Uterine (decidual) Casts

Cite this article as:
Tara George. Uterine (decidual) Casts, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32416

Lucy, 15, arrives in the ED sobbing hysterically clutching a wad of toilet paper. “I thought it was my period… only I had the worst period pains ever. I went to bed with a hot water bottle and it got worse and then… this came out”. She sobs, opening the tissue to show you a fleshy, pale triangular thing. approximately 5cm long.  “What is it? It’s disgusting. Have I got cancer? I’m not pregnant am I?”

Bodily secretions in tissues are rarely a source of delight but are common opening gambits. Vomit, faeces, sputum, vaginal discharge, worms, lice, blood clots and products of conception may be saved up and brought to the doctor to add colour to the history.    They present a challenge as often we don’t want to look. We don’t trust ourselves not to recoil or be disturbed and being presented with a “sample” early on can catch us off guard. It plays havoc with the “history, examination, management plan” structure we like to impose on our consultations.  In presentation terms, though, this is a gem of a presentation. We have an “Idea”, a “Concern” and it won’t be long before we elicit an “Expectation”.  Avoiding the enormous cue as it is thrust into your orbit, whilst tempting, risks dismissing the concerns. This can destroy any fledgling rapport and make the whole encounter even harder.  It is going to be necessary to take a history, but right now we have a distressed teenager, an unidentified object in a tissue and a lot of emotion. It may well be easiest to address this gift up front and just take a look. This is the time address the upset and the fear head on.

The “thing” looks like this:

A uterine or decidual cast occurs when the entire endometrial lining is shed in one piece. They are uncommon but frequently cause distress to the patient and can be extremely painful to pass.  A cast looks almost triangular in shape and if shed whole you can see the contours of the uterine cavity in a sort of fleshy model if you look closely.

Lucy tells you she had a Nexplanon contraceptive implant fitted about 6 weeks ago. She is not currently sexually active.  Her last period started the day before she had her implant fitted. She’s well otherwise with no past medical history. She had some light PV spotting yesterday and this morning but it has been light.  Since she passed the mass her pain has settled completely. Her observations are normal. She is happy to do a pregnancy test which is negative.  She just wants to know what it was, why it happened and if she can go home now.

The vast majority of uterine casts have no identifiable precipitating causes though there are case reports in association with Ectopic Pregnancy and they may be slightly more common in users of Hormonal Contraception though having had a cast is not a contraindication for continued use, nor are recurrent casts likely with continued use. The pain associated with passage of the cast is often severe – remember they are passing a 5cm mass through their cervix.

You reassure Lucy that this is not cancer, that she wasn’t pregnant and that this was a cast.  You explain what a cast is and that it is unlikely to recur.  She goes home much reassured and relieved.

You decide to send the cast to the lab for histology and a few days later a report lands in your in-tray which reads extensively decidualized endometrial tissue with minimal glandular structures lined by low cuboidal epithelium, consistent with a uterine or decidual cast. No chorionic villi were identified.

References

Nunes, R.D. and Pissetti, V.C., 2015. Membranous Dysmenorrhea–Case Report. Obstet Gynecol Cases Rev2, p.042.

Strauss, L., 2018. Fleshy Mass Passed Vaginally by a Young Woman. American family physician98(7), pp.449-450.

Lost Tampons

Cite this article as:
Tara George. Lost Tampons, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32273

Maddy is 15. She presents with a one week history of a brown smelly discharge from the vagina. Her period ended eight days ago. At first, she thought it was just some spotting tailing off but now it’s heavier and smelly. Maddy is a gymnast and swimmer and has used tampons since she started her periods at 13. She has never been sexually active. Shyly, she admits that she “felt up inside” herself and thinks there might be a tampon up there. She’s not sure she removed the last one at the end of her last period, but she’s scared by the discharge and has come to see you for help.

Retained tampons are a common presentation to the emergency department and to GPs. Most GPs will tell you that the first retained tampon case they encounter is a rite of passage into the “real world of GP” and is usually a learning experience.

Here are some top tips for your first time

Classic Presentation

  • May or may not remember having “forgotten” a tampon
  • Foul smelling PV discharge, often watery and brownish
  • Usually well but embarrassed however don’t forget the risk of Toxic Shock Syndrome (TSS) – you will need to check observations/sepsis criteria and if scoring high consider this within your differential

Top Tips for Managing

There are no official published guidelines….

Management consensus from a group of GPs nationally:

  • Firstly encourage her to try and remove it herself by bearing down on the toilet slightly and using her fingers to grasp either the string or the tampon itself.
  • Have a look with a speculum +/- a bimanual exam (preferably in someone else’s room because the smell will linger).
  • Pull it down (with sponge-holding forceps) to where she can reach herself and send her to the toilet to remove and dispose of it.
  • If you must remove yourself have a specimen pot half full of water to put in in and shut the lid immediately.
  • It may be sensible to check that there are no more up there, especially if the patient reports that this has happened before, or if she tells you she habitually uses more than one tampon at a time. This is not advisable or safe, but sadly not uncommon especially in adults with menorrhagia.

In the context of the emergency department and Maddy:

  • Reassurance is key – she is embarrassed. If you are embarrassed too this is only going to end badly.
  • Reassure her that exploring her own body, including her vagina is completely normal.
  • Remind her that the string is sewn through the tampon so it is unlikely to have fallen off. Feeling inside for it and pulling it down is likely to be effective.
  • Encourage her to go to the patient toilet in private and to try to bear down and pull on whatever is up there to get it out. Lots of teenagers are embarrassed and ashamed to have touched their own vulva or vagina. Understanding that this is okay may be all you need to give her the confidence to solve her own problem.
  • If this is unsuccessful and you need to examine her and intervene, make sure you have all the kit you need. In some departments this may mean you have to refer to Gynae for them so it’s worth knowing what they will do.

What you need

  • A chaperone/assistant
  • A room with a lockable door
  • Disposable gloves
  • Speculum – probably a small/”virgin” size for Maddy
  • Lubricating jelly
  • A specimen pot, half full of water
  • Sponge-holding forceps
  • A decent light source

What to do

  • Examine externally first. If the tampon is just inside the vagina you may well see it and be able to easily remove it.
  • Pass the speculum and have a look – if you see the tampon then grasp it with sponge-holding forceps, pull it out and put it straight into a specimen pot with water in and dispose. 
  • If you insert the speculum and cannot see the tampon but can see the cervix clearly it is probably worth pulling back slightly and reinserting to ensure you visualise the posterior fornix too.
  • If she is unable to tolerate opening the speculum blades a gentle bimanual examination may allow you to feel the tampon and grasp it between your fingers to remove it.

Provided she is well and her observations are normal, she does not need antibiotics or any follow up other than reassurance and safety netting. If she is sexually active and/or the discharge is profuse or typical you may wish to consider swabs. If she has symptoms of TSS or Pelvic Inflammatory Disease you need to manage as per these conditions.

Maddy and her mum disappear to the toilet in the department. They return 10 minutes later. Maddy is tearful and says the tampon is definitely there but she’s too scared to pull it down. She says it feels really low down and uncomfortable. You take her to a quiet lockable room with one of the nurses and the kit list above. Explaining carefully what you are going to do you examine her vulva externally and can see the tampon just inside her vagina. You use some forceps to remove it, and having been well-educated by this article you put it straight into a pot of water and shut the lid tightly. You chat about whether there is a possibility there might be another tampon up there and Maddy assures you that this is not possible. You discharge her from the department, relieved, with some safety netting advice about remembering to remove future tampons and to come back if the discharge persists or if she becomes unwell.

Period Problems: Menorrhagia

Cite this article as:
Tara George. Period Problems: Menorrhagia, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32371

Period problems in teens are common. They can cause significant disruption and distress to adolescents and their parents but rarely have a significant or medically worrying underlying cause. In this first of a series of articles I’ll try to provide a logical and systematic approach to assessing and managing period problems in teenagers (recognizing that this may often be able to be extrapolated to adults too for those of you who do not only see children).

Eloise is 14. She attends with her father complaining she is tired all the time.  When she saw her GP last, they arranged some blood tests – a FBC, haematinics, TFTs and coeliac screen.  The notes from the previous consultation are very sparse. It appears that mood (normal) and bowel habit (also normal) were discussed. Eloise’s dad had mentioned she eats a broad range of foods and is not vegetarian or vegan and she eats red meat 2-3 times a week. Her periods were not brought up by the last doctor. One of her aunts has coeliac disease is noted and that is why the GP had organised bloods. Eloise has come in today for her results.

Blood results showing iron deficiency anaemia

Iron deficiency anaemia (IDA) is common in young women. Paediatricians may be much more comfortable assessing dietary intake and encouraging iron supplementation or increasing iron in the diet than they are in talking about periods. 20-30% of all cases of IDA are caused by menorrhagia. Both NICE and the British Society of Gastroenterology advocate a trial of iron for menstruating females with iron deficiency, as long as coeliac disease has been ruled out and there are no red flags for cancer. Prescribing iron and advising Eloise to “eat more steak” isn’t going to address WHY she might have IDA. This could mean that she ends up on long term iron supplements unnecessarily. If she has menorrhagia significant enough to cause anaemia, it is likely to be having an impact on her education and her social life.

Approaching the subject is probably easier than you think, remembering if you are embarrassed the patient may well think there’s something to be embarrassed about”.

So let’s talk about periods….

First a little bit of nomenclature revision.

Menorrhagia – heavy periods

Dysmenorrhoea – painful periods

Oligomenorrhoea – scanty/sparse/irregular periods

Amenorrhoea – absence of periods (primary: failure to attain menarche by the age of 15 with the development of normal secondary sexual characteristics or failure to attain menarche by 13 with no development of secondary sexual characteristics. Secondary: cessation of menstruation for 3-6 months in someone who has previously had regular periods)

Intermenstrual bleeding (sometimes called metrorrhagia) – irregular and unscheduled bleeding including unexpected bleeding between periods

Menarche – the onset of menstruation (the last stage of female puberty)

The symptoms of problematic periods are not always found in isolation. Menorrhagia and dysmenorrhoea are very common and frequently coexist. It is not uncommon for periods to be irregular, painful and heavy especially in the first few months after menarche. In the UK, the average age of menarche is 12.9 years. The average girl will be in Year 8 at secondary school when she starts her periods. Most women will menstruate every 28 days, though irregular and prolonged cycles are common in early menstrual life.

The average period lasts for 2-7 days and on average 80ml of blood will be lost during the period. In developed countries a number of sanitary products are available. The majority of girls are likely to start off with disposable sanitary towels, though environmental concerns mean period pants and washable pads are gaining popularity. Tampons are often the easiest option for girls who do a lot of sport, especially swimming, and can be used from the onset of menstruation. Menstrual cups have a much greater capacity but can be tricky to get the hang of especially for young teenagers.

Absorbency of different products

What to ask in a history of menorrhagia

Start with an open question (recognising that lots of teenagers are much more comfortable with closed questions and giving specific answers): 

Tell me about your periods…

If you need to be more specific:

  • On average, how long do your periods last for?
  • How often do your periods happen?
  • Do you think they are heavy?
  • Does the bleeding change over the course of the period?
  • How often do you have to change your sanitary protection?
  • What sort of sanitary products do you use? (Pads or towels? Tampons? Period pants? Other?)
  • When did you start your periods?
  • Do you leak though your tampons/pads? If so, how often?
  • Do you pass clots? If so, how big are they?
  • How often do you need to change your pads/tampon at night?
  • Do you have to change your sheets/pyjamas?
  • Can you manage your period at school? How often do you need to leave lessons to change your sanitary product? Do you ever stay home from school because the bleeding is too heavy?
  • Are there activities you enjoy that you’ve had to stop doing because of your periods?

