THE burden of sickle cell anaemia can be huge on both the sufferers and caregivers in terms of the financial, medical and emotional demands, say Mwanza and Robert Chibilu.
The Lusaka-based couple is the parents of four children, two of whom have lived with the condition.
Late last year, their second-born son Lukumo, 11 underwent a lifesaving procedure in India to free him of the painful disease. Lukumo was diagnosed with sickle cell at the age of five and was often very frail.
In order for the procedure to be successful, Lukumo had to undergo a Bone Marrow Transplant (BMT) and needed a near-perfect match as a donor. His younger sister Luusa was able to provide the donation.
Last month, after five months of treatment, Lukumo returned home and medical follow-ups will continue.
The Chibilus say there is still stigma associated with sickle cell.
“It is believed sickle cell anaemia patients never grow to adulthood and as such the disease should only be managed until the victim dies. This belief is unfortunately also common among health practitioners. Some people believe the disease is caused by witchcraft,” says Robert.
“Others think it is a curse and is considered an embarrassing sickness. The stigma associated with the disease causes victims and caregivers/parents to avoid coming out in the open and seeking treatment.”
The couple, together with other sympathisers, has since established the Lukumo Ntambo Foundation – named after their children affected by the condition – to raise awareness, counselling, outreach, advocacy and where possible financial assistance for sickle cell anaemia patients.
ThisWednesday marks World Sickle Cell Day. Here, Robert and Mwanza share their story of caring for children living with the condition.
He had the first major (near-death) sickness at 9 months old that kept him in hospital for 12 days, with body temperature soaring as high as 40 degrees celcius.
After this he started going in and out of hospital with no diagnosis. He had about three blood transfusions by the time he was five years old due to low haemoglobin (Hb).
During this time he suffered a number of pain episodes and fevers, with high blood infections. In 2012 an electrophoresis test was done at UTH for the entire family to test for suspected sickle cell disease. An electrophoresis test is carried out to test for presence of sickle cell haemoglobin in the blood. This was after his younger sister Ntambo Yana tested positive in a sickling test. Ntambo had also started having frequent hospitalisations due to fever and pain episodes. So Lukumo and Ntambo Yana tested positive for sickle cell disease. The two other siblings tested negative.
Lukumo’s health started deteriorating as he grew. He walked late as a baby and he was usually frail and withdrawn. He usually exhibited some kind of post trauma stress due to near-death experiences. Frequent hospitalisations hampered his learning abilities, as he was missing school every now and then.
It wasn’t easy initially, not knowing the condition. We were all over in private hospitals paying huge hospital bills for treatment. After the confirmation of sickle cell, we did well to engage closely with doctors like Dr Kabamba at Levy Mwanawasa Hospital and others at the University Teaching Hospital, always consulting and learning from them, strictly following doctors instructions and avoiding over exposing the two children to cold, sun, rain, hyper activities, emotional stress and infections.
We googled on the Internet and read a lot about Sickle cell disease (SCD). We also spoke to others in similar situations. We have been open about the disease and this has helped a lot. A lot of people hide the condition as the disease, traditionally, attracts stigma. But hiding puts the patient at a disadvantage as often they are prevented from accessing immediate medical treatment until it is too late. Sickle cell is unpredictable and requires quick medical response.
For me as a mother, it was like a death sentence on my children. I struggled to accept the development. My husband on the other hand had relatives with SCD so it wasn’t as bad news for him as it was for me.
Reading and gathering information about sickle cell anaemia helped us to be positive and look after our children better.
UTH has a clinic for SCD. We started attending the clinics on appointments. For us at times we used Levy Mwanawasa Hospital as we lived near there.
The church, relatives and friends have been close at hand. Belonging to WhatsApp support groups has been very helpful too. For a condition like sickle cell you need a lot of support.
As Lukumo grew older, it was hoped that he’ll be getting better. He however started having frequent headaches which sometimes caused hospitalisation.
Headaches are not good in sickle cell patients as doctors say they’re an indication of an impending stroke. A stroke is not good for sickle cell patients as they make the condition worse. Strokes are common with sickle cell patients due restrictions in the veins.
By this time, to avoid any stroke, Lukumo had been put on serious blood transfusion protocols – weekly, bi weekly and monthly over a 6 month period.
Until recently, sickle cell had no known cure. Recent medical advances have discovered that bone marrow/stem cell transplant can cure sickle cell.
It is a new treatment and is the only known medical cure for sickle cell at the moment. Other methods such as genetherapy are also being studied.
Two doctors from two different hospitals in India visited Zambia at different times and talked about the possibilities of Bone Marrow Transplant (BMT).
The idea of BMT was exciting but the costs were however prohibitive and out of reach. Bone marrow transplant can cost as high as US$40,000 in India and could be higher elsewhere. We had doubts of outcomes also because the operation itself is new with few testimonials. It is a very delicate operation but has a 95 per cent success factor and 5 per cent critical factor. We searched for possible cheaper places.
Our family abroad came up with an idea of opening a Go Fund Me account to raise funds for Lukumo and Ntambo’s BMT procedure. BMT requires matched donor/patient genetically.
We did sibling and parent genetic match to see if we could find stem cell donors within the nuclear family (this is the best way to find a matched donor, but a donor can also be found outside the family). Siblings can easily give a full or 100 per cent genetic match, while parents can only give a half (Haplo identical) match each.
