chronicconditionsCheck Flashcards

Question

What is the role of GP in care of someone with CP?

Answer 1

arrange preventive health interventions (sexual health, cancer screening, immunisation, health promotion) and coordinate the multidisciplinary health team that will be required throughout her life. The pneumococcal vaccine and a yearly influenza vaccine are particularly important considering her respiratory risk factors. The NDIS enables access to therapy, equipment and personal support Madeline requires to participate in education, employment, recreational and social activities.

Answer 2

depression, diabetes, medications, anaemia, thyroid and endocrine disorders, spinal dysfunction and urinary tract infection

Answer 3

1. Lifelong hydrocortisone and fludrocortiosone replacement 2. Education from Endocrine Society of Aus and Addisons Foundation re: crisis managment, perioperative, pregnancy, intercurrent illness management medi-alert bracelet66,67,69 action plan for crisis management (Box 2 baseline bone mineral density assessment60 consideration of dehydroepiandrosterone treatment69 consideration of GP Management Plan and Team Care Arrangements for Chronic Disease Management.

Answer 4

Key aspects of a self-care plan for a patient with adrenal insufficiency:67 increase glucocorticoid dose during intercurrent illness recognise early features of adrenal crisis (eg nausea, vomiting, dehydration, hypotension) carry injectable hydrocortisone when away from medical care wear an alert bracelet or necklace carry a wallet card with details about their condition and treatment.

Answer 5

Screen all first degree relatives

Answer 6

IV fluids and 100 mg hydrocortisone IV, initially, then 50 mg IV, every six hours until stable 000 and ED is most imp

Answer 7

1. Was there any unusual activity, illness or fever in the 24 hours preceding the event? 2. Was there anything that could have provoked the seizure, such as alcohol, drugs or trauma? 3. Was the patient sleep deprived? 4. Is the patient taking any medications that can lower the seizure threshold, such as antipsychotics, antidepressants, antihistamines, analgesics, bowel preparations and drug withdrawals? In addition, it is important to ask about the patient’s past medical history, family history and social history.

Answer 8

It is important to inquire about symptoms present before the seizure (ie aura, which can be **autonomic [rising epigastric sensation], psychic [déjà vu or jamais vu], sensory [metallic taste, coloured spots, tinnitus, tingling] or lightheadedness)**. It is recommended that the patient is asked to describe the seizure. Did he lose **awareness?** Was he aware of sudden irregular jerks involving the trunk or limbs (myoclonus), regular shaking (clonic), stiffening (tonic) or spasms (dystonic)? A witness may describe impaired awareness such as absences (motionless stare, eyelid fluttering or disruption of awareness and activity) and automatisms (lip-smacking, fiddling, rubbing), pseudo-purposeful movements (dressing or undressing, hitting out), vocalisations and impaired communication.75 Any other physiological events that occurred during the seizure should be noted; for example, **tongue biting, cyanosis, lateral gaze, torticollis, urinary incontinence.** It is also essential to ask the patient how they felt after the seizure. Postictal confusion or drowsiness of more than 10 minutes is characterised by disorientation, poor concentration, short-term memory loss and decreased verbal and interactive skills.

Answer 9

**cardiac syncope** – arrhythmias, tachycardia, aortic stenosis, cardiomyopathy **noncardiac syncope** – vasovagal syncope, postural hypotension **migraine** **narcolepsy–cataplexy** **tremors** tics **movement disorders** **psychological and psychiatric** – pseudo seizures, hyperventilation, panic attacks, dissociative reactions **metabolic derangements** – hypoglycaemia, hypoxia, severe acid–base disturbances, febrile illness, hyperthermia, respiratory, liver and renal failure, intoxications and poisonings, drug and alcohol withdrawal **head injury** – acute concussive convulsions: extradural haematoma, subdural haematoma, raised intracranial pressure **cerebral infections** – bacterial meningitis, viral encephalitis **stroke** – embolism or thrombosis, subarachnoid haemorrhage, intracerebral haemorrhage

Answer 10

fasting blood sugar, renal function tests, liver function tests, calcium and magnesium, vitamin B12 and folate. Serum prolactin, *when measured **10–20 minutes** after a suspected seizure, can provide insight into the type of seizure experienced; this could be considered if Sosuke presented soon after another seizure.* A blood alcohol and urine drug screen for opiates and amphetamines can be useful *in selected cases*. **Cardiovascular screening is also recommended,** including an *electrocardiogram, 24-hour Holter monitoring and two-dimensional echocardiography.*79 **Electroencephalography (EEG) is an indispensable investigation in the diagnosis of epilepsy.** If _generalised 3–4 cycle per second discharges are present, the diagnosis is generalised epilepsy._ If the initial EEG is non-diagnostic, a sleep-deprived EEG should be obtained. _A computed tomography (CT) or magnetic resonance imaging (MRI) scan of the brain is important for patients who have had their first seizure episode and is mandatory for all patients with a history of trauma, focal seizure onset, suspected malignancy, fever, meningism or neurological signs._ As a rule, all adult patients who have had their first seizure should be considered for **MRI**

Answer 11

two or more unprovoked or reflex seizures occurring \>24 hours apart diagnosis of an epilepsy syndrome single unprovoked (or reflex) seizure and high risk of recurrence over the next 10 years.

