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Post by miriam on Dec 9, 2011 16:17:17 GMT -6
Hi Cooper,
Congrats on your new home. Many years of health and happiness to you all. Glad your little one's doing better.
We've an email group going with families of children with alveolar haemorrhaging syndromes. It's a very friendly, supportive group and people are very welcome to join.
Nollaig Shona. Happy Christmas
Miriam
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Post by cooper on Feb 6, 2012 16:15:23 GMT -6
Hi All, I just have a quick question. Our IPH boy who turned 7 last week has been bleed free for 2 1/2 years now and I just wanted to know what everybody thought or was doing about long term medication. When is the right time to start pulling back. He isn't on steroids and hasn't been for some time but like everybody we don't know whether the medication is keeping him in remission or whether it is him. It's a really fine line but we don't want him to be on chemo medication forever. Any thoughts or experiences would be greatly appreciated.
Best wishes, Cooper
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Post by miriam on Apr 11, 2012 16:20:58 GMT -6
Hi Cooper,
What's your son on now? 6MP?
Hope the countryside's suiting ye.
Regards,
Miriam
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Post by cooper on Apr 11, 2012 16:43:28 GMT -6
Hi Miriam,
Oliver is going great. He has been major incident free for 3 years now since he started the 6mp. We had a hiccup a year ago when he was on oxygen for 3 months which caused his co2 to build up and make him quite sick. Luckily he didn't have a bleed at that time and since our tree change he has been great.
The move has been great, he is now a sports nut and it's great to see him actually playing and running around outside instead of being cooped up inside on videogames.
How is your little one going?
Best wishes, Lori Cooper
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Post by tmujin3 on Aug 30, 2012 18:36:57 GMT -6
My daughter, 2.5 year old, was diagnosed with IPH in mid April this year and was discharged from hospital in beginning of following month. She was given steroid 4ml/kg when hospitalized and reduced to 2ml/kg per day at the time of discharge. Later in June, she reduced steroid to 1ml/kg per day since her HGB stayed over 14 and Retics at less than 2% from her lab results. A week ago, she was admitted to hospital as her Retics went up as high as 6.7, so our Doctors though it would be necessary to take her in to stop the bleeding with IV steroid. They done biopsy few days ago just to make sure she doesn’t have Capillaritis. No results about Capillaritis so far. Now she’s on steroid for 4ml/kg per day but no sign of improvement on the lab results. HGB still around 13.5 but Retics varies around 4.6 to 5.6 each day and would not drop to 2%. Doctors are planning on giving her 30ml/kg of steroid for 3 days to see if she responses to steroid. If not, they will look into other alternative treatment. I am concern of side effects on steroid as she already is showing cushioned face, gain weights, hair everywhere on her body, and shorter heights than her twin sister. Doctors are saying overall intake of steroid would be less and the risk of side effects should be less once 3 days IV steroid treatment takes place since it will be one time injection per month. I read all postings from the parents on this forum and kind of hesitate to get any steroid treatments. As I requested our Doctors to consider other alternative treatment as mentioned on this forum but they strongly feels 3 days mass amount of steroid should be tried at first. I tried to tell them the bleeding doesn’t seem that severe, so why not wait on giving her mass amount of steroid in short period of time. Any thoughts, your experience would be much appreciated it.
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Post by cooper on Sept 2, 2012 17:52:28 GMT -6
Hi tmujin3, sorry your daughter is going through the trials of IPH. In regards to steroids, a 3 day high dose pulse is effective in stopping the bleeding in the lungs and definitely has less side effects and is better for their bodies than an everyday dose. I know that steroids are scary but that is where we all started. In our case steroids were only used when my son was having a severe bleed but didn't work in an everyday sense to control the condition so we moved onto other medications are are now on 6MP (mercaptopurine) and this seems to be working well. If the steriods aren't effective in your daughter case you can trial other medications and at lease have a list of alternatives to try immediately. In regards to the side effects of the steroids, they will go away once the steroids have stopped or moved to monthly pulses. I would stick with your Dr's in the fact that if your daughter's IPH can be controlled purely by monthly pulses you would want to stick with that...some of the medications that others are on a very strong and quite nasty but we have to do what we can. Hang in there and best of luck with your daughter. I check regularly so if you have any questions or concerns, please ask, your not alone!!
