Child health

Patient information for Translation

For parents/ carers of children affected by

Congenital adrenal hyperplasia

(C.A.H.)

What is congenital adrenal hyperplasia?

The term congenital adrenal hyperplasia (often abbreviated to CAH) is a descriptive term for the condition:

• Congenital: means from birth

• Adrenal: the glands affected in this condition

• Hyperplasia: overgrowth or a thickening of the outer layer of the gland often occurs due to excessive work

The adrenal gland sits above each kidney.

It is made up of a medulla (middle), which makes adrenaline; this works normally in CAH.

The outer part of the adrenal gland is the adrenal cortex that makes three main hormones called steroids: cortisol, aldosterone and androgens.

It is the adrenal cortex and its hormones that are involved in CAH.

The three main steroids involved in CAH

• Cortisol keeps many of the body’s systems in good working order – this includes the circulation and control of blood sugar levels. Cortisol is particularly important in helping the body combat stress (such as infection or injury) and in raising a low blood sugar level (hypoglycaemia).

• Aldosterone helps to regulate the salt levels in the body.

• Androgens are sex hormones which are produced by both sexes, usually during puberty, which contribute to changes such as pubic hair development.

What happens in CAH?

• The adrenal glands are unable to make enough cortisol, and in some cases aldosterone. About 80% of children with CAH do not produce enough aldosterone.

• The body senses that there is no cortisol and attempts to stimulate the adrenal glands into making some more.

• This over-stimulation results in:

• the adrenal cortex thickening (hyperplasia)

• an increase in the production of androgens (the male hormone)

• Hence the hormone disturbances cause by CAH are:

• lack of cortisol

• lack of aldosterone

• excessive androgens

What medication does my child take?

• As the body does not make enough cortisol, or both cortisol and aldosterone, a tablet is required to replace them.

• The tablet to replace cortisol is known as hydrocortisone, or for some children prednisolone is used.

• The tablet to replace aldosterone is known as fludrocortisone. Occasionally extra salt (called sodium chloride) is needed.

What about the high androgen levels?

• Taking the prescribed medication means the body’s cortisol and aldosterone levels return to normal.

• This has the effect of the body ceasing to over-stimulate the adrenal cortex.

• With this, the adrenal cortex stops over-producing androgens, returning their levels to normal.

Do the tablets need to be taken for my entire child’s life?

• Your child’s treatment will last for their entire life.

• Also it needs to be taken at intervals throughout the day.

• Without treatment, your child, whatever age, will become very ill. In babies it is life threatening.

• As your child goes through adolescence, and in the years prior  to their transition in to adult care, the Paediatric Endocrine team will discuss with you strategies for allowing your child to become more independent in the care of their condition and medications.

Will my child develop normally?

• With regular treatment, your child will develop normally.

• Without treatment, the high levels of androgens will stimulate growth to occur too quickly. Early puberty can also happen.

What to do if your child is unwell

1. In the event of mild to moderate illness, e.g. cold, cough, sore throat, flu, tummy upset, double the dose of your cortisol replacement medicine (i.e. hydrocortisone or prednisolone) for the duration of the illness.
2. The hydrocortisone must be given by injection (into a muscle) if your child: is unable to swallow the tablets (e.g. due to continued vomiting); or feels drowsy

The Paediatric Endocrine Nurse Specialist will go through with you how to administer the injection, and help you practice until you feel competent.

If in doubt, it is always better to inject. Remember that no harm can come from giving an unnecessary hydrocortisone injection.

Remember to check the hydrocortisone injection (sodium phosphate) is not past its expiry date.

The dose of hydrocortisone injection is:

• babies and infants              25mg
• 1-5 years                           50mg
• over 5 years                      100mg

If your child continues to be ill and does not seem to be getting better, please go to the Accident & Emergency Department at your nearest hospital and take along your cortisol insufficiency therapy card.

You must always call an ambulance to take the child to hospital if they have had a hydrocortisone injection.

For more guidance on when to increase the dose of your child’s hydrocortisone or prednisolone, or when to give the intramuscular injection, please refer to the leaflet called Adrenal Insufficiency – when to increase your child’s dose of hydrocortisone.

Useful telephone numbers

Hospital switchboard:                                    020 3416 5000

Department secretary:                                    020 3594 0418

Clinical Nurse Specialist team:                      020 3594 1548

Specialist Registrar:                   bleep: 1147 via switchboard

Consultant:  call switchboard and ask to speak to the Consultant on-call for Paediatric Diabetes and Endocrinology

Useful support group websites

• Living With CAH: www.livingwithcah.com
• Pituitary Foundation: www.pituitary.org.uk
• Addison’s Self-help Group: www.addisonsdisease.org.uk
• British Society of Paediatric Endocrinology and Diabetes:

Large print and other languages
This information can be made available in alternative formats, such as easy read or large print, and may be available in alternative languages, upon request. For more information, speak to your clinical team.
 

এই তথ্যগুলো সহজে পড়া যায় অথবা বৃহৎ প্রিন্টের মত বিকল্প ফরম্যাটে পাওয়া যাবে, এবং অনুরোধে অন্য ভাষায়ও পাওয়া যেতে পারে। আরো তথ্যের জন্য আপনার ক্লিনিক্যাল টিমের সাথে কথা বলুন।

Na żądanie te informacje mogą zostać udostępnione w innych formatach, takich jak zapis większą czcionką lub łatwą do czytania, a także w innych językach. Aby uzyskać więcej informacji, porozmawiaj ze swoim zespołem specjalistów.

Macluumaadkaan waxaa loo heli karaa qaab kale, sida ugu akhrinta ugu fudud, ama far waa weyn, waxana laga yabaa in lagu heli luuqaado Kale, haddii la codsado. Wixii macluumaad dheeraad ah, kala hadal kooxda xarunta caafimaadka.

Bu bilgi, kolay okunurluk veya büyük baskılar gibi alternatif biçimlerde sunulabilir, ve talep üzerine Alternatif Dillerde sunulabilir. Daha fazla bilgi için klinik ekibinizle irtibata geçin.

یہ معلومات متبادل فارمیٹس میں دستیاب کی جا سکتی ہیں، جیسا کہ پڑھنے میں آسان یا بڑا پرنٹ  اور درخواست پر متبادل زبانوں میں بھی دستیاب ہو سکتی ہیں۔ مزید معلومات کے لیے، اپنی کلینکل ٹیم سے بات کریں'۔

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Reference: BH/PIN/289

Publication date: Nov 2020

All our patient information leaflets are reviewed every three years.

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