Jonathan (JonJon) Sherman
“Jonathan’s Journey” Facebook Page which is still active as he progresses in his medical journey and which you can find here https://www.facebook.com/jonathansjourneynj
He was a part of Steps Together from approximately 12/2015 through 12/2020.
Jonathan (JonJon) Sherman was born a month premature on 10/29/08. We were so excited to grow our family and his big brother Christopher was thrilled to welcome him home! It was clear from the beginning that there were going to be challenges ahead. Jonathan had a brain hemorrhage at birth and stopped breathing twice while in the NICU, so he was sent home on an apnea monitor for 3 months. At 10 weeks old, he had the first of many hospitalizations (and countless ER visits) for respiratory distress.
He has since been diagnosed with asthma, global hypotonia, Dyspraxia, Chiari 1 malformation, Marfan Syndrome, mild Hypotonic Cerebral Palsy, a large cyst on his brain, involuntary eye rolling with questionable seizures, chronic constipation, reflux, moderate obstructive sleep apnea, Periodic Limb Movement Disorder, iron deficiency anemia, ADHD, febrile seizures (which are now being monitored as they may have become Complex Partial Seizures), scoliosis which may require bracing and or corrective surgery in the future, bilateral pronation of his feet which require daily use of SMO's (braces), Sensory Integration Disorder, and a slowly progressive neuromuscular disorder has not been ruled out.
JonJon began receiving Physical, Occupational, and Speech/Swallow therapies in the first few months of his life and continues to receive different outpatient therapies weekly. Jonathan was evaluated at Johns Hopkins University Medical Center at their Hypotonia Clinic at 13 months of age with a main concern of ataxic gait. During that appointment, a keen eyed geneticist identified that Tom had a rare genetic condition called Marfan Syndrome and that he already had a heart aneurysm which requires daily medication and ongoing monitoring as it can be life threatening if it grows.
Genetic testing confirmed a few months later that both our boys also tested positive for the Marfan gene. This diagnosis led us down a new path of concerns, evaluations and treatment, not just for Jonathan, but for Tom and Christopher as well. They all take multiple medications daily and it is a huge monthly expense. Tom had to have major surgery in 2012 that took three months to plan as Marfan Syndrome makes them prone to hernias, as it is a connective tissue disorder that affects the entire body. He had a large, diaphragmatic hernia that allowed his transverse colon to come up and crush the bottom part of his right lung. He was recently diagnosed with another hernia which they are monitoring and has had several Marfan related medical issues to deal with himself.
One of the biggest struggles for us in dealing with Jonathan's multiple issues has been trying to piece together the puzzle that all of the different diagnoses has made. At times, it seems like every doctor we see has a different opinion about what the best treatment plan is. Seven years later, we are still seeing all of his many specialists and therapists, as well as continually having new ones recommended to meet JonJon’s unique needs. Many times that means traveling throughout much of New Jersey and even as far as Pennsylvania to go to The Children’s Hospital of Philadelphia and Johns Hopkins University Hospital in Baltimore, MD to see various specialists. As JonJon has gotten older, he has proven time and again to be a trooper and has gotten through a seemingly endless list of appointments. He has endured many ER visits, procedures, (such as CT scans, MRI's, EEG's and sleep studies) clinical evaluations, bloodwork, diagnostic testing, countless hospitalizations and ER visits and many therapies.
In December of 2015, they discovered he had a very large cyst in his brain which was increasing his intracranial pressure (Hydrocephalus) and causing him painful headaches, nausea, weight loss, loss of taste and smell, and personality changes for a big part of 2015. On December 23, 2015 he was admitted to the PICU after doctors decided he may have been developing hydrocephalus and that very day they performed the first of 2 brain surgeries/procedures & placed a probe directly on his brain to measure the intracranial pressure.
On Christmas Eve, 2015 he had brain surgery to drain the cyst as they were unable to remove it, but they drained it in order to decrease the pressure in his brain. So far, the results of the surgery have been very positive and many of the symptoms he had been experiencing have started to resolve. He will need follow - ups and sedated MRI's to ensure the cyst is not collecting fluid and pressing on his brain again, but we are very optimistic! He has been left with uneven pupil sizes post-surgery, as well as a hand tremor which tends to be worse as the day progresses or if he is tired.
Despite this daunting list of diagnoses and issues, JonJon is one of the happiest and friendliest kids you will ever meet. He does very well academically, loves to read and math is one of his favorite subjects. He enjoys playing sports and has played recreational soccer and baseball, too!. He has been through so much in his short life and handles it all with a positive attitude and a smile on his face. He is so charming and his spirit brightens the day of everyone he meets. He is truly an inspiration to us all!
September, 2021 Update: Jonathan is in the 8th grade and although he still has an IEP, he is in several Honor’s classes and enjoys learning very much! He had Surgery in January, 2020 and came through it like a champ. He continues to have many doctor and sepecialist follow – ups throughout the year, but he is no longer a “frequent flyer” in the ER which is awesome!
Further genetic testing determined that Tom (Jonathan’s Dad) has Loeys-Dietz Synrome, as opposed to Marfan Syndrome and so both Jonathan and his older brother were tested and came back negative for that gene! They did come back positive for a variant of the Marfan gene, but they are doing really well and if they continue to do well, they may be cleared by the Cardiologist altogether! Their next follow – up at Johns Hopkins University will be on 9/28/21…fingers crossed!
Jonathan was admitted for an overnight test on 9/24/21, but will be discharged on 9/25/21 and we are hopeful for a great report from the doctor once he has his follow – up appointment. Overall, our family is doing well after a very rocky few years. It has taken a long time to recover from all of this and so we are very grateful!