Stacy and Steve Corke
England, UK

OUR NAIT JOURNEY

1991 saw me having an awful pregnancy! I was huge although I ate little, had high BP and was covered in an itchy rash! I certainly didn’t bloom, more like a huge wilt. Everyone used to joke “you must be allergic to pregnancy” mmm funny how these words were not that far from the truth!

On my due day after a 36 hour labour my daughter Emily was dragged into this world with forceps and a ventouse! The silence was awful! I was exhausted and all I could see were the Doctors working on her in corner. we thought we had lost her! But eventually they managed to get her breathing! Her Apgar score was 1 at birth! She was rather bruised and this was put down to the delivery. Emily was perfect despite her traumatic entrance to the world.

1993 and I was expecting my next baby. But this time I looked and felt so well!  I was even looking forward to the delivery and hoping that this time things wouldn’t be quite so scary! This time things would be fine…. so at 36 weeks when my Midwife noticed that my bump had stopped growing I wasn’t unduly concerned as all previous checks had been fine, she was a good size and constantly kicked. So Monday morning I quite happily toddled off to the hospital on my own! they were just being cautious weren’t they?… As the ultrasound moved to her head my world fell apart! Even I could see with my untrained eyes that something devastating had occurred. The scan showed a big black mark covering more than half of her brain! the midwife didn’t have to say anything. I was immediately referred to Kings College Hospital in London to see fetal medicine specialist “Professor Kypros Nicolaides” the next day.

Professor Nicolaides explained that he didn’t think it was Hydrocephalus as was suspected by the local hospital, he suspected something worse as the damage was so severe. The scan showed that more than half of “Louise’s” brain had been destroyed and was filled with cysts. This had caused her head to swell all out of proportion. The valve that controlled cerebral spinal fluid at the base of the brain had collapsed. There was no way I would be able to have a normal delivery. He then offered me a termination! I remember looking at him and thinking what is this man on about? I’m 36wks not 24wks (the latest date in the UK for termination). He then explained that as the damage was so severe her quality of life even if she survived the birth would be devastating. It was legal, but I would have to have consent from my OB at the local hospital. This was the hardest decision I have ever had to make in my life! One I never thought I would have to face. I just wanted to run away, how could I decide to end my babies life! I could feel her moving around. How could this be real!

After much heart searching my husband and I made the decision to “help her go”. An appointment was made for the next day for us to see my OB to sign the forms and then back up to London the following day (Thursday) to see Kypros who would perform the procedure. No one slept those two days. It sounds really awful but I lay there all night just hoping that Louise would suddenly stop moving and I wouldn’t have to make the decision. I wanted it all taken out of my hands. Every little movement broke me further. Thursday arrived and even though I knew it wouldn’t be possible I wanted so much for the scan to show that everything was fine and it was all just a horrible dream. Louise was given a fatal injection to the heart. To allow Louise to be born naturally the fluid that had caused her head to swell had to be drained. Professor Nicolaides explained that he had suspicions as to what had caused such damage it was a condition called Fetal Maternal Alloimmune Thrombocytopenia and if as he suspected, and he was right, then future pregnancies could be affected by the same problems. The Haematologist concurred and blood samples of myself and my husband were taken. We travelled back to our local hospital in a state of shock. Not only was our baby dead and I had to give birth but the fact that, if the doctors were right, this could happen again!. Louise was stillborn the following day. She weighted 6lb 5oz. Sounds terrible but I was afraid to see her and at first glance she was so bruised and the damage to her head was obvious but then we could see beyond that and she looked perfect, with dark wavy hair and rose bud lips. Seeing Louise helped us to realise we had made the right decision and lift some of the guilt. The blood test results 2 weeks later confirmed that it was FNAIT and 100% of my future pregnancies would be affected. But Professor Nicolaides and Professor Mike Murphy (haematologist) agreed that although the condition was rare and not a lot was really known about it, they could and would treat me to try and prevent it happening again.

Four weeks later meant a check up with my normal obstetrician and for the final results of the post-mortem. It was all quite surreal! This fool of a Doctor sat there and told me that my baby had died as a result of Hydrocephalus and that “No it was unlikely to happen again, it was one of those things!” The fool had not even bothered to read my file let alone the post-mortem results! Lets just say I do not think he appreciated being told what I thought of his competence! and what I had learned in the past 6 weeks about FNAIT , which was a lot more than he had bothered to do.

