THEY ARE WORKING!

WOOHOOOOOO!!
SO, as many of you may recall ,we started weaning Faith from the hydrocortisol this past summer.  The first round of follow up bloodwork spooked her local doctor-her ACTH was at 209 and she then wanted to stop the weaning process.  I asked her to consult with Dr. Breault from Harvard, who graciously agreed to help. THey conferred and decided to continue Faith at the lower dose (she had been on 10 mg a day, the bloodwork was from the first stage of weaning at 7.5 mg day)  Well, we just got the followup bloodwork done again and   her ACTH is now 19!!!!!! Perfect!!!! Sooooo.... we have moved on to the next stage of weaning! She is now on only 5 mg a day and so far is doing well.. not feeling any effects!! Dr. says it is certain that her adrenals are working and is almost sure we will be able to wean her from the cortisol!! After that I will see what we can do to wean her from the florinef.... one step at a time! I got the news while driving and cried for joy all the way home! Thank you to so many who have been instrumental in giving me the courage and the knowledge that I needed to follow through with this process.   I love you all and wish you all good health and much happiness!!

Thank you Dr. Breault!

Yet another turn! I just got an email from Dr. Breaut, the Harvard pediatric endocrinologist who has agreed to follow along with and consult with the local endo on Faith's case... The email said that he and her local doctor had spoke at length this morning and that they have agreed to continue at the lower, first level of weaning, dose for three to six months then check things out to see if there has been any progress!!! I am so happy! I think he interprets things a bit differently and uses a wide variety of things to help make his determination, whereas doctors who are not as schooled in this weaning process only use the ACTH levels.  This awesome news!!!! YOU GO FEE!

Setback

Ok, so we got the test results after the first taper and it is not good. The local endo said that her ACTH was 200 and that she was not comfortable moving on with her taper or trying to wean her, but that we could try again in another year.  I am not happy with that.

So, being Tenacious J, as my husband calls me, I emailed Dr. Breault with the results.  He said it all depended on the context in which the bloodwork was drawn, which leads me to believe that all hope is not lost. She had not had any hydro since 330 the previous afternoon and I am sure that is why her ACTH was high.  So.... I am holding out hope that he says it is still okay to continue.... I am still waiting on a reply from him as to what he thinks. I just hope that he writes soon.  And that I can talk her dr. here into continuing if he wants to do so.

Am I being too pushy or stubborn? Wanting this too much for her? Am I putting her at risk? I am losing my mind trying to get this figured out.  Any advice from all the people who have "been there and done that"??

We started weaning today!

Ok, so, let the summer begin! And the weaning.  As many of you know I refused to start the weaning process until Faith had left the school she was in because I did not trust the nurse.  Well, today was the first day of her weaning.  She has been on 10 mg/day and we took her dose down to 7.5 mg/day.  We are to stay on that for 8 weeks, with bloodwork five weeks into it.  If the bloodwork comes out fine after the 8th week we go down another 2.5!! Wish us luck!

We spent about 4 hours in 100 degree heat today at the pool, I made sure she drank plenty of fluids and she is perfectly fine this evening.... But don't you know this mama is gonna watch her like a hawk!! I am so excited about this whole process and believe with all my heart and soul that this is going to work! If anyone has any words of advice about her weaning, please feel free to share!

Thanks! Joy and Faith

Got the ok to go ahead and wean!

Ok, so we got the last of Faith's bloodwork back and her ACTH is just a tiny bit on the high side... just over normal. Her local endo said she is still optimistic that we can wean her without any trouble.  Most other times her ACTH has been very high. This is the first ACTH test I recall having since they discovered her glands were regenerating and growing and the fact that her ACTH is down from what it normally is is a good thing! That tells me her glands are working to an extent, right? She is on a very low dose of steroids, so her ACTH being lower is an indicator that she is getting ALMOST enough cortisol... does this make sense to anyone else but me!?!?

Anyway, because I have such little faith in the nurse at Faith's school, we have decided to wait until summer to begin the weaning process.  I do not trust that she will be able to recognize symptoms,  or even treat Faith with enough common sense to keep her safe.  And the last thing I need is for her to go into a crisis just when we start the weaning process.  ANd the school nurse is definitely lacking in observational skills as well as common sense....only six weeks till school is out and then we begin!!! I am so excited, though frustrated that we must wait to begin... but, better safe than sorry.  I will feel much better keeping an eye on her myself when the process of taking her off steroids begins!!

Wish us luck!
Joy and Faith!

Miracles do happen!!

Hi Everyone!!

Well, for those of you who are following Faith's weaning, here is the next step! Good news... we got the results of her MRI back... she has "a lot of viable adrenal tissue!"  This in itself is a miracle, as several years ago we were told that her glands were completely calcified and would NEVER, EVER work again, so I shouldn't get my hopes up! HA!! The power of prayer is amazing!  We do not know how much of it is working and won't know until we begin weaning her and seeing how she responds.  When I spoke to her new local doctor, she told me she really thinks that the weaning is going to work.  We are only weaning from cortisol to start. IF that goes well then we will try to take her off florinef.  All in all the doctor thinks it will only take about a year or so... that seems like a short time to me, but she is only on 10 mgs right now, so maybe that is a reasonable time frame.  I pray that both the inner and outer portions of her adrenals regenerate and begin functioning so that we can take her off the florinef also.  But if not, even if the cortisol weaning is the only part that works, that will be fine with me!

