Our son has been having difficulty tolerating high dosage weekend dosing of Deflazacort (lots of behavioral side effects) We are concerned going to daily dosing because of long term side effects. Is anyone else using other dosing schedules such as alternate day dosing, 10 days on 10 days off, etc?

Thanks,
Nana

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Our son now takes 18mg of deflazacort every other day. He was taking it daily for 4.5 years. Consult your son's neurologist before changing his regimen. He may need to have his cortisol levels checked first.
Tina,

Thank you for your reply. It helps to know there are more options for dosing ( We have seen 2 different neurologists from different medical facilities and both only discussed daily and high weekend options for us) I will be contacting our current neurologist to ask about alternate day dosing. I was wondering why your son changed from daily dosing to every other day dosing.

thanks again
Nana
Nana,
I was hoping for a growth spurt over the summer, in addition to giving his kidneys, liver, and glands a break from the daily dose. Also, hoping to preserve his bones a bit more. He has only weakened a slight bit form the change, mostly noticeable ascending the steps and at evening time while getting off the floor.
There was a study conducted using the ADT regimen. I'll find it and post it here for you. The outome strength wise was not favorable ( which is why I omly planned on doing this mini study of my own over the summer months) and unfortunately it doesn't indicate the outcome of their weight, height, labwork, etc...

Interestingly, we did not increase our son's dose while currently on the ADT regimen. I was prepared to take him from 18mg daily to 24mg daily if I was concerned with seeing a dramatic drop in his strength level. This has not been the case so I've kept him at the 18mg daily dose level. He has been taking this dose which is equivalent to 12.5 mg of prednisone since he was 3 1/2 years old. Our son will be 9 in January. Overall, I'm happy with his status.

He has less of a cushingnoid appearance in his face and would say his appetite has lesson on his non-steroid days.
ADT® (Alternate Day Therapy)

ADT is a corticosteroid dosing regimen in which twice the usual daily dose of corticoid is administered every other morning. The purpose of this mode of therapy is to provide the patient requiring long-term pharmacologic dose treatment with the beneficial effects of corticoids while minimizing certain undesirable effects, including pituitary-adrenal suppression, the Cushingoid state, corticoid withdrawal symptoms, and growth suppression in children.
The rationale for this treatment schedule is based on two major premises: (a) the anti-inflammatory or therapeutic effect of corticoids persists longer than their physical presence and metabolic effects and (b) administration of the corticosteroid every other morning allows for re-establishment of more nearly normal hypothalamic-pituitary-adrenal (HPA) activity on the off-steroid day.
A brief review of the HPA physiology may be helpful in understanding this rationale. Acting primarily through the hypothalamus a fall in free cortisol stimulates the pituitary gland to produce increasing amounts of corticotropin (ACTH) while a rise in free cortisol inhibits ACTH secretion. Normally the HPA system is characterized by diurnal (circadian) rhythm. Serum levels of ACTH rise from a low point about 10 pm to a peak level about 6 am. Increasing levels of ACTH stimulate adrenocortical activity resulting in a rise in plasma cortisol with maximal levels occurring between 2 am and 8 am. This rise in cortisol dampens ACTH production and in turn adrenocortical activity. There is a gradual fall in plasma corticoids during the day with lowest levels occurring ab­•æmidnight.
The diurnal rhythm of the HPA axis is lost in Cushing's disease, a syndrome of adrenocortical hyperfunction characterized by obesity with centripetal fat distribution, thinning of the skin with easy bruisability, muscle wasting with weakness, hypertension, latent diabetes, osteoporosis, electrolyte imbalance, etc. The same clinical findings of hyperadrenocorticism may be noted during long-term pharmacologic dose corticoid therapy administered in conventional daily-divided doses. It would appear, then, that a disturbance in the diurnal cycle with maintenance of elevated corticoid values during the night may play a significant role in the development of undesirable corticoid effects. Escape from these constantly elevated plasma levels for even short periods of time may be instrumental in protecting against undesirable pharmacologic effects.
During conventional pharmacologic dose corticosteroid therapy, ACTH production is inhibited with subsequent suppression of cortisol production by the adrenal cortex. Recovery time for normal HPA activity is variable depending upon the dose and duration of treatment. During this time the patient is vulnerable to any stressful situation. Although it has been shown that there is considerably less adrenal suppression following a single morning dose of prednisolone (10 mg) as opposed to a quarter of that dose administered every 6 hours, there is evidence that some suppressive effect on adrenal activity may be carried over into the following day when pharmacologic doses are used. Further, it has been shown that a single dose of certain corticosteroids will produce adrenocortical suppression for two or more days. Other corticoids, including rnethylprednisolone, hydrocortisone, pednisone and prednisolone, are considered to be short acting (producing adrenocortical suppression for 1 1/4 to 1 1/2 days following a single dose) and thus are recommended for alternate day therapy.
The following should be kept in mind when considering alternate day therapy:

Basic principles and indications for corticosteroid therapy should apply. The benefits of ADT should not encourage the indiscriminate use of steroids.
ADT is a therapeutic technique primarily designed for patients in whom long-term pharmacologic corticoid therapy is anticipated.
In less severe disease processes in which corticoid therapy is indicated, it may be possible to initiate treatment with ADT. More severe disease states usually will require daily divided high dose therapy for initial control of the disease process. The initial suppressive dose level should be continued until satisfactory clinical response is obtained, usually four to ten days in the case of many allergic and collagen diseases. It is important to keep the period of initial suppressive dose as brief as possible particularly when subsequent use of alternate day therapy is intended.
Once control has been established, two courses are available: (a) change to ADT and then gradually reduce the amount of corticoid given every other day or (b) following control of the disease process reduce the daily dose of corticoid to the lowest effective level as rapidly as possible and then change over to an alternate day schedule. Theoretically, course (a) may be preferable.

