Caleb is still as "complicated" as ever and in his short time home, he is managing to build a reputation with the local fire department, as well as many pediatric units throughout Johns Hopkins Hospital including the emergency room, intensive care unit, acute care unit and the step down units. We have experienced several visits to the emergency room (2 G-J tube dislodgements, confirmation of 4th hernia, suspicious seizure activity, G-J tube malfunction, and trach infection with 103.1 temperature), two 911 calls with ambulance rides, and four hospital admissions. He has been put under anesthesia and recovered from a Bronchoscopy, Circumcision, Endoscopy, PH Probe Study, and two G-J Tube Replacements. Keeping Caleb healthy and free from the many germs that enter our house on a daily basis seems to be our toughest obstacle. Just the common cold and trach infections seem to set Caleb back for weeks at a time. Through all of this though, he continues to amaze and inspire us with his resilience.
Thankfully, Caleb is stable at the moment but he remains medically fragile. Caleb was born prematurely at 28 weeks and was diagnosed with a congenital diaphragmatic hernia. He has had three surgeries to repair persistent diaphragmatic hernias and he currently has portions of his bowel and spleen residing in his chest due to a fourth hernia. At the present moment, he has the following diagnoses: chronic lung disease, obstructive hydrocephalus that requires a VP (Ventriculoperitoneal) shunt, primary pulmonary hypertension, pulmonary hypoplasia, gastroesophageal reflux disease, congenital nystagmus causing some visual impairment, reoccurring tracheitis, and feeding difficulty with oral motor dysfunction. He is dependent on a tracheostomy, oxygen and a gastric jejunostomy (GJ) tube. He requires an awake and alert caregiver around-the-clock and must be monitored closely for respiratory distress, increased heart rate, oxygen desaturation, increase respiratory rate, wheezing, coughing, increased secretions, and infections in his GJ tube and tracheostomy stomas. In addition to constant monitoring, Caleb requires several interventions throughout the day including administering medication and inhalers 22 times, at least 20 water flushes, suctioning 10-15 times, tracheostomy and GJ tube care, tending to oxygen requirements, and alternating between J tube and G tube feeds per the doctor’s orders. These interventions, as well as many others may need to be performed more frequently when Caleb is sick and must be completed timely and with extreme care. As much as we try not to worry, it is nearly impossible. We are constantly reminded to always be cautious since just a slight change in Caleb's behavior could be caused by a worsening of his pulmonary hypertension, issues with the functioning of his shunt, or a shift in his organs due to the hernia...each of these being possibly fatal depending on the severity.
No matter how many setbacks there are, he continues to fight and his progress is slow, but steady. When he came home from Mt. Washington on June 16th, after spending eleven months in the hospital, he was vent dependent for twelve hours a day and required between 30-35% oxygen at all times (room air is 21%). After a few weeks of weaning and a sleep study, we are happy to tell you that Caleb has been vent free since the end of September and at healthy times, he is only requiring between 25-30% oxygen. As a result of Caleb's progress off the vent, they were able to downsize his tracheostomy in January, which forces him to do more breathing around the tube, rather than through the tube. The end result of downsizing is usually with the overall goal of decannulation; however, this will entail several bronchoscopy procedures (look inside the lung's airways), downsizes and capping trials to ensure that Caleb can clear his own airway effectively without the need for suctioning and to make sure there is no swelling, stricture, or obstruction of his upper airway. Also, decannulation may take longer for Caleb because he is still requiring oxygen and he has very little lung reserve (his saturations drop immediately upon removing his flow of oxygen). Although life at home would be much easier without a trach, there is no rush and removing it too early could be detrimental to Caleb. He just needs to grow and in time, the hope is that his lungs will get bigger and stronger. So, this brings us to the next topic of nutrition. Unfortunately, Caleb does not possess the coordination to suck, swallow and breathe (this is typically a learned behavior as an infant, but Caleb's complex medical condition did not allow him to master this skill) and he no longer accepts any sensation in or around his mouth (besides his own fingers). Oral aversion and feeding difficulty is and will continue to be one of our biggest challenges for months or even years to come. In the meantime, he receives his full daily nutrition through his G-J tube, where one tube ends in the stomach and the other extends into the jejunum (small intestine). When Caleb came home, he was on 24 hour continuous feeds through the jejunum port of his G-J tube. He has advanced to 12 hours through his stomach port and 12 hours though his jejunum port and we are slowly moving towards full stomach feeds, which would be amazing. This also takes time because of the greater risk for aspiration, but eventually Caleb could be on bolus (compressed) feeds that may help give him a sense of hunger. Hopefully, the G-J tube will be temporary and will allow Caleb to get enough nutrition to grow, develop, play, learn and recover at times of illness.