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Post by tmujin3 on Sept 7, 2012 15:55:35 GMT -6
Dear Cooper
Thank you so much for your input. It helps a lot. I have had read it many times not to miss any of your points.
My daughter is still at hospital and started high dose steroid of 30ml/kg since yesterday. She will be on weekly high dose for 8 weeks and wean down the daily dose. She's been on 6ml/kg for last four days and 4ml/kg for about 11 days prior to 6ml/kg. She still has Reticulocyte count of about 7%Doctors thought it would be better to give her high dose instead of 6ml/kg everyday due to side effect concerns. Reticulocyte just dropped only 0.6% today. But it's improvement, thanks to God!
When you said your son had a severe bleeding, what was his reticulocyte count and hemoglobin level? And how long did it take to stop the bleeding or the blood lab results to come in close to normal level?
Thank you.
tmujin
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Post by cooper on Sept 10, 2012 16:42:32 GMT -6
Hi Tmujin, I think we only ever did a retic count once and that was to make sure his bone marrow was working properly, which it was. His hemoglobin levels during the worst bleeds would be abount 4 but if he was already in hospital if they dropped to 7 then he would get a transfusion. He would stay in hospital usually 14 days each bleed but his last stay was 8 weeks. It would take usually about a month for his Hb to return to normal but as long as his levels were slowly rising he was allowed to go home (usually they were in the 90's). The physical side effects from the steroids will go away once your daughter reduces her dose and stops. I really didn't like the idea of being on them all the time so we decided to get out son off them as soon as possible as they weren't working anyway and he now has a note on his file not to commence steroids. Believe me Tmujin, my son has survived some horrific episodes, he has had over 40 hospital stays with 10 ICU stays. He has vomited blood, urinated blood, but at the end of the day it was a slow scary process but has has been major bleed free for 3 years now and is classed as being in remission now. We still have to watch him carefully, especially in winter, but he plays sport and lives the normal life of a 7 year old, even with all his lung damage. It does get better, you just need to hang in there. Where do you live, we are in Australia. My thoughts are with you and your family and I hope you daughter is back to health soon. Keep safe and well xx
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Post by Mattguest on Sept 26, 2012 11:01:13 GMT -6
Dear Cooper and all the other active writers,
thank you all so much for this forum. It has been a great help so far. My son was diagnosed with IPH some months ago. Reading some of the studies published in the internet, that was quite scary. Fortunately he seems to have a mild form of it, at least some of the doctors say that, since his Hb levels are almost normal and the percentage of iron-laden macrophages just a bit over normal if 25% in some areas can be called normal. They even had and kind of still have problems in signing the diagnosis because of that, although they have eliminated all other causes of the bleeding they can think of. Still, he is bleeding a bit almost every day despite pulse therapy with steroids and daily hydroxychloroquine. I have the feeling that the steroids just decrease the severeness of the bleeding if they help at all, but we haven't had the courage yet to try our luck without any medication and see what happens then. Reading this forum 6MP seems to be like a wonderdrug. However, I don't know how high the price of it might be in terms of side- and long-term effects. You seem to have some experience with it. Did it really stop the bleeding completely in your case? How did your son take it? Also my son experienced quite some pain in the lung area in summer, which he still complains of but has gotten kind of used to it. The doctors say it might be pychosomatic. I'm not so sure about it. Does anyone have experience with that?
Best wishes to all of you
Matt
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Post by cooper on Sept 29, 2012 18:14:40 GMT -6
Hi Matt,
Sorry to hear your son has IPH, I would highly recommend staying off the internet for information as it is quite outdated and not very positive at all. It would be lovely if your son has a mild form as at it's worst it isn't very nice, but it is amazing out resiliant these kids are. Our diagnosis was made final with a lung biopsy and if you haven't had one yet I imaging that would be where you end up.
