Medical Problems Of Those With MPS II

It is important that you consult with your medical practitioner regularly to monitor the progression of the disorder and enable various treatment options to be explored. Some common medical problems for individuals with MPS II fall into the following categories. This list is not exhaustive.  Please click on the headings below or scroll down to read all information.

• ENT and chest
• Heart
• Joints and carpal tunnel syndrome
• Anaesthesia

ENT and Respiratory Problems

Frequent coughs, colds and ear and throat infections are common problems for many individuals with Hunter syndrome. The tonsils and adenoids may also be enlarged and partly block the airway. Many ENT problems can be effectively treated.

Some children may have frequent ear infections and a degree of deafness is common and likely to get worse. Hearing loss may be conductive or nerve deafness or both (mixed deafness) and may be made worse by frequent ear infections. It is important for all MPS II children to have their hearing monitored regularly and for problems to be treated early to maximise the child's ability to learn and communicate. Nerve deafness is managed by the fitting of a hearing aid or aids. There are also adaptations to enable the hearing aid wearer to hear the class teacher, the television or telephone conversations more clearly. Details are available from the Australian Hearing Service.

Individuals diagnosed with sleep apnoea as a result of a sleep study may benefit from CPAP (continuous positive airway pressure). At night while the child or adult is sleeping, a CPAP machine which uses a mask over the lower face or a short tube, delivers air at a small positive pressure. It is often surprisingly well-tolerated even in young children and improved sleep seems to help children's daytime concentration.

Heart Conditions

Heart disease is fairly common in MPS II but may not develop or cause any real problems until later in life. The heart may be affected in different ways. The valves which open and shut as the blood is pumped from one chamber of the heart to another may be weakened by storage of mucopolysaccharides. The valves may fail to close tightly enough allowing small amounts of blood to leak back again. The muscle of the heart may also be damaged by storage of mucopolysaccharides (cardiomyopathy) and the heart may also be put under strain by repeated chest infections or by having to pump blood through stiffened and inefficient lungs (cor pulmonale). If necessary these conditions can be helped with medication.

Joint Stiffness and Carpal Tunnel Syndrome

Joint stiffness is common to all individuals with Hunter syndrome.

Individuals with MPS II sometimes experience pain and loss of feeling in the fingertips caused by Carpal Tunnel Syndrome. Carpal Tunnel Syndrome can be diagnosed with an electrical test called a Nerve Conduction Study. Early recognition and detection can ensure preservation of nerve and muscle function, as this treatment by surgery is safe and effective.

Anaesthetics for MPS II Individuals

Anaesthesia Any surgery for individuals with MPS II should not be undertaken lightly. Giving an anaesthetic to an individual with MPS II is a skilled task which should always be undertaken by an experienced anaesthetist. Adult sufferers should ideally wear some sort of identification bracelet with a contact medical person in case of emergency. With children, parents should inform their child's school and any person who looks after the child in case they cannot be contacted in an emergency. If you have to go to a different hospital in an emergency you should tell the anaesthetist that there might be problems with intubation. The MPS Society has more information on this important subject. 

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