Anyone still here who has a child with Hereditary Spherocytosis?

[zisforzebra]

There used to be a couple of us (I used to have a different user name) but there haven't been any posts on it for a while. Just wondering if there's still anyone around who knows about it and fancies restarting a thread.

Just bumping myself, sorry

yep, me ! I have it as does ds - I had my spleen out aged 6 but ds still has his and is on 5mg folic acid a day -he is very well and seldom poorly !

Yay! I have it too and had my spleen out aged 12.

DS2 (6) has it and is also on 5mg folic acid per day. We have yearly check ups with the paediatrician and he's been mostly okay but in the last month has been very pale and quite jaundiced on some days. Usually we have cycles of jaundice, anaemia and then he's well for a while. He's also very tired and often needs carrying to school and back as he's too tired to walk. I think we might need to call the paed for an appointment before his yearly one rolls round.

poor little boy - i know that feeling well- when I was six I spent a lot of time lying on the floor and being carried upstairs -I used to faint too.
Ds is checked every year- spleen felt and generally checked over,plus blood test. They have mentioned about taking it out - I thought they'd leave it alone, but it seems that to avoid gall stones they might...am not looking forward to that-ds is bad enough about blood tests.
He sometimes looks pale,they noted this in his last check up but I would recognise any symptoms I would think.
Are you on anti biotics ? I'm not, just have the yearly 'flu jabs/meningococcal and pneumococcal injections. My previous GP never bothered about all this,but since moving out to Hampshire 5 years ago they seem to take it quite seriously here- there's even a spleen nurse !!

A spleen nurse, I didn't know such a thing even existed! I'll have to move to Hampshire!

I get yearly flu jabs but haven't been called for a pneumovac for years. I had a nasty reaction to the last one so I'm in no hurry to repeat that.

No, I'm not on antibiotics now. I took daily penicillin up to aged 16 but now I just keep antibiotics at home to start as soon as I come down with anything. Ironically, I became allergic to penicillin a few years ago after a 10 day course for tonsillitis.

DS is really good with the blood tests, he even slept through them as a baby. I think he'd be better off getting his spleen removed but I'd like to put it off a bit longer if possible (partially because I told him that I got a pair of kittens after my operation and now he wants a puppy after his)

The paediatrician is happy to keep him ticking over for now but did note that his spleen was more enlarged at his last check than the previous one. He's great though and always assures me that he'll see DS any time I think necessary which is nice.

surprisingly lots of people without spleens here it seems !
I think the pneumococcal jab made my arm hurt a lot and i felt rotten after it,so am not looking forward to that one again. I'd rather they left ds's spleen alone. My brother had his out as a precaution aged about 13.

My sisters both got theirs out when they were much younger than I was. One of them was very jaundiced at birth and was transfused at six weeks. She was four when she went for surgery. The other was seven but had missed most of a year at school due to the fatigue.

I wasn't properly diagnosed until I was 11. I'd been tested twice before (I'm the oldest and was tested each time my sisters were diagnosed) but it seems they ran the wrong test. Flu kicked off the symptoms in dramatic fashion. My mum drove my siblings to school, leaving me a little under the weather on the sofa and when she returned half an hour later I was canary yellow with jaundice! From then on it was pretty acute until surgery six months later. The surgeon gleefully told me that my spleen was "the size of a rugby ball!"

How old is your DS?

ds was 13 in June, the last appointment which was in February was the first in which they'd mentioned spleen removal though. Am just looking at the letter to our GP and it just mentions his haemoglobin as being stable, palpable spleen tip and he has a slight pallor.
I had a transfusion before my operation - I have to say I have never felt as poorly since as I did then !

I know what you mean. The unbelievable tiredness was terrible. I just remember sinking to the floor because my legs couldn't hold me any longer and the tummy pain as my spleen was getting larger and larger. I threw up regularly everywhere with the pain. I'd definitely prefer DS to have his out before he gets to that stage.

you were probably aware of more than me ,being older when you were poorly - can't remember tummy pain,though I suppose i must've had it.
I would be quite nervous about subjecting ds to major op and GA unless his spleen was beginning to play up though.

Yes, I won't ask about it unless it becomes particularly enlarged or if starts getting pain from it. He had an unrelated op when he was 15months old and, although it only took an hour or so, it was a horrible wait for him to come back from surgery.

yes,that seems sensible- meanwhile let's hope they stay well

Yes! It's good to know that I'm not the only one here with HS though, I usually get quite a baffled reaction from people when I tell them!

! at least we have an interesting condition !

My son has it. He was sick a lot from age 12 onwards. Finally had his spleen out age 21, and his health is much better.

hi onmyfeet - is he the first one in your family with it ?

Can I join you? My husband has hereditary spherocytosis and we had genetic counselling last year as we'll be trying to start a family one day soon. He had rather a tough start to life, though you wouldn't know it to look at him now, and so it is something that I think about pretty often. He's 35 though so it would be fantastic to talk to people with more recent experience.

He was born at the end of the second trimester, very suddenly, and his parents were in such shock that they forgot to mention that his father has (much milder) HS and had had a spleenectomy at 20. My husband had his spleenectomy at 7 - not sure if that's unusually early or fairly common, these days.

Anyway - there's no evidence to link the prematurity to the HS (and I suppose if there had been, the HS would have died out in generations where such tiny babies didn't survive) but I do ponder on it. Finding this thread has really pleased me as while I'm able to read the technical clinical explanations on other websites, it's so much more easier to relate to your stories.

hi attenborough..my spleen was removed at age 6 -it was very neccessary as I'd been in a crisis or whatever it would be called and it had to come out (have never felt iller in my life !) There is usually a 50:50 chance of your child inheriting spherocytosis - my brother has it but his dd doesn't- my ds does. Some children do suffer from anaemia/jaundice and ds has been fortunate not to have any crises of illness in his 13 years-he was put on folic acid once he was diagnosed (as i recall this is when they're about a year-but my memory isn't brilliant)He was born 2 weeks early and was jaundiced and kept in special care under a lamp for a few days.

Thanks, MaryAnn. Glad your DS is doing well. Was he checked for HS as routine, when he was newborn? I presumed that that would happen.

yes,they thought he probably had it from the fact that he was quite jaundiced but did check -they will do for any offspring with an HS parent. Ds might sail happily through life without it troubling him,though I think they advise removal of the spleen at some point to avoid gallstones.

MaryAnnSingleton, yes, only ds2, although some cousins on my side had it. Dh & I have not had genetic testing so we don't know for sure it is from my side or not. He is doing very well since having the spleen removed, he used to be thin and gaunt and so pale he sometimes looked translucent. He has gained weight and has pink in his cheeks now.
He said about a month afterwards, that it was the first time he woke up without feeling foggy that he could recall.
He used to be sick and have migraines, but not now.

DS doesn't want to have any children, because he doesn't want any child to be ill like he was. He and his gf plan to adopt instead. She was adopted and is ok with this.

poor boy,being so poorly :-(
We are off next week to see consultant for annual check up and blood test with ds.

Sorry to keep posting, I keep thinking of something else to add. Ds gets flu shots each year but does not take antibiotics unless he gets a fever. He caught strep throat the first year he had the spenectomy and was hospitalized for 2 days, to have antibiotics via IV. But this was only because the doctor prescribed an antibiotic he couldn't tolerate and vomited too much for the antibiotics to work their magic. Once he got them into his system he rapidly improved.

keep posting -it's interesting to know how others deal with this