Eloise looks embarrassed and keeps looking at her dad. He is staring firmly at the floor looking as if he wishes it would open up and swallow him. You ask her if she would prefer to talk to you without her dad there and she nods. He takes his newspaper to the waiting area and you reassure him you’ll come and find him in a few minutes. 

Eloise tells you she started her periods at 11. They last 5-6 days on average and she has one around every 30-32 days. She uses tampons backed up with period pants as she often leaks. She uses SuperPlus tampons and on the first couple of days needs to change them every 45 minutes or so. This can be very difficult at school. She passes clots the size of grapes for a day or so each month. She has to set an alarm at night to wake her to change her protection every 2 hours but can end up with bloodstained sheets. She has stopped gymnastics and now only swims socially but not competitively. She was dropped from the squad because she wasn’t comfortable training when she had her period – the other girls had laughed when she had leaked during training. Worse still, when at a gala with lots of other teams, blood poured down her leg and she had been jeered by the crowd. She thinks her periods are heavy (heavier than all her friends) but her mum has told her this is normal and to stop making a fuss.

Whilst there is no truly objective “test” for menorrhagia, with this history and the marked iron deficiency anaemia, it is pretty straightforward to assume Eloise has menorrhagia. This is likely to be the cause for her IDA as well as affecting her sport participation, her sleep and her schooling. She had normal thyroid function tests (TFTs) as part of her tiredness workup (though it is worth noting that NICE do not recommend checking TFTs routinely in cases of simple menorrhagia). You might want to ask about other bleeding history like epistaxis, bleeding after dental extraction, family history and to consider testing for von Willebrand’s disease. NICE recommend this is for patients who have had menorrhagia from the start of their menstrual life. Most cases of menorrhagia at this age are, however, idiopathic.

Other factors to consider in your assessment

It is so important that Eloise feels listened to and heard. Her perspective is vital for compliance with any plan you make. You’ve already asked her if she thinks her periods are heavy. Now is a good time to continue to explore her ICE (“ideas, concerns and expectations”) by finding out how worried she is about her periods, whether she thinks they are a problem and if she has any ideas for what might be available to fix the problem.

Family history and past medical history are relevant here too in terms of management options as you might well want to consider the combined pill or tranexamic acid both of which are contraindicated if there is a first degree relative family history of venous thromboembolism or a known prothombotic mutation and the COCP is contraindicated if she has focal migraine. It is important to take into account the thoughts and feelings of Eloise’s parent as well during this assessment but remembering that at aged 14 she is likely to have capacity to make decisions some about her own care and be fully involved in the process.

Management of menorrhagia

The NICE guidelines on heavy menstrual bleeding contain a useful interactive flowchart for managing menorrhagia. The first line according to NICE is a levnorgestrel IUS (e.g. Mirena) but this is not always going to be the best tolerated or most suitable in a young teenager. Pragmatically in teenagers we are much more likely to opt for the second line options of tranexamic acid +/- NSAID or the combined pill.

Tranexamic acid (TXA) may be familiar to people who work in haematology or with major trauma patients as an antifibrinolytic. It is licensed for menorrhagia management to be taken as 1g three times daily for up to four days starting on the first day of the period. There are few contraindications but it cannot be taken if there is a history of VTE and should be used with caution if the patient is on the COCP because both increase thrombotic effect. TXA will reduce menstrual blood loss by up to 50%.

NSAIDs for managing menorrhagia often causes confusion as surely they make people bleed don’t they? It’s worth going back to basic pharmacophysiology and revising how NSAIDs act on prostaglandins.  NSAIDs are cyclo-oxygenase inhibitors and cyclo-oxygenase is the enzyme involved in production of prostaglandins. In menorrhagia most women will have increased levels of prostaglandins which, as you might remember, are powerful vasodilators. The local effect of prostaglandin on endometrial blood vessels causes increased bleeding. By reducing the level of prostaglandins using oral NSAIDs the blood loss volume will be reduced by up to 40%. NSAIDs will also have a significant effect on dysmenorrhoea which will frequently coexist with menorrhagia.

The COCP is frequently prescribed for menorrhagia. It is important to be familiar with the UKMEC guidelines when prescribing the COCP. Whilst the licensed regimen for COCP is to take for 21 days with a seven day break, the RCOG FSRH and most menorrhagia guidelines recommend using extended or tailored regimens. This allows for shorter pill free intervals and reduced numbers of bleeding days. Tailored regimens are associated with less frequent bleeds, and in many cases a reduced number of bleeding days.  Satisfaction with tailored regimens is high. 

Eloise seems delighted that you think her periods might not be something she simply has to “put up with”. As she isn’t sure about her family history you call dad back in and he confirms that he knows of no family history of clotting or bleeding disorders. Eloise has had several dental extractions for orthodontic work and has never bled much after these and has never had epistaxis. Eloise has never had a migraine. Her blood pressure and BMI are normal and after discussions of options you prescribe her the levest COCP using an extended tricyling regimen with a five day break after 63 pills to minimise the number of bleeds she experiences and the volume. You also prescribe oral iron and arrange a repeat haemoglobin and ferritin in 3 months, with follow up consultation in four months time.

Selected references

Heavy menstrual bleeding: assessment and management (2018, updated 2020) NICE guideline NG88

Goddard, A.F., James, M.W., McIntyre, A.S. and Scott, B.B., 2011. Guidelines for the management of iron deficiency anaemia. Gut60(10), pp.1309-1316.

Lethaby A, Augood C, Duckitt K. Nonsteroidal anti-inflammatory drugs for heavy menstrual bleeding. Cochrane Database Syst Rev. 2000;(2):CD000400. 

Nash, Z., Thwaites, A. and Davies, M., 2020. Tailored regimens for combined hormonal contraceptives. BMJ368.

Emergency Contraception for teenagers

Cite this article as:
Tara George. Emergency Contraception for teenagers, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31958

Olivia presents to the children’s emergency department at 3:40 one Monday afternoon still in her school uniform and with her friend Annabel. Annabel does all the talking to start with and tells the receptionist her friend has “a gynae problem”. She is somewhat assertive with the triage nurse arguing that Olivia needs to be seen by a doctor, in a private room and not in a cubicle with curtains.  They disclose that they are both 15 years old.  When you arrive in the room, Olivia tells you she’s come to A&E to get “the morning after pill”.

Requests for emergency contraception (EC) are a common reason for presentating to GPs, sexual health services and the emergency department. As a children’s doctor it can be a bit of a shock to the system to recognise that adolescents are just as likely as adults to need emergency contraception. There is often a learning gap in the paediatric medicine curriculum. In the UK, around 7% of all women of childbearing age have used emergency contraception in the past 12 months. 10-20% of sexually active teenagers acknowledge they do not always use condoms when having sex.  A request for emergency contraception in a teenager signals that contraceptive and sexual health needs are not being met.

What do you need to cover in the history?

  • Why does she think she needs emergency contraception?
  • When did the unprotected sex (UPSI) occur? (i.e. how long ago)
  • Where is she in her cycle? (i.e when was her last menstrual period (LMP)?)
  • Is her cycle regular and predictable?
  • How many other episodes of UPSI have there been this cycle?
  • Who did she have sex with?
  • Was it consensual? Do you need to consider Sexual Assault Referral Centre (SARC) or forensic medicine input? STI risk? Childhood Sexual Exploitation risk.
  • Was he a regular partner? (STI risk especially)
  • Remember she is 15 – the Fraser Guidelines apply here – does she have capacity to consent to sex? And to the treatment she is seeking? The NSPCC have a brilliant summary of Gillick Competence and Fraser Guidelines.
  • What contraception, if any, does she normally use?
  • Does she have a preference for the form of emergency contraception?
  • What is her weight? And BMI?
  • Is she on any regular medication which might affect the efficacy of oral emergency contraception?
  • What plans has she got for ongoing contraception?

Olivia tells you she had sex on Saturday night at around 2am.  She was staying over at her boyfriend’s house  Her boyfriend, Jack, and she have been in a relationship for about a year. He told her this morning that he thinks the condom might have split.  Olivia says she is normally careful to use condoms so feels pretty embarrassed about this situation. You ask a bit more about Jack.  Annabel tells you Jack is her twin brother. They are all in the same year at school and have known each other for years.  Olivia says her LMP was 10 days ago, her periods are really regular – every 28 days.  This is the only time since her LMP that she has had sex.  Jack is her only sexual partner ever and she is pretty sure he is hers too. Her BMI is 19 and she has no past medical history and is on no medication.

Now it’s time for a revision session on menstrual physiology*

*and on the life span of eggs and sperm without a good understanding of which, providing emergency contraception is really confusing

In a 28 day cycle the first day of the period is always referred to as day 1.  Ovulation then occurs around day 14 of a 28/7 cycle.  If the ovum is not fertilised within 12-24 hours, menstruation will occur 14 days later. The first part of the cycle is called the follicular phase and the second part the luteal phase.  The luteal phase is fixed in length in all women, if the cycle length varies the follicular phase may be longer or shorter than 14 days but following ovulation, if the ovum does not meet a sperm and implant, menstruation will always follow 14 days later

Sperm released into the female genital tract can live for upto 120 hours meaning if unprotected sexual intercourse (UPSI) has occurred any time from 6 days before the earliest possible calculated ovulation to 24 hours after ovulation there is a risk of pregnancy. If an ovum is fertilized by a sperm, implantation will occur 5-6 days after ovulation.

There’s a brilliant diagram in the BJFM article linked in the references section which shows dates, phases, risks and times of action really effectively.

These dates and timelines are crucial in establishing pregnancy risk and in supplying emergency contraception.  If ovulation has already occurred, a method which acts by postponing ovulation will be ineffective.  A method which works by preventing implantation cannot be used after the earliest possible implantation because it is unlikely to be effective but more importantly because it could be considered to be providing a termination of pregnancy which is only legal in specific situations.

What are the options for emergency contraception for Olivia?

There are 3 options licensed for emergency contraception in the UK.  The Copper IUCD, oral levonorgestrel (Levonelle) and Ulipristal (Ella-One)

The flowcharts and decision-making algorithms from the RCOG FFPRHC provide a really clear guide to prescribing.

The Copper IUCD is considered the gold ctandard first choice for EC because it:

  • Is the only method which is effective post-ovulation.
  • Is unaffected by body weight.
  • Is unaffected by enzyme-inducing drugs.
  • Provides ongoing contraception if required.
  • Has a failure rate of only 0.09% when used for emergency contraception.
  • Can cover multiple episodes of UPSI provided early in cycle.

Any copper IUCD can be used for emergency contraception though the “gold standard” for ongoing use is a device with a minimum of 375mm of copper, for example the T-safe 380a.  It is probably worth emphasizing here that this is not a Mirena IUS which cannot be used for emergency contraception.  A copper IUCD for emergency contraception can be fitted any time up to 5 days after the earliest possible ovulation in a cycle (so up to day 19 in a 28 day cycle). Fitting a copper IUCD for emergency contraception can cover several episodes of unprotected sex earlier in the cycle provided it is now before day 19 of a 28 day regular cycle. If a patient has had a single episode of unprotected sex after day 19 a copper IUCD can still be fitted.

