Life with CAH

At first diagnosis, Congenital Adrenal Hyperplasia can sound daunting. We’ve put together some information and ideas below to help to reassure you and show that, for the vast majority of people, having CAH isn’t life limiting.

Note – any products, services or companies mentioned are not endorsements and the CAH Support Group is not in any way affiliated to them. Other similar products, services or companies may be available. 

Managing illness and CAH (Sick Day Rules)

People with CAH need extra steroids if they are ill. This can mean either an increase in the usual dose of tablets (for illness), or in some circumstances, the use of an emergency injection kit (in case of trauma (e.g. broken bone) or more severe illness).

The Society for Endocrinonology have published Sick Day Rules (available on our Emergency Information page ), but please also refer to your medical specialist for specific advice.

Adrenal crisis

An adrenal crisis can be a life-threatening situation for someone with CAH. Signs of an adrenal crisis include extreme weakness, a significant drop in blood pressure, drowsiness or mental confusion.

It is essential to be vigilant to these signs and act without delay. More information is available on our Emergency Information page.

Emergency injection kits

We always recommend that someone with CAH has access to an emergency injection kit. Some useful information is provided below.

What should be in an injection kit?

As a minimum, an emergency injection kit should contain:

•Emergency vials of hydrocortisone. This can be hydrocortisone sodium succinate (Solu-Cortef,
Act-O-Vial), a powder that requires reconstitution or Hydrocortisone sodium phosphate (a liquid
preparation).
• Water for injection for Solu-cortef injections.
• Syringes (2ml) and needles suitable for intramuscular injection.
• Printed instructions on how to administer injection.
• Vial snaps or other protection for opening glass vials.

Some of our members carry hydrocortisone tablets, emergency contact information, and glucogel (a high sugar gel) incase of hypoglycaemia.

What else to carry in case of emergencies

Anyone who is steroid-dependant should carry a ‘steroid card’, to notify medics that steroid treatment must not be stopped and should be increased in illness or emergency. You can find a downloadable steroid card on our Downloads page.

As well as this, some ideas from our members below:

 Medical ID jewellery (e.g. a bracelet or necklace/dog tag). Medical ID jewellery helps to communicate medical information simply and quickly in an emergency to first responders, paramedics and doctors. Some suppliers include Medic Alert or Universal Medical ID. 

Emergency information – including family and medical contacts, current medication and past medical history (if travelling, translated to different languages). Some smartphones have a Medical ID app which can store details or current medications and medical history.

Daily medicine management

Managing daily medicine doesn’t have to be difficult. Some ideas from members include:

  • Reminder alarms for when to take medication (e.g on a watch, smartwatch or mobile phone).
  • Clearly labelled small plastic boxes or tins (or even camera cases or pencil cases!) to hold medication supplies or emergency injection kits in the car, on a bicycle, or in a bag.
  • Pill boxes for handbags, bags, rucksacks. (Some very nice looking pill boxes are available – they don’t have to be boring or clinical looking!)
  • Some members use small plastic pots or key rings to hold an ’emergency supply’ of medication when doing outdoor activities and hobbies.
  • Medical ID apps on certain smartphones to store your information and list of current medications.
  • Tabtime are a company who offer ‘medication management solutions’ such as alarm watches and pill boxes to help with managing your regular medication.
Vaccinations

The dose of steroids used in CAH are equivalent to the amount produced by the adrenal glands under normal conditions, and hence there is no reason for someone with CAH to be managed differently where vaccinations are concerned. The situation is quite different for people who are receiving larger doses of continuous steroids for different medical reasons.

Always consult your specialist about whether it is necessary to increase steroids prior to vaccination.

Regular tests

The most important measurements of CAH are that the person with CAH is healthy and well. In addition, for children with CAH other measurements are if they are growing along an appropriate percentile and that the bone age is satisfactory.

Monitoring varies from centre to centre and person to person depending on the severity of the CAH, and may include blood tests, urine tests, and for children, growth and bone age checks.

X-rays to monitors a child’s bone age should ideally be conducted on an annual basis after the age of three years. Prior to this there are so few epiphiseal centres to rate and it is difficult to ensure that you are getting the right answer.

Renin Renin is a hormone made by the kidneys and is important in control of salt balance. If salt replacement is inadequate, then renin is high. If salt replacement is adequate renin is low. Therefore, by measuring renin, the correct dose of fludrocortisone can be established.

There are no hard and fast rules about when a renin test should be done. If someone with CAH has normal blood pressure and electrolytes (and in children, if they are growing well), then about once a year is appropriate. If someone with CAH appears to have unsatisfactory metabolic control , then a renin test may need to be done more often.

DEXA (bone density) tests – for some adults with CAH, low bone densitiy due to long-term steroid use can be an issue. If low bone density is suspected, a DEXA scan may be required. 

Blood sugar

One of the responses to acute illness is that the blood sugars drop and cortisol levels go up to combat this. If you measure glucose levels in people with CAH who are drowsy, you often find they are low and that is when an injection of hydrocortisone should be given without delay. Injecting promptly in this situation lessens the severity and shortens the period of illness.

