Developmental milestones for children with PWS are typically one to two years delayed from that of an average child. Nolan started crawling and pulling to a stand just before his second birthday. Now two and a half, he is finally taking steps independently and can walk short distances without assistance. Nolan also has delays with fine motor skills and has Sensory Processing Disorder (SPD), also called Sensory Integration Disorder. SPD makes it difficult for Nolan to properly feel where his body is in space, and affects his depth perception and protective reflexes.
Speech
Nolan has delays with expressive language and auditory processing. We introduced him to American Sign Language when he was 4 months old. He started signing back to us when he was 15 months old, and now uses and understands more than three hundred signs. We have found the Signing Time video series to be a fun and easy way to learn new signs. Nolan's speech has really blossomed in the past year and he continues to use signs to supplement his communication. We continue to introduce new signs and encourage his expressive language skills in order to help minimize the frustrations associated with speech delays. In addition, Nolan's speech therapist uses a technique with him called PROMPT which uses tactile cues on the jaw, face, and under the chin to help develop or restructure speech production output.
Medical
There are many medical problems associated with PWS. Nolan was in Neonatal Intensive Care for the first three weeks of life, and has had three surgeries, with another on the horizon sometime in the coming months. He has endured numerous medical procedures including a spinal tap, EEG, reflux probe, two CAT scans, MRI, laryngoscopy, long-term naso-gastric feeding tube, long-term gastric feeding tube, bronchoscopy, barium swallow and fluoroscopy, orchidopexy, two month-long rounds of twice per week intramuscular HCG hormone injections, oxygen supplementation and long-term oxygen monitoring, sleep study, ultrasound, vision testing, several x-rays, and countless blood tests. He wears AFOs (foot braces) to support his feet and ankles in standing. In addition, he receives a daily injection of growth hormone every night before bedtime, which he will require for life. He has scoliosis, sleep apnea, torticollis, hypotonia, laryngomalacia, mandible asymmetry, testosterone deficiency and hypothyroidism in addition to the complex metabolic disorder that is the hallmark of PWS. He takes several medications daily and attends eight Early Intervention therapy sessions per week (physical, occupational, speech, music, and early education therapies.) We hope to start hippotherapy during the summer months.
Due to his low tone and very low metabolism, low activity level and decreased energy output, coupled with an intense desire to eat and seek out food constantly, Nolan is on a restricted 750-800 calories per day diet, and keeping his weight from spiraling out of control consumes our lives on a daily basis. Every portion of food is measured, and he is weighed several times per week. There are no cookies or other treats except on a very special occasion. A typical snack consists of half an apple and 1/3 cup of low calorie cereal. Due to a dysfunction in the part of the brain called the hypothalamus, Nolan's struggle with food and his need for strict calorie restrictions will be lifelong. His brain can not tell him that he has had enough to eat, so he will always feel hungry and will never feel full, even right after eating a meal. His access to food must be controlled and monitored around the clock, for life. He has very little chance at living independently unless a cure can be found for the relentless hunger.