transfusions and that it would take time!!
Luckily for us, the first Doctor we saw was only in SCBU as it was the August bank holiday weekend and we saw another Doctor the next morning. This doctor sat us down and explained that she thought that Elain’s platelets were falling due to Neonatal Allo Immune Thrombocytopenia – she had seen exactly the same in another baby at the hospital where she worked before down in South Wales, and explained what she thought was happening. She believed that there was an antigen in my blood that had been attacking and destroying Elain’s platelets. So both Aled’s blood and mine were taken and sent to the Platelet Lab in Bristol and that day, Elain was given a transfusion of ‘special’ platelets which were flown up from Bristol. Her platelet level rose to 93,000, but 6 days later, they had plummeted down to 6,000 again. As they had been so low, Elain was given a brain scan to check for brain damage but thankfully there was no intracranial hemorrhage (ICH), but those few minutes where the sonographer was looking at the screens and not saying a word were the longest few minutes of my life. Towards the end of our 2 week stay in SCBU, her platelet levels were staying above 50,000 for more than 2 days, so, as we lived 10 minutes away from the hospital we were allowed to go home, but had to go back every day for a full blood count. Elain and I practically lived at the hospital for the next 2 weeks as we had to go there every day by 8.30am in the morning so that we could get her blood tested, stayed for hours then waiting for the result and were either sent home or had to wait for another transfusion or immunoglobulin to arrive!! 7 days later, she was given her 3rd special platelet transfusion, as they’d gone down to 20,000. They rose to 150,000 but then 10 days later, they’d dropped to 16,000, and she was given her 4th special platelet transfusion, and they rose to 193,000. She very nearly had to have a transfusion through a vein in her head then as they couldn’t find an useable vein in her hands, arms or legs, and if it wasn’t for Dr Raj and Dr Ambica’s brilliance that day, she might not have had the transfusion at all, they said we’ll try one more time, but if not, we will have to transfer her to another hospital as she has no veins left. They got the cannula in just above her ankle, and I held her leg whilst she had the transfusion as the doctors had warned us if the cannula came out, she had no other veins left to use. Eventually, after a month, we got to go for a full blood count every 3 days as we knew that her platelet levels were staying up for a while in between transfusions. By then, I knew most of the doctors on the children’s ward and was nicknamed the ‘sweeteaze’ lady as I was giving that to Elain when she was having her blood taken!
Then, when she was 5 weeks and 4 days old, her platelet levels dropped again to 28,000, so the doctors decided she would have to go back again the next day for another transfusion. When we got there, the doctor said that he needed to see what the platelet levels were before doing the transfusion…we already knew if it was under 50,000 she would have another transfusion. The results came back…they were 59,000…I guess Elain was adamant that she wasn’t having another transfusion!!! The doctor explained that because they were taking blood from her heel for the full blood count as all the veins in her hands and feet couldn’t be used any more, the reading yesterday might have been a little misleading, so he asked us to come back in 2 days for another full blood count. We went back the next day, and Doctor Raj who was taking the blood asked me what I thought the platelet level would be today? I said I hoped it would be higher than 50,000 as it was my birthday in a few days time and that would be the best gift ever… the results came back a few hours later and I could tell that the results were good as the doctor did a kind of dance when he came towards me along the ward…I will never forget the smile on his face when he told me they were 113,000!!! Then they gradually rose to 184,000 by the time she was 2 months old and by the time she was 3 months old, the level was 231,000 and we were discharged.
Fast forward 2 years and I found out just before Elain’s 2nd birthday that I was pregnant again. Mixed emotions again, overjoyed, worried what would happen, how would they treat me, would the baby be ok?? During the 2 years since Elain had been born, as you can imagine, I had done a LOT of research into NAIT, and luckily, had found the yahoo group… I was so thankful to everyone on there for their support and advice, I felt that I, and they, knew more about NAIT than some of my doctors / consultants did – some had never even heard of NAIT, so I was very glad I’d found people that knew exactly what I was going through and could offer me such excellent advice! This time I was given an IVIG transfusion over the course of about 10-12 hours (8 bottles during the day) every 2 weeks from 20 weeks onwards at my local hospital and as well as the usual scans, had monthly scans at the Liverpool Womens’ hospital where my consultant, Dr Leanne Bricker was based. She was fab, very thorough, and helped me a lot, she was always there for me, and did a lot of research into the current treatment too.
