My friend is going for splenectomy

[tony1111]

my friend who is having thalassemia major is going to have splenectomy (removal of spleen) done because the doctor said the spleen is becoming too big and it makes the lifespan of the red blood cells short.

Have anyone here undergone the same or similar surgery? Please share here. 

[caraiti]

Yes I have.
My was done back in the 70's when I was 7years old.
It was suppose to be when I turn 10 but my spleen was becoming too big that they had to remove it sooner.
The spleen tends to break down the red cells and certain parts of the cell are sent to the marrow to make new ones.
The problem with Thalassaemics is that the red blood cells that our bodies produce are not sufficent and tend to die sooner.
The theory has been that if the spleen is taken out, our bodies are able to last abit longer in between transfusions.
In my case I was able to maintain my red blood cells for awhile but as you grow, your needs increase and so do the transfusions.
It is a straight forward operation. Your immune system would be as it was but your friend can talk to his doctor about that as he can go into more details over his personal management.
I can only talk from my own personal experience.

[caraiti]

here is some more information, I found out which might be helpful to you

Some information is from:- http://www.thalassaemiaindia.org

Spleen
Spleen is a soft, purplish organ about the size of one’s fist. It is located just below the diaphragm on left side safely hidden under the ribs.
It is not palpable while examining the abdomen. A normal spleen contains only 20-30 ml of red blood cells, a very large spleen may contain 1 litre or more.

When Splenectomy?
Increase of annual blood requirement by 50% or more over initial requirement.
Presence of massive splenomegaly.
When size of spleen is 6-8 cm or upto the naval, splenectomy should be considered seriously.

Why Splenectomy?
Patients with Thalassemia Intermedia & Major invariably develop splenomegaly due to ineffective production of blood cells in spleen and iron deposition.
This hypersplenism causes increased destruction of red cells resulting in increased blood requirement, increased iron load & enhanced risk of transfusion transmitted infections.
In addition to destroying the older red blood cells, the overactive spleen also destroys some white blood cells(WBCs), platelets and young red blood cells, further worsening the anemia.
The red blood cells circulating in the system of the patients with thal have an abnormal shape because they lack normal Hb molecules, and often become trapped in the spleen - which adds to the size of the spleen.

Also, the spleen may also attempt to counteract the body's anemia by producing red blood cells itself (also called extramedullary erythropoeisis), which further contributes to the spleens enlargement

Advantage of Splenectomy
Spleen removal might reduce your transfusion requirement
A non-splenectomised patient requires approximately 180 ml of red blood cells/kg/year while a splenectomised patient requires about 133 ml/kg/year

Risks
Splenectomy significantly increases the risk of serious infection and preferably be postponed to after 6th year of age
Patient should be immunized against Pneumococcal, Meningococcal A & C & Haemophilus influenza B (HiB), 4-6 weeks prior to operation.

After Splenectomy
Prolonged Penicillin prophylaxis (250 mg bd) or injection Benzethine Penicillin 6-12 lacs every 3 weeks should be prescribed post Splenectomy.
Post splenectomy fevers/upper respiratory infections/any infection should be managed promptly.
Broad spectrum antibodies including amoxycillin/co-trimoxazole should be started at home.
Patient should be hospitalised if fever doesnot subside within 48 hours.
Aspirin 50-100mg/day may be advised if platelet count exceeds 8,00,000/mm3

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[caraiti]

This was posted on the Thalpal.

May be we are on a breakthrough for spleen issues with thalassemia. Read below

From:- http://www.cooleysanemia.com/bodies/body407.php

Weill Cornell Study Examines Spleen in Thalassemia


Quote
May 22, 2008 - Researchers from Weill Cornell Medical College may have discovered the precise role of a gene in beta-thalassemia, which may help to prevent removal of the spleen in patients. The research is published in the latest online issue of the journal Blood, the official publication of the American Society of Hematology (ASH).

In thalassemia, hemoglobin -- the oxygen-carrying molecule on red blood cells -- is mutated and non-functioning, resulting in a low red-blood-cell count. Common symptoms include an enlarged spleen, called splenomegaly, caused by a buildup of malformed red blood cells within the body. The spleen works to filter out these unhealthy cells in order to protect the body from harm, such as in a stroke, but eventually the spleen becomes over-stuffed and is commonly surgically removed (splenectomy) in order to prevent a potentially fatal burst. Unfortunately, after the spleen is removed, patients are at a much greater risk for stroke and infections.

Dr. Stefano Rivella, the study's senior author and assistant professor of genetic medicine in pediatrics at Weill Cornell Medical College, in New York City, believes that he and his collaborators may have found a way around splenectomy. After giving mice with Cooley a compound called JAK2 inhibitor, the researchers found that the mice's spleens shrunk to normal sizes, and they began to produce normal red blood cells. The chemical (a similar compound is already in a Phase I clinical trial for myelodysplastic syndromes -- another blood disorder) blocks the activity of the JAK2 gene that is highly expressed in Cooley's anemia, and is believed to play a crucial role in the malformation of red blood cells.
After splenectomy, patients are considered immunocompromised, and so should be placed on lifelong prophylactic oral antibiotics. Patients should also be vaccinated against common viral infections, and should receive annual influenza vaccinations.

