spleenectomy for sickle cell child?
My boy is 23 months old and is sickle cell diseased SS. He developed his first spleen sequestration at the age of 12 months where his hemoglobin dropped to 4 and spleen measured 8 cm. His second attack was only 3 months later and since that time my son has been put on regular blood transfusion depending on his hemoglobin levels (i.e. if it drops below 8 then he gets transfused).
Initially the doctors told me that a spleenectomy is only done after 5 years of age but in some cases when a child sequestrates at such an early age then a spleenectomy might be considered earlier at 2 years old which might be the case with my son.
Now that my child is approaching his 2nd birthday i've asked his doctor if a spleenectomy will be done after my childs 24 months vaccination and he said no, they will keep him on regular blood transfusion untill he is 4 years old. This doctor is not the same doctor my son used to see over the first 19 months of his life and his previous doctor said that he favours doing a spleenectomy for my son at 2 years because the risk of regular blood transfusion is higher.
This doctor however thinks that the risk of spleenectomy before 4 years is higher than regular blood transfusion.
I am really lost and confused as to what is best keeping in mind that my son's iron levels are already high measuring 464 lats months but iron chelation will only be given when the levels are over 1000 according to the doctor.
I am really lost and dont know what to do or which doctors advise is better and my sons previous doctor moved out of the country so I can not reach him or get in contact with him and the current hospital my son goes to is really the best available hospital in the region where I live and it will be very difficult to meet another pediatric hematologist
- Anonymous7 years agoFavorite Answer
Lesher and Kalpathi (L&K)have been working together on "Removal of the spleen in patients younger than 4 years has been reported to carry an increased risk of postsplenectomy sepsis and has not been universally accepted." NOTE: They generally delay surgery until the child is at least 31 months old. L&K's 2009 article concluded that "The advantage of early splenectomy may outweigh the risks of long-term transfusion. Splenectomy in young children with sickle cell disease carries a low risk of postsplenectomy sepsis with appropriate vaccination and prophylactic antibiotics. We conclude that splenectomy in young children with ASSC is safe and effective, especially with penicillin prophylaxis and improved vaccination strategies." In 2010, L&K stated "Advances in immune prophylaxis, including new vaccines and daily penicillin, have made splenectomy a safe and effective treatment for children with ASSC."
Mouttalib (2012) and Vick (2009) both present strong cases for partial splenectomy, rather than complete removal.
The best way to reduce (but not eliminate) the risks is described in Howard's 2013 article in Lancet. Unfortunately, he lumped all the 1-6 year olds into one category. The Lancet paper is most likely to influence a British doctor.
Best wishes for a successful outcome.
-Howard J, Malfroy M, Llewelyn C, Choo L, Hodge R, Johnson T, Purohit S, Rees
DC, Tillyer L, Walker I, Fijnvandraat K, Kirby-Allen M, Spackman E, Davies SC,
Williamson LM. The Transfusion Alternatives Preoperatively in Sickle Cell Disease
(TAPS) study: a randomised, controlled, multicentre clinical trial. Lancet. 2013
Mar 16;381(9870):930-8. doi: 10.1016/S0140-6736(12)61726-7. Epub 2013 Jan 23. http://www.ncbi.nlm.nih.gov/pubmed/23352054
- Kalpatthi R, Kane ID, Shatat IF, Rackoff B, Disco D, Jackson SM. Clinical
events after surgical splenectomy in children with sickle cell anemia. Pediatr
Surg Int. 2010 May;26(5):495-500. doi: 10.1007/s00383-010-2587-4. http://www.ncbi.nlm.nih.gov/pubmed/20309565
- Lesher AP, Kalpatthi R, Glenn JB, Jackson SM, Hebra A. Outcome of splenectomy
in children younger than 4 years with sickle cell disease. J Pediatr Surg. 2009
Jun;44(6):1134-8; discussion 1138. doi: 10.1016/j.jpedsurg.2009.02.016. http://www.ncbi.nlm.nih.gov/pubmed/19524729
- Mouttalib S, Rice HE, Snyder D, Levens JS, Reiter A, Soler P, Rothman JA, Thornburg CD. Evaluation of partial and total splenectomy in children with sickle cell disease using an Internet-based registry. Pediatr Blood Cancer. 2012 Jul 15;59(1):100-4. doi: 10.1002/pbc.24057. Epub 2012 Jan 11. http://www.ncbi.nlm.nih.gov/pubmed/22238140
- Vick LR, Gosche JR, Islam S.Partial splenectomy prevents splenic sequestration crises in sickle cell disease. J Pediatr Surg. 2009 Nov;44(11):2088-91. doi: 10.1016/j.jpedsurg.2009.06.007. http://www.ncbi.nlm.nih.gov/pubmed/19944213