After 5 years we are still on hydroxycholorquine but have tried all other medications. The problem with steroids is the real side effects and if don't believe they are working you should asked to be taken off them. We did as they didn't make any significant difference and the negatives outweighed the benefits we were getting. It's hard but in the end we didn't want all the next problems after the IPH had been controlled (and yes, we didn't know that we would get to that point but he has and has no problems from steroid use). Unfortunately when changing medications you don't know whether it is the medication controlling the bleeds or the body. Everything takes time and you just have to be patient. For us we tried 6MP as a last resort as we had tried everything else. He started it (as a small caplet) in ICU and we haven't been back since. That was 3 years ago. If your son is having constant small bleeds, ask you specialist to look into Tranexemic Acid. It is used everyday for people who have excessive bleeding and it blocks the molecule that breaks down blood clots so the bleeding stops quicker. We have been on it for years and it is completely safe. It's definitely worth a try and I have always believed this significantly reduced the severity of Oliver's bleeds and stopped them quicker. As well as stopping the everyday microbleeds. His Hb is now constantly above 110.
Sorry I can't help with lung pain as my son doesn't have any. I'm pretty sure I have seen IPH-Net (the moderator) write about his daughter having this problem so you may want to message him with any questions. He may have some useful information for you.
I hope some of this helps and I am always happy to answer any questions you may have. Sorry I don't have many answers and each case is difference but I am happy to tell what has worked and hasn't for us.
Wishing you, your son and your family all the best.
Regards, Lori Cooper
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Post by tmujin3 on Oct 25, 2012 19:38:31 GMT -6
Hi Cooper,
Thank you for your input on my previous message. I live in Los Angeles California in US. My daugther recently got discharged from hospital about two weeks ago. Her reticulocyte was still high about close to 10% which indicated she has still has bleeding but they let us go. We were there for more than 8 weeks. Right before we were discharged, they started new treatment. She's now on Cytoxan, monthly IVIG, weekly high dose steroids, and weaning down on daily steroids. Her HB is normal and retics are reducing, the lab results seem to head for right direction.
As you mentioned on this forum, it seems keeping the white blood cell below 6 is helping not to bleed. My daughter is not on 6MP but after taking Cytoxan thru IV bi weekly her white blood cell is steady at below 6. We shall see if this continues.
Hope you and your son is doing well.
Thank you
tmujin
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evi
New Member
Posts: 10
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Post by evi on Oct 30, 2012 7:40:22 GMT -6
Hello to all, we made some ground research - now first results and questions. Please read the article in attachment! We are looking forward hearing from you and are ready to share our data and experience. Give us some feedback. Kind regards Evi and Hubert Attachments:
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Post by IPH NET on Nov 22, 2012 14:30:52 GMT -6
My daughter has presented with strange results. When she has a bleed the chest xray shows very little activity, we depend on her O2 sats to be over 90 if they are not we watch.. after a bleed her lungs are full of activity but she is feeling well. Go figure.
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Post by MattGuest on Nov 27, 2012 4:36:33 GMT -6
Dear IPH-Net,
although my son might be much better than your daughter having O2 saturations above 95 he shows a similar condition. Before he is bleeding he is having a lot of pain. After the bleeding the pain is much better and he is just tired. Also, can you do me a favor? My account with the username "Matt" is still pending, so I can't pm anybody, however I would really like to do so.
Best wishes.
Matthias
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Post by IPH NET on Nov 27, 2012 15:42:06 GMT -6
Matt, Check your account again. It is activated. If you have any issues let me know. Dear IPH-Net, although my son might be much better than your daughter having O2 saturations above 95 he shows a similar condition. Before he is bleeding he is having a lot of pain. After the bleeding the pain is much better and he is just tired. Also, can you do me a favor? My account with the username "Matt" is still pending, so I can't pm anybody, however I would really like to do so. Best wishes. Matthias
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