Benjamin Shaffer
TrachKid Profile
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Ben is our first born child. He arrived seemingly healthy at 37 weeks on May 2, 2002. One day later, while still in the hospital he arrested in my arms. He was rushed to the NICU and revived after 20 minutes of no signs of life. He recovered slowly only to arrest again 4 days later. He was again revived after 15 minutes. He continued to desaturate without obvious cause. Many tests were performed with no definitive results. At 4 weeks a tracheotomy, gastrostomy and Nissen Fundoplication were performed. The trach resolved the saturation issues. Amazingly an MRI showed only minor spots on the brain stem. He sustained liver damage and had a severly abnormal EEG. After learning how to care for Ben's new specific needs, we brought him home from the NICU after 6 weeks. We originally had 50 hours of home nursing per week but it was increased to 90 hours when it was discovered that Ben had developed hypertrophic cardio myopathy at 3 months of age. The myopathy was resolved with blood pressure treatments once it was discovered that Ben's constant respiratory distress had led to high blood pressure which enlarged his heart. Ben was connected to humidified air and c-pap whenever asleep or sick to prevent secretions from pooling in his lungs. It was an unconventional use of the C-pap but it worked for him. Keeping water out of the tubes and out of his lungs was a constant struggle. Ben bounced in and out of the hospital for the next three years with pneumonias and other infections. We discovered early on that a g-tube fed diet of only pediasure was not right for Ben. He had turned orange from all the added vitamin A. So with the help of his dietician, we developed a mix of our own. Ben really wanted to communicate and clung to every bit of sign language we could teach him. He began using a passy muir speaking valve at around 1 year and slowly worked up to wearing it full time while well. He had a muscle biopsy performed to look for genetic issues, but none were found at that time. He had his tonsils and adenoids removed at 2 1/2. He was physically delayed in all areas, but functioned well cognitively. He received developmental, occupational, speech, and physical therapy in our home through First Steps for three years. A week before his 3rd birthday after all sleep studies, swallow studies, and observations were passed, Ben was decannulated successfully.
Today Ben is a thriving 6 year old in the first grade in a regular education classroom. He still struggles with respiratory issues mostly in the form of asthma and is frequently ill. He cannot swallow well and has some dietary limitations and oral aversions. He has visual perception difficulties and sees a vision therapist for this. His right hip has moved out of its socket and suddenly began walking on the right side of his foot. He will be having surgery soon to try to correct. He struggles with poor tone, a weak core and vestibular issues so he continues to receive occupational and physical therapy. His speech has improved dramatically, but he has the diagnosis of sound system disorder, so he receives speech services as well. Ben became a big brother to Alex a few weeks after he was decannulated. Ben's G-button was removed just after turning 4.
Ben has been an incredible light in our life. He has shown us great strength and determination. Despite all the stress, difficulties, red tape to cut, fighting for care and insurance, sleepless nights, and the pain of watching a loved one suffer, Ben's trials have been a challenge that gave us gifts untold. I discovered this website one night about 1 1/2 years into the struggle. I cannot describe what a God-send it was. I finally felt as though I were no longer alone. I hope this site continues to be a beacon for others for years to come.
Update 12-4-08
Ben suffered a few setbacks recently. He suddenly began walking on the side of his foot and lost bladder and bowel control. He required a tendon transfer and osteotomies on his right foot to correct structure. After a spinal MRI we learned he had a tethered spinal cord, requiring surgery to correct. An abnormal EEG and further studies on a muscle biopsy from infancy showed that he may have dystroglycanopathy ( a rare form of muscular dystrophy). We feel a definite peace at finally getting answers to mysteries that have eluded us for over six years. Ben is doing well after his last procedure and is walking better than ever. He will have a brain MRI tomorrow. We are anxious to compare it to the one he had as a baby.
12-9-08 It took 6 years, 7 months, and 6 days but today Ben was diagnosed with a form of congenital muscular dystrophy called dystroglycanopathy. His MRI was normal (white spots gone!), but EEG still shows abnormal activity in the brain. He is recovering well from tendon transfer, osteotomies, and spine surgery to remove a lipoma that had tethered his spinal cord. He is walking better than ever with only the assistance of an AFO and lift. We are pursuing gene testing as an attempt to isolate the gene responsible for all this trouble.
History
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- Member for
- 3 years 29 weeks
Location
- Country
- United States
- State/Province
- Missouri
