Different Electrolytes like Sodium, Potassium, Chloride, Bicarbonate and Calcium which may be positively charged or negatively charged, need to stay in a state of balance. Aldosterone instructs the cells to retain Sodium and let go of Potassium. If Aldosterone is deficient or missing, as is the case of Congenital Adrenal Hyperplasia, the body will do the reverse. It will instead lose Sodium and hold Potassium.
Low Sodium will lead to lack of water in the body, low blood pressure and reduced blood circulation. It can even lead to complete cessation in blood circulation, or “shock”. Low Sodium levels can cause “Hyponatremia”, which can even lead to seizure or death. In a normal human body, when the kidneys sense low Sodium or low blood volume, it releases a hormone called Renin. When Renin is released, it triggers the release of another hormone called Angiotensin, which instructs the Adrenal glands to produce Aldosterone. Aldosterone in turn tells the kidney to reabsorb excess Sodium. In salt wasting CAH, the Adrenal gland cannot produce Aldosterone. Thus, the body cannot reabsorb Sodium or get rid of excess Potassium. In salt wasting CAH, low Sodium leads to various other events, finally ending in an Adrenal crisis.
Aldosterone also instructs the kidneys to get rid of excess Potassium. In salt wasting CAH, without Aldosterone, Potassium levels rise. When the body cannot release Potassium, it can lead to “Hyperkalemia”. This can cause your heart to beat irregularly, which can also lead to shock and death.
Flurocortisone works by retaining sodium in the body of someone with CAH. For it to work properly, someone with salt wasting CAH must consume enough salt. Only those who are the salt wasting form of CAH must take Flurocortisone. If they are not salt wasting, they run the risk of developing high blood pressure.
Unlike the Glucocorticoid dosage, Mineralocorticoids, Flurocortisone, does not require as much balancing. Usually a typical dose is arrived at, and prescribed, and not adjusted very much. Whereas an excess dose of the Glucocorticoids can have a harmful effect on growth, a slightly excess dose of Flurocortisone does not badly impact growth. If it is too high, blood pressure may rise and the excess salt is released by the body through urine.
Monitoring Mineralocorticoid Treatment in salt wasting Congenital Adrenal Hyperplasia
Blood pressure monitoring
In case the Flurocortisone dosage is high and the body holds excessive salts and fluids, it can be monitored by checking if the blood pressure is high. If so, the dosage may be lowered. If the Flurocortisone dosage is too low, the body will not hold enough salt and fluid, in which case, the blood pressure will be too low. But, a normal blood pressure reading is not always an indication that the Flurocortisone level is adequate. The blood pressure only gives clues about salts and fluids.
As Renin is produced by the kidney which in turn triggers the Adrenal glands to produce Aldosterone, if the Flurocortisone dosage is too low, there will be a high Renin level. In which case, the Flurocortisone dosage will need to be increased. If the Flurocortisone level is too high, then the Renin levels will be too low. In which case, the dosage will need to be increased. Each lab may have their range of normal Renin values. To interpret Renin level readings, normal range of that lab should be compared.
In the salt wasting CAH, monitoring Electrolytes and finding a major imbalance in them in times of illness, can suggest that an Adrenal crisis is about to occur. At those times, the dosage of Flurocortisone may be additionally required, or a consumption of salt may be needed.
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: