Spinal Muscular Atrophy
Spinal Muscular Atrophy (SMA) is a condition which affects the nerves of the spinal cord and which prevents nerve impulses from travelling between the brain and the muscles. There are several different types of SMA and, although there is no cure, research is ongoing and certain types of the condition can be managed by physiotherapy and other methods.
Adam has a mobile standing frame which he uses to enable him to stand upright to strengthen his muscles and to aid the development of his feet and hips.
Many conditions are covered by the term Arthrogryposis, which denotes multiple joint contractures (fixed and stiff joints). Adam has regular physiotherapy sessions to treat his condition, which affects his ankles, knees, hips, thumbs, wrists, elbows and shoulders. He has had courses of hydrotherapy and in 2009 had surgery on his knee.
Update - 16th November 2012: Some of the professionals involved in Adam's care are now querying whether Adam's symptoms are consistent with a diagnosis of Arthrogryposis. However no alternative diagnosis has yet been agreed upon.
At birth, Adam’s hips were found to be stiff. An ultrasound scan revealed that he had bilateral hip dysplasia, meaning that both his hips were dislocated. At three days old, therefore, Adam was fitted with a Pavlik Harness, designed to hold his legs in the optimum position to correct his dislocated hips. After several weeks an ultrasound scan revealed that his right hip had corrected itself but that the left hip had not. X-rays showed a blockage of tissue had formed in the socket part of the left hip joint and that surgery would be required to correct this. At 13 months of age Adam was admitted to the Bristol Royal Hospital for Children for hip surgery. Following the operation he spent 4 months in a Hip Spica Cast, a plaster cast from his chest to his left ankle and right knee, with a small hole for nappy changes.
We soon became quite adept at fitting nappies into the hole, although a lot of time had to be spent trimming nappies to the correct size. When the cast was removed, the hip was found to have responded well to the treatment and to be developing normally. However it is possible that Adam will require further surgery.
Update - 16th November 2012: Adam had surgery on his left hip in April 2012. See 2012 News for full details.
Physiotherapists use a scale from zero to six to grade the severity of clubfeet, with zero meaning no deformity and six meaning the most acutely affected. At birth, Adam’s feet were both assessed as “six”, so at one week old he began a treatment regime which followed the Ponseti Method. Developed by Dr Ignacio Ponseti of the University of Iowa, this involves gradually changing the shape of the foot by manipulation and plaster-casting. Adam spent several months with both legs in plaster, visiting his physiotherapists weekly for further manipulation and recasting. As part of the treatment, he was also twice admitted to the Bristol Royal Hospital for Children for tenotomies, operations to cut the Achilles tendons of both feet in order to lengthen the heel cord. Following the casting phase of the treatment, Adam had to wear a "boots and bar" device for several months.
Although initially the treatment was successful, Adam’s feet lost almost half of the correction that was originally achieved. He was therefore referred to Naomi Davis at the Royal Manchester Children's Hospital, who began a fresh course of Ponseti treatment, including regular casting. This necessitated frequent trips from Cornwall to Manchester which were exhausting for both Adam and his mother. However, the results have been excellent and Adam's feet have now regained much of the correction formerly lost. He resumed wearing the boots and bar at night, but this made it harder for him to sleep and also began to put pressure on both his knees and his hips. In May 2010 therefore, Naomi Davis decided that, while he should still wear the boots at night, he should stop wearing the bar, to prevent further problems. During the day Adam wears supportive splints which allow him to wear regular footwear, much to Adam's delight - he has frequently expressed a desire to own a pair of slippers and now he does.
Update - 22nd July 2016: We have now been advised that Adam's talipes have regressed to the point where casts will no longer be effective, leaving surgery as the only option. See our 2016 News Archive for details.
Scoliosis is a lateral curvature of the spine which affects as many as one in twenty children. Although it is not caused by bad posture, Adam has been given a lycra suit to wear to help hold his body straight (see 2010 News for further details). It is hoped this will help control the condition, although further treatment may be necessary.
Update 16th March 2015 - Adam had scoliosis surgery in November 2014. See our 2014 News Archive for details.
ASD - Autism
Early in 2015, Adam was diagnosed as having Autistic Spectrum Disorder, a condition that is estimated to affect one in every hundred people in the UK and which affects his behaviour and his ability to cope in some social situations.