Evan's story – by Caron James
My son Evan was an extremely boisterous 3-year-old little blondie, always on the go, always happy and loving preschool. He woke me up one night with a nosebleed, he had never had one before, so I wasn't worried, it stopped within 5 minutes, so we just carried on. A few days later, he had another nosebleed, but this time, it did not stop. I knew something was not right, so I made an emergency GP appointment, they said children's noses are extremely sensitive and prescribed some sort of cream.
We were also sent for a blood test 'just to be sure' within a couple of hours I took Evan to A&E. The consultant called us in and Evan dramatically vomited blood. The consultant pushed a button and the room was suddenly swarming with doctors, stripping Evan down to his underwear and putting in an emergency cannula, which was very traumatic for Evan. They said it looked like leukemia and my heart stopped! He was covered in petechai and bruises, even more so than the few I'd seen and put down to him being a clumsy boy.
His blood results came back while we were still in the emergency room. His platelets were 2. That is when I heard ITP for the first time, this complicated sounding illness, I'd never heard of. Evan was admitted to the ward and as we passed the playroom, Evan got excited and tried to run. He collapsed and I carried him to his bed. The doctor came in and explained the illness in more detail and said our GP had called in an absolute panic saying his patient has 2 platelets, the doctor said, "don't worry, he's already here". We watched Evan like a hawk and he slowly got less and less responsive. We called the nurses and again my boy was surrounded by doctors and nurses, they gave him fluids directly into his cannula and he eventually came round. To this day that was the scariest moment of my life, it seemed as though Evan was lifeless forever. He was given a blood transfusion and platelet transfusion and we were in hospital for 3 days. We were sent home with a bag full of medication and steroids. I was still trying to process what had happened and tried to educate myself about ITP as much as I could.
We were back and forth to hospital doing regular blood test and having consultants’ appointments. I was told Evan would likely recover from ITP within 3 months, he didn't, then I was told 6 months, still, he was struggling to maintain double figures and he was off and on steroids at varying doses and he had rounds of ivig. He responded well to treatments thankfully, but it was always a quick fix. He had a fall and hit his head badly and because he had single figures, he was sent for an MRI. They wanted me to leave him because of the radiation but Evan was scared so I refused to leave. I had to wear a bomb proof dress and neck guard and watch my tiny boy go into the biggest machine. He was so brave and laid perfectly still. There was no internal bleeding. Thank God.
After his 1-year diagnosis anniversary we were referred to the paediatric haematologist in Southampton hospital and Evans case was discussed with Dr Grainger in Manchester. We discussed treating Evan with either rituximab or eltrombopag. The decision was ultimately in my hands and I struggled with the decision. I did not want my child on any medication. He was frequently visiting hospital, he was quite a celebrity, he loved going to hospital, but was missing so much school and was beginning to fall behind. Turns out, the threat of treatment, made Evans immune system behave and his count began to rise. He managed to get triple figures without meds and just before Christmas when Evan was 7, we were told Evan was "likely in remission" it was literally all I had wanted to hear for the last 4 years! And we were all SO relieved. Evan's remission lasted 1 whole year, the following November, Evan came downstairs with blood blisters in his mouth. My heart sank, I could feel the weight of my boy’s condition hit me again like a ton of bricks. We went to hospital and his platelet count was 2. He was given 3 different courses of steroids, but each time he weaned off, his platelets would crash. Evan was sent for a lumber puncture. The results confirmed ITP which was expected but it was a relief to know nothing else was lurking. Evans count remained dangerously low, then one evening, he went for a wee and his urine was pink. We rushed him to hospital. They asked for a sample and his urine was RED. It had clots in it and I somehow managed to calm Evan who was obviously shocked and worried, the nurses in turn, tried to calm me. Evan was given steroids by IV and we were admitted. The steroids gave Evan good numbers but we were back within 8 weeks with blood blisters and he was given ivig another 4 times before the consultant said "now is the time for treatment" I had to make a decision so I discussed Evans options again directly with Dr Grainger, we spoke for over an hour and between myself, Evans consultant and the specialist, we decided to put Evan on eltrombopag. Evan was upset that he would potentially not get to go to hospital so regularly, so I bribed him with a weekend at Legoland. It has been such a relief to me that Evan has taken his condition like a champ and has not felt poorly. My philosophy is, if Evan's well, I am well. He is a strong boy and I must be strong for him.
It took 3 months, but Evan reached triple figures and his dosage was reduced. I was comfortable with how things were going and so were the doctors. His liver function tests were coming back normal and he was having no side effects except for not being allowed any dairy after 5pm, this drug was magic. Things were great for 6 months, then on 23 march, days after lockdown and the added worry of this pandemic, Evan was covered in bruises. We called the community nurse for a blood test, his count had crashed to 23, he had not been unwell, there was no reason for the crash. His medication was increased, and his count is slowly rising. He currently has 58 platelets. I check the inside of his mouth daily for blood blisters and check for bruises. I am constantly aware of his ITP and know how unpredictable it can be. I've found the ITP support association a great source of information and assurance and I have become friends with some ITP mum's via Facebook pages, which has been so nice to talk to others who know exactly how you feel. The doctors are hoping that puberty might spur Evan into remission. I remain ever hopeful.