On November 6, 2014 I noticed multiple bruises on Connolly's legs. But with having 3 little boys four and under in the home that are rather rough, tough and ornery I wasn't really too concerned about them. I mean bumps and bruises are a daily occurrence. Over the next few days the bruising began to multiply...It was on his face, his arms, his back, his legs and multiple other places. I had also noticed that he had some red dots (petechiae) throughout. I decided to take Connolly in to see his pediatrician on Monday November 10th, 2014. They took a blood draw to run a CBC. The results came back with his platelet count being 9 (9000). I was told the lowest his platelet count should be is 210 (210,000). With a platelet count that low he was at risk for nonstop bleeding under the skin, internally and/or through an injury on the skin. He was life transported via ambulance to Children's Hospital. Admitted to the hospital our lives became a blur. Doctors, nurses and more coming one after another or in groups. It was believed he had I.T.P. (Idiopathic thrombocytopenic purpura). Which is a bleeding disorder in which the immune system destroys platelets, which are necessary for normal blood clotting. Persons with the disease have too few platelets in the blood. The treatment plan was to give him an IVIG infusion. Which is intravenous immunoglobulin (IVIG), also known as gamma globulin. This treatment runs hours and caused Connolly some nasty side effects. It takes 24 hours to begin to see any changes in the patients’ counts. During the labs on November 12, 2014...We found that not only had his platelet count not gone up, but dropped even further (to 6). He was also becoming anemic and his white count was going down...He tested positive for RSV and Rotavirus. Discussion of performing a bone marrow biopsy were in the works, but the Hematology Oncologist wanted us to try another IVIG infusion, in hopes that it would fix the issue. Unfortunately, it did not and Connolly had to undergo blood and platelet transfusions. This was done due to how severely low his counts were and to perform the bone marrow biopsy which took place on November 13th, 2014. Steroids were started that same day in hope to slow down his platelet destruction and give his body a chance to recoup. On Friday November 14th, 2014 we were given the news that he tested negative for Leukemia. Steroids were stopped on Sunday after finding there was no change in his blood work. On Monday November 17th, 2014 we were given the news that he has Aplastic Anemia. Aplastic Anemia is a disease of the bone marrow (essentially hollow bone marrow). It happens when the bone marrow stops making enough red blood cells, white blood cells and platelets for the body. Any blood cells the marrow does make are normal, but there are not enough of them. Connolly has a very severe form of Aplastic Anemia. Which puts him at risk for life-threatening infections or bleeding. After being given this diagnosis we were also told that we were still awaiting a third test from the bone marrow biopsy and it could take up to two weeks. We also discussed having a central line placed for his future transfusions. On Wednesday November 19th, 2014 Connolly underwent his second platelet transfusion and surgery to place his central line. In recovery Connolly had a bad reaction to anesthesia as he was waking. He threw himself about and ripped part of his new line and had to be taken back to surgery to fix the issue. His downward spiral with anesthesia continued through the night. Let's just say it's was a very long night. On Thursday November 20th, 2014 Connolly needed a second blood transfusion. Later that evening one of the Hematology Oncologist paid us a visit. She was there with the final bone marrow biopsy results. Connolly was found to have MDS (Myelodysplastic Syndrome). Myelodysplastic syndrome (MDS) is a rare disease of the blood, only occurring in four out of every 1 million children. Usually it develops in older patients (60+), it can occur at any age. It develops in the bone marrow, which produces the three major blood cells. This disease occurs when the bone marrow does not properly produce sufficient numbers of healthy red blood cells, white blood cells and platelets. These blood cells lose their ability to mature and function properly…