With my 2nd child, I was actually given the IVIG in the same Haematology and Cancer Day Unit where my Mum had been treated for her cancer for 3 years, which was a hard concept to begin with as I’d been there a few times with her when she was having her chemo. When she died, they offered us the opportunity to donate a framed photo to the unit, that they would hang up on the wall in the ward, so being into photography, I went and took a photo of Llanberis, which is the area where mum grew up, and took that in, never expecting to see it again. So when I got to the ward on the first day to have my IVIG, they told me to go to sit on a bed in the corner, and there I was wondering where they’d put the photo (+ it was a distraction rather than thinking about the IVIG!), I couldn’t see it anywhere, until I got up to stretch my legs, and there was the photo, right above the bed where I was told to sit – the only place I hadn’t looked!!! I know it’s a little corny, but I like to think that it was my mum’s way of watching over me!!!
I was really lucky with my 2nd pregnancy too, apart from heartburn after the IVIG and a little nausea, and the fact that I couldn’t bear the thought of eating salad cream (!!!) I was fine. My consultant at my local hospital wouldn’t agree to a c-sec for ages saying that this wouldn’t affect my baby’s platelets, but mine (I had to explain to him that it was Allo not Auto Immune Thrombocytopenia!!!) and I should keep an open mind etc and everything would be fine with a natural birth, until I went there one day armed with all the case studies and advice I’d had from all the people on the yahoo group and he eventually agreed with me!
Siôn, my son, was born by c-sec at the end of March 2009, he seemed healthy and no ‘purple freckles’ at all!! He was taken to SCBU as a precaution and I was wheeled into the recovery room. My body went into shock as he’d arrived so quickly into the world, and as I was laying there, the nurse came in with the incredible news that his platelet levels were 130,000!! I was so happy!! He was tested daily (I insisted on heel prick tests this time as I didn’t want all of his veins to be used up in case he needed a transfusion. His platelets rose to 133,000 on day 1, 146,000 on day 2, but fell to 93,000 on day 3, and went down to 50,000 on day 4. As his platelet levels were dropping, he had to have 2 immunoglobulin transfusions when he was 4 and 5 days old, and although they were down to 50,000, he was nowhere near the 4,000 level Elain had been when she was born. His platelets rose again to 130,000. He was also given a brain scan and again, thankfully, no ICH. This time I asked the doctor to tell me exactly what he was seeing…there was no way I wanted to go through the silence I had when Elain had hers. We were allowed home after a week in SCBU as his platelets were now 194,000 but we had to go back to the hospital in 3 days…they’d gone up again to 257,000!!! We then went every week and then when he was a month old, we were discharged as his platelet levels were 335,000 and staying up.
I count myself extremely lucky to have two NAIT survivors – such beautiful, healthy children. I will always be eternally grateful to all the doctors and nurses at SCBU and Alaw Day Unit at Ysbyty Gwynedd and the Fetal Medicine Unit at Liverpool Women’s Hosptial who treated Elain and Siôn, and gave them such an amazing start in life, but I also count myself extremely lucky that I’ve found so many people who understand exactly what NAIT is about, without all of them and the advice that I had, my NAIT story could have been very different. It’s weird to think that none of us would have known of each other if it wasn’t for NAIT, but because of it, we have an unexplainable connection, and although I haven’t met any of the other NAIT families YET(!), I feel that I know some of them so well!!
Let’s hope one day that doctors start testing for NAIT as part of the usual tests during pregnancy so that no one else has to go through what we’ve all been through…
Nia, Aled, Elain and Siôn
North Wales, UK