Succor for children with sickle cell

With the training of radiologists, pediatricians, hematologists from the six geo-political zones in Nigeria on the use of TCDs, the risk of children who are prone to having stroke can now be early detected and prevented.

Nigeria is home to the largest number of children with Sickle Cell Disorder (SCD) and a devastating complication of SCD is a high prevalence of strokes in children with sickle cell anaemia. Although interventions for stroke prevention are feasible in identified children with Transcranial Doppler (TCD) screening for determining those at high risk of developing strokes but TCD facilities in Nigeria are no more than 3 in a large country with 36 semi-autonomous states.

To this end, a Train-the-Trainer (TOT) capacity building training programme was held recently at the National Sickle Cell Centre, Lagos.

Over 40 million Nigerians are healthy carriers of the sickle cell gene (Hb AS), while over 150, 000 babies are born each year with sickle cell anaemia (Hb SS). About 1 in 14 children with sickle cell anaemia have a high risk of developing strokes (overt or silent).

Sickle cell anaemia is a genetic (inherited) blood disorder in which red blood cells, which carry oxygen around the body, develop abnormally. Rather than being round and flexible, the sickle red blood cells become shaped like a crescent (or sickle). These abnormal red blood cells can then clog sections of blood vessels leading to episodes of pain which can be severe. These episodes are called sickle cell crisis (also known as a vaso-occlusive crisis).

11-year old Desmond Donkgwap has come a long way. At age nine, a sudden stroke put him in a coma and he had to be admitted in the hospital.

“There is nothing new to managing sickle cell because I’m SS too but it was really challenging when he had stroke,” Ruth John, 43-year-old Desmond’s mother told Daily Times.

“I wasn’t there when he had the first stroke because he was with my younger sister. They were in the car and the next thing, she realizes that he wasn’t talking. She thought he was joking and she called to inform me. I had to rush home from work and I realized that he wouldn’t answer me while talking to him. He was in pains.

“I took him to the hospital and I couldn’t believe it when the doctor told me it was a minor stroke. Afterwards, he was admitted in the hospital and taken care of.

“I was surprised he had another stroke in August this year again. There was no symptom to tell me he was at risk of having stroke at all,” she said.

Continuing, she said, Desmond’s academic performance has been poor after the stroke attacks. “He used to come 1st to 3rd in his class but now, his performance is poor. Though, he has improved ever since we started coming to Sickle Cell Foundation Nigeria (SCFN).”

An associate professor of radiology, Kofo Soyebi said, “if there had been a TCD scan, the case of Desmond would have been prevented. With the scan, you see whether there is a risk and if you pick any, there are management.

“There’s what they can give-whether blood transfusion or hydroxyl urea which help them stay without the sickening blood for a while but you have to keep doing that. Why they have stroke is because the blood cell is abnormal, it will now flow and block a vessel. When that happens, that part that is not getting blood dies.”

However, going for TCD scan depends on what is found, if what is found is standard risk- the risk that any SS patient can have, then the patient will be told to come back the next year for another scan.

The second category is conditional risk, “for certain situation, the patient might be prone to stroke but such patient is advised to take plenty of fruits, and advised to come back in two weeks. If it is still conditional, then the doctor tells the patient to come every six month, but if it is high risk, immediately you see the doctor,” explained Soyebi.

“The stroke can happen in the brain, bone and the organs of the body. If it happens in the brain, the child can’t move hand, the child can’t support himself,” the associate professor said.

Adding, Chairman of the SCFN, Prof. Olu Akinyanju said, if a child has a stroke, he has 70% of having another stroke within 2 years.

“The way to prevent it is to give them blood transfusion every 3 week to put A blood in them so that the S is pushed down to 30% but that’s a very challenging thing in a country like Nigeria. Fortunately, if you take hydroxyl urea appropriately, it can also prevent stroke.”

Experts’ Experience

One of the trainees, who is a Lecturer/Consultant Hematologist at the Barau Dikko Teaching Hospital, Kaduna State, Dr Ijeh Ifeoma said, she sees patients with sickle cell anaemia every day “and I see what they go through, I can’t pretend to know how they feel but as a human being and as a doctor, I can empathise with what they suffer and I’ve actually had to manage a lot of patients who already had a stroke

“On the average, the paediatric clinic which runs every week with the younger children, they have about 80 patients per week and that’s just the scheduled visit, not those who come with acute problem. On the adult and adolescent side, we see about 25-30 patients per week. Between 5-10% of my attendee already have a stroke. The problem has always been that you can’t always know beforehand who will have it and that’s why this equipment will add value but what we’ve been doing so far is the patient education. We educate them that the earlier they come to the hospital after they have a stroke, the better the chances of recovery.

“So, imagine that each week, a different person comes and that is not even a tip of the iceberg because Nigeria has so many challenges-financial, social cultural and lots of people don’t even access these facilities. A lot of people are suffering at home or going to their road side patent medicine store or utilize alternative medicine. There are so many people out there that we are not yet reaching, so we are hoping that things like this will open up the door to more people and be more willing to access healthcare.”

At the conclusion of the training, new TCD equipment was given to representative Institutions from each of the zones, which will in turn use the equipment provided to develop capacity on use of this equipment in their zones. This will result in: increased numbers of TCD Trainers; number of TCD Sonographers; six additional TCD centres across Nigeria thereby increasing access to this facility; strengthening available database which can be available for further studies; integrating TCD screening into routine management of children with SCD.

Dr Oliemen Peterside, a pediatrician at Niger-Delta University Teaching Hospital, Bayelsa state said stroke in children with sickle cell is common, “we see patients in the clinics with stroke. It’s a very devastating experience for both the child and the parents because it impairs the child’s quality of life and movement and we know that if there is a means that it can be prevented, it will be very good.

“Before now, in my clinic, we do not have this TCD machine and we didn’t even have the skills to use the machine, so we just counsel them on general prevention, we didn’t know who is at high risk or at standard risk or low risk. We just assume that all our patients with sickle cell has a risk and we gave them general prevention counseling and those that eventually come down with stroke, we refer them to Lagos to come for TCD scanning.”

She said, averagely, 50-100 patients for sickle cell clinics, “some come and stop coming, some get above 17 years and go to the adult clinic. I think between 5-10% of them eventually come down with stroke.

“Now, we have been trained and given a machine to go home with and from now on, we will scan all our sickle cell patients and categorise them into the risk category and for those that are at high risk, we will take measure to prevent stroke. It will drastically reduce the incidence of stroke in our patients.”

Dr Rachael Akinola, Associate Professor of Radiology at the Lagos State University Teaching Hospital, (LASUTH), Ikeja, said, the experience she had at the training was an eye-opener.

“It made a lot of influence to me, I would have regretted not coming. It is for one to diagnose possible stroke in sickle cell patients and it will help us a lot because of the prevalence of the incidence in Nigeria.

“Being able to diagnose it early will help the early management and prevention of the stroke. When I get back to LASUTH, I will make them realize we need this machine urgently.”

Way forward

Soyebi who is also the Principal investigator of the project said, “we’re doing letters to state governors and commissioners of health and we need more of TCD scans. The machine can’t be more than $20,000, We can afford it, even buying one for a state is not enough.

“What bothers me was the day someone came from Port Harcourt for a test at the centre here and I thought people need not travel for such. Every town should have one TCD, at least every state, so that people won’t have to travel far to access it.”