It is unlikely there is a service within your emergency department for fitting copper IUCDs. This means that if a copper IUCD is the chosen option you are going to have to arrange for Olivia to attend a contraception and sexual health clinic or her GP if they have a coil fitting enhanced service. She’s on day 10 of her cycle so this is possible as she doesn’t “need” it until day 19 but are you sure she will go there? Not having had children is NOT a contraindication to having a copper IUCD fitted but it can be extremely uncomfortable and she may need a cervical block or gas and air to tolerate the procedure.

Oral EC  in the form of Levonorgestrel and Ulipristal both work by delaying ovulation. This is an important piece of information to bear in mind because if your patient has already ovulated oral emergency contraception is not going to work. It is also worth being aware of for those patients who believe life begins at fertilisation and who may be ethically or religiously opposed to preventing implantation via the use of a copper IUCD but who would find the postponement of ovulation (as per other hormonal contraceptive measures also) acceptable.  The flowchart in the FFPRHC guidelines is really useful to consult every time you have a case of this sort.  

Ulipristal (Ella-One) is second-line after a copper IUCD for the majority of situations when emergency contraception is needed. It does have a number of interactions, most importantly with enzyme inducers and progestogens. It is contraindicated in asthma if the patient is taking oral steroids.  It is important to note that if a patient has taken a progestogen within the preceding 5 days ulipristal will not work. It is not suitable in the case of “missed pills” requiring emergency contraception and it is not possible to “quickstart” POP/COCP/depo progestogen/Nexplanon if you give ulipristal.

Levnorgestrel is the only emergency contraception available both over-the-counter from pharmacies and on prescription in the UK.  It should really only be used if the risk of pregnancy is low and copper IUCD and Ella-One are both not suitable/not available. It is given as a 1.5g stat dose (though this should be doubled to 3g if she weighs over 70kg or has a BMI over 26 and cannot have Ella-One). If levonorgestrel is given the clinician can (and if reviewing the FFPRHC gold standard and NICE CKS advice, should) “quickstart” alternative contraception immediately with condoms until the next period.  The patient should do a pregnancy test three weeks after use if she has not had a normal period.  If your patient is on an enzyme inducer and declines IUCD, levonorgestrel is unlicensed but is the only other option as she cannot use ulipristal.

What else do I need to consider?

Any teenager who has had unprotected sex is at risk of STIs as well as pregnancy. There is no role for doing swabs urgently as they will not pick up STIs contracted as a result of this episode of unprotected sex.  Your patient should be signposted to a Contraception and Sexual Health (CASH) Clinic or her GP surgery for swabs in 3 weeks. You may want to write some free-text on your discharge letter to ensure the surgery are aware of this, and if the department isn’t too busy you might even want to ring her GP surgery and book her in for an appointment with the practice nurse for swabs.

Every teenager presenting needing emergency contraception has unmet ongoing contraceptive needs.  Whilst provision of such contraception may well be outside the scope of your role in the Emergency Department, basic counselling about ongoing contraception with signposting to CASH/GP and some relevant patient information leaflets such as those from www.fpa.org.uk should be within the scope of all clinicians.

If you have any concerns about a young person’s sexual activity and think there is any risk of sexual exploitation, abuse or inability to consent to treatment or to the sexual activity they have disclosed you need to raise this with the safeguarding lead in your department before you let her leave

You supply Olivia with ullipristal and some written counselling information about reliable contraception. You phone her GP surgery and get her booked in for a telephone appointment with a GP to discuss contraception a few days later and an appointment with the practice nurse for triple swabs for an STI screen in three weeks. Olivia rings her mum whilst in A&E and tells her what has happened. Her mum comes to pick her up and is engaged with the suggestion for ongoing follow up and contraception.

References

https://cks.nice.org.uk/topics/contraception-emergency/

https://www.fsrh.org/standards-and-guidance/documents/ceu-clinical-guidance-emergency-contraception-march-2017/

https://www.bjfm.co.uk/emergency-contraception-which-option-when-part-1

https://learning.nspcc.org.uk/child-protection-system/gillick-competence-fraser-guidelines#heading-top

Burack, R., 1999. Teenage sexual behaviour: attitudes towards and declared sexual activity. The British journal of family planning24(4), pp.145-148.

Using your HEADS-ED

Cite this article as:
Sarah Edwards. Using your HEADS-ED, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31842

Paediatric mental health (MH) admissions to emergency departments and hospitals are increasing worldwide, including the UK, USA and Australia1–6. COVID-19 has changed this somewhat, at least in the UK, with a decrease in presentations in the first national lock down7. As the UK and the rest of the world return to the “new normal” in 2021, it is likely that paediatric MH presentations could rise back to pre-covid levels. 

These presentations can be challenging to manage in the emergency department. A variety of tools have been developed to help with history taking and disposition, including the HEEADSSS, HEADS-ED, Risk of Suicide Questionnaire (RSQ) and many others. Here we take look at a recent paper looking at the utility of one of these scoring systems, the HEADS-ED.

Cappelli M, Zemek R, Polihronis C, Thibedeau NR, Kennedy A, Gray C, Jabbour M, Reid S, Cloutier P. The HEADS-ED: Evaluating the Clinical Use of a Brief, Action-Oriented, Pediatric Mental Health Screening Tool. Pediatr Emerg Care. 2020 Jan;36(1):9-15. doi: 10.1097/PEC.0000000000001180. PMID: 28538605

What is HEADS-ED?

The HEADS- ED was developed in 2012 as a tool specifically to obtain a psychosocial history from adolescents in the ED, when it was found found to predict psychiatric consult and admission to inpatient psychiatry with a sensitivity of 82% and a specificity of 87% (area under the receiver operator characteristic curve of 0.82, P < .01). This was a promising finding. 

What was the aim of the paper?

There was a composite primary aim:

  1. To examine the utility and decision validity of the HEADS-ED tool for Paediatric Emergency Department (PED) physicians in guiding consultations to psychiatry and crisis services for patients presenting with mental health concerns.
  2. To examine the concordance between PED physicians and crisis intervention workers (CIWs) in communicating the level of need and action required amongst a sub-sample for the patients. 

Where was the paper set?

This study was conducted in the PED at the Children’s Hospital of Eastern Ontario, Ottawa, a tertiary hospital with 70,000 annual visits a year. 3100 (4.5%) of these are related to mental health concerns. Approximately two-thirds of the MH patients are seen by the PED physicians who either discharge to the community or request a consultation with specialised MH services. Those who don’t need any medical care (other 1/3rd ) are referred directly to the CIWs.

What did they do? 

The HEADS-ED was added to the charts of every child aged 12 to 17 presenting to the ED with a MH concern, completed by the PED physicians. 

Only adolescents who had a complete HEADS-ED were included in the study. If they were younger than 12 or older than 17, or if their presentation was not with a MH concern, then they were excluded from the study.

What did they find? 

There were 2704 mental health presentations during the study period. After various exclusions for wrong age or incomplete or no HEADS-ED assessment, 639 adolescents were included in the study. Of those 140 (22%) were seen by the CIWs.

How good is HEADS-ED at predicting consultation or admission?

The study team looked at how well the screening tool corresponded to consultation for full psychiatric assessment and subsequent admission to inpatient care. 254 (39.7%) children and young people required a consultation by the CIW or psychiatrist. 96 (15.3%) were admitted. 

Chi-squared was applied to each of the seven HEADS-ED items to examine whether the scores correlated to request for consultation and subsequent admission.

  • Inpatient admission was highly associated with higher scores in education, activities and suicidality
  • Consultations with CIW or psychiatry was associated with higher mean HEADS-ED score (mean 6.91)
  • Those who did not need a consultation had a mean score 4.70 (n=254)
  • Those who were discharged had a lower mean score than those admitted (5.28 vs 7.21).

As the HEADS-ED score increased, the likelihood of admission did also. 

How reliable is the score at predicting admission?

  • A HEADS-ED score of 8 or more and a suicidality score of 2 led to 164% more requests for consults from the PED team (relative risk, 2.64; confidence interval, 2.28–3.06) 

How well did the PED and CIW scores correlated?

140 patients had the HEADS-ED completed by both PED physicians and CIWs. The PED physicians rated patients higher on all HEADS-ED items and composite scores compared with CIW; however, not all were statistically significant. 

Agreement on ratings ranged from 61.7% to 92.9% with the highest agreement being suicidality and lowest agreement being activities and peers.

Bottom line – Should I change my clinical practice after reading this paper?

Maybe.

The HEADS-ED can be useful in helping take a psychosocial history in adolescents in the PED.

This may help confer concern when referring to the MH team. It cannot currently be used as a risk assessment as this was a single centre site, in Canada. More work is needed to understand its external validity.  

Final words from Andy Tagg

Patients with mental health concerns are increasing in numbers. Rather than skip over them for something easy it is important that we all get comfortable with asking uncomfortable questions. One of the challenges of formal tools is that they rend to direct the conversation and turn it into a tickbox exercise rather than a free-flowinng conversation. Clinicians need to be able to jump from topic to topic as they develop rapport with the child or young person in front of them.

With four times as many exclusions as inclusions I wonder how well the clinicians did if they did not use the tool. My first instinct would be that clinical gestalt, in experienced clinicians, would be as useful, if not better than the HEADS-ED tool. Where I see the potential value is for those healthcare workers with less experience, that might need a little guidance along the way.

References

1. Lo CB, Bridge JA, Bridge JA, et al. Children’s mental health emergency department visits: 2007-2016. Pediatrics [Internet] 2020;145(6). Available from: https://doi.org/10.1542/peds.2019-1536

2. Irteja Islam M, Khanam R, Kabir E. The use of mental health services by Australian adolescents with mental disorders and suicidality: Findings from a nationwide cross-sectional survey. PLoS One [Internet] 2020 [cited 2021 Jan 3];15(4). Available from: https://doi.org/10.1371/journal.pone.0231180

3. Lawrence D, Johnson S, Hafekost J, et al. The mental health of children and adolescents: Report on the second Australian child and adolescent survey of mental health and wellbeing [Internet]. Austrialian Government; 2015 [cited 2021 Jan 3]. Available from: https://www1.health.gov.au/internet/main/publishing.nsf/content/9DA8CA21306FE6EDCA257E2700016945/$File/child2.pdf

4. Tolentino A, Symington L, Jordan F, Kinnear F, Jarvis M. Mental health presentations to a paediatric emergency department. Emerg Med Australas [Internet] 2020 [cited 2021 Jan 3];1742-6723.13669. Available from: https://onlinelibrary.wiley.com/doi/10.1111/1742-6723.13669

5. Williamson A, Skinner A, Falster K, Clapham K, Eades SJ, Banks E. Mental health-related emergency department presentations and hospital admissions in a cohort of urban Aboriginal children and adolescents in New South Wales, Australia: findings from SEARCH. BMJ Open [Internet] 2018 [cited 2021 Jan 3];8:23544. Available from: http://bmjopen.bmj.com/

6. UKParliment. Written questions and answers – Written questions, answers and statements – UK Parliament [Internet]. UIN 181292. 2018 [cited 2021 Jan 3];Available from: https://questions-statements.parliament.uk/written-questions/detail/2018-10-18/181292

7. Ougrin D. Debate: Emergency mental health presentations of young people during the COVID-19 lockdown. Child Adolesc Ment Health [Internet] 2020;25(3):171–2. Available from: https://doi.org/10.1111/camh.12411

8. Cappelli M, Gray C, Zemek R, et al. The HEADS-ED: a rapid mental health screening tool for pediatric patients in the  emergency department. Pediatrics 2012;130(2):e321-7. 

