Q: My 2 week old is in the hospital and the doctors told me to put salt in her bottle to replace her lost salt. The baby has been gagging and crying every time she eats. Is there another way to do this? Is there a pill we could crush up and put in a syringe and squirt in her mouth like they were doing with another medicine?

I’ve tried the bottle but it was like drinking the ocean. The doctors are encouraging me not to breastfeed.

A: You should definitely breast feed if you want to!

If your baby is rejecting the salt, then it’s possible that she is not a salt-waster or perhaps doesn’t need as much salt as you are giving her. Babies who are salt-wasters need and crave salt — they generally gobble up salt like it’s candy! If she does need salt supplementation, then sodium chloride (NaCl) comes in 1 gram tablets (by prescription) that are easily dissolvable in water. Depending on how much sodium your doctor wants her to get per day (the usual amount for an infant with salt-wasting CAH is 1-2 grams per day), you can dissolve the total amount in water and then divide it into 3-4 doses given by syringe. I would not recommend putting it in her bottle because then she won’t want to eat anything! You can start with 1 gram of NaCl (one tablet) and dissolve it in 15 mL of water, then give her 5 mL by syringe three times a day. Or, if that is still too salty, you can dissolve it in 30 mL of water and give her 10 mL by syringe three times a day. If necessary, you can try to add a little glucose to the solution so it doesn’t taste as salty, but you should ask your doctor about that.

Q: I have a 7 year old daughter who has been diagnosed with NCAH recently. She is taking hydrocortisone 10mg/day. I shaved her armpit hair 5 days ago. I noticed her armpit hairs are growing and are dark today. Her doctor said hydrocortise would stop the growth of her armpit hair. How long does it take to stop the growth of her hair? Also, she has hair on the outer nose like a black head. Please let me know a safe way to remove her armpit and facial hair. Thank you.

A: The hydrocortisone will bring down the hormones that are causing the hair growth fairly quickly (usually within a month or so). However, the lifespan of a hair follicle is about 6-9 months, so the hair follicles that are already present will remain for the duration of their life cycle unless you do something like electrolysis or laser, which I would not recommend for your 7 year old. Once the level of androgens (the hormones from CAH that cause the hair growth) have come down, you will start to see less hair growth, but it can take up to one year to really see a difference. In the meantime, you can try Nair (a cream that dissolves the hair) or continue shaving, although shaving may cause chafing. You could also consider waxing, which would pull out the hair from the root, but it may be too painful for your 7 year old. Some people also just trim the hair, which is another option. If your daughter has blackheads or acne on her face, acne will resolve more quickly (1-3 months) once the androgens are lowered. In the meantime, you can use a facial cleanser containing salicylic acid and topical benzoyl peroxide as needed.

Q: My granddaughter will be three next month. She has salt wasting CAH and was born with ambiguous genitalia. She has developed massive amounts of calcium in the tissue of both kidneys, and her kidneys are not growing based on the last ultra sound. Is this a normal occurrence with CAH? Does the cortef or florinef cause this condition? We were told she will need a kidney transplant at some point. She is also on blood pressure medication due to thinning cortex. Lastly, my granddaughter has yellowing of the teeth and she thinks it may be caused by the medication.
Any information will be helpful.

A: Nephrocalcinosis (excessive calcium in the kidney) can be caused by conditions where there is too much aldosterone or when a patient is being treated with too much Florinef (fludrocortisone), which is used to replace aldosterone in patients with salt-wasting CAH. If your granddaughter is hypertensive, that is another indication that she may be receiving too much Florinef. There is always the possibility that she has another underlying condition apart from her CAH that is causing the nephrocalcinosis, but it would be a good idea to make sure that her labs do not indicate that she is receiving too much Florinef. Laboratory indicators of too much Florinef would be a very suppressed (undetectable) plasma renin activity or a low potassium level.

Discoloration of the teeth may arise from some antibiotics (such as tetracycline or doxycycline). You mentioned that your granddaughter is taking medication to lower her blood pressure. Some anti-hypertensive medications may also cause discoloration. I am not aware of any relation between hydrocortisone or fludrocortisone and discoloration of the teeth.

Q: My 18 year old son has been living with CAH, 21-hydroxylase, salt wasting….since birth obviously, and is now morbidly obese.  I have researched the HCG diet and cannot find any information relating to the safety or counter productivity of trying this…would love some feedback

A: I would be cautious of the HCG drops that are included in the diet plan.
The diet plan itself is fine, but HCG is a hormone produced by women during pregnancy to help maintain the placenta. It is similar in structure to LH (luteinizing hormone), which is produced by the pituitary gland and stimulates testosterone production in males. If your son takes HCG, the concern would be that his own LH production would decline, and his fertility may become impaired. Males with CAH are also at risk for benign growths consisting of adrenal tissue in their testes (called “adrenal rests”), and the HCG could potentially make them grow, which could again lead to infertility or impaired testosterone production. Truth be told, the weight loss that some individuals experience with the HCG diet is related more to the diet and exercise plan than to the HCG itself. There are many meal plans available that provide a set number of calories per day that would produce the same results.

Q: Does the excess intake of salt in a Salt-wasting, Classical CAH patient, place increased stress on an infant’s colon/GI system, leading to increased occasions of blood in the stool?

A: Steroids in very high doses can cause inflammation of the lining of the stomach (called gastritis), which could potentially lead to dark blood in the stool (the color would be dark due to digestion and passage through the intestines). However, the steroid doses generally used for the treatment of CAH are more in the physiologic range, so they should not cause bleeding.

If the blood is bright red, the more likely culprit is anal fissures, which are fairly common infants and can be a result of hard stools. Your pediatrician should be able to tell you if your infant has anal fissures pretty easily.