Researchers are still learning about the health effects that stem from years of living with reduced or deficient globin proteins. An emerging realization is that removal of the spleen can cause an increase in the risk of life-threatening blood clots. Splenectomy is common for thalassemia patients because their red blood cells are crippled or dead, so the spleen has to work overtime and can become enlarged.

Researchers disagree on the degree of risk associated with splenectomy. A study by Dr. M. Domenica Cappellini, a co-author of this research, found that 30 percent of splenectomized thalassemia intermedia patients developed clots. However, a recent study of 8,860 splenectomized patients with thalassemia major and thalassemia intermediate found that the rate of thrombolytic events was 1.75 percent.

Surgical technique has played a role in developing clots (thrombosis). A recent study found that patients who underwent open splenectomy had a 19 percent chance of developing life-threatening clots, whereas patients who had laparoscopic surgery had a 55 percent chance.

Researchers are only now discovering why splenectomy leads to clots. Injury of endothelial cells may lead to a coagulation cascade, involving the activation of endothelial adhesion proteins, monocytes, granulocytes and platelets. Alternatively, the splenectomy might result in an imbalance between coagulation factors and anticoagulation factors. Dr. Cappellini has recommended short-term anticoagulants such as heparin after surgery, and prophylactic anticoagulants any time thalassemic patients are exposed to thrombolytic risk factors such as surgery, prolonged immobilization or pregnancy. Oral contraceptives should be avoided because they carry their own risk of thrombosis.

This study was funded by grants from the National Institutes of Health (NIH), the Carlo and Micol Schejola Foundation, the Roche Foundation for Anemia Research (RoFAR), the Cooley's Anemia Foundation (CAF), the Children's Cancer and Blood Foundation (CCBF), the Associazione per la Lotta alla Talassemia di Rovigo (AVLT), and the American Portuguese Biomedical Fund.

Dr. Rivella's co-authors and collaborators include Drs. Ilaria Libani, Ella Guy, Luca Melchiori, Raffaella Schiro, Pedro Ramos, Laura Breda, Amy Chadburn, YiFang Liu, Matteo Porotto, Patricia Giardina and Robert Grady -- all of Weill Cornell Medical College in New York City; Dr. M. Domenica Cappellini, as well as Dr. Libani, of Centro Anemie Congenite, the University of Milan; Dr. Maria de Sousa, as well as Dr. Ramos, of Iron Genes and Immune System (IRIS) Lab, Oporto University, Portugal; Drs. Thomas Scholzen, Margrit Kernbach, Bettina Baron-Luehr and Johannes Gerdes, of Research Center Borstel, Germany; Dr. Eliezer Rachmilewitz, of E. Wolfson Medical Centre, Israel; and Dr. John Hood, of TargeGen, Inc., San Diego.


About Weill Cornell Medical College
Weill Cornell Medical College -- Cornell University's Medical School located in New York City -- is committed to excellence in research, teaching, patient care and the advancement of the art and science of medicine, locally, nationally and globally. Weill Cornell, which is a principal academic affiliate of NewYork-Presbyterian Hospital, offers an innovative curriculum that integrates the teaching of basic and clinical sciences, problem-based learning, office-based preceptorships, and primary care and doctoring courses. Physicians and scientists of Weill Cornell Medical College are engaged in cutting-edge research in such areas as stem cells, genetics and gene therapy, geriatrics, neuroscience, structural biology, cardiovascular medicine, AIDS, obesity, cancer, psychiatry and public health -- and continue to delve ever deeper into the molecular basis of disease in an effort to unlock the mysteries behind the human body and the malfunctions that result in serious medical disorders. The Medical College -- in its commitment to global health and education -- has a strong presence in such places as Qatar, Tanzania, Haiti, Brazil, Austria, and Turkey. With the historic Weill Cornell Medical College in Qatar, the Medical School is the first in the U.S. to offer its M.D. degree overseas. Weill Cornell is the birthplace of many medical advances -- from the development of the Pap test for cervical cancer to the synthesis of penicillin, the first successful embryo-biopsy pregnancy and birth in the U.S., the world's first clinical trial for gene therapy for Parkinson's disease, and, most recently, the first indication of bone marrow's critical role in tumor growth. For more information, visit www.med.cornell.edu.

[caraiti]

To Tony1111,
I have posted as many info I could get at this stage, there is also another topic that I had posted before.
I hope that the information helps and although it might be over whelming to your friend to read...
my advice is read and make a list of what he wants to ask his doctor that way he is more informed and better prepared for his operation.
Apart from my scar, there is no sign to say I had the operation and as for getting sick, I just make sure I try to keep well, have my flu vaccines and if I start getting sick I go to my GP for a check.
My boss reckons that healthy people have more sick days than me so I must be doing okay.
Tell your friend I wish him all the best and that he will be out before he knows it.

[amanda]

Treatment for thalassemia depends on the severity of the condition. Thalassemia may be mild, moderate, or severe. A splenectomy, which is surgical removal of the spleen is needed for severe cases.