Other useful resources 

Andrew Tagg. Mental Health Screening, Don’t Forget the Bubbles, 2019. Available at:

https://doi.org/10.31440/DFTB.21114

Henry Goldstein. Adolescent Inpatient Psychiatry, Don’t Forget the Bubbles, 2017. Available at: https://doi.org/10.31440/DFTB.11391 

Ester Sabel. The ABC of Self-Harm in Young People – A Psychiatric Approach to Resuscitation. 2019. https://www.rcemlearning.co.uk/foamed/the-abc-of-self-harm-in-young-people-a-psychiatric-approach-to-resuscitation/  

Period management in young people with disabilities

Cite this article as:
Tara George. Period management in young people with disabilities, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31396

Ellie is 14 years old and she has athetoid cerebral palsy. She has limited use of her hands and is in a wheelchair. She started her periods 3 months ago and she finds them very distressing. She comes to see you with her mum to ask about options to help. Mum asks if “ she should go on the pill to help” because that’s what mum was given 30 years ago for her own menstrual problems.


Why might we be thinking about this topic?

Menstrual disorders are common (10% of all teens will need treatment for heavy menstrual bleeding for example). Dysmenorrhoea is very common in all young women and can be extremely disabling or frightening especially if an adolescent does not have the language skills to articulate the issue or the receptive understanding to have it explained to them. Periods and especially period products may be extremely difficult to manage for a young person with a physical or mental disability


Triple incontinence” (bowel/bladder/menstrual blood) can have serious consequences for skin integrity. There are a number of strategies to help but gynaecology is often not part of the paediatric curriculum. Here at DFTB we are going to try and fix this.


Sexuality and sexual behaviour are not the exclusive privileges of the neurotypical but, unfortunately, the risk of abuse, coercion, sexually transmitted infections (STIs) and pregnancy is higher in people with physical or learning disabilities.



What do we need to know from Ellie and her mum?

A clear menstrual history is key here but most important is focusing on their ideas, concerns and expectations. We’ve already heard from the mother that she thinks “the pill” could be the solution but we need to know more about what is happening, what is bothering them and what they think might help.

  • Is the issue simply periods per se and the manual dexterity challenges of managing pads/tampons independently?
  • Does she have heavy bleeding?
  • Are her periods significantly painful?
  • Are there concerns about relationships and sexual activity?

Remember Ellie is a teenager. She’s likely to have crushes, to fancy boys (or girls) and to be exploring her own sexual identity. This can be really hard to come to terms with as a parent of any child, but as the parent of a child with a disability, this can be particularly frightening. There is a huge taboo surrounding the idea of disabled people having sex (more to come in a future article).


Ellie tells you she has had three periods so far. She bleeds for 4-5 days each time and gets a bit of lower abdominal cramping. This settles with paracetamol or ibuprofen. She needs her sanitary pads changing every 3-4 hours and this is really upsetting as her hands won’t let her peel the sticky strips from the back of the pad and she has to get someone else to do it for her. She’s in a mainstream secondary school with some writing and mobility support but her 1:1 teaching assistant can’t help her with personal care so on period weeks her mum has to come into school 2-3 times in the day to change her pads. Ellie finds this humiliating as she is really independent with everything normally. Ellie knows about sex and relationships and has a bit of a thing for a boy in her French class. She’s open and aware of pregnancy and STIs having been to lots of PHSE lessons recently and does not want, or need, contraception currently.



What options are there for helping Ellie with her periods?


The key thing here is not to over medicalise the situation but to recognise that being a menstruating adolescent with physical disabilities can be challenging.

Consider period pants

Liaise directly with school nurses

Medication based approach

Progestogen only pill

  • Shouldn’t be used as a first line agent as erratic bleeding is common
  • It needs to be taken daily
  • 50% of young women will have amenorrhoea by six months with desogestrel

Combined Pill

  • Commonly used, such as the 150/30mcg Levest
  • Prescribers need to consider relative contraindications such as immobility, BMI and enzyme-inducing medication and absolute contraindications such as a history of migraines or family or personal history of venous thrombo-embolism
  • Targeted regimes (bi- or tri-cycling) may be used to induce the longest period of amenorrhoea
  • Loestrin can be crushed and flushed down a gastrostomy tube if swallowing is a challenge

Transdermal contraceptives

  • E.g. Evra
  • They have the same relative and absolute contra-indications as the combine oral contraceptive pill
  • Weekly application is easier than daily tablets
  • A sequentially tailored regime can be used to manage bleeding

Depo progestogen

  • 70% will have amenorrhoea by 12 months
  • This is the most commonly used method
  • Sayana-Press may be able to given by the carer – it is a subcut injection, that is less painful and easier for the teen.
  • Known side effects include weight gain, which may further impede mobility or challenge carer
  • If the teen is of lower BMI, and relatively immobile, it may increase the risk of osteoporosis necessitating the use of supplemental medication

Intra Uterine Devices (IUDs)

  • E.g the Mirena. It works by slowly releasing progestogen into the uterus
  • Licensed for 5 years
  • No drug interactions
  • Amenorrhoea common and dysmenorrhoea tends to improve
  • The uterine cavity needs to be at least 6cm
  • Needs a degree of mobility (and consent) to fit in a routine clinic
  • It may be more appropriate to fit under GA

Contraceptive implant

  • E.g. Nexplanon (etonogestrel)
  • Only a 20% amenorrhoea rate and erratic bleeding is common, though it is usually lighter and less painful
  • The 3-year license means better concordance
  • There is a risk of the teen picking at the implant site, so it may be better to implant in the dominant arm, by the triceps
  • Removal can be tricky so current guidelines suggest putting in a second implant and just leaving the old one in place



Ellie and her mum decided to try some period pants and to speak to the school nurse about changing facilities at school. After another two months she’s still struggling on days 1 and 2 with changing her pants but can manage a whole school day in one pair of “nighttime” pants on days 3-5. She opts to try the combined pill with a tailored regimen allowing day 1-2 of her bleed to be scheduled for weekend days.

Selected references

Dickson J, et al. Contraception for adolescents with disabilities: taking control of periods, cycles and conditions. BMJ Sex Reprod Health 2018;44:7-13

PEM Adventures Chapter 2

Cite this article as:
Team PEM Adventures. PEM Adventures Chapter 2, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.30926

Are you ready for another PEM adventure? This time the stakes are a little higher. Join us on another journey (with an inbuilt time travel machine) as we manage Grace…

Teenager holding mobile phone

Meet Grace. Grace is a 15-year-old vegetarian environmental activist. She’s thrilled because she’s recently hit a TikTok following of 10,000 – social media is SO the way to spread the word.

She spent yesterday at an illegal climate strike rally outside parliament. Buoyed up on the adrenaline of a thrilling protest, she and some buddies went back to her friend, Zak’s house where they celebrated in style with vodka pops. But this morning, horrified by the fact Grace was impossible to wake, Zak called the emergency services.

Meanwhile, you’ve just fished a pea out of a child’s ear when the red phone rings. Hearing the pre-alert, you mobilise the team and prep a bay in resus. Minutes later, Grace is wheeled in with Zak in tow and she’s transferred to a trolley.

Whiteboard containing vital signs

Your SHO, Lucy, does a primary survey:

  • A: Tolerating an oropharyngeal airway. No stridor or stertor.
  • B: Self-ventilating in 15L O2 via a non-rebreathe mask. Respiratory rate is a bit raised but her chest is clear and she doesn’t have any other signs of respiratory distress.
  • C: Warm and well perfused, heart rate 68 with normal heart sounds and normal pulse volume. Blood pressure is 115/70 and capillary refill time is less than 2 seconds.
  • D: GCS 7, made up of M4, V2, E1. Pupils are size 3 bilaterally and normally reactive to light. Tone is generally low but reflexes are normal and plantars are down going.
  • E: No rashes, no bruises and Grace is currently afebrile.

Lucy gets Grace’s mum’s number from Zak and phones her to get a bit more information. Grace is a healthy adolescent with no significant past medical history. She’s not on any medications, is not allergic to anything and is fully vaccinated. She’s been completely well with no fever, cough, coryza, or any other symptoms. She did have a cold sore a few months ago – could that be relevant?

Grace’s parents, who had gone away for the first time since covid-lockdown lifted, are running to the train station to make their way back home.

Back in resus, you put in a cannula, and send off some bloods: FBC, U&E, LFT, CRP, blood culture and an alcohol level.

Her venous gas shows a pH of 7.47, pCO2 of 2.7, bicarb 14, lactate of 2.7 and normal glucose.

Blood gases showing respiratory alkalosis

That’s odd, you think to yourself, a respiratory alkalosis with some metabolic compensation. You pause for a second and work through your list of possible causes.

  1. Could this be a central cause of hyperventilation? A bleed? A tumour? A meningoencephalitis? You put up a request for CT brain. 
  2. Could this be a respiratory cause? Asthma? Pneumonia? Pneumothorax? Better get a chest x-ray too.
  3. Could this be sepsis? You prescribe ceftriaxone and add acyclovir. There was that coldsore after all…
  4. Pregnancy?
  5. Endocrine or hypermetabolic cause? Maybe DKA? No… her blood sugar’s normal. Or thyrotoxicosis?
  6. Maybe it’s something toxicological? You remember, from your undergraduate days, learning that salicylates cause a respiratory alkalosis.

You add a salicylate level, and paracetamol for good measure, add thyroid function and ask for a catheter urine for beta HCG and a tox screen.  

But her catheter urine doesn’t give you any extra clues. Grace’s urine beta-HCG is negative, her tox screen is negative and her dip is negative.

The resus nurse gently touches your elbow and quietly says, “Do you want to call the anaesthetist?

Good question, you think to yourself. Her GCS is 7 and she’s tolerating the oropharyngeal airway, but she’s breathing well for herself at the moment. What do you want to do?

There are some compelling arguments not to intubate; Grace is maintaining her airway and she’s obtunded and may have seizures – if you give her a paralysing agent as part of her RSI you’ll never be able to tell. Sure, if you really want to monitor for seizure activity, AND you’re in a a tertiary centre with a PICU with capability of CFM or EEG monitoring, you could keep arguing you can monitor for seizure activity while she’s intubated and ventilated, but it takes a while to set up, and time is of the essence.

So you make the brave decision not to intubate. 

You later decide it was less brave and more foolhardy. While Grace is in CT she drops her GCS further and then has a respiratory arrest, which quickly deteriorates into cardiac arrest. The scanner is a terrible place for CPR. While you’re trying to run an arrest on a narrow CT bed you wish you could go back in time and make that choice again. Luckily for you, the inbuilt PEM adventures time travel machine can do just that. In you hop and whizz back to resus.

Close the toggle and this time click on the ‘intubate’ choice.