Since coming home, Caleb has been a very busy little boy. You would never believe the set of instructions Caleb has for just one full day. He also has his own personalize calendar to help keep track of his therapy sessions, milestones and many appointments! Multiple times a week, Caleb has physical, occupational and vision therapy in our home and we go out for additional therapy most weeks. We visit his pediatrician once a month and he has regular follow-up appointments with his general pediatric surgeon, cardiologist, pulmonologist, ophthalmologist, neurosurgeon, ENT and GI doctors. We've even created a method to remember important notes and follow-up questions from doctors appointments and set monthly reminders to order medications, trach supplies and feeding supplies. Somehow, we have managed to keep it all together!!!
One word that best describes Caleb is "happy". I often wish that everyone could smile as much as Caleb, who loves to show off his FIVE teeth. This little boy is poked and prodded at from the moment he wakes up until he goes to bed and even in the middle of the night. From administering feeds, medicine, and flushes, suctioning, and changing his trach or trach ties, he still smiles. Whether he is laying in his crib at home or recovering from a procedure at the hospital, he will get the biggest grin from ear to ear with just a whistle or click of the tongue from his daddy. Caleb is very content hanging out in his crib, playing on the floor, watching cartoons on the IPAD, following his mobile, snuggling, interacting with Brody and we are just now introducing the jumperoo since he is gaining more head control. He's also just started to really play with his toys if they are placed next to him, but he hasn’t mastered finding them on his own yet. Unfortunately, his CDH diagnosis, arriving three months prematurely, and his length of stay in the hospital can all impact his ability to develop. In addition, shortly after birth, Caleb was diagnosed with a brain bleed and hydrocephalus and required high amounts of oxygen that could further contribute to a delay in his progress. It is hard to imagine, but a healthy twenty month old toddler is typically crawling, walking, talking, eating and playing by now. However, Caleb still struggles to sit up and roll over on his own. We don't know exactly what this means for Caleb, but we can only hope that he will one day do all of those normal toddler things. In time, Caleb will write his own story!
Caleb was able to experience a lot of firsts since being home...his birthday, many crazy get togethers with family and friends, Ravens/Orioles games, Thanksgiving, Hanukkah, Christmas, a huge Christmas tree with a lot of bright lights (picked by daddy and big brother), and he even had a special visit from Santa and the local fire department. Just having him home this year has made these things extra special. He was also pictured in the November edition of the Baltimore Magazine with his physician, who was being recognized for the services she provides to children with complex medical conditions. Since keeping Caleb healthy through the winter was most important, we didn't venture out except for appointments. As the warm weather approaches, we are looking forward to walks outside, play dates, outings to watch Brody play baseball, and whatever other adventures present themselves. Come on Spring...
Overall, Caleb has made remarkable progress. He has beaten the odds and overcome many obstacles. The journey ahead will be long and hard, but we will be right beside him to love him, comfort him and guide him along the way. Every day Caleb is home with us will be another day cherished.
Big brother update – Brody continues to do amazing in first grade and he especially loves math and of course, recess! This winter, Wii Mario Kart was a favorite and he played on a winning basketball team. Baseball will be starting soon and we have been practicing every night. He still loves golf and the golf channel is often on and accompanied by the putting green in our family room. It melts my heart to see my boys together and especially when Brody gives Caleb a kiss on the forehead for no reason at all. I long for the day when they are chasing after each other...