Because of the advantages of ADT, it may be desirable to try patients on this form of therapy who have been on daily corticoids for long periods of time (eg, patients with rheumatoid arthritis). Since these patients may already have a suppressed HPA axis, establishing them on ADT may be difficult and not always successful. However, it is recommended that regular attempts be made to change them over. It may be helpful to triple or even quadruple the daily maintenance dose and administer this every other day rather than just doubling the daily dose if difficulty is encountered. Once the patient is again controlled, an attempt should be made to reduce this dose to a minimum.

As indicated above, certain corticosteroids, because of their prolonged suppressive effect on adrenal activity, are not recommended for alternate day therapy (eg, dexamethasone and betamethasone).

The maximal activity of the adrenal cortex is between 2 am and 8 am, and it is minimal between 4 pm and midnight. Exogenous corticosteroids suppress adrenocortical activity the least, when given at the time of maximal activity (am).
In using ADT it is important, as in all therapeutic situations to individualize and tailor the therapy to each patient. Complete control of symptoms will not be possible in all patients. An explanation of the benefits of ADT will help the patient to understand and tolerate the possible flare-up in symptoms which may occur in the latter part of the off-steroid day. Other symptomatic therapy may be added or increased at this time if needed.
In the event of an acute flare-up of the disease process, it may be necessary to return to a full suppressive daily divided corticoid dose for control. Once control is again established alternate day therapy may be re- instituted.
Although many of the undesirable features of corticosteroid therapy can be minimized by ADT, as in any therapeutic situation, the physician must carefully weigh the benefit-risk ratio for each patient in whom corticoid therapy is being considered.

Nana,  Have you gone to a different schedule yet and if so, what is the schedule?  

Tina,  So are you saying the best time to give steroids is between 2 am nad 8 am  or 4 pm to midnight.  Sorry, I just cannot follow the above, little confusing for me, sorry.  can you tell me how you dose your sons?  My son is on 40 mg every other day, but i just realized that maybe that is too high and the nurse gave us the wrong dosage because his new prescription bottle says 30 mg EOD.  please tell me what you think.

Donna, We did change to alternate day scheduling Sept. 2010 and it improved my son's behavior significantly.  He still has problems with ADHD symptoms but his behavior is much more managable.  I was told it is best to give the steroid in the morning because tends to follow the body's usual pattern.  We actual give our son his dose in the evening because it tends to make him too hyper to concentrate on his school work if we give it in the morning.  Are there any reasons you are trying alternate day dosing versus other schedules?  Dosing is usually calculated based on weight- is he taking prednisone or deflazacort?  Let me know if you have any more questions.  best wishes to your family

:) Nana

Donna Cicardo said:

Nana,  Have you gone to a different schedule yet and if so, what is the schedule?  

Donna, Are you using double the daily dose every other day? The daily dose is 0.9 mg/kg, so your 40 mg dose seems very high. I was under the impression that ADT will just be the 0.9 mg/kg dose every other day. Does one need to double that?

Have any of you notice if the boys have good vertical growth while on every other day regime? I do know that the weekend regime does preserve growth and minimizes other side effects, how about the alternate day one?

Well I was wrong, we decreased alex down to 30 mg every other day and today, he could barely stand and was in alot of pain.  Called the doctor back and he said based on his weight of 118 lbs (53.6 kg) he needs to stay on 40 mg every other day.  If you multiple 53.6 x 0.9 mg/kg that equals 48.5 mg of prednisone.  I think after seeing him struggle so much today we will keep him at 40 mg.  We have decided to join an aquatics center to help with weight control and to hopefully lose some weight.  Hope this helps everyone.  As far as some of his side effects, he takes prozac for depression and since being on that it has made a complete uturn nad he is so much better.  As far as his hives, the doctors said (after seeing 4 doctors) they have all concluded that he has chronic urticaria, which does not come from DMD but probably comes from his extreme allergies to foods, molds, and pollens.  If you ask me, I believe an every other day dose is a good regimen.  Thanks so much for your comments, it has been very helpful.   We have alos started him on idebenone and his fvc lung functoin increased by 5%.  next we are going to slow start him on Bvitamins and glutathioine and amino acids.  good luck everyone.

 

Prednisone is 0.75 mg/kg/day, so 40 mg is the right dose for his weight. 0.9 mg/kg is for deflazacort. Thank you!

Thanks Ofelia, mistook the 0.9 for the 0.75.  that makes alot more sense. 

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