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…The only treatment for Pediatric MDS is a Bone Marrow transplant. Connolly’s 4.5 year old brother, Broderick, would be his best chance at a successful transplant. Until a donor is found he would continue to get blood and blood product as needed to support his needs. He would need to be seen every Monday and Friday in clinic to have blood draws, be given any transfusions that may be needed and to monitor his health and central line. We would have to have a home health nurse on call for any of his central line needs. We were told that Connolly no longer has any type of immune system and it would essential for us to keep him as sheltered as we could. No large groups, no eating out, no shopping, no house guests, no anything…just home, doctors and hospital. At home things needed to be hospital sanitized…hand sanitizer pumps everywhere, antibacterial soap, paper towels and hospital masks to become a new normal. Connolly’s 16 year old sister, Gwendolyn, was/is required to wear a hospital mask at school all day and sanitize constantly. Anyone from our household would need to wear a mask in public settings to avoid catching a bug and bringing it home to Connolly. He would be required to wear a helmet when moving about, playing or out of the home to protect his head. If he sustained an injury to his head, even a slight bonk, it could result in a massive internal bleed and he could die. We were given a new normal for Connolly…It consisted of low blood counts, blood and platelet transfusions and severe risk for internal and external bleeding. He could go home after he stabilized more and most of his care would continue through clinic at Children’s Hematology Oncology and with UNMC Pediatric Hematology Oncology/Bone Marrow Transplant team. After he went home we would have strict guidelines to watch for. We were instructed that Connolly was to return to Children's emergency if he had a temperature of 100.4, loss of appetite, inability to tolerate prescribed medications, specific symptoms such as increased bruising and/or bleeding, low energy or weakness…and to call with any question or concern. I felt faint. We had just been given the worst case diagnosis. I was in complete shock and hadn’t a clue how to begin to comprehend. I immediately became overwhelmed and lost. We were told that we would be given a ton of information in the upcoming days in the hospital and in clinic after Connolly went home. That there were other factors that hadn’t been factored in regarding his illness. That we would have to have some genetic testing completed, blood typing of immediate family and a few other things put into place to have a complete diagnosis. That there still were a few factors that could make this even more severe and unfortunately we had to wait on insurance to approve the testing and then the lengthy time it took to get the results. Connolly remained in the hospital until Sunday November 23rd, 2014. He came home on medication for his nausea, an antifungal for his central line, a medication to prevent PCP and Pneumonia and his normal heartburn medication. On Monday November 24th, 2014 Connolly has his first clinic appointment. During this appointment his 5 day old central line quite working and had gone bad. They had to remove it that day. They set up a new surgery to place another central line for the following Monday December 1st, 2014. Unfortunately Connolly needed to be rushed dot the emergency room on Friday November 28th, 2014 due to him screaming owie and pointing to his tummy for over an hour. He was admitted back into Children’s due to having spells of Intussusception and swollen lymph nodes. He required a third platelet transfusion on Saturday November 29th, 2014. While in the hospital Connolly began to have eating, drinking and potty issues and to be safe they kept him for further monitoring. His central line was placed on Monday December 1st, 2014 right in the middle of his chest. Different anesthesia was used to try to avoid the violent outbursts he had been having in the previous surgeries. After surgery he had troubles waking, but once awake he did fine for about 1.5-2 hours. During this fine and awake time frame Wesley (dad), Broderick (brother) and Penny (Mom, myself) went to the hospital (UNMC) that would be handling his transplant to have our blood drawn to begin blood typing to see if any of were a part match or full match for bone marrow. These results would take at least a week. We then rushed back across the city to Children’s where Connolly was. It turned out just in time as he then began falling asleep mid activity and then waking 10-30 seconds later to pick up right where he left off. He then fell asleep and began having spells where he just stopped breathing and I would gently to shake him to get his breathing again. They put leads on him to monitor his vitals and also his blood oxygen levels. This continued through the night and he was given a special steroid to bring down swelling from the breathing tube used during surgery…in hopes that that was the issue. The spells began to lessen and he was allowed to go home on Wednesday December 3rd, 2014. Our next step was to go to clinic on Friday. Here is a website that has some decent information on the MDS for the pediatric side. http://www.danafarberbostonchildrens.org/Conditions/Blood-Disorders/Myelodysplastic-Syndrome.aspx?_vsignck&_vsrefdom=myelodysplasticsyndrome&gclid=Cj0KEQiA5K-kBRDZ9r71gOvlxOMBEiQAwkK52O55GG9fuuFPwGSfKF4ipWENUYrvJmaKuDEDtub7PmgaAqnq8P8HAQ
