Sickle Cell Disease Care Center at FMC Keffi - Report Overview

REPORT FROM NORTH CENTRAL MDG SCD CENTRE, FMC, KEFFI By Dr Chinatu N Ohiaeri MBBS; FMCPAED, MPH Sickle Cell Support Society of Nigeria (SCSSN) Conference, Enugu. August, 24th2017

Introduction Sickle Cell Disease (SCD) is an inherited condition arising from presence of abnormal Haemoglobin S in the red blood cells and its reaction to low oxygen conditions by sickling Leading to a host of reactions including vascular endothelial reaction, Vascular occlusion among other pathophysiologic mechanisms. Gives rise to a variety of symptoms, signs, and complications such as Severe anaemia, Bone pains, Vaso-occlusive crisis, Acute Chest Syndrome, Predisposition to Sepsis, Stroke and Silent Brain Infarcts etc

Introduction-2 It occurs worldwide but it is prevalent in Africans, Carribeans, Asians and mediterraneans. In Nigeria, it is estimated that 1 in 4 people carry the sickle cell gene. It a significant cause of morbidity and mortality among the populace. Poverty, ignorance as well as weaknesses in the National health system compound the problem.

Background-1 The Sickle Cell Disease Care Centre in Federal Medical Centre, Keffi commenced operation in the year 2011 It is one of the first four (4) Special Sickle Cell Disease Care Centres designated by Federal Ministry of Health in 2011 to cater for North Central zone of Nigeria. Presently, there are six (6) special SCD Centres in each geo-political zone in Nigeria. The Sickle Cell Disease Care Centre at FMC Keffi offers holistic care to children, adults and families with sickle cell disease.

Background-2 2011-2015 Activities included Screening for SCD for both neonates and above, routine and acute care and counselling. Training for program staff on the use of Vnbs HPLC machine, institution of protocols for sample collection processing and result analysis and interpretation as well as disclosure of results Participation in the production of the national Policy document on the management, control and prevention of SCD. Hosting of the 2014 National WSCD celebration with HPLC training for participants from all over the country

Background-3 2016 Awareness campaign & Lecture at Keffi NYSC Orientation camp for the world SCD day celebration with Free screening for 200 NYSC members and point of care testing for 20 of them 2-Week Training on the use of Trans-Cranial Doppler (TCD) machine for screening of SCD clients aged 2-16years to monitor blood flow in the cerebral vessels as a means of preventing stroke. (3 Radiologists and 1 Paediatrician under the auspices of Sickle Cell Foundation in Lagos). TCD Machine donated to the centre at close of training) 2017 Ongoing Planning for a sensitization and training workshop for PHCs in the state as part of the World SCD day celebration.

SCD patients attended to from January to July 2017 Month Children SEX Adult 15 years Sex 0-14 years and above M F M F Jan 47 25 22 33 17 16 Feb. 71 34 37 20 6 14 March 53 29 24 29 15 14 April 56 34 22 17 11 6 May 53 26 27 24 11 13 June 60 33 27 28 14 14 July 48 25 23 37 15 22 Total 388 206 182 188 89 99

Graph of SCD Patients both the children and adult from January to July, 2017 100 90 80 70 60 50 40 30 20 10 July Jan Feb Mar April May June

NBS done from January to July 2017 No. of NBS HbAS trait Other Hb SCD Remark done Variants identified Jan 10 2 Nil Feb 21 4 Nil Mar 4 1 1 Nil (FAD) Apr 11 3 Nil May 3 0 Nil Jun 13 3 Nil Jul 22 4 Nil Total 84 17 1 0 FAD (20.24%)

Screened 692 new born samples received from FMC Asaba Screened 96 new born samples received from UATH, Gwagwalada. Validation / QC for a study for a point of care testing Non determinate results due to poor sample collection Total of 874 New born samples screened in 2017

Summary of NBS from 2013 -2017 Total sample received and screened 1203 FMC Asaba 692 (57.52%) UATH Gwagwalada 96 (7.98%) FMC Keffi NBS 415 (34.50%) 522 sample received HbSS identified -1 (0.24%) HbAS carrier state 90 (21.69%) Hb S/ + thalassaemia (SFA) 2 (0.48%) Other Abnormal Hb traits - 7 HbAC 4 (0.96%) HbAD 3 (0.72%)

Other services offered Services Available Target population Available Remarks SCD N-B Screening 0-9mths Yes Free Hb electrophoresis All Yes Fee for service OPD/Consultation All Yes Free In-patient treatment All Yes Fee for service Nutritional Counseling 0-14 yrs Yes free Genetic Counseling Adult Yes free Investigations & treatment All Yes Fee for service Routine drugs All Yes Fee for service

Trans-cranial Doppler Scan (TCD The Centre was part of the TCD workshop held SCD centre Lagos in 2016 A grant from Radiological Society of Northern America (RSNA) to University of Lagos through Dr Kofo Soyebi Three Radiologists and a paediatrician were trained on the use of the TCD to categorize the risk of stroke in children 2-16yrs The TCD machine is being validated presently to generate standard values for children in our environment since June 2017 Values from 12 children attending our SCD clinic have so far been obtained and mass screening in Primary schools in Keffi is part of the plan to generate the standard normal values for the environment in record time No abnormal velocity detected so far

Scope, activities and Status of SCD Program in FMC Keffi-4 Logistics & Funding for a. Nationwide/Regional SCD Advocacy Yet to be worked out b. National/Regional Community awareness Yet to be worked out c. Sample collection/Transport Yet to be worked out d. Basic Funding/equipment Take off grant in 2011

Constraints Lack of Political will at the 3 tiers of government Lack of a nationally co-ordinated NBS programme for SCD despite a robust, clear and disseminated policy document Space Capacity Building Lack of equipment such as the D10 HPLC Machine for SCD screening older clients No Sustained annual funding Limited Blood Transfusion resource for use in hypertransfusion Inaccessibility and Unaffordability of some important drugs such as Hydroxyurea Poverty & Ignorance

Recommendations Sufficient fund should be allocated to the Centre Allow the centres to be integrated into the hospital service delivery system and charge appropriate fee for service Fund raising from multinational co and NGOs Research and projects grants Retraining of the Staff in the Sickle Cell Disease Centre. Create/activate the appropriate logistics for sample collection and transportation Increased Community awareness to be encourage Formation of consortium for NBS for SCD the 6 centres

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