[text] hunter

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                    <h1 class="c:white fw:700 fs:lg mb-3 mb-lg-4 lh:3">Disease progression</h1>

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                            There isn’t a typical disease course in Hunter syndrome, as each individual has their own unique set of genetic mutations contributing to the disease.²

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                            However, progressive decline is inevitable in Hunter syndrome as GAGs accumulate. Although there isn’t a typical disease course, there are common trends in the progression of the disease.²

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                                            <p class="c:white fw:900 fs:xs:2">NEURONOPATHIC</p>

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                                            <p class="c:white fw:900 fs:xs:2">NON-NEURONOPATHIC</p>

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                    <span class="text:under c:white fw:900 d-block">Neuronopathic</span>

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                    In Hunter syndrome patients with neuronopathic involvement, behavioural problems including

                    hyperactivity and aggression may start from the second year of life. These behavioural issues can

                    become very serious, disrupting the quality of life of both the patient and the family. Patients

                    with neuronopathic involvement can also be obstinate and unruly; these behaviours can be exacerbated

                    by the hearing difficulties and sleep disturbance-induced fatigue.³

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                    Neurodegeneration due to GAG deposition leads to fine motor skill impairment in some patients, with

                    a median onset at 4 years old. Children may become particularly boisterous if their tolerance to

                    pain has increased due to loss of sensation (dysaesthesia). However, behavioural problems typically

                    only continue until the age of 8 to 9 years, at which point neurodegeneration causes cognitive

                    functions to wholly decline. Around this point, the ability to walk and talk diminishes. At the same

                    time, cognitive decline means that learning and maintaining new skills will become harder.³

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                    Severe neuronopathic decline leads to the complete loss of the ability to communicate and chew or

                    swallow, as well as extreme cognitive disability and the inability to control gastrointestinal

                    function.^3,4

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                    Advanced nervous system symptoms will also be observed in patients as they decline cognitively. Many

                    patients suffer from carpal tunnel syndrome, which has a median onset at age 8 years.⁵ A few

                    patients develop communicating hydrocephalus, which exacerbates the cognitive decline and can result

                    in behavioural changes, headaches and vision disturbances.³ Patients can also suffer from spinal

                    cord compression due to dura mater thickening, which leads to a general muscle weakness, loss of

                    sensation, neurogenic bladder dysfunction, and further deterioration of fine motor skills and gait

                    problems.³

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                    <span class="text:under c:white fw:900 d-block">Non-neuronopathic</span>

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                    As a patient with Hunter syndrome grows, the non-neuronopathic symptoms will become increasingly obvious and debilitating. The characteristic facial features and prominent brow begin to show around 2 years of age.1 Patients can be of average height for their first few years, before growth begins to slow, eventually leading to a short stature.³

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                    A noticeable consequence of Hunter syndrome is that patients require numerous surgeries, even before the underlying disorder has been diagnosed.⁶ The repeated ear infections and hernias, and presence of enlarged tonsils and adenoids are early symptoms of Hunter syndrome that require treatment, and many Hunter syndrome patients will undergo multiple hernia repairs, tympanostomies, adenoidectomies and tonsillectomies before Hunter syndrome is identified.⁶

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                    After these early symptoms, the disease continues to progress, and patients will often suffer from auditory, ocular and dental complications that require more surgeries. Other common developments are joint contractures and skeletal deformities, which gradually limit mobility, and eventually prevent the patient from walking and standing erect.³

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                    The management of Hunter syndrome patients and their symptoms requires that physicians are aware of the special underlying and multisystemic nature of Hunter syndrome. Therefore it is extremely important to diagnose Hunter syndrome early in order to ensure timely and appropriate management.³

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                    Learn more about the specialist multidisciplinary management of Hunter syndrome patients in <a class="opacity:7 c:white fw:900" href="monitoring-management.html">Monitoring and management</a> section of this website.

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                    Cardiac and respiratory complications can be life-threatening. Cardiac involvement through valve disease is common in Hunter syndrome patients, from a median onset age of 6 years, and cardiac complications which develop include abnormal heart frequency, cardiomyopathy, left and right ventricle hypertrophy, arrhythmia, hypertension, and ultimately, heart failure.^5,7

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                    Respiratory tract infections such as pneumonia are common and can develop to be life-threatening. Respiratory failure, cardiac arrest, pneumonia, and cardiac failure are the most common causes of 60% of Hunter syndrome patient deaths as recorded in the HOS.⁸

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                Remember – if you suspect Hunter syndrome: REFER TO A SPECIALIST TODAY

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