General (6)
€31m for mental health services, but St. John of God's withdraws independent living skills courses from School
Written by Super UserAs was reported in the Independent, the independent living skills courses has been withdrawn at St. Augustine's Sallynoggin, by the board of St. John of God's. I was under the impression that people were now being looked after in the community, instead of by the service providers under the new directions initiative being put in place by the HSE. Even more strange is the statement by the board which states that no one is to lose their job as a result but will be found other jobs within St. John of God's. If you feel the same, click on the link below to send a pre-formatted email to Finian McGrath TD Minister for Disability Services.
A child with Down syndrome may have weak muscle tone (hypotonia). He or she may also have ligaments that are too loose (ligament laxity). This leads to excessive joint flexibility.
Associated findings include:
- Developmental delays. A child with Down syndrome will eventually reach the same growth milestones as other children. But, he or she may be slow learning to turn over, sit, stand, walk or do other physical activities.
- Joint instability. Children with Down syndrome may have joints, such as the hip or knee, which slip out of place or become dislocated. Joint laxity in the neck may be excessive. This can lead to compression of the spinal cord.
Other Problems. Down syndrome may also lead to other problems, such as flat feet and bunions.
The neck
In some people with Down syndrome, the upper part of the spine (cervical) is abnormal under the base of the skull. Muscles are weak and ligaments may be looser than they should be. Potentially, this can cause bones (vertebrae) in the neck to press on the spinal cord, leading to an inability to coordinate muscle movement and weakness.
Any progressive changes in a child with Down syndrome should be brought to the doctor's attention. These changes may include:
- Weakness;
- clumsiness and tripping;
- Walking with stiff legs;
- having a stiff neck, neck pain, and headaches.
The doctor may recommend taking X-rays of your child's neck to look for joint laxity.
In some cases, a child may be kept out of contact sports or other activities that put stress on the neck, such as high jumping, diving, gymnastics, and using a trampoline.
The teeth of a child with Down syndrome usually come in later than the teeth of most children. The first tooth usually appears between 12 and 48 months. Fifty percent of children with Down syndrome are missing one or more teeth.
Health Checks for People with Down's Syndrome
There are many medical problems that are commonly found in people with Down's Syndrome as well as cardiac conditions. These are usually not major problems in themselves, but they can affect health and development and should be screened for so that early diagnosis and treatment can be made. Sometimes doctors are not fully aware of these issues in relation to Down's Syndrome and they may put them down to being part of the syndrome itself or even related to the heart condition.
It is important for everyone with Down's Syndrome that these medical problems are recognised and treated, but particularly for those with cardiac issues as developmental delays may be attributed to poor health and hospitalisation due to the heart and thus other problems may be overlooked. So be persistent in asking that the relevant tests are carried out according to the guidelines issued by the Down's Syndrome Medical Interest Group (www.dsmig.org.uk).
Heart
About 47% of babies born with Down's Syndrome have congenital heart defects and not all of these will exhibit symptoms in the first few months of life. Accordingly all babies diagnosed as having Down's Syndrome should be screened for cardiac problems and this should be done within the first six weeks after birth to ensure that serious abnormalities can be treated at the optimum time.
Clinical examination alone is not sufficient and should be carried out together with either an echocardiogram (performed by someone with appropriate paediatric experience) or with an electrocardiogram (ECG) and chest x-ray (again these should be examined by someone with appropriate paediatric experience). Where problems are suspected as a result of these tests, there should be immediate referral to a paediatric cardiologist so that if surgery is necessary it can be performed before pulmonary vascular disease develops.
It is also known that there is an increased incidence of mitral valve prolapse (where the mitral valve balloons back into the left atrium when the left ventricle pumps, sometimes allowing blood to leak backward) and aortic regurgitation (where the aortic valve allows backflow from the aorta to the left ventricle) in adults with Down's Syndrome. This puts them at risk of endocarditis and antibiotic cover will be required when any dental procedure is carried out.
The Down's Syndrome Medical Interest Group recommends echocardiogram screening early in adult life for everyone with Down's Syndrome, and prophylactic cover (medication to guard against endocarditis) for anyone in a risk situation who has not had an echo as an adult.
Cervical spine (atlanto-axial instability)
A small number of people with Down's Syndrome are at risk of spinal injury due to a weakness in the joint at the top of the spine. There is currently no way of screening for this weakness and there is no reason to exclude sporting activities as the risk is small, although care needs to be taken when giving anaesthetic to people with Down's Syndrome and caution is advised should they be involved in a road traffic accident.
Should a person with Down's Syndrome develop unexplained neck pain, pain behind the ear, weakness in the arms and legs or problems with bladder or bowel functions, immediate referral should be made to the GP with a view to urgent investigation of the upper neck.
Dental
Annual dental check ups are very important for anyone with a heart condition as oral hygiene plays an important part in the prevention of bacteria entering the blood stream where they can cause endocarditis. (see Dental Care Topic Note).
Growth
Although it is recognised that people with Down's Syndrome tend to be shorter than their peers, there are occasionally medical problems which can affect growth which need to be identified and treated. Regular weight and height checks should be made during the first two years of life and then annually throughout childhood and on a regular basis in adulthood. These should be recorded on the specific growth charts that are available for Down's Syndrome.
Failure to gain weight as a baby or sudden weight loss or gain later in life needs to be taken in consideration with general health and nutrition as it may indicate an underlying medical problem that needs investigation.
Hearing
A large percentage of people with Down's Syndrome have significant hearing loss, most frequently due to glue ear (otitis media with effusion). First assessments can be carried out a few months after birth, but there should be provision for continued assessment and treatment of any hearing loss as hearing plays such a huge part in speech and language development.
