Sedation

General paediatrics

[NICE guideline]

Ensure that healthcare professional and assistant available and trained, with resus and monitoring equipment available.  Should have a record of practical experience of sedation under supervision, WBAs etc.

Document informed consent.

Confirm time of last food and fluid – fasting not required for minimal sedation eg nitrous oxide, or where child will maintain verbal contact.  Else 2-4-6 fasting rule.

Prepare child and  family psychologically.

For painless procedure eg imaging, use choral (under 15kg), midazolam.  For painful procedures, use nitrous oxide, oral/IN midazolam +/- local anaesthetic.

If unsuitable, ketamine or IV midazolam (+/- fentanyl) – combination of opioid and ketamine requires anaesthetic training.

[http://www.nice.org.uk/guidance/CG112/chapter/1-Guidance]

Antihistamines

Allergy, General paediatrics, Immunology

First generation have prominent CNS effects, usually drowsiness (which an be useful, eg to help sleep in chronic itch) but can be hyperactivity, paradoxically!

  • Ketotifen – age 3+ only. Sugar free 300ml bottle (Zaditen) is only £17. Give with food. 1mg per 5ml, dose is 1mg twice daily from age 3 so 250 (1.25ml)-500 (2.5ml) mcg below that?
  • Promethazine hydrochloride (have to specify HCl as also comes as teoclate) 2+ for allergy but 1/12+ for sedation! Unlicensed for sedation! Phenergan 100ml sugar free £4.
  • Hydroxyzine 6/12 and over, but special order only. Risk of long QT.
  • Alimemazine – BNFc gives dose from 6 months (specialist use only and unlicensed until 2). Solution is minimum £89 a small bottle and often higher.

Second generation have lower incidence of these side effects. BSACI peanut and tree nut guidance specifies “long acting antihistamines with rapid action eg cetirizine”. Resus council anaphylaxis guideline says “non-sedating oral antihistamines in preference to chlorphenamine” may be given following initial stabilisation.

Benadryl (brand name) can be either cetirizine or acrivastine.  Latter marketed as “fastest acting allergy capsule” – I suspect the only allergy capsule.

Tmax (plasma) lowest for Rupatadine (0.75hr), levocetirizine (0.8), cetirizine (1).  Chlorphenamine is 2.8, worse than diphenhydramine (best of the sedating antihistamines at 1.7).  Loratadine (1.2) and Acrivastine (1.4) not far behind; Desloratadine has a range of values from 1-3, which is unhelpful, Fexo is definitely slow at 2.6.

In terms of clinical effect on wheal (because tissue distribution different from plasma), cetirizine, acrivastine, levocetirizine equivalent (1hr), diphenhydramine, fexo, desloratadin 2 hrs, CPM 3hrs (!).  Rupatadine is 2 hrs, despite the short Tmax!

No particular reason to think formulation matters. Possibly liquid might work directly in the mouth and throat, but only if you don’t swallow it immediately (gargle!?).

Some examples of capsules beating liquids for specific drugs (!?) but prob not much difference between absorption from tablet, liquid etc. Prob easier to swallow liquid if oral angioedema, but liquid bolus might actually be harder than tiny tablet…

[J Investig Allergol Clin Immunol 2006; Vol. 16, Supplement 1: 3-12]

Congenital pigmented naevi

Dermatology, General paediatrics

Particularly giant or multiple lesions usually present at birth but can be shortly after.

Number of naevi increases through childhood, but high volatility, eg over 3yr period, about a third change pattern, sun burn increases number. Halo pattern of disappearance  (with depigmentation) rare.

Can be associated with neurocutaneous melanosis; if suspected then neuroimaging is advisable, seeking evidence of leptomeningeal melanotic lesions.

Malignant change in childhood is possible, but only really in lesions greater than 1% BSA size.  Melanoma detection relies on morphology eg ABCDE criteria (asymmetry, border irregularity, color variegation, diameter (6 mm, and evolving).  ABCDE very low specificity in kids but melanoma does present in children (and pigmented naevi are risk factor).  Dermoscopic changes in blood vessels important.

Beckers naevus tends to present at puberty,  usually torso or upper arm, irregular, hairy.

