This is your chance

[miriam]

Hi Ann,

I hope you're keeping well. There's a very useful website uptodate.com that has a summary on IPH and treatment.

Send a message or via the bulletin board if you wish any help.

Regards,

Miriam

[cooper]

Hi everybody,

Just wanted to wish you all a merry Christmas and a wonderful new year. Hope you all have a safe and happy holiday and wish you all a very healthy 2011.

Best wishes,
The Cooper Family

[miriam]

Many thanks Cooper - a lovely thought. Hopefully we'll all have a lovely Christmas and a great year ahead.

Miriam

[evi]

Hallo at all,
we are from Italy. My son – now he is 5 jears old – was diagnosted with IPH 3 jears ago. But just with 7 mouth he had a pneumonia the frist time and a jear later the next one. So we think that this was probably the IPH.
Just for a long time we are looking for connection with other people, who have experience with this disease, but only today we found your net by chance and we are very gratefull for it.
The first time was very difficult, many times in hospital (3x intensive care ward intra 4 mouth), blood tests, medicals with side effects and above all the awful fear to lost our child. Now we learned to live with the IPH and to watch our son and to interpret the symptoms. At the moment he is well, he goes to the nursery school, he can do anything as the other children, too. But there are also episodes with serious bleedings into the lung. At the moment we are trying to determine the prednison therapy, so we will see…
We gave him only prednison and omeprazol for the stomach. Our doctor dissuaded from immuran and other immun suppressive medicals. Normaly he has to take 0,5 mg / kg body weiht (bw) every second day. When he had a haemorrhage, we enhanced the dose of prednison on 2 mg per kg bw 3 times/day. In the first time we started to analyse the defecations (we are both biologists). And so we found out an interrelation between eating bananas and amid in the defecation and in sequence often a haemorrhage. So we don´t eat bananas any more. But the haemorrhages didn`t stopp at all, alltough now they come scarcer. In the last year any morning we measured the urine with combour test (cobas). On this way we were able to recognize little haemorrhages also when our son didn`t have any other symptoms.

This is a short summary of the last 3 years. The very new information for us is the treatment with IGs and with tranexamic acid. Can you tell us more about it. Do the IGs work, because every cold and every cough seems to trigger the IPH. And so we often are in fear especially in the cold seasons.

Regards,

Evi

[miriam]

Dear Evi,

Welcome to the site. Blood in the stools (melaena) occurs after the lungs bleed as you know. For my daughter, it can be several days after a bleed before she gets melaena. I have an oximeter for my daughter and I check her oxygen once or twice daily. Her oxygen drops about 12 to 24 hours before a significant bleed. I find it very useful. Our oximeter is just a small handheld one. On a number of occasions, I brought her into hospital when her oxygen dropped because I knew that a bleed was coming so she was actually an inpatient when she bled. I find it useful to be able to tell the hospital staff what her oxygen is usually like because sometimes it only drops slightly (and at times not at all).

Keep in touch.

Take care,

Miriam


Evi caro,

Dare il benvenuto al luogo. Il sangue negli sgabelli (il melaena) accade dopo che i polmoni sanguinano poiché lei sa. Per mia figlia, può essere diversi giorni dopo un sanguina prima che prenda il melaena. Ho un oximeter per mia figlia e controllo il suo ossigeno una o due volte quotidianamente. Il suo ossigeno fa cadere circa 12 a 24 ore prima che un significativo sanguini. Lo trovo molto utile. Il nostro oximeter è appena un piccolo uno portatile. Su un numero di occasioni, l'ho portata nell'ospedale quando il suo ossigeno ha fatto cadere perché ho saputo che un sanguina veniva così era effettivamente degente quando ha sanguinato. Lo trovo utile a poter dire il personale di ospedale che il suo ossigeno è di solito come perché a volte solo fa cadere un po' (ed a volte niente affatto).

Tenere in contatto.

Prendere cura,

Miriam

[evi]

Dear Miriam,

thank you for your answer. I hope your doughter goes well.
We also have an oxymeter, but the oxygen is always on 96 – 98 % when my son does not bleeding. So we use the oximeter only when we have a hunch that he is bleeding. But still before the oxygen drops, the heart frequency enhances (over 130 -150!). This ist the moment to go to hospital.
In the first time he always felt so bad, that there was no doubt whatever that. But in the last time he had bleedings also without significant signs. But with the mensurations of the urin we can prove micro-bleedings - mostly in the course of a cold. In this case we enhance the prednison dosis.
By testing the stool we never found blood, only a huge amount of starch before the bleeding. The doctors confirmed the high starch-sugar into the stool, but they had no ecplications for that. So we began to keep a tally of all he ate, every bite! And there exist a significant coherence between eating bananas – starch and bleedings. Maybe other people have an incompatibility also. As you know, there is described a coherence bitween IPH and Coeliac disease… Anyway, we needed much time to ricover this. So we would be glad if this info could help an other person with IPH.

Best wishes
evi

[miraclemom]

My daughter was diagnosed at 1 and is now 7. She was hospitalized over 40 times, and had a really rough time from age 1 to 4. Then she was episode free for 2 years. before relapsing in 2010 June, and December. She is doing great now. What I have observed, is that her last few episodes came after stressful events. Once when she had surgery on her feet she had a bleed. The only treatment she is on at home is orapred, and prevacid daily, albuterol/pro air as need, and I hate the side effects. She is very small and looks like she is 3 or 4. Also her bones have been affected resulted in deformity in the legs which was corrected surgically. Her skin too is thin and peels. However, she is very active (does ballet, tap, and jazz - even competes) and excells academically. She is on daily dose of orapred, and efforts to wean her resulted in relapse. Whenever she has a bleed, she is admitted and given increased dosage of orapred by IV. It is interesting to know of others experience, and other medications being used. I was excited to read the success stories. Thanks for sharing.

