Anyone with experience of having Hereditary spherocytosis

[Naz2009]

My DD is 4 years old at age 3 she was diagnosed with Hereditary spherocytosis both husband I don't have it and neither do family members. Doctors have said it's a mutation in her gene.
She is on 5ml iron and 5ml folic acid daily.
Yesterday the consultant informed me to look out for slapped cheek syndrome and said I should ask the teacher to let me know if a case comes up in DD class or bubble. As it can be serious for HS patients.
When my DD has jaundice she is the opposite of being tired.
She'll have extra energy but not in good way. She'll get upset quickly. Very emotional. Have bursts of energy. Won't be sleeping in time. Waking up early. Yet will complain of leg pain and her appetite goes.
After a couple of days. One jaundice starts to clear up. She will be her normal self.
Just want to know what others have experienced. What advice I can get
Thank you

We have a family history of HS. My dad had it, me and two sisters have it and one of my DSs has it. We've all had our spleens out over the years to stop the jaundice/anemia/well cycle.

DS had his operation when he was almost 10. They were reluctant to do it (it used to be almost routine to remove the spleen as a treatment but the guidance changed) but he'd stopped growing and was anemic for much of the time. It was done by keyhole and it was a quick recovery. He's been well since, takes daily penicillin and has his vaccinations.

DS and my sisters' cases were diagnosed when they were very little but mine didn't manifest until I was 11. I went from feeling fine to being quite unwell quickly. I remember abdominal pain and vomiting with the enlarged spleen, jaundice and absolute exhaustion. I've been well since my spleen was removed when I was 12 though.

I'd advise letting school know that she might be more tired on some days and cutting her some slack. DS was allowed to sleep on a cushion in the corner at school if he needed to.

Hope that helps but happy to try and answer any questions.

Yes we have a history of it in my family too. Spleens removed, blood transfusions and the same supplements. I’ve found it gets easier with age. Symptoms less pronounced.

@BeBraveAndBeKind @Thethingswedoforlove thank you ladies for replying back.
Correction my DD is on 10mg of folic acid and 5ml iron daily. She has had 6 weeks of vitamin D and since then after consulting with her consultant I've been giving her vitamin D chewy vitamins.
She often complains of leg and sometimes arms hurting. Have you experienced this and what helps relieve it.
She will wake up crying and kicking and DH or I have to go into her room massage her limbs and she goes back to sleep.
Consultant explained when she jaundice and low blood count. The marrow in her bones realises premature cells to make up for low cells. This causes her pain.
She has started reception school and I have found she doesn't tell the teachers she is tired etc but then comes home and I can see the exhaustion which then leads her to becoming unwell with a common cold etc.
I have told her teachers but sadly as she is academically doing so well. They forget she has HS and perhaps needs an extra eye on her well-being.
I've also found when DD is distressed she gets jaundice right away. Is this something you can relate to?
For eg her dad went away for business trip for 5 days last year and she fell ill.
She started school and was being bullied and she got ill with Jaundice.
DD has always had a 300ml water bottle which she knows she has to finish before bed. That's not including the fluid she drinks during meal time.
I've informed the school to remind her to keep drinking from her bottle. As it's the only way to get rid of the toxins from her system.
So far I've not had experience of anaemia. Only find she gets jaundice a lot and also when she does get a common cold more than likely she needs antibiotics to help her.

We found that it was very much a cycle of jaundice - anemia - feeling okay. DS always did very well academically but on some days was so exhausted that DH had to carry him home from school. Just keep reminding the teacher that she has this difference and they need to take it into consideration for her.

It can affect people differently too. My sister was transfused as a baby and would fall asleep in random places. My other sister presented much more like my DS and had her splenectomy because she'd stopped growing and started to lose weight. We found that DS would have days where he'd be too tired to eat very much or would feel sick. It might be worth getting a diary and just making a few notes about how she is/what she's eating/how much sleep etc to see if you can see any pattern.

How often are you seeing the haematologist?

@BeBraveAndBeKind that's a very good idea to keep a diary.
I've found stress always causes DD to get jaundice and fall ill.
If her days has had to go away on a business trip for few days. She'll get jaundice, starting school this year, a big change in her life abs being bullied caused her back to back jaundice and illnesses due to it.
She sees the hematologist every 6 months.
This year both appointments where over the phone. However when I feel she is very unwell or I worry about anemia. I can take her into children's hospital and get a blood test done right away.
This is all new to me. DH and I don't have it neither do family members. So far I've found she gets jaundice. Almost every 4/6 weeks. Worse case she has had jaundice back to back in the space of 6 days after recovery.
She hasn't been anemic yet.
Bilirubin levels are always elevated every time she has had blood test. That's expected as she has been very yellow. Causing me alarm and taking her in for tests.