There are some compelling arguments not to intubate; Grace is maintaining her airway and she’s obtunded and may have seizures – if you give her a paralysing agent as part of her RSI you’ll never be able to tell if she’s seizing. 

But there’s something niggling you… Grace is heading for a CT scan and the LAST thing you need is for her to arrest in the scanner.

And yes, it’s true, there is a risk you could miss a seizure if she was paralysed, but you can give her a long-lasting anticonvulsant to prevent seizures. 

So… you decide to follow your gut and make the decision to intubate.

Thankfully the anaesthetist is nifty with a tube and she’s already drawn up the RSI drugs – fentanyl, ketamine and rocuronium in a 1:1:1 ratio (that’s fentanyl 1mcg/kg, ketamine 1mg/kg and rocuronium 1mg/kg). She’s intubated without difficulty. 

Grace has bilateral equal breath sounds and a mobile chest x-ray shows the tube to be in a good position, with clear lung fields and normal heart size. You mentally cross respiratory causes of an alkalosis off your list.

You’re doing great.

The anaesthetist asks you, “How should I ventilate Grace? Should I match her raised respiratory rate?

That’s a good question, you think to yourself. What should you do?

This is a very good question and you’re not sure you know the answer. Grace is hyperventilating for some reason, and maybe mimicking this is the right thing to do…

But, you’re worried about her ultra low pCO2. At 2.7 it’s likely to be causing cerebral vasoconstriction and hypoperfusion. It’s time to start some simple, proactive neuroprotective measures.

On reflection, you decide it would be better to slow Grace’s breathing so resolutely you turn back to the anaesthetist and ask him to SLOW Grace’s respiratory rate to keep her end tidal CO2 tightly between 4.5 and 5; you want to prevent secondary brain injury.

He nods his assent, while tilting the head of the bed up to 30 degrees.

But, remembering a great DFTB post by Costas Kanaris, you know you can do more than that to neuroprotect. As well as maintaining normocapnia and nursing her at 30 degrees head in line, Grace needs strict normothermia and hypoxia should be avoided at all costs. She needs vigilant glucose monitoring, tight circulatory monitoring and support and an anticonvulsant to prevent seizures. 

Close the toggle and move on to the next part of the story.

You think this through. The alkalotic pH doesn’t matter quite so much, what’s really troubling you is Grace’s pCO2. With a pCO2 of 2.7, there’ll be huge amount of cerebral vasoconstriction and hypoperfusion. It’s time to start some simple, proactive neuroprotective measures.

Resolutely you turn back to the anaesthetist and ask him slow Grace’s respiratory rate to keep her end tidal CO2 tightly between 4.5 and 5; you want to prevent secondary brain injury and so now’s the time to start some neuroprotection.

He nods his assent, while tilting the head of the bed up to 30 degrees.

But, remembering a great DFTB post by Costas Kanaris, you know you can do more than that to neuroprotect. As well as maintaining normocapnia and nursing her at 30 degrees head in line, Grace needs strict normothermia and hypoxia should be avoided at all costs. She needs vigilant glucose monitoring, tight circulatory monitoring and support and an anticonvulsant to prevent seizures. 

Great choice! Close the toggle and move on to the next part of the story.

With fortuitous timing, CT ring down to say they’re ready for Grace.

Satisfied that A, B and C are all stable, you turn to take the brake off the trolley when Lucy, your SHO, asks, “But do we only want a plain non-contrast CT?

That’s a good question, you think to yourself. Is that all I want? What neuroimaging will you choose?

“Yes”, you say to Lucy. “A non-con CT is quick and will show us most tumours and bleeds. She can have an MRI later to get a bit more detail.” 

But,” your SHO counters, “a non-con CT won’t always detect an ischaemic stroke. Perhaps we should ask for a CTA too?

You remember a case from a few weeks ago, a little boy called Tomas. You’d bookmarked the RCPCH Stroke in Childhood guideline on your phone. You quickly bring it up and Lucy’s right, the guideline says to consider stroke in children with focal neurology, speech disturbance, focal seizures, severe headache, cerebellar signs… and unexplained decreased conscious level.

Smiling gratefully at Lucy you pick up the phone and ask the radiologist if you can add a CTA. They say yes.

Minutes later, Grace has her CT with CTA… but it’s normal. No abscess… no tumour… no bleed… and no stroke.

Well that’s good news for Grace, you think to yourself, but it doesn’t give you any much-needed clues.

Great work. Close the toggle and move onto the next part of the story.

You know what”, you say to your SHO, “let’s ask for a contrast-enhanced CT. It’s still quick and will give us a little more detail than a non-con CT.

But,” she counters, “do you think we should be considering stroke in our differential? Perhaps we should ask for a CTA too?

You remember a case from a few weeks ago, a little boy called Tomas. You’d bookmarked the RCPCH Stroke in Childhood guideline on your phone. You quickly bring it up and Lucy’s right, the guideline says to consider stroke in children with focal neurology, speech disturbance, focal seizures, severe headache, cerebellar signs… and unexplained decreased conscious level.

Smiling gratefully at Lucy you pick up the phone and ask the radiologist if you can add a CTA. They say yes.

Minutes later, Grace has her CT with CTA… but it’s normal. No abscess… no tumour… no bleed… and no stroke.

Well that’s good news for Grace, you think to yourself, but it doesn’t give you any much-needed clues.

Great work. Close the toggle and move onto the next part of the story.

You know what”, you say to your SHO, “let’s ask for a CT plus CTA. The CT will show us most tumours and bleeds and she can have an MRI later for a bit more detail, but we should consider stroke in our differential, and to detect that we need to add angiography to our CT.

You think back to a case from a few weeks ago, a little boy called Tomas. You’d read the RCPCH Stroke in Childhood guideline and remember that it says to consider stroke in children with focal neurology, speech disturbance, focal seizures, severe headache, cerebellar signs… and unexplained decreased conscious level.

Smiling gratefully at Lucy you pick up the phone and ask the radiologist if you can add a CTA. They say yes.

Minutes later, Grace has her CT with CTA… but it’s normal. No abscess… no tumour… no bleed… and no stroke.

Well that’s good news for Grace, you think to yourself, but it doesn’t give you any much-needed clues.

Great work. Close the toggle and move onto the next part of the story.

You haven’t ruled out infection. So, when you’re back down in resus, you ask Lucy if she’d like to do the LP.

Really? Is that safe with her low GCS?” she questions. 

What do you think? Should you LP?

It’s fine,” you reply, “she doesn’t have physiological signs of raised ICP: she’s not bradycardic or hypertensive, she’s not posturing and she didn’t have focal neurology. Plus, her CT doesn’t look like there’s cerebral oedema.

Feeling reassured, Lucy picks up the spinal needle and performs an LP. 

But it’s not your finest decision. Grace cones and arrests. 

Luckily for you and Grace, there’s an inbuilt time travel function in your PEM adventure and you return back to resus just as your SHO asks if it’s safe to LP Grace.

You have a strange feeling of déjà vu, while a little voice tells you that although a normal CT is usually reliable for ruling out raised intracranial pressure, this isn’t failsafe and it might be safer to defer the LP for when she’s a little more stable. You’ve already started the ceftriaxone and acyclovir, so this time you decide that the LP can wait until she’s a bit more stable and can have an MRI first. 

Thank goodness for that time machine! Close this toggle and move onto the next part of the story.

Lucy’s right. Although a normal CT is usually reliable for ruling out raised ICP, this isn’t failsafe and there’s no rush to get CSF now. You’ve already started ceftriaxone and acyclovir anyway. And when she’s a bit more stable she can have an MRI to check the LP’s safe. The LP can wait for now.

Great teamwork! Close the toggle and continue the next part of the story.

You’re still not sure what’s causing Grace’s low GCS though. Maybe the bloods will help. So you log in to the computer to check Grace’s results.

Results showing a mild transaminitis

Huh, you think to yourself. Grace’s FBC and CRP are normal; it’s sounding less and less like infection.

Her urea is low and her liver enzymes are raised, with a slightly prolonged INR.

Her salicylate and alcohol levels are undetectable. This isn’t feeling so toxicological anymore.

You mull this over with Lucy. Maybe this is a viral picture. There was that cold sore…

Just then Maureen, the ED cleaner, pops her head into the office. “Might this be of any use?” she asks. She’s holding the RCPCH Decreased Conscious level guideline.

You quickly flick through. Bloods… imaging… you’ve done pretty much everything it suggests. But then you take a closer look at the list of bloods it suggests. And there, in black and white, it says ammonia.

Of course!” you say out loud. “That would explain the respiratory alkalosis!

You draw off an ammonia sample, get it on ice and ask Raymond, the dashing porter, to run it down to the lab. You give the lab a ring so they can get the machine primed.

While you’re waiting for the result to come back, Zak comes running over. He’s just been looking in Grace’s backpack for her mobile and found a high protein Diet book. Apparently she’s been trying to lose weight for TikTok. Could it be relevant?

The cogs begin to whir… Hang on a minute… A high protein diet in a vegetarian environmental activist?

The lab phones with Grace’s ammonia level.

It’s over 500! And normal is less than 40.

It all falls into place. Selective vegetarian… Recent protein load… Raised transaminases… High ammonia… This is all beginning to sound a bit metabolic.

But what should you do about that ammonia? As far as you can see, the DeCon guidance only tells you to take it, not what you do when it comes back at over 10 times the upper limit of normal.

Just a sec,” says Lucy scrolling through her mobile phone, “The British Inherited Metabolic Disease Group have got this covered. They’ve produced a whole range of easy access emergency guidelines, including this one, for the management of an undiagnosed hyperammonaemia.”

It says, turn off protein catabolism by giving a 10% dextrose bolus followed by a dextrose infusion to provide an alternative energy source. If her glucose climbs, add insulin but don’t reduce the dextrose – otherwise, she’ll just start breaking down more protein. And, finally, mop up that ammonia with scavengers like phenylbutyrate and sodium benzoate.

The words ‘ammonia scavengers’ remind you of another post you read on Don’t Forget The Bubbles, about the different types of metabolic conditions, how they present and the various treatment strategies. You make a mental note to read it again later to remind yourself of the differences between an amino acid and organic acid.

Meanwhile, you hastily prescribe…

  •       A 2ml/kg bolus of 10% dextrose
  •       a dextrose infusion
  •       And those ammonia scavengers, sodium benzoate and sodium phenylacetate

Grace is subsequently diagnosed as having a urea cycle disorder. You’re amazed to discover that although most diagnoses are made in neonates, diagnoses are sometimes made in adolescents and adults presenting encephalopathic after a big protein load or when catabolic, such as after trauma, childbirth, major surgery, major haemorrhage, critical illness, rapid weight loss or simply after switching to a high protein diet. This is particularly true for ornithine transcarbamylase (OTC) deficiency, which although is X-linked, can present in symptomatic female OTC carriers. Little diagnostic clues include autoselective vegetarianism (that protein makes them feel a bit ‘ugh’) and subtle or behavioural difficulties from chronic low-level hyperammonaemia.

You bookmark a fantastic review article to read later and flick back through your undergraduate biochemistry textbook to remind yourself about urea cycle defects… and hastily close it again when you remember how little you knew even then, at the prime of your undergrad years.

Wow, what a shift. You pack up your stethoscope and head home, reflecting on your day as you walk to the bus stop.