Answer 12

All patients who have had an initial seizure should be referred to a neurologist for a **semi-urgent neurology assessment in 4–6 weeks and advice regarding commencement of anti-epileptic medication.**

Answer 13

Patients should be advised of **first aid measures**, which they can discuss with their families. It is also important to discuss **activities that may affect the patient’s safety such as using heavy machinery, working at heights, being around water, sports, recreation and general safety issues.** Some lifestyle measures will help reduce seizures and give patients a sense of control. It is recommended that a **patient who experiences seizures follows a ketogenic (high fat, low carbohydrate, adequate protein) diet84 and avoids illicit drugs of abuse.** Sosuke should be advised to get **adequate sleep, as lack of sleep is a common trigger for seizures. He should be encouraged to maintain a healthy lifestyle by practising muscle relaxation exercises, breathing techniques, meditation and obtaining regular exercise.** It is important to discuss any psychological symptoms such as **mood and emotions,** as even patients with well-controlled seizures have higher rates of depression than the general population. _Suicide rates_ are _triple that of the general population, especially in the first six months after diagnosi_s. Sosuke should be made aware of the availability of professional support and therapy. Epilepsy management plan

Answer 14

An epilepsy management plan should also be discussed, including emergencies, safety, risks, medications and monitoring. Well-controlled epilepsy has a good prognosis. It is considered resolved if the patient is seizure-free for 10 years, including five years off anti-seizure medications.80

Answer 15

Sudden unexpected death in epilepsy (SUDEP) is a rare complication of epilepsy. It occurs in benign circumstances, usually at night, and is unrelated to seizure duration. Although rare, a discussion about SUDEP, its causes and how to reduce its risk is recommended. If appropriate, this discussion can include the family.

Answer 16

Carbamazepine is a narrow-spectrum hepatic enzyme–inducing medication. Common side effects of carbamazepine are **dizziness, diplopia, blurred vision, ataxia, sedation, rash, weight gain, nausea, hyponatraemia and benign leukopenia.** Rare side effects are **agranulocytosis, aplastic anaemia, Stevens–Johnson syndrome and heart block.** It is recommended that patients taking carbamazepine undergo r**egular monitoring of sodium, liver function tests, full blood examination and electrocardiography**. However, in general, routine monitoring of anti-epileptic medication levels is not required

Answer 17

Long-term enzyme-inducing anti-epileptic medications can result in loss of bone density. Sosuke should receive supplemental vitamin D and calcium and undergo periodic bone-density measurements.

Answer 18

1. FAMILY MEETING A consultation with Sosuke’s close family could include discussing transport, living arrangements, safety and help with paperwork. 2. providing a letter of support to access government-funded financial assistance 3. referred to a disability service provider for retraining and suitable employment.

Answer 19

Significant nausea, abdominal pain and confusion indicate a possible adrenal crisis Hypotension is another early feature of adrenal crisis.

Answer 20

Valproate is not known to cause Stevens–Johnson syndrome.74

Answer 21

SUDEP is rare complication with an annual incidence rate of 1.2 per 1000 people with epilepsy.89 It occurs in benign circumstances, usually at night, and is unrelated to seizure duration.

Answer 22

Patients with seizures have higher rates of depression than the general population. The risk of suicide is tripled in the first six months after diagnosis.

Answer 23

Cerebral palsy can sometimes be diagnosed as early as age 3–6 months in infants at risk (or even shortly after birth if problems are severe), using a combination of clinical evaluation, magnetic resonance imaging (MRI) of brain and validated structured assessment tools such as the General Movements Assessment and Hammersmith Infant Neurological Examination.8,24 The average age of diagnosis of cerebral palsy in Australia is between 12 and 24 months.24 Early diagnosis enables early referral for intervention when brain plasticity is highest.24

Answer 24

People with cerebral palsy have only one thing in common: a movement disorder.12 Movement disorders in people with cerebral palsy can range from mild to severe, and can affect one or both sides of the body. Any number of limbs may be affected.12,21,23 Movement disorders in people with cerebral palsy can include one or more of:21,13,27 spasticity – the most common, affecting about 85% of people with cerebral palsy dyskinesia – also common. Includes dystonia (sustained muscle contractions, often causing twisting, repetitive movements or abnormal postures), athetosis (slow writhing movements of distal limbs) and chorea ataxia – uncommon hypotonia – uncommon. The combination of spasticity and dystonia is common. 21,23 Most people with cerebral palsy can walk; only a minority have severe cerebral palsy and permanently use a wheelchair.8 In high-income countries:8,26 two-thirds of people with cerebral palsy will walk three-quarters will talk approximately half will have normal intelligence (approximately 45% will have intellectual disability).