Polymicrogyria

General paediatrics, Neurology

One of the most common malformations of cortical development.  

Bilateral perisylvian polymicrogyria (BPP) is the most common subtype , causes the congenital bilateral perisylvian syndrome:

  • oromotor dysfunction,
  • cognitive impairment,
  • epilepsy.

Many possible causes eg vascular or hypoxaemic insults (eg twin-to-twin transfusion syndrome) and congenital cytomegalovirus infection. Genetic causes also important – some syndromes eg 1p36.3 and 22q11.2 deletion syndromes. PIK3R2  gene defects are the most common genetic cause of BPP in patients with normal head size, and the second (to  PIK3CA ) most common cause of polymicrogyria associated with large head size. RTTN gene  also associated with isolated BPP.

[Lancet Neurology, The, 2015-12-01, Volume 14, Issue 12, Pages 1182-1195]

Spiritual care

Communication, Generic

See also Difficult conversations.

Taking a spiritual history

  • do you consider yourself religious or spiritually minded?
  • where do you get inner strength from?

[Larry Culliford podcast]

[RCPsych leaflet]

All staff play a role in spiritual care.  Definition – “Allow people to explore their innermost feelings and ask the most difficult questions about suffering, illness and death”. Aim to help those in need find peace of mind.

Many levels – speaking with dignity and respect, training in bereavement, specialist spiritual care provided by department of spiritual care and wellbeing.  Spiritual care volunteers also available.

[NHSL spiritual care guidelines]

[Scottish government guidance CEL 2008.]

Staff care also important eg reflective practice, mindfulness, Schwartz rounds etc.

LGBT issues

Ethics

Sexual orientation vs sexual practices vs gender identity.

“Coming out”  means primarily acknowledging your own lesbian, gay, bisexual or transgender identity to yourself.  Coming out to others is not a one-off experience, LGBT people have to make decisions on whether to (or not) disclose, often on a daily basis. This can be an ongoing source of stress and distress.  Heterosexism – assumption of heterosexuality, +/- judgment of its superiority in terms of moral value.

Harassment in the workplace can lead to the organisation and/or the individual being found liable and having to pay compensation.

The umbrella term transgender includes transsexual people and transvestites.

A trans-man is someone who transitions from a female label at birth to a male gender identity.  When the transition is complete, their trans identity could be considered a part of their past medical history, rather than an on-going identity.

Trans vestites (medicalized? “Cross-dressers” better?) have no desire for any permanent transition but enjoy aspects of the opposite gender and may have a temporary identity including a different name.

Trans sexual protected under Equality act by EU gender directive 2007.

It is the impact on the individual that determines whether bullying, harassment or discrimination has occurred, not the intentions of the perpetrator.

Gender recognition act 2004 allows trans sexual people to apply for full legal recognition of their acquired gender (evidence must be provided).  It is also a crime to disclose previous gender without express permission.

Under legislation it is also illegal to discriminate against someone on the basis that they are heterosexual!

[Good LGBT practice in NHS document, Stonewall Scotland]

Dyschezia

Common, Gastro, General paediatrics, OPD

Baby strains and cries to pass stool but it comes out soft or not at all.  Functional gastrointestinal disorder thought to occur in 0.9 – 5% of infants under 6 months (Rome IV criteria).

Due to poor co-ordination of pelvic floor muscles with increased intra-abdominal pressure generated during stooling. Seen in babies up to 9 months.

Studies have reported symptoms of discomfort around passing normal stool in up to 18% of babies, not all of these children will strictly meet the diagnostic criteria for dyschezia.

Differentiating from true constipation etc requires a clinical history and a normal clinical examination, with the key difference being that the stool is not hard in dyschezia.

No medication (or any form of rectal stimulation) required, can be expected to resolve spontaneously.

Urate

Community, General paediatrics, Genetics, Metabolic, Neurology

Product of amino acid metabolism.

In developmental delay, an abnormally high or low result is significant viz:

  • Glycogen Storage disorder
  • Purine disorders eg Lesch Nyhan
  • Molybdenum Cofactor deficiency – other features are microcephaly, hyperekplexia

High urate levels can also be risk factor for urolithiasis (stones in urinary tract)