Grace has taught you the importance of…

Reaching for the RCPCH DeCon guideline when looking after a child with an unexplained low GCS.

Not ever forgetting to send an ammonia in an encephalopathic child, young person or even adult; these tricksy urea cycle disorders can present in adulthood. If the ammonia comes back high, BIMDG have a handy guideline telling you exactly what to do.

And, remembering that a normal CT does not ALWAYS rule out raised ICP. In a child with low GCS, put away that LP needle and neuroprotect instead.

But what happened to Grace? Let’s jump in the time travel machine and find out…

Your epic diagnosis of a late presenting metabolic disorder was the talk of the ED. The RCPCH DeCon poster was put up in the ED staff room and from that point onwards, everyone remembered to check an ammonia in a patient presenting with an unexplained low GCS. 

Lucy was nominated as employee of the month. This shift was a pivotal moment in her career as she decided PEM was her vocation.

The ammonia scavengers did the trick and Grace made a full recovery.

Grace focussed her efforts on reducing plastic waste in hospital and successfully petitioned for the introduction of plastic-free PPE, reducing plastic waste during the COVID-19 pandemic by an incredible 275%.

She hit 3 million TikTok followers (and you’re one of them).

This PEM adventure wouldn’t have been possible without some help from some amazing people. Thank you to Roshni Vara, Consultant in Paediatric Inherited Metabolic Disease at the Evelina London, Costas Kanaris, PICU and retrieval consultant at the Royal Manchester Children’s Hospital and Jon Lillie, PICU and retrieval consultant at the Evelina London Children’s Hospital.

Here are some of their wise words of advice…

As Costas says in The N of 1 matters, we’ve outlined our take on Grace’s case and how we’d manage her in our own resus bays. Medicine’s not always so clear cut and there are often different approaches to the same problem, but this is our consensus on minimising risk using, as Costas says, a rational, evidence-based and pharmacologically prudent approach (I love that phrase Costas!)

Should we intubate Grace?

Grace is self-ventilating but the fact that she is tolerating an oropharyngeal airway means some of her airway reflexes have gone. Scanning a child with a GCS of 8 or less, without securing the airway, puts them at risk. If they vomit, they aspirate. If they stop breathing and arrest in the scanner, the CT room is one of the least fun places to run an arrest, perhaps second only to an elevator. Are there any counter-arguments? Yes, and they’re soft.  One is “this patient is encephalopathic/obtunded and may have seizures; if the child starts fitting we won’t be able to tell as they’ll be paralysed”.  Costas says he usually stands his ground and says that if someone is worried about seizures then the child can be given a long-acting antiepileptic. Levetiracetam is his preference, although phenytoin would work just as well unless there’s suspicion of an overdose of an arrhythmogenic agent. The last thing you need is to tip this child into an arrhythmia.

When should a lumbar puncture be performed in a child with a decreased conscious level?

CT is a useful tool for ruling out raised intracranial pressure before proceeding to lumbar puncture. And we’d agree. But Grace has a low GCS and this changes the picture.

If we take a look at the full RCPCH DeCon guideline it dedicates a whole section to answering the question about LP in decreased conscious level. So, let’s start there.

The DeCon guideline advises a lumbar puncture if your differentials are viral encephalitis or tuberculous meningitis and advises that we consider lumbar puncture when our differentials are bacterial meningitis, sepsis, or the cause of the low GCS is not known. This is cloaked with the phrase “when no acute contraindications exist” and this is key. So what are those contraindications?

  • Signs of raised intracranial pressure: dilated pupil(s), abnormal pupil reaction to light, bradycardia, hypertension or abnormal breathing pattern.
  • A GCS equal to or less than 8, or a deteriorating GCS
  • Focal neurology
  • A seizure lasting more than 10 minutes with a GCS less than 13
  • Shock or clinical evidence of meningococcal disease
  • CT or MRI suggesting obstruction of the CSF pathways by blood, pus, tumour or coning.

What’s the evidence? Well, it’s mostly been derived by expert opinion, and there aren’t many people who’d dispute them.

But what about when you have a normal CT? The radiologists can look for midline shift and for signs of impending herniation by assessing the position of the cerebellar tonsils. So, surely that can rule out raised ICP, allowing an LP to be done?

The DeCon guideline quotes a study published in 2000 that showed that in 124 CT scans from 65 children with traumatic brain injury, CT had an excellent sensitivity of detecting raised ICP of 99.1%, with a specificity of 78.1%. But, a 2019 revision to the guideline says that no further evidence about the sensitivity or specificity of CT in detecting raised ICP in children has been found. None. Although the sensitivity in the one quoted study was very high, it was felt that one study, in children with traumatic brain injury, could not be extrapolated to all children with a decreased conscious level. And so the guideline states that a normal CT scan does not exclude raised ICP. If other contraindications are present, don’t use a normal CT to justify LP.

What does this mean in practice? Well, in a child with a GCS of 8 or less, like Grace, there’s no rush to do an LP. It’s unlikely to change your management acutely in the ED. Her infection can be treated empirically and once she’s more stable, and you have more information including, potentially, an MRI, she can then have an LP for PCR.

What neuroimaging should we do?

That’s a good question, answered beautifully by an article by Hayes et al, published in 2018. Although this article focuses on neuroimaging for headaches, it has a great section on when you might choose each type of scan.

We’d all agree that the ideal imaging to look for a brain tumour is an MRI. It gives excellent detail about the brain tissue as well as other intracranial soft tissues and the extra-axial CSF spaces.

But, if you want a quick answer, or your access to MR is difficult, a non-contrast CT can be performed easily from the ED. If there’s no possibility of a later MR, then contrast-enhanced CT might be better as it gives more detail, but it’s more radiation – this is one for discussion with the radiologist.

CT is very sensitive in detecting blood, and it can be done quickly, in an emergent setting from the ED. So, in children with thunderclap headache, when you want to exclude subarachnoid hemorrhage, a non-contrast CT will be your first choice scan. If blood is detected, then add in arterial imaging: CT or MR angiography (CTA or MRA). Contrast is injected and images taken in the arterial phase.

CTA or MRA are also useful in the investigation of suspected stroke. In practice, you need an answer fast, particularly if the child’s within the thrombolysis window and could be a candidate if there’s evidence of ischaemic stroke, so a CTA is a more practical scan. The CT component looks for blood or large areas of parenchymal infarct, while the angiography looks for filling defects in the arteries that could indicate a thrombus.

If you’re looking for intracranial extension of infection, such as from an orbital cellulitis, mastoiditis or a brain abscess, then a contrast-enhanced CT will highlight suppurative collections.

And if you suspect a venous sinus thrombosis, such as in children with coagulopathies, sickle cell disease, infective spread from meningitis / mastoiditis / sinusitis, or secondary to dehydration or renal failure? Then you need to look at the venous spaces. CT or MR venography (CTV or MRV), when contrast is injected and images obtained in the venous phase, will give you the answers you need.

And what ARE the causes of a respiratory alkalosis?

There are a few! Here are the main ones:

  • Central: brain tumours, meningoencephalitis; stroke
  • Respiratory: asthma, pneumonia, pneumothorax, PE
  • Sepsis
  • Pregnancy
  • Endocrine and hypermetabolic cause: DKA, thyrotoxicosis
  • Toxicology: salicylates 
  • Hyperammonemia: liver and metabolic disorders 
infographic of causes of respiratory alklosis

We would LOVE your feedback about these DFTB PEM adventures so if you can spare a minute, please complete our survey at www.tiny.cc/DFTBpemadventure or use your smartphone to let the QR code take you straight there. We timed ourselves completing it and it takes less than a minute. Thank you.

Select references

The management of children and young people with an acute decrease in conscious level. A nationally developed evidence-based guideline for practitioners. RCPCH. 2015 update, with 2019 revisions. Management of children and young people with an acute decrease in conscious level – Clinical guideline | RCPCH

Undiagnosed Hyperammonaemia. Diagnosis and Immediate Management. British Inherited Metabolic Disease Group. Last reviewed 2017. The major causes are as follows (bimdg.org.uk)

Hirsch, W., Beck, R., Behrmann, C. et al. Reliability of cranial CT versus intracerebral pressure measurement for the evaluation of generalised cerebral oedema in children. Pediatric Radiology 30, 439–443 (2000). https://doi.org/10.1007/s002470000255

Expert Panel on Pediatric Imaging:, Hayes LL, Palasis S, Bartel TB, Booth TN, Iyer RS, Jones JY, Kadom N, Milla SS, Myseros JS, Pakalnis A, Partap S, Robertson RL, Ryan ME, Saigal G, Soares BP, Tekes A, Karmazyn BK. ACR Appropriateness Criteria® Headache-Child. J Am Coll Radiol. 2018 May;15(5S):S78-S90. doi: 10.1016/j.jacr.2018.03.017. PMID: 29724429.

Mitani H, Mochizuki T, Otani N, Tanaka H, Ishimatsu S. Ornithine transcarbamylase deficiency that developed at the age of 19 years with acute brain edema. Acute Med Surg. 2016;3(4):419-423. doi:10.1002/ams2.214

Summar ML, Barr F, Dawling S, Smith W, Lee B, Singh RH, Rhead WJ, Sniderman King L, Christman BW. Unmasked adult-onset urea cycle disorders in the critical care setting. Crit Care Clin. 2005 Oct;21(4 Suppl):S1-8. doi: 10.1016/j.ccc.2005.05.002. PMID: 16227111.

Kanaris C, Ghosh A, Partington CG389(P) A case for early ammonia testing in all encephalopathic patients: female patients with x-linked ornithine transcarbamylase deficiency. Archives of Disease in Childhood 2015;100:A158-A159. http://dx.doi.org/10.1136/archdischild-2015-308599.343

Summar, Marshall. (2005). Presentation and management of urea cycle disorders outside the newborn period. Critical Care Clinics. 21. IX-IX. 10.1016/j.jccc.2005.08.004.

Metabolic presentations part 2: children and adolescents

Cite this article as:
Taciane Alegra. Metabolic presentations part 2: children and adolescents, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.28592

Neurological symptoms in a healthy teen

Jane, 14 years old, is brought in by ambulance, unconscious after a 20-minute generalized tonic-clonic seizure at home. She’s wheeled into resus, and while she has a cannula inserted, you take a history from her father. You learn that she has been a healthy child who’s never had a seizure before, with no chronic conditions, no history of drug abuse, no acute illness, and no sick contacts. She’s a vegetarian and enjoys dancing. She’s started a new ‘intermittent fasting diet’ and yesterday hadn’t eaten since brunch. She went to bed early and this morning her mother was woken early by strange sounds coming from Jane’s room and found her seizing on the floor. 

Her primary survey shows that she’s maintaining her airway, is tachypnoeic with oxygen saturations of 98% in air and clear lungs, a normal cardiovascular examination and a GCS of 10, with global hyperreflexia.

This adolescent has an acute onset of neurological symptoms. The differential diagnoses are broad, but her symptoms were precipitated by a new diet that required prolonged fasting. This case is a red flag for a metabolic condition! 

The RCPCH Decreased Consciousness (DeCon) guideline lays out an approach to the child with a decreased conscious level, including differentials, investigations and management (take a look at the DeCon poster and summary guidance). 

You send some bloods and, as suggested by the RCPCH DeCon guideline, you include an ammonia.