Stay tuned for the next episode!! Next week the rest of her bloodwork comes back and once the doctor checks her ACTH levels, she will set us up on a weaning schedule!!

Thanks for tuning in!!
Joy and Faith

HIstory

I have been asked by some of my AI friends to start a blog on the weaning process of my daughter, Faith Evangeline.  So.....here goes....

Any of you that know me, know my daughter and her history. But for those of you who don't let me give you a little bit of background.  Her name is Faith and she is ten years old.  She is perfect in every way... except one.  Her adrenal glands don't work.  She is considered adrenal insufficient.  Not Addison's in the normal way, from steroid use or autoimmune issues. Her dysfunctional adrenal glands were the result of an adrenal hemorrhage at birth.  We had an especially rough delivery in which she crowned for hours and they could not get her out of the birth canal. They used every means imaginable and finally were able to extract her from my body... but not without a good deal of trauma.   

She looked like the perfect little baby girl, until the bruising and jaundice set in.  Her delivery caused a great deal of physical trauma.  They kept her in the hospital at first so they could monitor her sugar level, because I was gestational diabetic.  Then after a day or two they began to notice that there were problems with her bloodwork... It took two weeks of her being in ICU and numerous doctors trooping through the hospital nusrery, ordering multiple tests, and then shaking their heads in confusion. Everything looked fine according to the tests they were performing. Finally her savior doctor came through- he was a stand in for the endocrinologist who had already seen Faith and come up empty handed.  He asked the staff if anyone had thought to test this baby's adrenal glands.  The other doctors and nurses acted as if he had no sense, but they must appease him, so they went ahead and did the tests to satisfy him.  Lo and behold! The tests came back and indicated that she had had a total bilateral adrenal hemorrhage... in other words, both adrenal gland had exploded, essentially... One doctor likened it to two soldiers fighting a war to the bitter end.... they were wounded, but kept fighting, and once the fight was over, they gave up the ghost. Her adrenals did the same thing as they were trying to keep her alive during an exceptionally horrid birth.  Poor little fellas fought to keep my baby alive for almost a full 24 hours before giving up the fight once she finally entered this world.

We were told for the first three years that there may be hope that her adrenal glands would heal themselves and begin to function.  It had been known to happen for another of the doctor's patients who had had a similar situation at birth.  But when she was three, her doctor called after reviewing some routine bloodwork and told me that he didn't believe her adrenals would heal at this point.  

For the next 3 years we still held up hope that someday we would find that her adrenal glands somehow began to work.  Because her case was a bit unusual, her endocrinologist referred us to a doctor at the NIH and off we went to Maryland in the hopes that the doctors up there could help us find a way to get her glands up and running!!  That first visit in 2006 was disheartening.  We were told that her adrenal glands had completely calcified and that there was no hope of them ever working again. I should resign myself to the fact that she would be on the steroids all of her life. (Any of you who know me, know that I am not that easily resigned to something that does not suit my fancy, and this definitely did NOT  suit my fancy!)  

In 2008 we went back to the NIH for a follow up exam with the doctors there.  They scratched their heads in confusion when the MRI and bloodwork results came back. Apparently it "looked" as if there was some living tissue... ever so small... in her glands, and some adrenal function... ever so little... I was told not to get my hopes up, as this was probably not the case.

But, this was the miracle I had been praying for! Granted, her glands were not functional... only just beginning to come to life! But it was a pinpoint of light in this dark spot on her life.   Two years later, in 2010,  I took her back to the NIH yet again.. This time the test results confirmed that there was definitely living tissue and function of her adrenal glands!!!

Well now!!   Let's fast forward to late 2010 when, in sharing thoughts and stories with the two wonderful yahoo adrenal groups to which I belong, I discover a doctor at Harvard who has been successful in helping to wean other AI patients off of their steroids and who everyone has much confidence in.  (That happens so little in the world of adrenal patients) So, being one of those moms who won't take NO for an answer, especially when it comes to my child's health, I contacted this doctor and a new local endocrinologist, as we had moved to another state and could no longer be seen by her savior doc.  They both agreed to take on her case and possibly try weaning her off the steroids.  Everything in the past two years points to the fact that she can be weaned successfully, so that is what we are praying for.  

Soooo.... here we are... in the early stages of possibly weaning her off the steroids and getting her adrenal glands to function after spending her whole life as an AI patient.  I understand that this will take time and patience. But I also understand that there may be no need for her to stay on this medication and to be AI all her life.  She has never had a crisis, nor has she ever come close to one.  She is more healthy than she should be, by rights...and she is thriving! This gives me the encouragement to move ahead with the weaning process and the hope that she will get through it with little trouble. 

I feel the need to go through with this process because I feel that if I did not I would not be doing everything that could be done to insure her best health.  

We went to the local endo last week and she is on board to begin the weaning process.  She will be consulting with the Harvard doctor and we will soon be on this adventure. We will have an MRI done at the end of March along with bloodwork, and bone age and bone density scans to see where she is.  After that the local endo plans to set up a weaning schedule and off we will go!! Wish us luck.