NAIT Explained: The Reason Behind the Infusions
On day one when we visited James in the Neonatal Intensive Care Unit (NICU), the doctors explained he was born with a platelet level of 13, which is classed as ‘severe’. Platelets are a component of the blood essential for clotting, and normal levels are between 140 and 450. Low platelet levels obviously cause bleeding into tissues and subsequent bruising, with severe cases causing irreversible intracranial haemorrhage (bleeding on the brain), resulting in long-term disability or death. The medical staff were describing how each contraction I had would have been like a punch to our poor little man in the womb. James was born extremely swollen and bruised, mostly around his head and bottom (both of which were ‘stuck’ in my small uterus – his head was up the top and his bum was down in my cervix). He also had petechial (red/purple) spots which are caused by broken capillary blood vessels, and an ultrasound showed a small grade 1 intracranial haemorrhage, but fortunately there was no long-term damage. This is where it truly started to sink in just how lucky we were – even the most knowledgeable doctors who really have ‘seen it all’ were calling this a miracle!
James had immediately received a platelet transfusion overnight but he had not responded – his platelets only rose to 26. The doctors then began questioning whether James had a condition called ‘Neonatal Alloimmune Thrombocytopaenia (NAIT)’. I’ll have a go at describing this, but I totally understand if it doesn’t sink in – it’s taken me a while to make sense of it all! In short, when James was formed, he inherited a type of platelet antigen from both myself and Scott. The antigen he inherited from Scott (which was different to mine) was recognised by my body as ‘foreign’, and my immune system created antibodies to destroy them. These antibodies in my blood crossed the placenta, came into contact with James’ platelets and caused them to slowly decrease. This process generally starts from 10 weeks’ gestation and can eventually deplete the baby’s platelets completely. Do not get this confused with RhD (rhesus) negative blood types – this is completely different and relates to platelets rather than red blood cells. NAIT is a relatively uncommon condition which is not routinely tested or screened for in pregnancy - the Women’s & Children’s Hospital in Adelaide only see about 6 cases per year.
The doctors requested Scott and I have our blood tested immediately in order to determine if this was definitely what had caused James’ low platelet levels. In the meantime, they found specific platelets from a donor in Melbourne and had them sent over on ice immediately. They divided this one donor bag into three and gave James a transfusion as soon as they arrived, and his platelet levels began to rise, increasing the likelihood of NAIT. In the meantime, Scott and my blood results confirmed what the doctors had expected – our platelet types were the ‘typical’ setup of NAIT, hence why James required specific platelets the same as mine (which didn’t have the causative antigen) in order to increase his levels. James received the three transfusions which were enough to initially raise his platelets to a ‘normal’ level, but this also meant they had used the supply if they needed to transfuse again. Despite my offering, the doctors were very reluctant to take and use my platelets unless absolutely necessary, as I had just had major surgery and my levels were also depleted due to bleeding. They instead found a donor on the registry in Sydney and contacted her asking her to kindly donate that day in case they were required, and they were again sent over on ice immediately. This is my not so subtle hint to please donate blood if you don’t already – James’ only hope were those specific rare platelets, and we will forever be in debt to those two kind-hearted selfless people who took their time to save a life – and this time it was our little man’s life.
Back on that first day in NICU, my favourite doctor and James’ biggest fan (let’s just call him Doctor Awesome) explained to us that James’ early arrival into this world was a blessing in disguise and if he had stayed inside just a little longer his platelets may have depleted completely and caused irreversible (if not fatal) internal bleeding...not to mention that he probably wouldn't have survived a vaginal delivery – even now this news is inconceivable. My case was the talk of the nursery. I had medical students reviewing our case, and the doctors were all unbelievably impressed by our little miracle - James needed to come early, and we love him for having the courage and strength to do so.
Fast forward to now. They say the recurrence rate of NAIT is very high (88-97%) with progressively more severe manifestations in subsequent pregnancies. So while this baby might not have this condition (depending on which platelet type he inherited), we are assuming the worst and treating regardless. Treatment is pretty intense (and expensive). I will receive weekly intravenous infusions of immunoglobulin from 20 weeks until delivery, starting tomorrow. IVIg is a solution of human plasma proteins from approximately one thousand blood donors, and contains the typical antibodies found in the normal population. The doctor explained that these antibodies ‘flood’ my blood and ‘trick’ my body to stop creating the specific antibodies which attack the baby’s platelets. This in turn decreases the risk of internal bleeding. The infusions take approximately 4-6 hours and are done under close observation, with the main side effects being severe headaches, body aches and vomiting. Also, these infusions cost the hospital over $8,000 a pop, meaning a total cost of up to $160K just for the infusions throughout my pregnancy. All I can say is thank goodness we live in Australia, if we were anywhere else we quite literally wouldn’t be able to have another baby. At around 34-36 weels (or however far I make it) I will need to have a c-section to prevent any bruising or bleeding to the baby, in case the treatment hasn’t worked – yes there’s a chance that the baby could still be born with low platelets, but thankfully this is rare. Obviously the birth will be closely monitored and bubs will require urgent blood tests and medical treatment in the nursery initially.
I’m anxious for tomorrow – mostly as I don’t understand the process yet and am unsure how my body will respond, but I am so thankful we are aware of this condition and treating it appropriately. I will be drinking lots of fluids and taking preventative panadol in the morning, and will keep you all updated throughout the day. I know this was more of an educational blog and was probably extremely boring, but I only hope I can raise awareness and help someone else who may be frantically googling for a ‘real-life’ experience…and at the very least, you will have a better understanding about this condition and the treatment process as you follow our pregnancy journey and develop your own love for our puggle!