Hi, my name is Donna and I am the heterozygous carrier of ALPS that I passed on genetically to my precious son Devin. I am not in any way trying to scare anyone, but I need to share our experience to draw awareness to this disease. Our story is also one of the warrior stories posted, so you may know us already. I am so sorry we are all part of this ALPS and ITP community, as we know how devastating these diseases can be. Devin and I have both suffered ITP symptoms several times in our lives. He more than me. We got to the point that we knew exactly what to do when we saw petechia or mouth blisters or bruising. Call the hematologist and order a CBC. If platelets were at an unsafe level, be admitted to hospital for IVIG and Rituxan until safe levels. Unfortunately, in September of 2019, Devin awoke with ITP symptoms. We went straight to ER and had CBC done, his platelets were 2000, so we went by ambulance to St. Louis Children's Hospital for IVIG and Rituxan. Even though Devin was 24 years old, his ALPS was diagnosed at this hospital when he was 3 years old, so we continued care here. They kept him overnight and sent us home because the treatments can take several days to weeks to work. He started a horrible nose bleed on Wednesday evening and we paged the hematologist on call..SHe said nosebleeds with this low of platelets is not uncommon, if it doesn't stop in 10 minutes call back. Well it did stop. Looking back now, I regret with my whole soul, not realizing the mouth blisters were worse than any other occurrence and the nose bleed too, was a sign I wish I had said "we aren't comfortable this time it seems worse, can we please come over for a CT scan?" But being desensitized to the symptoms from previous occurances, we went back to watching our movie and thanked God the nosebleed stopped. The next day was uneventful, he laid around and rested, but no more nosebleeds. We were supposed to return Friday morning for another CBC then Monday have another IVIG and Rituxan if platelets not safe . Friday morning our world stopped. Devin actually. texted me at 5:35 am that he could not move his left arm or his left leg. I ran downstairs to find him having stroke like symptoms. We called an ambulance and took him to the local hospital where they did a CT scan and saw that my Devin had suffered an Inter-cranial brain hemorrhage and was in need of emergency surgery to relieve the pressure. They airlifted him to St. Louis Children's Hospital where the Neurologist and team of surgeons took his platelet count and told us that his platelets were 2000 again and therefore could not safely have the life saving brain surgery because he would bleed out and they would not be able to stop it. They did an emergency splenectomy because they could clamp those arteries to control bleeding and over the course of 8 hours transfused 24 bags of platelets, n-plate and any options available to try to raise platelets to do the brain surgery. After the splenectomy and n-plate, the platelets seemed to be rising some, so we had much needed hope for a minute. Then the ALPS kicked in and the liver started taking over platelet destruction in place of the spleen! Steroids, hours of immune suppressing measures could not stop the ALPS from continuing to destroy them and at 9:00 that evening our precious Devin was no longer responding to stimuli and was placed on a ventilator.. two weeks later we had to say goodbye. We are lost and broken. The reason I felt I needed to post this is so that you are aware that ITP as a secondary to ALPS can be life threatening in the event of an ICH. Please advocate and do not leave the hospital until platelets are at least above 10,000 and safe monitoring are in place. I now live not only with the loss of my best friend and precious son, but also with the survivors guilt of being the genetic carrier of ALPS to my son, even though I had no idea until ITP appeared to us 10 years ago, that I even had the gene defect. I pray for all of you on this nightmare journey and that research continues to progress to hopefully one day have ITP secondary to ALPS protocol safely in place. Please make sure to have a CT scan if the bleeding is great and refuse to leave the hospital until you feel the active bleeding symptoms and platelets are at a safe level. Feel free to ask me any questions you may have and I apologize if my post has scared or upset anyone. I needed to share to hopefully save someone else from this devastation.