My husband and I decided that we did want more children and were willing to try again. And 1994 saw us expecting another little girl “Melanie”. The thing was way back then not a lot was really understood about how early the antibodies crossed the placenta so treatment would start after a PUBS (percutaneous umbilical blood sampling)-also known as fetal blood sampling (FBS) – where a blood sample is taken from babies cord thru the mothers abdomen and baby is then transfused with platelets to ensure that they are high enough). This would be performed at 21 weeks to see how the counts were holding up. During the 2 hour journey to the hospital Melanie made some very odd rapid movements which unsettled me, and that first PUBS did nothing to alleviate my fears! I was sure I could see a tiny dark patch that shouldn’t be there! but as we were looking at such an immature brain I tried to put my worries out of my head, although I did think the Doctors didn’t seem entirely happy. Melanie’s platelet count was only 30,000 (they should be over 150,000) she was transfused with platelets to bring them up to a safe level. An appointment was made to return the following week for another PUBS. Waiting a week was hell for me, trying to act like everything was fine, I didn’t mention my fears to my husband or family. Why should they feel as bad as I did when there was a chance I was wrong but in my heart of hearts I knew I wasn’t. The next scan confirmed what I thought I had seen, the damage was more visible “Melanie” had suffered a Massive ICH and as I was so early into the pregnancy the damage had totally destroyed her brain stem. She was still alive but only thanks to my body, Kypros explained that she could not survive. Again I had a terrible decision to make. I was induced at my local hospital the same day and “Melanie” was born the following day weighing 1lb 2oz, she looked like a perfect doll. Following “Melanie’s passing we met again with Kypros and Mike Murphy-consultant haematologist and they explained that it wasn’t known that the antibodies would cross the placenta so early in a pregnancy, treatment was new but they thought that if I decided to try again they would start me on steroids and possibly IVIG to try and lower my immune response.

Losing two babies within the space of a year saw me trying to cope. I hated the fact that I had chosen to end their lives and no way was I ever going to go through that again. I then had what my husband called a bacon moment!!! I was trying to cope too well, acting like I was fine etc and it got too much! One day I couldn’t open a packet of bacon and I finally broke down. Happy tabs being the order of the day!

Fast forward to 1999 and morning sickness!! Yep! unplanned of course. This time we saw Professor Nicolaides and a new Haematologist immediately, and it was decided that I would start on prednisone at 14 weeks. A PUBS would be taken at 20 weeks. The first PUBS showed that the counts were starting to drop, “George” was transfused and I was to start having IVIG once a week. The following week a second PUBS was performed , the platelet count was only 71k so another transfusion. BUT this time it all went wrong. “George’s” heart started to fail. Kypros battled for an hour to try and save him, but there was nothing he could do. He was too early to deliver. I couldn’t believe that I would be doing this again, Why me?………..Why my Baby?…….. my world was falling apart yet again and there was nothing I could do! I felt so sick, so in shock! Travelling back to the local hospital to be induced… how I hated that labour ward. For the third time I would be coming home without my baby. The midwives were lovely and made sure I was looked after but it didn’t ease my pain. “George” was stillborn the following day he weight 1lb 4oz a perfect lil man.

I threw myself back into work and college and put all thoughts of babies out of my head. The following year (2000) I had just completed a Counseling course and was busy working for a local AIDS charity when I started to feel decidedly unwell. This went on for a couple of weeks, finally it clicked NOOOOOO! Not again, I was scared witless, I couldn’t handle losing another baby everything was too raw. I knew the odds would be stacked against us, could I really go through it all again? Could I stand to lose another baby? But then could I really not give this baby a chance, at least it deserved that much.

This time it was another Haematologist, Dr Alec Mygovic at Kings, who initially wanted to just start on steroids at 14wks, but I refused and said as the counts with George had dropped by 20 weeks he needed to offer something else. The steroids obviously didn’t work that well on me. I was not prepared to risk anything on guesses. It wasn’t as if we would get another go! After a lot of debating! We reached a compromise and I started weekly IVIG from 15weeks. It was agreed that my normal pregnancy checks and IVIG would be done by the local hospital but everything else would be handled by Kings College and Kypros. The BBC were at Kings College at the same time making a programme about the fetal medicine surgeries performed by Professor Kypros Nicolaides and because NAIT is supposed to be so rare they asked us if we would agree to be filmed during our treatments. I agreed as I thought they would work in our favour, no one’s going to screw up if it’s on camera! So my life then revolved around IVIG once a week at my local hospital, 8 bottles of IVIG would take 6/7 hours if I was lucky!! I think the longest day I went in at 9am and finally was released at 2am, The IVIG wasn’t ready, there was no one to put a new vent in when my vein collapsed!! I was not impressed and caused a ruckus!! After that they seemed to get things in order! The headaches from the IVIG were awful, and I felt so water logged but it was a small price to pay.

