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HEREDITARY SPHEROCYTOSIS

csmp
2 Recommendations

Does anyone know of any new research or treatment for
HEREDITARY SPHEROCYTOSIS?
My husband, our two children, and now our grandchild has this blood disorder.

37 replies

3timehsmom

Hello,
All 3 of my children and their father have this blood disorder. The father and my 2 oldest have had their spleens removed and my 4yr old is about to start his immunizations for his spleen removal as well.

If I can be of any help just let me know.

Stephiesdad

My daughter, born 8.5.08 was diagnosed with HS. My wife has HS and had a splenectomy at age 7. Our daughter just had her first transfusion last week. We can't find any families who have kids with HS and the Pediatric Hematologists in our area have very little info for us. We live in CA 40 minutes south of San Francisco. Can anyone recommend Dr.s who specialize on this blood disorder?

MerisMom

Hi Stephiesdad

My daughter has HS as well. I highly recommend Children's Hospital in Oakland. Meri had her first transfusion at about 5 weeks old. They have a really stellar HemOc unit there and deal with HS all the time.

In fact, even after we left the Bay Area and moved to Atlanta, I still called our Oakland Doc for advice and she was happy to talk to me.

Here is her contact information:
ALISON T. MATSUNAGA, MD
Specialty: Pediatric Hematology/Oncology
Gender: Female
Primary Office
Pediatric Hematology Oncology Medical Group
747 52nd St
Oakland, CA 94609
Phone: (510) 428-3377
Fax: (510) 601-3916

3timehsmom

Hi Stephiesdad,

My ex-husband has HS and we were told the odds for our children was 50/50. All 3 of our children have the disorder and have had a tough time. I was curious, how old is your daughter? Only my youngest had to have a transfusion early on, at the age of 3 months, he is now almost 5yrs old and in a month received 5 transfusions. I have become more inquisitive as I begin to realize that this may never go away in our family. Stay on top of your little one's health and let me know if I can answer any questions. We have a great hematologist but we are in Atlanta, GA.

Stephiesdad

Thanks so much for the recommendation. Was your pediatrician familiar with HS. Can you recommend someone in the Bay Area?

Stephiesdad

Our daughter was born on August 5th, 2008. She's had 1 tranfusion so far. Our older daughter doesn't have HS and we were told 50/50 as well. I was curious what age they recommend having a splenectomy? My wife was 6yrs old and that's what our Hematologist has suggested for our daughter. I've also been reading about partial splenectomies. I'm glad to hear there are other people out there dealing with this. My wife and I feel like we're on an island.

3timehsmom

Yes, I understand the "island" feeling. Trust me when I say I feel like I am an honorary PHD in this disorder. I hope I can offer some help to you.

The "new" age for recommended spleenectomy is age 6 at the earliest. My 14yr old daughter had hers out at 5yrs old. My middle child who is 10 now had his out 1 month shy of his 5th birthday. My 4yr old will turn 5yrs old in November and he is now beginning his immunizations for his spleenectomy to take place either around Christmas or spring break.

My 4 yr old just got over "parvo" which caused his count to drop to 4 and he was not producing any red cells. He has just started pre-k and until this he had never been in a childcare facility. He continues to get sick a lot so the doctor decided to go ahead and get the spleen out. Right now he is having stomach aches that are being contributed to his spleen being enlarged and causing discomfort in that area.

Please let me know if you have any general questions as I will be more than happy to offer any information I can pass along. I will also say that even though I wish my children were perfectly healthy, there is always something worse they could have had. Best of luck to your and your baby.

MerisMom

Our bay area ped was not that familar with HS.

My husband's family is one of, if not THE, family that most early HS research was based on. My husband had his spleen removed by Dr. Diamond himself, one of the foremost researchers on HS, in 1970, I think.

My daughter's doctors, both in SF and here in Atlanta, have indicated that her spleen should stay in until it becomes necessary to remove it, if ever. Her hemoglobin numbers tend to hover around 9.5, although they often drop to about 7 when she's sick. (She was diagnosed with a far more serious disease, totally unrelated to HS earlier this spring and her hemoglobin dropped quite a bit but we avoided any transfusions). Her reticulacyte count is always quite high.

I've also recently learned that many, if not most, HS patients also have their gallbladders removed due to gallstones. Meredith has gallstones, but we're just going to wait and see what happens with that.

I haved a lot of confidence in our doctors at Emory/Children's Healthcare of Atlanta. We are very lucky!

tracy41

My daughter of 4 has just been dignosted with heriditary spherocytosis, 3 weeks ago.
The docters have her on Pre-K and Folic Acid,
what are the benefits of taking these medicines?
They are wanting to re-view and make a discussion
when she turns five in a years time to remove her
spleen.
Please can you tell me how severe the operation is to remove the spleen?
I live in South Africa, Cape town and am finding it difficult to find a support group.
I feeling scared and lonely, not knowing what to
expect.

Many thanks and kind regards

MerisMom

Hi Tracy

I'm sorry you've gotten this news, but if it's any consolation, I don't think you'll find it to be overly intrusive in your life. My daughter takes folic acid daily as well - I believe it's to boost red blood cell production in the bone marrow. I'm not sure what Pre-K is.

Our medical team has never been very eager to remove her spleen, so I can't say how the operation may go, but I know from discussing gallbladder removal that it probably can be done laproscopically meaning much smaller incisions and much shorter recovery time.

If they remove her spleen, will she being prophylactic antibiotics? That was the recommendation we heard when we last discussed it, but that was years ago.

Does your daughter's spleen frequently become enlarged? I have to say I'm surprised that she wasn't diagnosed until 4 - she must have done pretty well up until now! That's a good sign, right? Does her father have it?