Regular hospital visits

As a child with CAH grows, it’s important that they are monitored to ensure that their treatement is as good as it can be. Typically a child will be assigned to a paedriactic endocrinologist who will check their progress every 6-12 months (this varies from centre to centre, and child to child) and change their medication as required. Read more in our ‘Health and Emergencies’ section’ about the regular tests that can be expected.

Growth, height and weight

Growing & height  – With careful treatment it should be possible for children with CAH to grow at a rate sufficient to achieve an adult height within the normal range. However, even with the very best treatment, it is not always possible to achieve perfect growth in CAH, and some individuals may end up shorter adults than they would otherwise have been. Once someone with CAH has stopped growing and the bones in their body have fused, nothing else can be done to get more growth. 

Weight management – The dose of hydrocortisone required to control the CAH may make it difficult for some children to keep their weight down, and this can be a particular problem for girls at adolescence. It may be helpful to have the input of a dietician and/or to have a full hormone evaluation – you should discuss this with your specialist.

Telling your child about their condition

When asked “When is the right time to tell a child about their condition, and who should tell them?”,  the overwhelming response from our membership was ‘the parents/carers and not the doctor’. The support of an endocrine nurse could be very helpful, especially if the parent/carer finds certain aspects of the condition hard to explain.

Parents/carers invariably find that children develop at different rates and there is no magic age. However, children’s questions should always be answered and it’s important to be open and honest. They’ll only retain what they can understand so it’s likely that you’ll have to tell them more than once and add more information as they get older.

Nursery and school with CAH

The majority of children with CAH attend mainstream education and have fulfilling school lives, taking part in all the normal activities and going away on trips etc. We have made a ‘CAH school letter’ available for download which contains information for schools & carers with guidance on care & emergencies for children with CAH. Find it on our Downloads page.

Bed-wetting and CAH

A lot of children wet the bed and this simply reflects normal delay in bladder maturation. However, in CAH bed wetting should always raise the question of under-replacement with fludrocortisone. In this instance we make quite sure that the fludrocortisone is sufficient (checking that the plasma renin is properly suppressed). If a child with renin suppression on a good dose of fludrocortisone, with no symptoms of salt craving, but is still wetting the bed, then it is safe to conclude that he/she is showing ‘normal’ bed wetting tendency.

Employment & Benefits
  • Career choices – While most careers are perfectly possible with CAH, some may be restricted such as the armed forces. Of course, questions relating to specific careers should be directed to the appropriate professional or representing body.

  • Flexible careers – Some people affected by CAH find that having a flexible employer can help to make life a little easier. Flexa Careers was created in 2019 to help people find truly flexible work, and help forward-thinking companies to stand out from the crowd.

  • Information for your employer – The Addison’s Self-Help Group have produced guidance for employers called ‘When an Employee Has Addisons’, which highlights some of the key points around work and adrenal insufficiency. View it here.

  • Benefits and financial support – People affected by CAH may be entitled to benefits and/or financial support. For more information please see the following links:
Paying for medication

In the UK, people with CAH are eligible for free prescriptions via a Medical Exemption on the grounds of hypoadrenalism. Speak to your GP or pharmacy for more information.

See also: https://www.nhs.uk/nhs-services/prescriptions-and-pharmacies/who-can-get-free-prescriptions/

Driving licenses and the DVLA

CAH isn’t listed as a ‘notifiable condition’ for the DVLA.

For more information please see https://www.gov.uk/driving-medical-conditions

Sports

For most recreational competitive sports, the treatment methods used in CAH are unlikely to breach any rules . At higher level competition, the World Anti Doping Agency state that some treatment methods used for CAH would require a ‘Therapeutic Use Exemption’ in order not to break ‘Prohibited Substances’ rules.
If you are at all unsure, contact the governing body or organisation that represents the sport in question.

Everything else

Alcohol and CAH – We’ve not heard of any problems among our adult members with CAH when they’ve drunk alcohol in moderation. While drinking to excess has it’s usual risks, including dehydration, it can also affect someone’s ability to take medication orally.  

Body modifications and CAH – We have some adult members with CAH and a wide variety of tattoos and piercings. Their advice has always been to keep blood sugar high and take extra medication if they feel it’s required before or during the procedure.

Coming soon.

Hear some of our members via our artificial intelligence videos talking about their experiences of living with CAH.

The CAH Support Group is affiliated with Society for Endocrinology, an organisation that supports clinicians, scientists and nurses who work with hormones throughout their careers. They engage policy-makers, journalists, patients and the public with hormone science to encourage informed health decisions, and to demonstrate the value of endocrinology to the wider world and also maintains a public information website, You and Your Hormones.

All content on this website (livingwithcah.com) is copyright the CAH Support Group unless otherwise stated.

The CAH Support Group cannot be held responsible for the content provided by any external links unless stated.

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