Hi sorry to hear it, OP.
I don’t have experience but just wanted to say... My friend has it, his father had his spleen removed etc. I don’t know what difference it made to his childhood but I can tell you that he is now one of the fittest people I know, thinks nothing of doing a half marathon for a jog or cycling hard for hours.
Hope all goes well with your little one.

I need advice. My DD is only 4 and I'm super worried about her going back to school next week.
I'm concerned about the new variant of covid and how safe is she.
Should she shield due to her condition. Since starting school, twice her year group has had to self isolate. Due to someone getting covid In her class/bubble.
Now during the Xmas break, there have been 3 more positive cases in her school and those children have been told to isolate during their holidays.
We are in Tier 3. Have been since second week of September. Ever since February this year. I've not visited any family or friends and not allowed anyone over.
I didn't even allow it on Xmas day. Purely for DD safety.
I work from home, DH is an estate agent so has to go office. As he is out. Primarily he been doing the shopping to.
I'm concerned due to her condition. Should I shield her. Am I being too over protective.

Call the haematologist's secretary and see if you can have aa quick call with them to discuss it.

DS and I are on the shielding list because if not having a spleen rather than because of the HS directly. We're in tier 3 and the advice for DS is that he can continue to go to school at the moment but if we move into tier 4 he'll have to stay home.

It's new to you and scary so I'm definitely not going to say that you're being over protective. It sounds like you're doing all the right things. It might help you feel more confident if you seek out as much reading as you can about what causes it etc. There also used to be a support group on Facebook. I left Facebook a couple of years ago so I don't it they're still going but it's worth a look.

I was 'lucky' because I grew up with it and knew what to expect with DS. I'd seen my mother advocating for treatment for us and so was able to do that for DS when the time came.

@BeBraveAndBeKind thank you for all your advice. I've read up on it loads. I'm not on social media, so i can't join a FB group.
You're very fortunate to be in the shielding list.
I pray my DD can also get on it.
I've decided to wait today to call the consultant. As I want to know what Borris decides about closing schools today.
There is this strong gut feeling inside me saying NO to school.
Last 2 weeks before the Xmas holidays. My DD fell ill. Had jaundice and then started to cough with fever. Covid was negative. GP gave antibiotics. Last week of school she wanted to go to school. As wanted to take part with the Xmas activity's. I sent her with antibiotics and second day the teacher told me. She had forgotten to give her the antibiotic after lunch and told me to give it to her when I took her home.
That night DD fell worse and threw up blood. She ended up in A&E turns out she had croup and had steroids for 3 days.
I can't imagine what she would be like if she got covid.
I just don't know how to explain and word it out to the school about my concerns of having DD In school.

I'd talk through your concerns with the consultant (after today's announcement) and see if they'd support you with a letter that you could share with the school. It's important that the school understand that her condition may affect her attendance record long term anyway or you'll be getting endless calls from the attendance officer.

We were lucky in that DS was always desperate to go to school so they knew that if he was off, he was really unwell and therefore didn't call about his attendance record.

Had a long discussion with DH. He said if you shield her and send her later. What's the chances she won't get the virus then once she is back. Risk is always there.
He said hope for the best and let her go to school, as she enjoys it.
He is right about her having a chance to pick up the virus when she is back at school after shielding. But I'm hoping the chances of that would be reduced.
I'm still going to call GP at 8 and get a consultation booked in and have a discussion with him/her see what their professional opinion is on the matter.

He's got a point but I'd be led by your doctors.

We've just moved into tier 4 so DS should be staying home (he's in sixth form so has a delayed start back anyway). I'm just hoping that he can get the vaccination in the next month or so so he won't have to be at home for too long. He still loves school and I'm worried about the impact to his mental health if he has to be at home when all of his friends are at school.

But if you wait before sending her back to school then maybe the majority of adults will have had the vaccine and it won’t be so rampant. For the sake of a few months if the drs say to I’d keep her off. Even if the drs didn’t think it was necessary I think I’d still keep her off.

I honestly can't thank you all enough. For taking the time to reply and advice me.
I've got the letter. Doctor has made it very clear about her condition and the consequences if she was to get covid. For that reason he has advised to shield for 12 weeks.
I feel so much more relaxed now. I'm glad I followed through with my guy instinct.

That’s good. Seems far safer.

That's good news. Hopefully the landscape will look very different in 12 weeks.

@BeBraveAndBeKind I do hope so.
Not sure how she'll cope once I tell her she sing going back to school. She is only 4 yet keeps talking about her friends and how she wants to go back to schools and play what games with whom. Your DS will be very upset no doubt. Fact he is a teen he must be super gutted. It's the age they want to socialise meets friends etc. I can understand your concerns in regards to mental health.
Keep safe. Happy new year

I hope it all goes okay for you both. Stay safe.