Hang in there. I totally understand how scary this is, especially with a new diagnosis. But the real risks involved with HS are not knowing about it. Once it's been diagnosed, it's very treatable, and your lovely girl is going to be just fine. <hugs>

tracy41

Hi Merismom
Thankyou for your letter and comfort in it.

My daughter is taking Folic Acid once a day, and the
Pre-K is pencillian antiboic. The docters will review her situation next year, with regards to the removal of her spleen. My worries are, that I need to get as much info
with regards to this operation, and if they do it what are
the down falls, how dangerous is it etc.

Her spleen has not reached a "enlarged" state yet, and has not had a blood transfusion yet either.

I conceived my daughter throu our Infertiliy Clinic,
with the help of a sperm donar. My husband at the time had a vasetomy and could not have children,
which I dearly wanted, so went that route. We got
divorced before she was 8mths old, and he has no
contact with her. I have been single since and love
her more than anything else in the world. Divorced
and single with a small child, and only to hear the news now that she is ill, is going to scare anyone
away!! I have been told that we are, only given what
one can handle, I wonder somtimes in the cold light of dawn if this true....
Thankyou again for your kindness and HUGS

csmp

Hello Stephanie's Dad and everyone else,

We have never met another family with HS. We had a wonderful phyician named Thomas Miale in the beginning which was 1979.
Did your wife have any problems with her pregnancy? My daughter with HS has not had a child yet. I know that she and her husband would like to know if there will likely be any problems. They have only been married a few years but the time is coming to decide. Since I don't have it I can't be of any help to her.
Our son was diagnosed in May of 1979 at ten months. He was born jaundiced but there were no tests done to find out why. I had toxemia and we were both in the hospital for a week.
After many dissagreements with our doctor we changed phyicians and the new pediatrition did blood work and thought our son had lead poisoning. We were refered to Cook Children's Medical Center in Fort Worth. Dr. Miale found the HS and through searching through my husbands medical records found that when his spleen was removed at in 1972 at age 19 it was discovered. My husband's parents had chosen to not tell him.
Our son had many problems including gall stones and had his spleen and gallbladder remover at age 10.
My son's daughter age 4 will be tested again soon. They have changed physicians to see one that he saw when he was small.
She will now get help and information from the Cook Fort Worth Children's Hematology lab.

My pregnancy with my daughter was perfect. Everything went well and she was not jaundiced. She was diagnosed at three months. She was able to keep her spleen until age 13.
Our son had Human Parvo Virus when he was 7, and an unrelated surgery at 2 1/2, other than his spleenectomy those were the only times he had blood transfusions.
Our daughter had the virus too but did not need transfuions. During her laproscopic spleenectomy it was discovered she had an extra spleen that was hidden. The doctors had to open her up to access it. She has not had gall bladder trouble since her surgery.
Both of my children were on prophalatic penicillin before and after their surgeries. They both became allergic to it during adolescence and had to stop. We make sure that any illness with fever is watched closely. They are remarkably healthy now.
My best advice to anyone is to make sure that you find a pediatrition who sends patients to a hematology clinic regularily for check ups. Call the children's hematology clinic nearest to you to ask for a recomendation for a pediatrition who works well with them.
The things I am reading on this site about the differences in ages for spleenectomy surprise me. I was able to stay home with my children and not put them in day care. This was at Dr. Miale's request. When school time came along they were ill more often than the other children their age and missed many days at a time. I kept up their lessons at home when they were out.
Now I am doing the same thing for my granddaughter while my son and daughter-in-law work. I really feel that this is why my children were able to delay the removal of their spleens for so long. They were both classified as moderately severe.
Best wishes to you all, and any information on having HS during pregnancy would be very appreciated.

Stephiesdad

Both pregnacies went smoothly. My wife's platlet count was high but that's to be expected.

blessedbe1985

look this up on Clinicaltrials.gov they have a trial that is recruiting right now

Rose77

Hi, my daughter has been diagnosed with HS last week, she is 7 years old and we have been visiting doctors for almost 2 years till finaly they reach to HS diagnose they told us her spleen is large but they will not remove it now, maybe later in the future, her Hemoglobin level is between12-10 so they said it consider to be with min. normal limit, I want information about the spleenctomy? what should I expect ?I'm afraid that the doctor will tell us that she needs this operation in our next vist or near future.

mardia

Has anyone heard of research that suggests inherited spherocytosis and constant hunger are connected?

3timehsmom

I do not have anything new but my 5 yr old is headed to surgery in the morning (Dec 29th) to have his spleen removed. He is the last of my 3 children to have this procedure. Their father has it as well. Their father's niece is pregnant, due in May, and I will be very curious to see if her child has HS.

Thanks for keeping this topic alive.

csmp

I pray that all goes well with your son, and that you are comforted during your wait during the surgery.
My granddaughter is doing well even though she started preschool.
Let us know how things go this morning.
God bless you all.

3timehsmom

Thanks you csmp for your kind words. My son is home now and doing very well. Hopefully he will be like the rest of our crew and his HS will now be a minor blip on his health radar :)

Best wishes to everyone for a safe and Happy New Year!

IAS

My newborn was diagnosed at birth Now 8 months old had 3 blood transfusions in 1st 3 months of life, currently on iron and folic acid daily with regular trips to hospital for blood tests), but now my 6 year old has all the symptoms of it. She has been confirmed as anaemic. Going to doctors tomorrow to find out HB etc etc, I am praying its not HS.
Thanks for all the above info I have just read on here. - Its great to know I am not alone in this too.

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