My granddaughter has hs she is 3: and had 6 transfusions already, last week dr told us they are concerned at her heart murmur as it is very loud so we've now been referred to cardiologist, her spleen is measuring at 7cm nd she says pain in her hip , h/ b is 7.2 at the moment so think
She'd may need another top up!!! It's worrying for me as both my 18 and 23 years old daughters and husband have all had their spleen and gallbladders out as both girls also had gallstones at 5 not very nice to see them in pain!! We've been told that Hollie will have spleen gallbladder and appendix out at the same time!! Very concerned about the heart murmur though , It's nice to know I'm not alone and advice is available, I knew straight away when my girls h b dropped below 7 as they were lethargic very pale nd sleeping all the time , and wee smelling strong and looking like tea , big hugs to mums looking after children with h s I know how u feel xx Well Hollie has a valve in her heart that needs closing over as her blood is going back into one of her chambers and been informed that this will be in the next 6 months, she has 2decaying front teeth due to meds that weren't sugar free and also been told that they have to come out before her op,but dentist won't do it because of her heart problem !!! So will have to go in specialist hospital to remove them!!! Worried sick but know tht she will be fine x
Worriedmum12 Worriedmum12
3 Responses Sep 23, 2012

Thanks ladies:) I haven't noticed it since so maybe it was the detergent on the cloth diapers making her urine smell. Idk. It's not brown though like you said. So having a 'crises' is that when their hb drops?
You see, my daughter is only 3 months old- idk if her case is mild or severe right now- the drs don't even know. They say that she is so young and that we need to wait until she is 6-12 months I guess to see if her body is going to level out. Right now it's looking like frequent transfusions. When she wa born she had super high jaunduce 13.4 at 12hrs old. High for the age. Then after she had to stay a couple of days we got it down and then it would return to 16.4 which was the highest. She was so yellow for over a month and then she turned pale. I didn't catch it:( my husband is a red head and my oldest daughter has my skin so I just thought she got his skin color. At 9 werks her hb was a 4.7 with a bili of only a 2.5. she was rushed to tx children's and got 2 transfusions. After discharge we did weekly CBC and when she was 13 weeks she dropped to a 7 and was systematic (fast breathing and heart rate, not nursing as well, extra sleepy, etc)
She got more blood and her hematologist confirmed that she has spherocytosis after checking mine and my husbands blood. She is the first in the family to have it.
I can't seem to really find answers on how her quality of life will be- if she will catch viruses more than others, Idk how to protect her or what I should be doing:( my oldest daughter is so healthy and my youngest is the opposite. I hope I don't miss the signs - she is just a happy baby I wish I could fix her.

Hi once she has her spleen removed she will be susceptible to infections as has no immunity then, so will need hib / phneumovac / menigeccocal and flu jabs throughout her life to protect her , my daughters have hib nd phneumovac every 5 years now but are both fine , just ensure she is vaccinated if you travel to other countries if any protection you need, but my husband has the same disorder he is now 53 and leading a normal life , you won't miss any signs sounds like you're doing a good job already x

Thank you:) her hb dropped from a 9.2 last Tuesday to an 8.2 today so I am pretty positive she will need another unit of blood next week or a few days after:(

I've also read somewhere that if she gets sick (like a virus or something) that her rbc will bottom out and she will need to go inpatient and get more blood. I wonder how true that is or if it is only in kids with severe hs. Idk
Taking this day by day.
I'm glad I found this site:) there isn't really a lot of groups that talk about this- I'm glad that I can understand what we are facing through real life experiences since it is no where in our family:)

Hi the dark urine is. Build up of the bilirubin sometimes my girls urine went like that before they had a "crisis" when they went pale or yellow with yellow tinged eyes nd blood count dropped, they still get dark urine now in their 20's when they are ill after having the spleen removed at 5 I wasn't concerned its just a sign they are unwell, but I you are concerned always call the doctor just to put your own mind at rest x it's worrying for you I know and mum always knows best x

My daughter is 3 months old and has had 3 transfusions since birth. I was wondering about her urine odor and if I should be concerned or do something about that? Should I call her dr? It doesn't really look brown but I do notice a light brown color in an evening diaper every once in a while but not all the time and mostly when she is in disposables (I use cloth most of the time on her and the cloth diaper changes.

Sorry only just got the rest of your thread-Some people with spherocytosis have it mild and don't need operation but some are chronic , your doctor will tell you , my daughters and granddaughter were and are severe cases who are chronic, now my daughters never had transfusions just became ill and yellow and pale with temps high and gallstones so had spleens removed at 5 and haven't looked back , after their op they had growth spurts and first thing I noticed when they came back from theatre was rosy cheeks that I hadn't seen before, but I knew when hb was below 7
Now my granddaughter is different , she as had loads of transfusions and when she becomes pale lethargic and sleepy with dark urine I phone up the hospital and arrange bloods to be done and if its below 7 they transfuse, you can actually feel when the spleen is enlarged , a doctor showed me this, on the left under the rib cage it feels harder than the rest of their belly which is soft, but my hospital is great I can phone if concerned and they will do blood test that day I I need them to.

Hi there. I can see this is an old thread but i'm in need of just a chat! My daughter is 2 years old and was diagnosed with HS at a week old. She is very poorly and has severe HS. Just 3 weeks ago she had a transfusion and already her haemoglobin has dropped to 7.1, she is booked in or another blood transfusion tomorrow. The condition is not in our family and we are learning as we go but its so scary. Her consultant is concerned at the minute as she went a whole year without needing a transfusion(had around 10 in her first year) and has now suddenly become bad again. Her liver is enlarged now and so is her spleen. She also has an atrial septal defect(heart condition) that requires surgery. She suffers from seizures and fainting. But the doctors tell me that all these 3 different conditions are not connected in ay way. I really think differently and feel like there is a connection, does anyone else with this condition suffer with seizures or have a heart defect?? I believe this condition needs more research and not enough is known about it. My daughter is always bouncing around no matter how low her HB is, but as a mother all i do is worry, her tummy is so so big and swollen. Her eyes are always yellow and she is as pale as pale can be. Any advice would be great. Thankyou