In order to recover from any of these situations, much higher than normal doses of Cortisol is required. Someone with CAH, who does not produce Cortisol even in a normal situation, is unable to recover from any of these situations. They are unable to generate any response to illness or trauma.
Adrenal crisis is a medical emergency and potentially life-threatening situation requiring immediate emergency treatment. It is a constellation of symptoms that indicate severe adrenal insufficiency caused by insufficient levels of the hormone cortisol. Without Cortisol, there may be low blood flow, creating “shock”. There could even be organ damage or brain injury. Adrenal crisis can even result in coma or death.
In CAH, an Adrenal crisis can occur due to delayed diagnosis or at times of illness or stress. At times like these, due to the inability of their body to produce the large amount of Cortisol required to fight illness, they require Stress Dosing.
Stress dosing of Hydrocortisone is given in times of physical stress to avoid an Adrenal crisis, and its subsequent dangers. Minor illness like common colds may not require stress dosing, but very high fevers for more than a day will surely require it. Very high fevers that are prolonged, as in a Virus, and when the temperature goes higher than 101, will need stress dosing through every day of the high fever, and should continue for one more day after the temperature is normal. In cases where antibiotics are prescribed, the fever may come down in a day or so. In those times, the stress dosing is only given until the fever is present.
Dental surgeries may require stress dosing, but not minor dental cleaning work.
In times of accidents, a minor injury where someone with CAH seems to be mentally normal does not require stress dosing. But, major accidents where they seem to be unconscious or disoriented or in shock will definitely require stress dosing.
In times of Surgery, normally Cortisol requirement goes up by about 5 times the normal requirement. In CAH, stress dosing may be started by IV, prior to the anaesthesia and surgery, and continue till the surgery ends. Afterwards, where they are able to take oral medication, stress dosing may be continued for a day or so. In case there are no complications like fever afterwards, they can go back to the normal dose. Otherwise, they are given stress doses until they are back to normal.
In case there is bone breakage, stress dosing is surely given.
Excessive vomiting or diarrhoea, they may not be able to take oral medication even in a normal dosage, leave alone a stress dosage. In those times, they should be taken to the Emergency Room. As it is, vomiting puts those with CAH under tremendous stress, all the more if they are salt wasting. In the case of salt wasters, due to deficient Aldosterone, they can also dehydrate. In those times, they may require to stay in the hospital under IV fluids and IV Glucocorticoids.
Even in the Hospital, it is important that you share information and ask questions a lot. Remember that in an Emergency Room, not everyone present at that moment will be fully aware of CAH and its repercussions. If the Endocrinologist is not present or not accessible right away, keep sharing information all relevant information such as normal dose, what occurred, how your child appears compared to normal and so on. Your instinct may be important at such times.
The school which a CAH child goes to must be fully informed as soon as they are admitted. They should also be continuously updated about dosage changes and what to expect in terms of appetite, sleep, energy levels or exercise. They should be requested to notice subtle changes and inform.
In case of travel far away from the regular Paediatric Endocrinologist or the regular Hospital, make sure that you carry adequate medication to cover for stress dosing if required, medication for fever, vomiting, diarrhoea and any other medication which might be required. Make sure that you carry the medical file in case you have to rush to a new Hospital for a crisis. Try to always have access in emergencies, to either your regular Endocrinologist, or someone whom they recommend to contact in those times.
In the life of someone with CAH, precaution is the best savior. Try to avoid all emergencies or a crisis by taking due care.
1-hydroxylase deficiency Congenital Adrenal Hyperplasia is usually diagnosed by elevated levels of 17-Hydroxyprogesterone, with or without low Cortisol levels in a patient’s blood sample.
The primary issue in Congenital Adrenal Hyperplasia is the inability of the adrenal glands to create enough cortisol in all clinical forms of CAH and in addition, inability to make enough aldosterone in the salt-wasting form.
This treatment is given for anyone with CAH, irrespective of whether they are salt wasting or not. It has to be sufficient to replace the deficient or missing Cortisol, and at the same time, it has to prevent the Adrenal glands from over producing Androgens.
In the case of someone with salt wasting CAH, in addition to the Glucocorticoids, they also require a Mineralocorticoid or Flurocortisone, to replace the deficient Aldosterone which is required to regulate salt and fluid levels and to maintain normal blood circulation.
We understand how challenging it can be for parents of CAH children, who need to manage so many things.
In the first of a series of handouts for parents, we have created an Emergency Awareness Handout Pack that includes a handout for Schools and a bonus handout for parents.
by C. Y. Hsu and Scott A. Rivkees MD
CAH is an inherited disorder of the adrenal glands. Its effects can be wide-ranging and serious-from problems of ambiguous genitalia in baby girls; to problems with growth and physical development; to problems with infertility and hormone imbalance.
by Peter C Hindmarsh and Kathy Geertsma
Congenital Adrenal Hyperplasia: A Comprehensive Guide addresses how hydrocortisone works, what can go wrong, and how to correct it, also explaining why the timing of doses and measurement of cortisol from a dose is extremely important.
We have created a Facebook page called Omkar's Journey with Congenital Adrenal Hyperplasia to chronicle all possible events and scenarios in the life of a child with CAH, with a view to let new parents know what to expect.
Mobirise themes are based on Bootstrap 3 and Bootstrap 4
Publish your website to a local drive, FTP or host on Amazon S3, Github.
The Congenital Adrenal Hyperplasia Support India group on Facebook is a closed group and is for discussion and sharing of information relevant to Congenital Adrenal Hyperplasia only. This is a safe place for parents whose children have CAH to exchange and seek information that might help them deal with the problem. Note that posts which are not relevant to CAH and which are advertisements for sales of products, services or groups will be deleted and the person posting the message will be removed from the group without notice. If you wish to join this group: