Note: This is pretty matter-of-fact with very little emotion expressed. The reason for that is this is a component of a grant application I am in the process of submitting. I did not go into great detail surrounding some of his experiences, simply for the purpose of brevity. As I have promised on many occasions, the real story of Jaime will someday come about, but this isn’t it…
Jaime was delivered by emergency c-section six weeks early, after experiencing severe distress in utero, despite a completely normal previous 34 weeks (for a Type 1 diabetic mother). He was transported within hours to the Children’s Hospital of Philadelphia’s Cardiac ICU because he had a coarctation of the aorta, which was quickly determined to be the least of his many issues. He had severe hyper-bilirubinemia and received two full-volume exchange transfusions, where all of his blood was removed and replaced with donor blood. He also received multiple whole-blood and platelet transfusions during that time. He experienced seizure-like activity a few times, which were then considered myoclonic activity.
At one-week old, he was moved from the CICU at CHOP to the NICU back at Cooper, where he was born, due to his cardiac diagnosis no longer being seen as an issue (CHOP’s NICU was being remodeled at that time and there were no beds available, so they felt Cooper would be the next-best choice). A few hours after he was extubated and placed on a nasal cannula in Cooper’s NICU, he went into severe respiratory distress. He had developed a systemic staph infection that had gone undetected. Because the respiratory distress was dismissed as a reaction to medication several times, despite obvious physical distress, cyanosis, and a drop in O2 saturation to 80%, it was left untreated for 45 minutes. By the time he was intubated again, he was in such distress that the tube “nicked his trachea” and his lungs filled with blood. I was there for the entire time and was told that he was not going to live through the night. He did live, though, and was in critical condition for a week on an oscillating ventilator (basically a machine that gives over 200 tiny breaths per minute in order to help the lungs rest and heal). At the end of that week, when he was deemed stable enough, he was removed from the oscillating ventilator and transported back to CHOP’s NICU upon my demand. Once there, members of the NICU staff began to see a significant increase in seizure-like activity, so he had an MRI which showed a major brain injury. He was diagnosed with severe Cystic Periventricular Leukomalacia, the leading cause of Cerebral Palsy. Basically the grey matter around the ventricles in the brain were deprived of oxygen and died. The injury was vast and the Neurology team prepared us for the worst.
Jaime came home from the hospital after six weeks of intensive care, on his original due date. He was on a nasal-gastric feeding tube and a breathing monitor. He came off the feeding tube and monitor at four months and began therapy with Early Intervention. He began to progress, though much more slowly than I could have anticipated.
When he was six months old, he began making strange jerking motions and screaming incessantly. When I took him to the emergency room, they told me he had an ear infection and gave him an antibiotic injection. When he was still screaming and jerking a day later, the pediatrician examined him and said he did not have an ear infection and sent him to the emergency room to have his bowels examined. They told me there that he was constipated and sent us home. Two days later he was still screaming. His father noticed that his eyes were rolling in his head when he had the jerking motions so I told the pediatrician and he sent me directly to the emergency room at the Children’s Hospital of Philadelphia for myriad tests, including an EEG. After three days of testing, Jaime was diagnosed with hypsarrhythmia, or infantile spasms, a devastating form of epilepsy, and Cortical Vision Impairment, which is frequently seen with infantile spasms and renders the patient legally blind.
He was treated unsuccessfully with oral medication for the infantile spasms and was then treated with a daily steroid injection (ACTH, or Acthar Gel) for seven weeks. Fortunately, the steroid injections worked, but the devastating effects of the infantile spasms were already apparent. Jaime had regressed to the level of a four month old. The first time I ever saw him smile, he was 11 months old. I will never forget that moment as long as I live. It was then that he regained his eyesight as well.
Jaime was finally given an “official” diagnosis of Cerebral Palsy (chronic static encephalopathy, characterized by spastic quadriparesis, microcephalus, and psychomotor retardation). He is now almost six years old. He cannot sit up on his own and cannot roll over. He is non-verbal, but he uses one syllable babbles and grunts. He will communicate some of his wants and needs by shaking his head “no”, clapping, crying/whining, or grunting and reaching. Because he has been working with a yes/no button, he starting to understand how to push buttons to get what he wants (i.e. on toys, as long as the buttons are easy enough to push). He is in a wheel chair and attends kindergarten in our district’s special education program where he receives physical therapy, occupational therapy and speech therapy, in addition to his daily education by his multiple disabilities teacher and “specials” with his inclusion class.
Jaime is a little love bug. He laughs and smiles all the time and claps his hands. He loves music and goes nuts when anyone sings or dances on TV. At this very moment, he is rocking in his chair with his hands in the air, squealing, and laughing along with the Laurie Berkner Band! He has been showing everyone just how extreme his love of music, particularly playing instruments, is. Since he has started to use his left pointer finger, he has started “playing” any type of piano he can get “pointy” on! He is quite the little Beethoven. And his face lights up every time he sees his mommy. Everyone who meets him falls madly in love with him and his beautiful blue eyes and impossible-to-ignore smile. He knows it, too. He loves to pose for the camera and he frequently admires his reflection in my car door.
Most recently, he has learned to say “hi”, and uses it appropriately, but only when he feels like it, of course (he is very strong-willed, which explains his immense strength and determination in his will to live). Jaime also began to imitate his speech therapist saying “ho ho ho”, though he has only said it to me once or twice (on Christmas morning – my favorite gift this year).