Thyroid
There is a higher incidence of thyroid problems in people with Down's Syndrome than in the population as a whole - Hypothyroidism (underactive thyroid) is more common although Hyperthyroidism (overactive thyroid) is also found. Either condition needs to be treated as the thyroid gland is responsible for controlling the body's metabolism and incorrect levels of thyroxin (the hormone secreted by the thyroid gland) can affect growth and development as well having some uncomfortable side effects such as lethargy, weight gain or weight loss and palpitations.
Clinical examination alone is not sufficient to identify thyroid problems in those with Down's Syndrome. Regular blood tests of thyroid function need to be carried out.
All newborn babies in the UK are routinely given the Guthrie test which screens for many disorders including hypothyroidism. For those with Down's Syndrome regular testing at least every two years should be carried out from age one. Currently this usually requires blood to be taken with a syringe, but annual testing using fingerprick blood, which is far less traumatic, is being evaluated and is likely to become the method of choice in many areas. Annual testing is preferable in early childhood and introduction of the fingerprick test means that this can be achieved with little upset to the child.
Vision
There is a high incidence of vision problems in people with Down's Syndrome, these include not only long and short sight but also poor focusing at near, squints, congenital cataracts, nystagmus and keratoconus. Vision should be checked and monitored regularly throughout life. Eye examinations are possible even in very young children unable to read a letter chart.
The Down's Syndrome Vision Research Unit is studying eye defects and visual development in individuals with Down's syndrome and their effect on learning. You can read the latest research findings at www.cardiff.ac.uk/optom/eyeclinic/DownsSyndromeGroup/downssyndromemain.html.
Anaesthesia
Although some procedures are possible using local anaesthesia and / or sedation, given the added difficulty of communication and understanding, it is likely that patients with Down's Syndrome will be given a general anaesthetic even for these.
Whether a general anaesthetic is required for heart surgery or another operation the same principles apply. When anaesthetising anyone with Down's Syndrome the anaesthetist needs to be aware of concerns regarding atlanto-axial instability (an increase in mobility in the top of the spine which is found in some people with Down's Syndrome) and also the possibility of a narrow oesophagus which may make intubation (insertion of the tube into the windpipe which is used to provide oxygen during the operation) more awkward. If the patient has an unoperated heart condition and the operation is not cardiac related it may well be suggested that the surgery is undertaken at the heart unit where everything is on hand to deal with any potential problems. In some people with Down's Syndrome, anaesthesia is not safe because of high lung artery pressure (pulmonary hypertension).
Before surgery
Whatever the operation, the anaesthetist will visit on the ward before surgery, ask questions about the patients' health and medication, and explain what the anaesthetic will involve. They will have discussed the operation with the surgeon and use this information and what you tell them to decide on the best type of anaesthetic to use. They may prescribe some medicine to be taken just before going to the operating theatre, which is called a pre-med (pre-medication) that may include a sedative to make the patient relaxed and sleepy.
For a child, the nurse will put some local anaesthetic cream on the backs of their hands, so that it doesn't hurt when the cannula is put in in the anaesthetic room. Anyone having a general anaesthetic will not be allowed to eat or drink for several hours before the operation, to reduce the chance of them being sick during and after the operation - they will have a sign over their bed saying 'Nil by Mouth'.
In the anaesthetic room
This is a room adjoining the operating theatre, where patients are put to sleep. It is usually possible for a parent to stay with a child until they are asleep, and may well be possible with older people with Down's Syndrome if you ask.
Once in the room an anaesthetic assistant will attach leads to monitor heart rate and oxygen level during the operation - these just stick to the skin and do not hurt. The anaesthetist will insert a cannula (drip needle) through the skin into a vein in the hand or arm, through which the anaesthetic and any other medication can be given.
The patient may be asked to breathe some oxygen from a mask whilst the anaesthesia is injected into the cannula making them go quickly to sleep. Alternatively they may be asked to breathe anaesthetic gas through a mask which will take a couple of minutes for them to drift off. Once they are asleep anyone who went with them to theatre will be asked to leave.
Finally before going into the theatre, the anaesthetist will insert a plastic tube through the mouth into their windpipe to help them breathe during the operation. Once asleep the anaesthetist may also insert a central line and arterial line which are thin tubes or cannulae, often inserted through the groin or side of the neck, into a vein or artery. They allow monitoring of the blood pressure, and can also be used for giving medicines or fluids, and taking blood samples. The patient will be kept asleep with anaesthetic gases and will also be given painkillers before they wake up.
In the operating theatre
During the operation the anaesthetist will closely observe the patient's heart and lung functions and other aspects of their general care, as well as controlling the anaesthetic. At the end of the operation the anaesthetist allows them to wake up gently, by which time they are usually in the recovery area, or stays with them until they get to Intensive Care if they will remain sedated as after open heart surgery.
After the operation
After the operation patients will wake up in the recovery room of the theatre suite for minor operations, or may be taken asleep to the Intensive Care Unit after major surgery (see Intensive Care Unit Topic Note). There will be a nurse looking after them and monitoring them while in the recovery room, until they are fully awake and safe to return to the ward.
The anaesthetist will prescribe painkillers, should they be needed, for when they get back to the ward. They may also be on a drip to provide fluid until they are eating and drinking again.
Things to think about
- Make a list of any questions you have so you don't forget them when you get to the hospital.
- Try to remember any previous operations so that you can tell the anaesthetist of any reaction their may have been.
- Think about any allergies to food, medicines or everyday items to tell the anaesthetist. Ask close relatives about allergies (particularly to anaesthesia) as they can run in families.
- Bring any medicines taken regularly into hospital so that the doctors can see exactly what is being taken.
- If there is a problem or you think there is something that would make it easier for the patient to cope with an operation, do talk to the staff, they may be able to assist if they understand why it would help.