At 18 weeks we found out we were having another lil man “Jack”. At 20 weeks we were back at Kings for a scan then PUBS/transfusion. All was OK.  The platelet count had dropped quite a bit , but not dangerously low and we were to return in 3 weeks. The 3 weeks flew by. I couldn’t settle. I just wanted to know how things were, would I be seeing another ICH?! Finally we were back at Kings, the scan showed all OK and the PUBS & transfusion went fine, but the platelet count had dropped dramatically in those 3 weeks and was only 34,000. So weekly PUBS and transfusions became the order of the day! The weekly routine from then on was IVIG followed by an Ultrasound scan to check no bleeds had occurred and breathe again!  PUBS/Transfusion all OK, breathe again. Until next week! Everything was going fine until week 27 (December 22) when within a week the platelets had dropped down to 19k. Luckily there was no sign of an ICH. IVIG and platelet transfusions were upped to twice a week. The problem was this was it was now the holiday season with lots of bank holidays. Fitting all the appointments around these were a nightmare. I just seemed to live at the hospital with IVIG and PUBS. Christmas was awful, we tried to keep upbeat for the sake of Emily our daughter who was only 9, but it was hard, all I wanted to do was make the next treatment. Then things started to get out of hand, twice weekly transfusions and twice weekly IVIG were not keeping Jacks counts high enough. The counts started to come back dangerously low at 9k the next one 7k. At 29wks “Jack’s” count was 9k and he had only received a platelet transfusion 48 hours prior ( which had boosted him to over 180k). Things were so out of control now Prof Kypros agreed we couldn’t carry on, there was no way “Jack” would make it to the target 34 weeks, he had to be delivered.

It was agreed that less than 2 days later “Jack” would be delivered by c section at Kings College. “Jack” was born at 16.46 on 11 Jan 2001 weighting 4lb 2oz feet first! He gave a squeal and then was rushed off to special care I had a fleeting glimpse of a very bruised lil man. His platelet count was 10,000. That night “Jack” very nearly lost his fight, he was bleeding into his lungs, they couldn’t start the surfactant as he was having a platelet and blood transfusion and the ventilator couldn’t sustain him. They were ready to come down and get me to say goodbye! Luckily the hospital had on trial a new type of ventilator that rather than pump air into the lungs (which was a problem because of the bleeding) it vibrated the oxygen into his lungs. Seeing Jack for the first time the following day was a shock, he was swollen from oedema,  covered in petechia and bruises, with his whole body vibrating from the ventilator. Although he looked so small and sick I was able to stroke his tiny little hand. Then I knew he would make it, he was safe. Within 4 days he was moved to a standard ventilator he was still very sick but improving, It was so hard leaving him at a hospital when I was discharged a week later, but I had Emily at home who had been shoved from pillar to post the past few months, she needed me too, we lived 2 hours away but as soon as he was stable they promised they would have him sent to our local hospital. Jack was transferred to the local hospital at 12 days, his platelet count was holding and even starting to climb again. He kept having bradycardia episodes but was finally discharged at 5 weeks old weighing 5lb 3oz.

While all this was going on the BBC were there filming it all. Jack had ten platelet transfusions in all and one blood transfusion in the womb.
Jack went from strength to strength with no real health issues, apart from a heart murmur and asthma. He did have a ‘stridor’ (he used to make a funny noise in his throat from all the tubes) and when he was about a year he used to pass out/faint if hurt!! (learning to walk!! ) The pediatrician explained that it was that his immature brain couldn’t handle stress so it would ‘cut out’!! Very scary the first few times it happened but he did outgrow it. Jack will be 14 in January, the heart murmur has now resolved as they said it would, and he no longer needs his asthma meds, he is fit and healthy, we have never wrapped him in cotton wool and we won’t stop him from doing what he loves best!!! “Motocross!!!!! To see him racing a motorbike across a track no one would even guess what a start he had. When I watch the BBC programme about Jack, I can’t believe that we actually went through that and that Jack is here. It seems like another world, I still don’t know how we managed to stay sane! But for all the stress and heartache Jack certainly makes up for it.

As for the professionals who helped us I can’t thank them enough. They gave me the most precious gift!!

Stacy Corke

This video link looks back at the treatment I received to ensure Jack survived!

14 December 2014.