Sickle Cell Disorder (SCD) is prevalent in tropical Africa especially in Nigeria. We have about 2-3 percent of the Nigerian population having this disorder and about 30 per cent of the population carrying the gene. Record also show that Nigeria has almost 200,000 of infants born annually with sickle cell disorder, and this is a big problem in Nigeria. LARA ADEJORO writes.
At the sickle cell clinic of the Lagos University Teaching Hospital (LUTH), Idi-Araba, Lagos, the number of sickle cell patients is overwhelming, one doctor can see up to an average of 30 patients in a day.
However, Dr Olusola Olowoselu, a haematologist and stem cell transplant physician at LUTH says bone marrow transplantation is cure to the disorder. Hence, the patient will live a normal life free from the complication of sickle cell disease however the patient’s genotype remains unchanged, he/she can still transmit the affected gene to his offspring. We usually advise such patient not to marry someone carrying the trait or someone with the disorder so that their children will not be affected.
” Sickle cell anaemia is a severe disease which is an inherited haemoglobin disorder. Instead of having the normal adult haemoglobin that is very soft, doughnut shaped and able to transverse the blood vessel easily with a life span of 120 days, the haemoglobin in this case is sickled and confers on the red cell the rigidity and insolubility. The clinical implication of this is that instead of the blood to move freely in the blood vessel as it occurs in an individual with normal haemoglobin, it becomes slow and gets stuck in the blood vessel. This then give rise to the clinical consequences associated with this disorder. For an individual to have this disorder, he/she must have inherited from both parents a double dose of the abnormal gene that codes for the haemoglobin S. When an individual inherits one abnormal haemoglobin gene from one parent, we call that sickle cell trait and such individual under normal circumstances is haematologically stable and free from the clinical complications or crisis associated with this disease.,” Dr Olowoselu said. Explaining further, he said, the presentation of the disorder can be acute or chronic.
Early Signs and Symptoms
If a person has sickle cell disease (SCD), it is present at birth. But most infants do not have any problems from the disease until they are about 5 or 6 months of age. Early symptoms of SCD may include: swollen wrist especially the hand when they are still young, that is the first sign and as they grow up, they come down with pain all over the body called sickle cell pain or crisis. Sickle cell patients present with anaemia, some of them have acute chest syndrome and some develop stroke which is known as cerebrovascular accident, some have persistent painful erection of the penis that usually occur around the age of puberty. All these can cause unpredictable setback to the patients.
The management of this disorder involves multidisciplinary approach and it revolves around prevention of complications and aggressive treatment if they occur. The caregiver, parents, paediatricians, psychologists, surgeons, haematologists have to be involved in the care of sickle cell anaemia.
“It is necessary that the patient must avoid precipitating factors like dehydration, stress, extreme of cold and all infections must be treated adequately. The use of hydroxyurea was a major breakthrough in the management of sickle cell disease; it reduces the number of frequency of crisis in them and dramatically improves their clinical outlook. Another treatment that serves as a cure is Bone Marrow Transplantation (BMT) which we refer to as haematopoietic Stem Cell Transplant because we realised that the source of this is not only from the bone marrow, it can also come from peripheral blood (when you draw blood from the vein), at the same time it can be gotten from the umbilical cord blood of a newborn.,” Olowoselu said.
Bone marrow transplantation is a treatment modality in which graft or stem cell called Haematopoietic and progenitor stem cell are infused to the patient to normalise their haematopoiesis and there are three types of bone marrow transplantation based on the sources of the stem cell. It can either be allogeneic, autologous or syngeneic types.
He said, ” When it is allogeneic type, it means the transplant from one individual to another individual. When it is autologous, it is about giving yourself what has been taken from you. Syngeneic type is when the transplant is between two identical twins.
” In sickle cell, autologous is not practicable; allogeneic is what we do presently. It’s a very complex medical procedure, donors will be selected through a molecular testing called HLA (Human Leukocyte Antigen) typing to know the person that is genetically compatible with the patient this is very important. However, one can get someone who is not 100 per cent compatible with the donor but at least with some degree of match for the transplant. Afterwards, the individual’s stem cell would be wiped out by chemotherapy to remove the defective cell from his body and to create space inside the bone marrow so as to introduce a normal stem cell and infuse it just like infusing blood, this is usually done in an isolated room with positive pressure. From that day, the patient’s immune system will be monitored to ensure he/she doesn’t have infection.
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Adding, he said, The success rate of the stem cell transplant is about 90 per cent. Though a few persons have complications like having what we know as Graft versus host disease and infectious complication. The mortality that may arise from this procedure is about 10 per cent. After a successful transplant the patient will live a normal life free from the complication of sickle cell disease however his genotype remains unchanged, he can still transmit the affected gene to his offspring. We usually advise such patient not to marry someone carrying the trait or someone with the disorder so that their children will not be affected. Our concern is that the patient after the transplant will no longer have crisis or complication resulting from the crisis. If what is left for the patient is who to marry, then he/she can choose someone who is AA; marrying someone who is AS is not advisable but if marrying an AS individual is the patient’s choice, then the wife while pregnant can do prenatal diagnosis to find out if the unborn child is an SS carrier.
” The number of sickle cell patients in sickle cell clinic is overwhelming. In LUTH, when we start a sickle cell clinic in the morning, you might not leave the clinic until 5pm or 6pm and one doctor can see up to an average of 30 patients in a day. Apart from placing them on the routine drugs like folic acid and Paludrine we also counsel them on how to avoid precipitants and live a healthy life so as to reduce the frequency of crisis,” he noted.
Cost of BMT
The expert said, The cost of treatment is prohibitive. In America, it’s about $150000 to $200000 which is about N40 million to N50 million. In Europe, it’s about N30 million. The Mediterranean Institute of Haematology, Italy in collaboration with LUTH, Sickle Cell Foundation Nigeria (SCFN) as the facilitator has done free transplant for fifty (50) Nigerian sickle cell patients; and this is free of charge.
Challenges
Olowoselu said one of the challenges is that, ” Patients often can’t afford medications as low as N1000. Patients may not have money to come to the clinic for their routine check-up.
“I was attending to a patient who had leg ulcer as a result of sickle cell complication sometimes ago but unfortunately, he couldn’t get money to get what he needed to dress his wound. A lot of them come from poor homes and that’s a big challenge.” The implication of this is that majority of the patients can’t afford the potential cure of this condition.
However, the Lagos State Governor, Mr. Akinwunmi Ambode has pledged the support of the state government to see to the completion of the Bone Marrow Transplant Centre at LUTH, which will go a long way to save the lives of those suffering sickle cell disorder.
By: Lara Adejoro
Daily Times News
ABUJA: Training Schedule for Basic Life Support BLS, Pediatric Advanced Life Support (PALS), Advanced Cardiovascular Life Support ACLS, First Aid, CPR, AED
PORTHARCOURT: Training Schedule for Basic Life Support BLS, Pediatric Advanced Life Support (PALS), Advanced Cardiovascular Life Support ACLS, First Aid, CPR, AED
LAGOS: Training Schedule for Basic Life Support BLS, Pediatric Advanced Life Support (PALS), Advanced Cardiovascular Life Support ACLS, First Aid, CPR, AED
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