Some points to remember

Common things are common: sepsis, CNS infections, intoxication (prescribed and recreational drugs), and primary seizure disorders should all be considered here, but extend your differentials to conditions that can be individually rare but are common as a group: metabolic diseases.

All children presenting with a decreased conscious level, regardless of age, should have an ammonia sent as part of their initial investigation in ED… this could be a case of an undiagnosed urea cycle defect. 

In late onset urea cycle defects, acute metabolic encephalopathy develops following metabolic stress precipitated by a rapid increase in nitrogen load from: 

  • infection
  • trauma
  • rapid weight loss and auto-catabolism
  • increase in protein turnover from steroids
  • surgery and childbirth
  • or other precipitants of protein catabolism.

Adolescents and adults with an undiagnosed urea cycle defect may be completely fit and well, but may have chronic symptoms such as headache, cyclical vomiting, behavioural difficulties, psychiatric symptoms or mild learning difficulties.

They may be selective vegetarians, restricting their protein intake.

Between episodes patients are relatively well. However, acute presentations can be fatal or patients may be left with a neurological deficit.

For more information about cycle urea disorders, check out Metabolic presentations part 1.

The take home

Always send an ammonia in any child presenting with an acute encephalopathy or decreased GCS.

Disorders involving energy metabolism

Next up is Liz, a patient with a diagnosed metabolic disorder.

Liz is a 3-year-old girl from the countryside, who is visiting her grandmother in the city. She has had diarrhoea since yesterday and started vomiting last night. In the last 3 hours, she hasn’t been able to tolerate anything orally. There has been no fever or respiratory symptoms and she is passing urine as normal. Her 5-year-old cousin has similar symptoms. 

Her Grandmother informs you that Liz has MCAD deficiency and her emergency plan was tried at home, without success. Liz is not usually treated at your hospital and you don’t have her chart. Unfortunately, Liz’s grandmother didn’t bring the plan to the hospital. 

Liz looks tired and is mildly dehydrated, but smiles at you. Her heart sounds are normal and her chest is clear. She has increased bowel sounds, a soft abnormal with mild diffuse pain on deep palpation and no masses or organomegaly. She’s afebrile but tachycardic at 165, her capillary  refill time is 3 seconds, and her systolic BP is 104mmHg.

You put in a cannula and measure bedside glucose and ketones. Liz has a hypoketotic hypoglycaemia.

What is MCAD deficiency?

Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is the most common fatty acid oxidation disorder in Caucasians in Northern Europe and the United States. Most children are now diagnosed through newborn screening. In fatty acid oxidation disorders, the body can only partially break down fat.

Let’s recap some basic biochemistry: in prolonged fasting, the body’s normal response is to break down fat to create ketones, as an alternative source of energy. However, children with MCAD deficiency can’t produce large amounts of ketones, so their ketone response is not appropriate to the degree of hypoglycaemia. 

Clinical symptoms in a previously apparently healthy child with MCAD deficiency include hypoketotic hypoglycemia and vomiting that may progress to lethargy, seizures, and coma, triggered by a common illness. Hepatomegaly and liver disease are often present during an acute episode. These children appear well at birth and, if not identified through newborn screening, typically present between 3 and 24 months of age, although presentation even as late as adulthood is possible. The prognosis is excellent once the diagnosis is established and frequent feedings are instituted to avoid any prolonged periods of fasting (Merritt and Chang, 2019).

Children with fatty acid oxidation disorders (medium, long and short chain defects) have typical acylcarnitine patterns. This is one of the reasons acylcarnitines are sent as part of metabolic and hypoglycaemia work-ups.

What is the priority in acute presentations?

Children who have MCADD, like Liz, need extra calories when sick. The most important intervention is to give simple carbohydrates by mouth, such as glucose tablets or sweetened, non-diet beverages, or intravenously if needed to reverse catabolism and sustain anabolism. In Liz’s case, she’s vomiting all oral intake so cannot tolerate oral carbohydrates, so the intravenous route is necessary. 

The key priorities are:

  • Correct hypoglycaemia immediately with 200mg/kg glucose: 2 ml/kg of 10% glucose or 1ml/kg of 20% glucose, over a few minutes. 
  • Treat shock or circulatory compromise with a bolus of 20ml/kg 0.9% sodium chloride. 
  • Give maintenance fluids with potassium once the plasma potassium concentration is known and the child is passing urine. 

Where can you find resources?

The British Inherited Metabolic Disease Group, BIMDG, has specific guidance on their website.

Disorders involving storage of complex molecules

Mike is 12 years old, presenting to the ED with cough and fever. He has been coughing for 10 days, worse progressively in the last 5  and febrile for the last 3 days. He’s been lethargic since yesterday and even when afebrile he looks unwell. His appetite is poor and he has been “sipping some apple juice”. You learn from his mother that he has a condition called Mucopolysaccharidosis (MPS) type I and is receiving treatment with “the enzyme”. Every now and again, “he is chesty and needs to come to hospital”. 

You examine Mike. He’s pink and well hydrated, but looks sick. You notice that he is shorter than an average 12 year old boy, has hand contractures and coarse facial features. 

He has a soft systolic cardiac murmur with good pulse volume. On auscultating his chest you hear creps and rhonchi on the right side. He has mild hepatomegaly and an umbilical hernia. 

His temperature is 37.5ºC, heart rate is132, respiratory rate 30, and oxygen saturations are just 88% in air.

A bit about mucopolysaccharidoses (MPS)

In mucopolysaccharidosis disorders, the body is unable to break down mucopolysaccharide sugar chains. These mucopolysaccharide sugars build up in cells, blood and connective tissue: hence the name, ‘storage disorders’.

In general, most affected people appear healthy at birth and experience a period of normal development, followed by a decline in physical and/or mental function.

As the condition progresses, it may affect appearance; physical abilities; organ function; and, in most cases, cognitive development. 

Most cases are inherited in an autosomal recessive manner, although one specific form (Type II) follows an X-linked pattern of inheritance. 

Specific treatment can be provided via enzyme replacement therapy or haematopoietic stem cell transplantation in the early stages. 

Presently, enzyme replacement therapy is available for MPS I, II and VI and is given as an intravenous infusion either weekly or biweekly, depending on the disease. 

Both enzyme-replacement and haemotopoietic stem cell treatments still have gaps and few clinical trials supporting them. (rarediseases.info; Dornelles et.al, 2014).

What treatment should be started in the ED?

Patients with Mucopolysaccharidosis don’t require any emergency treatment in the ED for their underlying metabolic disease. They are, however, at increased risk of respiratory infections.

Mike is likely to have a community acquired pneumonia and needs to be treated accordingly with oxygen and antibiotics.

References

Adam , HH. Ardinger, RA. Pagon, S. E. Wallis, L. J. H. Bean, K. Stephens, & A. Amemiya (Eds.), GeneReviews® [online book]

Merritt JL,  Chang IJ. Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency.  GeneReviews® [online book], June 2019. Available at  https://www.ncbi.nlm.nih.gov/books/NBK1424/

Genetic and Rare Diseases Information Center (GARD) https://rarediseases.info.nih.gov/diseases/7065/mucopolysaccharidosis 

Dornelles AD et al. Enzyme replacement therapy for Mucopolysaccharidosis Type I among patients followed within the MPS Brazil Network. Genet Mol Biol. 2014

Subtle Signs in Safeguarding: Giles Armstrong at DFTB19

Cite this article as:
Team DFTB. Subtle Signs in Safeguarding: Giles Armstrong at DFTB19, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.22259

Giles Armstrong reminds us that curiosity is needed for all of our young patients and that without it, we cannot truly care for them. We have to be the detectives and be prepared to ask the questions, not just to the routine questions, but to the unspoken ones. Giles presents us with some challenging, but very realistic scenarios, in which it is easy to miss the subtle clues if you don’t look for them.

This talk was recorded live at DFTB19 in London, England. With the theme of  “The Journey” we wanted to consider the journeys our patients and their families go on, both metaphorical and literal.

If you want our podcasts delivered straight to your listening device then subscribe to our iTunes feed or check out the RSS feed. If you are more a fan of the visual medium then subscribe to our YouTube channel. Please embrace the spirit of FOAMed and spread the word.

iTunes Button
 

 

Preserving fertility in oncology patients: Dan Yeomanson at DFTB19

Cite this article as:
Team DFTB. Preserving fertility in oncology patients: Dan Yeomanson at DFTB19, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.22384
iTunes Button
 

 

Dan Yeomanson is a paediatric oncologist dealing with teenagers and young people with cancer. Whilst the future ability to become a parent is not something that immediately springs to mind when a new diagnosis of cancer is made, you can be sure it is on the mind of the parents and the patient in front of you.

Of the ten patients that are diagnosed with cancer on any given day in England, eight will survive and one will be rendered infertile by their treatment.

 

DoodleMedicine sketch by @char_durand

This talk was recorded live at DFTB19 in London, England. With the theme of  “The Journey” we wanted to consider the journeys our patients and their families go on, both metaphorical and literal.

If you want our podcasts delivered straight to your listening device then subscribe to our iTunes feed or check out the RSS feed. If you are more a fan of the visual medium then subscribe to our YouTube channel. Please embrace the spirit of FOAMed and spread the word.

iTunes Button
 

 

Communicating with children with additional needs: Liz Herrieven at DFTB19

Cite this article as:
Team DFTB. Communicating with children with additional needs: Liz Herrieven at DFTB19, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.21387

Communication is vitally important in so much we do as clinicians.  Without good communication we can’t hope to get a decent history, properly examine our patient, explain what we think is going on or ensure appropriate management.

How to be an LGBTQIA+ young persons ally

Cite this article as:
Dani Hall. How to be an LGBTQIA+ young persons ally, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.23101

You know what the rainbow symbol is, right? It’s become synonymous with LGBT+ identity, solidarity and support and from Pride week to day-to-day living you’re likely to spot one fairly regularly. But, you probably also know that LGBT+ people are a minority group within our society, with huge inequalities in provision of healthcare, which is tragic given that this group have huge healthcare needs. As a step towards tackling this, national health services like the NHS and HSE (Ireland’s health service) have incorporated the rainbow with their logos and put them on a pin to show that the wearer is an LGBT+ ally, someone who an LGBT+ person can feel comfortable talking to about issues relating to sexuality or gender identity, someone who will listen to them with respect, someone who’ll create a safe space for discussion.

 

If you’re reading this post, you’re probably an advocate for LGBT+ rights. You might even have an NHS or HSE rainbow badge. Great! But wearing a badge is only one step towards being an ally. The wearer must feel confident that they will treat the young person with respect and that they’ll know what to do if a young person discloses to them. The wearer may be the first person a young person has ever felt confident enough to open up to about how they feel; it may be one of the most important moments of that LGBT+ young person’s life.

So, how can you be an LGBT+ young person’s ally?

You don’t need a rainbow badge (of course you don’t) to be an LGBT+ young person’s ally. But there are a few things that will help you on your way.

First, you need to understand what LGBT+ means.

Let’s start with some definitions.

 

+: inclusive of all identities (queer, questioning, intersex, asexual, pansexual amongst others), regardless of how people define themselves.

There are some key definitions of sexual and gender orientation and expression. Let’s go through them:

Sexual orientation is a description of who we are attracted to romantically or sexually, such as lesbian, gay, bisexual, asexual, heterosexual.