[Rick]

Hi all, it has been a while since saying hello, my son Devon is now 11, actually at karate tonight and is doing well. Still on IV steroids every 6 weeks but has grown and caught up to the rest of his class mates (was on daily steroids and didn't grow for 2-3 years early on). No major bleeds or hospital visits other then his yearly bronch (last one was good). Had a ct scan last year and that didnt show anymore new scarring in his lungs so that was very positive. I wish everyone a happy new year and I promise to check in more often.

[miriam]

Hi Rick,

Delighted about the good news about your son. I was interested to hear that your son gets yearly bronchoscopies. My daughter only had one at the time of diagnosis her illness five years ago but she unfortunately bled quite badly after it. Thankfully her most recent CT was really good.

Hi Evi - interesting what you have noticed about bananas. I haven't noticed that with my daughter. She has been tested for coeliac disease but doesn't have it. It sounds like having coeliac as well can be help with the way haemosiderosis for those effected can improve with the coeliac diet.

Regards,

Miriam

[cooper]

Hi Evi and Rick, welcome and welcome back.

Evi, in regards to your query about IVIG's and tranexamic acid, my son is on both. We have been through all the medications and is now on 4 weekly IVIG's, the tranexamic acid, hydroxychloroquine (as an antifibrotic) and mercaptopurine (6MP). Since this started he hasn't had a serious bleed and has been hospital free for 16 months. He is no longer on any steroid as they didn't lessen the severity or frequency of his episodes.

I believe the tranexamic acid is a must have, and I find with the IVIG's he no longer has random viruses that cause bleeds. Still has self limiting bleeds with colds but not full episodes. Whether this is helped by the IVIG's I don't know.

If you want any further information, please let me know.

Regards,

Lori

[miriam]

Hi Lori,

My daughter isn't on tranexamic acid - using tranexamic acid makes sense to me. I'm just wondering if it'd be useful for us to know how many of our kids are on it so that we can share the info and the possible results with the physicians.

Regards,

Miriam

[Riccardo]

Ciao A tutti , scrivo dall'Italia ,anche nostra figlia Arianna ha la malattia. E' stata diagnosticata da quando aveva 8 anni ma con probabilità già da quando aveva 4 anni era malata.
Al primo ricovero aveva il valore di emoglobina a 2,7 (.........) ora ne ha 14 ed è sotto terapia steroida ( Deltacortene ) e immunosoppressore ( Azatioprina ) tutto sommato stiamo procedendo abbastanza, teniamo però sempre sotto controllo quando ha raffreddori o bronchiti che sembrano scatenare la malattia.
Ciao a tutti spero in qualche notizia nuova da qualcuno.

[miriam]

Dear Riccardo ,

You are very welcome to the site. Glad to hear that Ariadne is doing better now. It must have been very frightening for you when her Hb dropped so low. Infections seem to take a toll on our kids. Thankfully my daughter has been doing much better this Winter. She's had infections without a bleed. hopefully the worst of the Winter's over now.

She's on oral acetylcysteine (trial), oral cyclophosphamide, hydroxychloroquine orally, steroids, septrin prophylactically, azithromycin Monday / Wednesday/ Friday (trial as anti-inflammatory), calcichew D3 forte, ranitidine, lansoprazole, flixotide inhaler, and pain control medications (for nerve pain in her scar post surgery). She gets the flu vaccine annually and has had pneumovac.

Keep in touch,

Miriam

Riccardo caro,

Lei è molto benvenuto al luogo. Spero che sua figlia fa meglio ora. Deve aver spaventato molto per lei quando il suo Hb ha fatto cadere così basso. Le infezioni sembrano portare un pedaggio sui nostri bambini. Grazie al cielo mia figlia ha fatto molto migliore quest'Inverno. Ha avuto delle infezioni senza un sanguina. È sull'acetylcysteine orale (la prova), il cyclophosphamide orale, l'hydroxychloroquine verbalmente, gli steroidi, il septrin profilatticamente, lunedì di azithromycin / mercoledì/ venerdì (la prova come anti-infiammatorio) il calcichew D3 l'attitudine, il ranitidine, il lansoprazole, l'inalatore di flixotide e le medicazioni di controllo di dolore (per il dolore di nervo nella sua chirurgia di palo di cicatrice). Prende il vaccino di influenza e ha avuto annualmente il pneumovac.

Tenere in contatto,

Miriam

[riccardo]

:)Dear Miriam , thanks for you answer , i'm very happy.
For now in very good but we have fear for arrive the influence , here very very cold in winter and much snow.
I hope for other notice from you , thanks.
I'm sorry whit you for my english not perfect.
Salute Riccardo

[miriam]

Dear Riccardo,

It's a long road so it's helpful for us all to have one another. Many thanks to IPH Net for setting up the site. I can't speak Italian, although we holidayed at Lake Garda a few years back. It was beautiful. I'm using the online translator so I expect there could be quite a few mistakes.

Take care,

Miriam

Riccardo caro,

È una strada lunga dunque è utile per tutti noi avere l'un l'altro. Molti grazie alla Rete di IPH per prepara il luogo. Non posso parlare l'italiano, sebbene noi l'holidayed a Garda di Lago pochi anni di nuovo. Era bello. Uso il traduttore in linea dunque mi aspetto ci potrebbe essere degli errori parecchi.

Prendere cura,

Miriam