His mom, Julie shared their story of bruising, hospitals, treatments and determination to live a full happy life in spite of ITP....
We jokingly refer to Samuel as our million dollar baby. Being a preemie and spending a month in ICU will earn you that title.
A year after he was born there was little sign that his life started out fierce and fighting. Cruddy ventilator lungs was the only lasting sign. Samuel was your average 3yr old who loved life and left nothing unexplored.
The first bruising we noticed was in 2009. I remember telling my older son that he needed to not be so rough while they are playing. ( awful, I know) It was winter and the boys had been playing in some unexpected OK. snow. Later that day he developed huge bruises across his lower back that seem to get worse and spread out by the day. I was at one point even worried the school would think we were beating our child! I still never had any alarms in my head going off and chalked it up to great outside fun and a clumsy boy.
A couple months later, I watched Samuel lean over to pick something up and barely hit the corner of his eye. He didn't even flinch and I thought nothing of it. Moments later, I look at him and there is a huge ball on the side of his eye that is black and blue. Bleeding inside his eye and blackened the other side of his face and corner of his other eye. Then the blood looked almost like it was slowly moving down his face. I had never seen anything like it!
Finally some alarms went off. His brother was going to pediatrician the next day for a followup and I decided I should probably take him with me and just
let him take a look at his face. Our awesome pediatrician did bloodwork just to be safe and when he came back he said that Samuel had only 5k platelets and normal was 150k and above. He then proceeded to tell us that he is at risk for inter-cranial hemorrhage and that he could not rule out leukemia. He told us to head to the hospital and that they would be waiting for us. Ugh. In my head I am thinking, ok, what is a platelet? What did they do? How did a followup appt. end up with us going to the hospital fearing for our child's future? We head home to pack a quick bag and drop off our older son.
At the hospital we waited half the day and night. They gave us a diagnoses and said they were pretty sure it was not leukemia. I've never been so relieved. Samuel began his first of what would be 30 plus infusions of IVIG. The infusion lasted 8 hrs and nasty side effects followed with fever, vomiting and migraine.
We were given all the odds and percentages for ITP and we were hopeful as the remission rates for his age were great. Only 4% will go on to be chronic as children.
A week later his numbers were in the 400k range and our hemoc. told us she thought he was in remission. Yeah!
Two weeks later we went for a followup. I noticed bruising coming back and after a CBC, his body had already chewed through most of the 400k platelets and was down to 60. This became a pattern over the next year. Every two-three weeks he was in hospital getting IVIG infusions. It was definitely a lifestyle change. Weekly blood draws and missing out on fun things because he was in the danger zone. As a Mom and friend, I became the most unreliable person. We could never truly make plans at school or otherwise because we simply did not know what our week would entail.
After a year, we realized that Samuel is part of the 4% who's ITP is chronic. We explored another treatment option called mercaptopurine . This was an oral chemotherapy pill given to leukemia patients as a maintenance drug. Scary, but it was shown to have good results with long remissions. It would be the first time Samuel learned how to swallow a pill and he did it like a champ. He took it at night at certain specific times. He did not have many side effects, mostly a nauseated stomach. After 4-5 months I began researching the drug and found many disturbing facts about secondary leukemia risks while taking this drug. It was not helping his numbers and his doctor took him off.
We began discussing splenectomy and vaccinated him and prepared. We decided on doing a bone marrow biopsy to be sure prior to surgery to make sure we were not missing anything. That was tough and glad it was done. During this time we started noticing bruising and bleeding with higher counts. I would assume he was low, take him in for labs and numbers would not match the symptoms. This began a whole series of testing putting splenectomy on hold. Aggregation testing and platelet function testing were coming back abnormal. So not only did his body fight off his platelets, the ones he did have were not functioning. Many, many tests were done for months, specialized tests sending as far as as Wisconsin with no real answers.
In Feb. of 2012 I was getting Samuel's bath ready and I could hear him commenting on how his pee looked funny. I turned and looked and there was a stream of dark brown. It didn't even register what it was at first. You get that familiar pit in your stomach and I knew we were in for a long night. I called our doctor and she said to head to children's and they would be waiting to admit him. This began the scramble of packing bags for the hospital and getting our son Ethan somewhere. We are good at not panicking at this point, but the unknown of "what now" is what can drive you crazy. He was in the hospital 3 days until the bleeding stopped. He had CT scans, MRI and ultrasounds. His platelet count? 80k! Samuel for sure got everyone's attention. The bleeding was from the kidney so a nephrologist was called in. Due to the bleeding, he could not do a kidney biopsy. He did other testing for kidney function that was normal. The hematologist blamed the kidneys and the nephrologist blamed ITP. At this point we were scared that we did not have a clue at what we were dealing with nor did anyone else.
We decided to try Rituxan. IV chemotherapy was given once a week for a month. This also unfortunately did not nothing for his counts and was a very long month and rebuilding of his immune system and side effects. I had already been researching, trying to find a platelet specialist. Boston kept coming up and before long we were headed there for a second opinion. They were super excited to meet Samuel and the first conversation was spent discussing our approval on putting Samuel on their new study for testing. Although very insightful, our options were still limited. Both Boston and his local doctor are concerned with the possibility that there is something underlying going on that has not surfaced even though he has been tested for everything under the sun. He is an awesome doctor who I am thankful we were lead to. He has discussed Samuel's case with rooms full of oncologist / hematologist around the world who met at oncology conferences for their opinions. How awesome is that!
So now we are in somewhat of a holding pattern. We have had to stop using the IVIG because of the chemical induced meningitis he was having. The most recent treatment he had is Winrho. It was nothing short of a nightmare. He was a very
sick little man and we had some scary moments with this treatment.
This brings us to our current date. We will be going to consult about starting NPlate. There is positive information regarding this drug. Although I am very skeptical to keep experimenting with these new drugs. It was shortly after stopping the 6mp that he began the weird bleeding symptoms. Coincidence? I am ready to pull out his spleen and be finished but his doctors are still worried there are other issues. There is actually a test that can help determine whether it is spleen or liver that destroys the platelets. Of course this test is only offered outside of the U.S. Why? Well there are two thoughts:
1. Not enough need for the test and the isotope that is used in the test is very hard to get and the lab has to be certified to get it which is very expensive and hard. This was concluded from a Red Cross research facility that used to perform the test but can no longer because of the isotope. We had an interesting conversation for sure.
2. The test just is not a good tool.
That brings us up to date!
Who is Samuel? He is not the poor 6yr old that has ITP. He is the most happiest, energetic, kindhearted child. He takes every needle and treatment in stride and can be sick as a dog and wake up smiling. We are not dramatic in speaking with him about his disease and after three years he does not really know any different. We find something fun out of every hospital stay and doctor visit. ITP is not who he is and to speak to and see Samuel, you would not even know he is sick and that's our goal everyday. God gives his toughest battles to his strongest soldiers and Samuel is a survivor and a fighter.