Gender orientation describes an internal sense of being male, female, neither or both, a psychological sense of who we are and who we feel we are.

Transgender: a person’s gender identity is different from the gender they were assigned at birth.

Cisgender: a person’s gender identity matches the gender they were assigned at birth.

Non-binary: a person who doesn’t identify as exclusively male or exclusively female.

Gender expression is a description of how we portray ourselves to the world; how we act, speak, talk and dress. It ranges from feminine, through androgynous, to masculine.

These definitions are explained perfectly by the Genderbread person.

 

By Sam Killerman from itspronouncedmetrosexual.com

 

OK. So you know a bit of the lingo. What can you do to be an LGBT+ young person’s ally?

 

Don’t make assumptions

We live in a heteronormative environment (where being heterosexual is considered the norm) and people often make heteronormative assumptions. This means we may make an unconscious assumption that heterosexual is ‘normal’ without even realizing we’re doing it. The example on HSEland’s LGBT+ Awareness and Inclusion e-learning module is a classic example of this…

Katy is an 8-year-old girl who has been brought to the emergency department by her two mum’s, Jill and Freda. She’s called into triage and, after inviting them to sit down, the triage nurse asks, “Which one of you is Katy’s mum?” Jill replies by saying, “Actually, we’re a family with two mothers.”

The triage nurse made a heteronormative assumption here. A more inclusive approach would have been for the triage nurse to ask Katy to introduce each of the ladies accompanying her. But, until we can shake heteronormative assumptions, it can be easily done; if you do make a heteronormative assumption, apologize and move on. And make an effort not to make a similar mistake next time.

Another assumption that’s often made is about a young person’s sexual identity based on their sexual behaviour.

Suzy is a 15-year-old girl who attends the emergency department with abdominal pain and dysuria. Her urine sample is dipped – no nitrites or leucocytes, but her beta-HCG is positive. She’s pregnant. With this information in hand, you go in and, after some gentle questioning (you’re pretty good at building rapport), you ask her how old her boyfriend is.  She looks at you with disdain and replies, “I don’t have a boyfriend.” Blustering a little, you ask whether she and her boyfriend have broken up. “I’ve never had a boyfriend,” she replies.

Suzy is attracted sexually and romantically to girls and has a girlfriend called Melissa.  And you’ve just lost her trust by assuming she was straight.  Adolescent lesbian and bisexual girls are also at risk of unintended pregnancies and acquiring sexually transmitted infections.

Yet another assumption people make is cis-normativity, the belief, or unconscious assumption, that that it is ‘normal’ to be cis-gendered.  It’s explained all too well by Emily, an 11-year-old transgender girl in the Mermaid’s #IfIHadAVoice video.

 

 

Once you feel you can actively make an effort not to make any assumptions, what else can you do to be an LGBT+ young person ally?

 

Use inclusive language

It can feel artificial to start with, but try and break the heteronormative barrier and ask a young person what their chosen gender or pronouns are.

Samuel is a transgender boy. His assigned gender at birth was female and the name on his birth certificate is Samantha. He has breast buds and looks feminine. You introduce yourself to Sam and his mother, Sandra, but as you’re explaining to Sandra that you’d like to speak to Sam alone, you say, “Would you mind stepping outside the room, while Sam and I speak together first? I’ll call you back in after I’ve examined her.” Sam looks stricken and Sandra gently explains that Sam is a transgender boy and uses the pronouns he/him.

If you accidentally misgender someone, apologize and correct yourself.  We often don’t know what name or pronouns someone would like us to use, and it’s safest to assume nothing and ask (and I mean ask everyone, because you will be caught out if you don’t), “How would you like us to record your details in the medical record?”

What about sexual orientation?  A sensitive way to ask a young person about their sexual identity is to ask if they have a partner or if they’re in a relationship. If they don’t have a partner, ask them if they’re attracted to boys, girls, either or neither.  Let’s think about Suzy again.

You’re about to see Suzy, a 15-year-old girl who attends the emergency department with abdominal pain and a positive urine beta-HCG. You call her into a cubicle and introduce yourself. After taking a history of her presenting complaint and past medical history, you start a HEEADSSS assessment (more on that later). Even though you know Suzy is pregnant, you know it doesn’t mean she’s heterosexual. As you start talking with Suzy about sexuality and gender identity, you ask her, “Are you attracted to boys, girls, neither or either?”

See what you’ve done here? Suzy can now tell you that she’s in a relationship with Melissa without breaking that rapport you’d already established, paving the way for further exploration about her sexual behaviour.

 

Reassure the young person their sexual or gender identity will be kept confidential

Let’s take a step back in time. Suzy may not feel comfortable telling you about her sexual orientation as she might be worried about whether you’ll keep this information confidential. Confidentiality is a huge one. We may feel torn between sharing information about a young person who’s at risk and maintaining confidentiality. Before you start taking a history, explain to a young person that anything you discuss will be kept confidential and private, between the young person and the team looking after them, but if you discuss anything really serious, like suicide or that someone was abusing them, then you’ll come up with a plan together to get the help needed. But, and this is an important but, even if there’s something that you need to seek help for, you’ll keep their sexual or gender identity confidential if this is what they want – this is private to them and you shouldn’t be outing the young person against their wishes.

 

Adapt the HEEADSSS assessment

We mentioned the HEEADSSS assessment.  HEEADSSS is a structured psychosocial history tool.  But when you use it, adapt it.

 

H: Home environment

Up to 40% of young people experiencing homelessness internationally are LGBT+ with figures estimated to be as high as 45% in Canada and 24% in the UK, with similar patterns reported in Ireland. A report by the Albert Kennedy Trust found that more than two-thirds of LGBT homeless young people in the UK have experienced familial rejection, abuse and violence and shockingly almost 1 in 10 16 and 17 year olds have undergone or been offered conversion therapy.

E: Education / Employment

Bullying is rife in schools. 1 in 2 LGBT+ young people in the UK and Ireland experience anti-LGBT bullying at school while 1 in 4 Irish LBGTI young people skip school to avoid anti-LGBTI bullying.

E – Eating disorders

Eating disorders are sadly also much higher in LGBT+ young people. Transgender young people are 4 times more likely to have an eating disorder compared to their cisgender peers. That’s 15% of transgender young people of any sexual orientation reporting an eating disorder (data from a national sample of almost 300,000 young American students). But it’s not just transgender young people at risk of eating disorders: any LGBT+ young person is at higher risk of an eating disorder of any type.

A – Activities

Enquire about activities. LGBT+ young people are less likely to participate in sports  than their heterosexual peers. Enquiring about their leisure time may reveal risk-taking behaviour (we’ll come to that under S for Safety).

D – Drugs and alcohol

Drugs and alcohol are also a problem for LGBT+ young people. Not only are LGBT+ young people more likely to use drugs and alcohol than their heterosexual peers, LGBT+ people under the age of 13 are more likely to have tried alcohol or marijuana than heterosexual young people aged 12 and under.

S – Sexuality

LGBT+ young people are more likely to have had sexual intercourse, have had sexual intercourse before the age of 13 and less likely to use birth control than their heterosexual peers. Birth control, that’s a big one. Remember I said earlier that teen pregnancy occurs in lesbian teenagers just as it does in cis-gendered adolescent girls? The same is true for STIs. I don’t need to say that questions around sexuality and gender identity must be asked sensitively, in a non-judgmental way, without assumption, about sexual identity and gender identity.

S – Suicide, depression and self-harm

It’s widely quoted, but mental health difficulties are much higher in LGBT+ young people, very likely related to a feeling of isolation and non-inclusion and as a result of verbal and physical abuse. More than 50% of Irish LGBTI young people aged 14-18 have self-harmed; 2 in 3 have seriously considered ending their life and tragically 1 in 3 have attempted suicide. The most common age for an Irish LGBT person to attempt to take their life is 15. These are shockingly high. But Irish LGBT+ young people mental health statistics mirror those across the world, in the UK, Australasia and North America.

S – Safety

Being LGBT+ can be lonely. LGBT+ young people are more likely to use dating apps to meet people.  You can just imagine the risk this exposes them to: unsafe sexual encounters, child sexual exploitation, and grooming. Statistics support this. These young people are more likely than their heterosexual peers to be physically or sexually assaulted.  Risky behaviour doesn’t end there. LGBT+ young people are also more likely to undertake another risky behaviour, such as not wearing a seatbelt.  Gently explore risk-taking behaviour.

 

I ask myself, “Why are all these problems seen in young people with an LGBT+ identity?” It’s likely due to minority stress – the stress associated with being treated as a minority group within our society.

 

Be an ally

Knowing the different LGBT+ terms isn’t important. What is important is listening with respect, not making assumptions and creating a safe space for discussion. An ally supports equal rights for LGBT+ people and let’s face it, we’re in healthcare because we want to help people. Healthcare is for everyone.

You may be the first person an LGBT+ young person meets in their acute healthcare. You may be the person they confide in. Their interaction with you may be one of the most important moments of their life

 

 

Where can I find out more?

Watch:

Thom O’Neill’s Be a supr doc for LGBT+ youth, SMACCDub

Aidan Baron’s Crash course in LGBTQI+, DFTB17

Read:

Butler G et al. Assessment and support of children and adolescents with gender dysphoria. Arch Dis Child 2018; 103 (7): 631-636

O’Neill T, Wakefield J. Fifteen-minute consultation in the normal child: Challenges relating to sexuality and gender identity in children and young people. Arch Dis Child Educ Pract Ed 2017; 102: 298–303

Salkind J et al. Safeguarding LGBT+ adolescents. BMJ 2019;364:l245

 

 

Selected references

Charlton BM et al. Teen pregnancy risk factors among young women of diverse sexual orientations. Pediatrics. 2018: 141(4); e20172278

LGBT youth homelessness: a UK national scoping of cause, prevalence, response, and outcome: the Albert Kennedy Trust, 2015

UK Government Equalities Office. National LGBT Survey: Research report. 2018. https://www.gov.uk/government/publications/national-lgbt-survey-summary-report

Higgins et al. The LGBTIreland Report: national study of the mental health and wellbeing of lesbian, gay, bisexual, transgender and intersex people in Ireland. 2016. GLEN and BeLonGTo

Diemer EW et al. Gender identity, sexual orientation, and eating-related pathology in a national sample of college students. J Adolesc Health 2015;57:144–9.doi:10.1016/j.jadohealth.2015.03.003

Kann L et al. Sexual identity, sex of sexual contacts, and health-related behaviors among students in grades 9-12 – United States and selected sites, 2015. MMWR Surveill Summ. 2016;65:1–202

Calzo JP et al. Eating disorders and disordered weight and shape control behaviors in sexual minority populations. Curr Psychiatry Rep. 2017; 19(8): 49

School Report. The experiences of lesbian, gay, bi and trans young people in Britain’s schools in 2017. Stonewall.

Bidell MP. Is there an emotional cost of completing high school? Ecological factors and psychological distress among LGBT homeless youth. Journal of Homosexuality. 2014:61(3);366-381

Abramovich IA. No safe place to go: LGBTQ youth homelessness in Canada: reviewing the literature. Canadian Journal of Family and Youth. 2012:4(1);29-51

https://www.hse.ie/eng/services/list/4/mental-health-services/connecting-for-life/publications/lgbt-ireland-report.html