Bone marrow transplant is the only proven therapy that can cure sickle cell disease. It is built on the premise that sickle cell disease is a blood dyscrassy with a genetic inheritance.The bone marrow is home to the process of erythropoesis where new blood cells are made. All formed blood cells(erythrocytes, leukocytes, and platelets) have their genesis from a single blood stem cell known as Hemocytobast. Most blood cells have specific life spans and therefore have got to be replaced; for example erythrocytes (red blood cells) have a life span of 120 days. A person with sickle cell disease  has a very short erythrocyte life span due to destruction of red blood cells during sickling.

It therefore makes sense that ridding the body of a bone marrow that produces sickled erythrocytes (due to a genetic predispostion) and replacing it with a normal one from a donor who is free of sickle cell will provide the cure. A successful bone marrow transplant begins with identifying a matching donor. An intensive workout for both the patient and the donor is undertaken. Both donor and the patient have to be screened for multiple viruses, bacterias, and other pathogens. Other diagnostic tools are also used to help rule out any potential causes of complication during the therapy itself. Such diagnostic studies may include MRI, chest X-rays, KUBs, Trans-cranial Doppler, Ultra sounds, and CAT scans.

The American heart Association recommends that sickle cell patients under 15 years of age, who have one or more sickle cell crises, or who may have had acute chest syndrome, or who are at risk of cerebral vascular accidents (strokes) are ideal candidates for bone marrow transplant. Unlike other transplants like kidney or heart transplants, a bone marrow transplant needs to have a matching of above 90% to be successful (HLA compatability).

The therapy begins with wiping away the patient's bone marrow with chemotherapy and replacing it with a sickle cell free bone marrow. The biggest threat during the therapy is infections. Chemotherapy suppresses the body's immunity by wiping away not only the erythrocytes but the leukocytes and of course the platelets; thus robbing the patient of any ability to fight diseases. Prophylactic antibiotics, antivirals, and anti-fungals are initiated. Strict sterile techniques for any invasive procedures and the magic hand washing is observed. Most bone marrow transplant centers are manned with trained health-care professionals who observe the necessary precautions. Reverse isolation is also initiated and all measures are taken to protect the patient during this difficult time.

The major complication of bone marrow transplant is rejection. Host VS graft disease or Graft Vs Host disease is always looming out there. Patients have to be started on anti-rejection medications which depending on individual response/progress may be permanent or may be discontinued. Follow up with the provider for at least a year may also be recommended.

Our family's experience

Our daughter who is seven years now has been battling sickle cell since birth.During her seven years of life she has been hospitalized 35 times not counting the numerous emergency room visits, and follow up visits to the sickle cell clinic in Detroit, Michigan and the Hematology/Oncology clinic in San Antonio, Texas.In June last year 08 we took the bold decision to go ahead with a bone marrow transplant.This followed worrying results from her trans-cranial Doppler which predicted that she was at risk for a cerebral vascular accident (stroke). We were given two options: starting her on chronic blood transfusions or doing a bone marrow transplant. Chronic blood transfusions can deter CVA but also come with their own complications including iron overload and possible transfusion of contaminated blood.

Back in 2003 when her brother was born we were able to have his cord blood stored in a cord blood bank in the hope that one day it may help in treating his sister.We also saved cord blood from the youngest one who was born in 2007. For the rarest of luck, both siblings were perfect matches with big sister!!! We therefore went ahead and had the procedure done at Methodist Children's Hospital in San Antonio on the 29th of October 2008.Today she is 86 days post transplant and she is free of sickle cell as evidenced by two DNA analyses done so far.She had her marrow from the five year old brother including his cord blood saved at his birth in 2003.

Perhaps the best outcome from this experience is the hope and interest that it generated in Uganda when her story was broken.Parents ans patients who up to now had given up to fate were once again excited that there is a cure after all for sickle cell disease!! Granted that this therapy is still beyond the majority of Ugandans but who can tell what the future holds!!!

Ruth Nankabirwa who is the chairperson of the Sickle Cell Association of Uganda was in the United States when our daughter was getting her bone marrow transplant.Following her return to Uganda she was able to organize a press conference in which she communicated to the press the good news.We have recieved numerous e-mails from parents in Uganda and the United kingdom who want to know how they can have the same procedure for their children.

What makes the procedure expensive is that patients need to stay in the United States or Europe for at least a year after the procedure for closer follow up by the transplant team.Like any tissue transplant, the danger for a rejection looms high and closer and frequent monitoring is very important.

About 96 ml of preserved cord blood from son was infused into my daughter