Last September our son William, rolled his ankle on the edge of the pool, which ended up breaking both his tibia and fibia clean across. So we started examining his bone health as this is something that shouldnt happen just from a rolled ankle. We had a bone age xray performed which showed that his bone age is that of a 5 yr old (he is 8 in August) and further xrays showed the effects that he has been on steriods now for 4 years ie, ragged edges on his spine, and smaller less dense cortexes? for his long bones.
It was suggested that we try Pamidronate infusions to increase the bone density to minimise the chances that he break more bones. We are fully aware of the potential side effects, but also know that there is minimal data when it comes to these infusions in children.
Has anyone elses children undergone these infusions, and have they seen benefits from it yet?
Haven't heard of anyone doing this.
I assume you're on calcium and vitamin D supplements, and monitoring results regularly by blood tests and DEXA scan? That's the standard protocol.
My son just had his first infusion of IV Zolendronic Acid a couple of weeks ago. He is 18. He started taking steroids at age eight. At that time he was also prescribed weekly fosamax. He had been experiencing quite a bit of back pain and had a bone scan. It showed he had compression fractures of the lumbar spine. It was recommended that he switch from the oral bisphosphonates to the IV. The first infusion is done in the hospital, he stayed overnight to keep an eye out for side effects. The infusions can be done on an outpatient basic after the first one.
I am curious though, what is the reason that your son's doctor would not start your son out on an oral bisphosphonate (fosamox or Actonel) first? According to the paper that I got from the Nephrology department if you do IV Pamidronate the first infusion is done as an inpatient and requires a two night hospital stay. They do blood work before hand to check calcium and vit D levels. My son has had no side effects from either the fosamax or the IV Zolendronic Acid. He has been experiencing less back pain since the infusion.
We have been doing supplements for some time now with things like Vit D etc. We have been having regular blood tests and were recently involved in a supplemet trial until he broke his leg which was monitoring everything including having DEXA scans.
When he broke his leg our specialist suggested we see an endocrinologist who ordered xrays and blood tests including a Deoxypyridinoline test (gives an indication of the bone reabsorption) which was really high, although its hard to judge as the reference ranges are more established for adults.
After all the results were looked at the endo suggested the Bisphosphate infusion. We are aware that the first infusion is as an inpatient for 2 days and will give him flu like symptoms the first time. He needs to be in hospital so that they can closely monitor his calcium levels as they can fluctuate.
At the moment the plan is to do the infusion once every 3 months for a year and then re-assess, with dexa scans, xray and blood tests.
As for why the Endo went for the infusion over the tablets, I cant remember now but I will put something here when I do.
Also on a side note I have included an interesting article which may have already been put up here before but Im not sure.
The TL:DR version is that there is some evidence that Bisphosponate therapy combined with steroids increases survival times, however it is a retrospective study and there needs to be more studies/data to really prove this.
My 10yo gets quarterly pamidronate infusions. He just had his second earlier this month. He's done a few years of Fosamax, but it we all felt it wasn't doing the job anymore. Sam hasn't had any side effects from the infusions. Haven't had a follow-up DEXA yet, so I'm not sure how well it's working. He at least hasn't broken any more bones. He fractured 4 last year.
Adding that Sam did his first infusion as outpatient. It took all day, but I'm glad we didn't have to stay. He also did not have any flu-like symptoms from it.
Keith, not sure if you were asking me but we go to Nationwide Children's in Columbus Ohio. Dr. Jerry Mendell (he now sees Kevin Flanigan) originally prescribed the weekly Fosamax at the same time he started prednisone for my son. He took it for ten years until switching to the infusion. They thought as Laurie said that the Fosamax wasn't doing the job anymore and the IV infusion would be more beneficial.
Keith Van Houten said:
Out of curiosity, what hospital are you working with?
Im in Australia in Brisbane, and we see our specialists at the Mater Hospital, or otherwise at the Montrose clinics.
I am sorry I am not able to add to the conversation or offer help....instead I need your help! Our three sons were diagnosed with DMD in November in 2012. We live in Ireland and were told that it is up to us if/when to start our seven year old on steroids. The plan is to start them next week - however I am concerned as the only thing they are doing as a baseline is to check if he has immunity to chickenpox. There has been no mention of bone scans or other blood tests and we were told the use of 'supplements' will be up to us! I understand that Vit D and phosphates should be prescribed? Can anyone tell me what we should be prescribed and what baseline tests/scans etc we should ask for. Many thanks,
Best wishes, Paula
I am truly sorry learning your three sons have DMD. My two sons were affected and we recently lost my older son at age 20. My younger son is 18 and still strong.
Please urge your clinic/physicians to take a baseline bone density scan (DXA) for all of your sons and monitor this for changes in bone health. Additionally, speak with them about a calcium supplement as well as Vitamin D3 which enhances the metabolism of calcium. Being careful with diet is important, avoiding sugary drinks and eating healthy appropriate portions is helpful.
Parent Project Muscular Dystrophy conducted a number of meetings with physicians familiar with the endocrine aspects of DMD and developed Endocrine Information Sheets for families which can be found mid-page following this link: http://www.parentprojectmd.org/site/PageServer?pagename=Connect_con...
The decision to use steroids is challenging, yet currently there are no other medicines that slow the progression of weakness.
I wish you and your family the best.
I am so very sorry to hear that your son has died. This is such a horrific disease Brian; we had never even heard of it until November. So much work is happening around the world and I just hope and pray that the miracle we all crave with arrive soon. It is just so difficult to articulate the pain we all experience every minute of every day. We shall keep your family in our prayers.
Thank you very much for taking the time to reply - we appreciate it very much.
Warm regards from Ireland.
Paula & The Boys
Just an update! We went ahead with the infusion, which was changed to zalidronate at the last minute. This was because the Zalidronate was stronger and would last for 6 months meaning he wouldnt require another infusion until then. It also meant that the infusion was only 30 mins rather than 4 hours.
As was expected he reacted to the infusion with spiking temperatures, nausea and vomiting and body aches etc. but was in hospital for the night aswell as the next day so he was well monitored. His calcium levels dropped quite low aswell but this was expected too.
This was all 2 weeks ago now and he has recovered well as if nothing had happened. his calcium levels have come back to normal, and we only have 1 more test to do to check the effectiveness of the infusion. Once we get the 6 monthly checks done I will update how his z-scores are going aswell.
My son has been doing Pamidronate Infusions since last July. It's every 3 months for about 4(or more)hours. This July will be our last set of 3 days, then in October, we go to 2 days(still every 3 months) then next April, we will do 1 day and that will be it. He only had 1 reaction and that was on the very first day. Nothing major, just upset stomach and a bit of diarrhea. They have been doing bone density scans and can see the progress. Everything is done in the Day Medicine Unit, so we go home as soon as the infusion is done for the day.