Answer 25

n addition to movement disorders and associated conditions, the most common medical disorders in people with cerebral palsy are:12 epilepsy (approximately 40%) visual impairment (approximately 15%) hearing impairment (approximately 7%). _Approximately 25% of people with cerebral palsy have communication difficulties._ **The majority of people with cerebral palsy do not have an intellectual disability.** Health professionals should not assume someone who is unable to speak has an intellectual disability. In an Australian population-based cerebral palsy registry cohort, intellectual disability was present in 45% of participants and was associated with non-ambulation, a quadriplegic pattern of motor impairment, and epilepsy.

Answer 26

Interventions supported by clinical trial evidence include Learning Games Curriculum for infants with diplegia, constraint-induced movement therapy for infants with hemiplegia, bimanual therapy for infants with hemiplegia, and Goals–Activity–Motor Enrichment (GAME) for infants with diplegia, hemiplegia or quadriplegia

Answer 27

The goals of epilepsy treatment in people with cerebral palsy are to improve quality of life, reduce the frequency, length and severity of seizures and reduce risk of death due to epilepsy.14 It may not be possible to completely prevent seizures.14

Answer 28

premature birth, low birth weight, multiple birth, maternal infection during pregnancy and birth complications (eg oxygen deprivation during labour or delivery).13

Answer 29

intrauterine infection (eg rubella, cytomegalovirus, toxoplasmosis), vascular events (eg middle cerebral artery occlusion), brain malformations (eg cortical dysplasias), or post-neonatal causes events including hypoxic events (eg near drowning), head trauma and severe brain infection (eg meningitis).

Answer 30

MRI and genetic assessment

Answer 31

**Referral to a clinical geneticist before conception is highly recommended to determine if more genetic factors have been identified since Emma’s last test.** Constantly improving gene sequencing techniques are enabling genetic causes to be identified in a much higher proportion of children with cerebral palsy.7 The risk of cerebral palsy in subsequent pregnancies is increased in a family in which one child has cerebral palsy, after controlling for a history of perinatal injury and risk factors.28 However, the absolute risk is low, particularly when no genetic cause has been identifie

Answer 32

Routinely booking longer appointments for people with cerebral palsy is one strategy to help reduce healthcare disadvantage that they commonly experience due to their complex needs, communication difficulties and psychosocial issues.15,16 It also allows time to educate and support parents/carers.16 Annual comprehensive assessment is recommended for people of any age with cerebral palsy.19 Those with an intellectual disability are eligible each year for an MBS Health Assessment: item 701 (brief), 703 (standard), 705 (long) and 707 (prolonged).3

Answer 33

Oral baclofen is the most common agent used in the management of generalised spasticity. **Intrathecal administration via a pump implanted under the skin is more effective than oral administration**, because **oral baclofen does not readily cross the blood–brain barrier.** It is suitable for a small minority of patients with severe spasticity and/or dystonia unresponsive to oral medication and where the movement disorder is impacting on quality of life. Other medicines used in the management of generalised spasticity include dantrolene and diazepam.27 The role of diazepam is limited because doses sufficient to induce muscle relaxation in spasticity may cause drowsiness.27

Answer 34

Nasogastric feeding or gastrostomy should be considered with cerebral palsy if satisfactory weight gain cannot be achieved, if the length of time taken to feed the person is excessive, or there is evidence of aspiration.11 Nasogastric feeding is a short-term strategy, but Con needs a long-term strategy to maintain a healthy weight throughout his adolescence.

Answer 35

As people with cerebral palsy age, their musculoskeletal function tends to deteriorate. To slow this deterioration, attention must be paid to: Exercise to maintain general fitness, core strength and limb coordination and strength. Both aerobic and strength exercises are important. A physiotherapist can prescribe an exercise program for Stephen to address his needs and can oversee the implementation of this program with the assistance of a personal trainer and/or exercise physiologist. Support for the person’s gait to maximise independent mobility and to minimise falls. Orthotics, shoes, and walking aids need to be reviewed. Stephen may need a motorised chair or scooter for long distances. A physiotherapist can advise and assist with this. Social and occupational engagement. People need friends and meaningful activities in their lives - so they have places to go and people to see. Social isolation can lead to physical deconditioning. An occupational therapist can work with the physiotherapist on this.

chronicconditionsCheck Flashcards

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