Luusa, the youngest sibling, was found to be a full genetic match with Lukumo. The closest for Ntambo was her mother Mwanza at 70 per cent.
The Go Fund Me account did not yield as much as anticipated. So as family, we turned to other ways for raising funds for the two children.
An older sister in Drumheller, Canada organised a fundraising initiative under the “Africa in the Valley” outfit, which was a one time event and it managed to raise initial funds enough for one child to start the BMT process.
Lukumo, whose condition had become worse than Ntambo’s, was chosen to undergo the BMT first, as advised by doctors.
The LukumoNtambo Foundation worked hard to help family prepare for the procedure in India after settling on one hospital.
The foundation initiated a fundraising drive locally by lobbying corporates and individuals. Due to low sickle cell disease awareness levels, it was difficult to convince most donors and garner much funds to augment the funds already raised elsewhere. A number of institutions and individuals, however, came forward and donated and were quite helpful especially during the outpatient period after discharge from hospital.
The government does not openly sponsor sickle cell patients for such medical procedures at the moment despite the sickle cell situation being desperate … the cost being the factor. This is all the reason societies and foundations should step up to help alleviate the condition and plight of sickle cell victims.
BMT was to be done in India at Fortis hospital in Gurugram, Haryana by Dr Vikas Dua.
Luusa was to donate her bone marrow stem cells to Lukumo.
Screening tests were done on the two (donor and patient) upon arrival in India to ensure all infections were treated and ruled out before BMT could begin.
Blood exchange was done on Lukumo because his sickled cells were quite high. Chemotherapy was then administered on him for 8 days to kill all sickled stem cells in his body.
In the meantime, Luusa was being prepared for harvesting of the bone marrow stem cells by a special medical procedure.
The stem cells harvested from her were put in Lukumo’s blood stream for engraftment.
The donor stem cells had to start engrafting before he could be discharged from hospital. Hospital reviews started right in India every other day, then weekly over a 120-day period, post-BMT.
After this period, he was released from India and he is back home in Zambia recovering. He will continue bi-weekly blood tests with a local lab, then monthly till he is 13 months post-BMT.
He is currently in isolation at home, and a very strict hygiene protocal has been prescribed. This is to avoid infections as the immunity is compromised during chemotherapy. During the period he is in isolation, his food must be freshly cooked and eaten immediately. His environment must be neat and sanitised all the time. He must not be exposed to the outside or public. Windows must be kept closed most of the time and visitors kept to a minimum.
He was in hospital for 25 days before being discharged. He however was in a rented apartment near the hospital as an out-patient for 120 days.
The medical procedure was successful. The tests are showing that he is on the mend and he is generally looking livelier, stronger and more active. He looks more confident and ready to interact. He’s had no pain episodes since the bone marrow transplant. He says he feels so strong now, but has to learn to live without pain which he lived with before and got so accustomed to.
In an effort to help raise the funds for the medical procedure in India, sympathizers came together after learning about Lukumo and Ntambo’s story and the efforts of the Drumheller community in Canada.
A fundraising vehicle called Lukumo Ntambo project was birthed.
This brought on board a number of people from different professions and walks of life including doctors, lawyers, bankers, child advocates and so on.
These people have a passion for helping raise awareness for sickle cell disease and its treatment.
It was later felt that the project be turned into a foundation to go beyond Lukumo and Ntambo so that society at large can benefit from the experience. This spelt the establishment of the ‘Lukumo Ntambo Foundation’ named after the pioneering children.
The objective of the foundation is to provide “healthcare assistance” in the areas of sickle cell disease awareness, awareness to treatment, counselling, outreach, advocacy and where possible financial assistance.
The foundation targets patients, caregivers, relatives, schools and workplaces.
It is registered with PACRA by guarantee and is organised around a committee of members. The four guarantors act as directors.
Mr Chibamba Kanyama is the foundation’s goodwill ambassador.
Sickle cell disease is an inherited genetic blood disorder that affects people.
No single parent with a sickle cell trait can bear a child with sickle cell. The disease can only manifest in a child if both parents carry sickle cell genes or traits.
There are a lot of lives being lost to sickle cell today in Zambia because despite the disease being prevalent in Zambia (especially Northern, Luapula, North Western and Western regions), it is often given less attention and is believed to be incurable.
As a foundation, we would like to partner with the government, corporates, donors and individuals to create better outcomes for sickle cell patients in both urban and rural areas the same way much attention is given to Malaria, HIV and AIDS etc.
From the statistics gathered, it is clear that sickle cell disease is on the increase and if not checked will soon reach alarming levels that will eventually overwhelm the health sector in managing it.
Sickle cell patients are more likely to suffer from renal failure.
A sickle cell patient’s internal organs deteriorate faster than normal with time as they overwork to handle chemicals in the body that sicklers take every day through medication.
The government should therefore start considering helping sickle cell patients, with lasting treatment such as BMT while the condition is in its early stages.
We are also looking for volunteers to act as sickle cell disease ambassadors, ie young people, musicians, and so on.
For more information on the Lukumo Ntambo Foundation, contact 0962712819 or follow @lukumontambofoundation on Facebook page.