Sickle cell crisis leads youngster to launch child-health NGO

Mwenda’s mother, Lelemba Phiri, chief marketing officer for a Cape Town-based financial services company, says persistent coughing, general weakness and unusually light-skin as an infant, set her first-born apart.

Worried, she took him in to see her grandfather at the country’s main hospital. Professor Chintu conducted a number of tests, diagnosing a chest infection and a mild urinary tract infection. Mwenda’s haemoglobin levels were however particularly low (6 grams per decilitre compared to normal levels of 14) so her grandfather conducted more tests to rule out Endemic Malaria, HIV and Leukaemia before ordering the specialised tests for Sickle Cell Anaemia. Some 48 hours later the results came in; positive for Sickle Cell Anaemia.

“While it was a shock, we couldn’t have had a better start to our journey than my grandfather’s counsel on all the implications and requirements of life-long, pro-active management for the disease. Before the diagnosis it was really hard because I just wanted to know what was wrong with my baby,” she adds.

Just over two years passed with the family instantly handling even small infections without further incident. The Phiri family couldn’t take a common cold for granted, and the constant use of antibiotics, plenty of hydration in the hot Zambian summers and blood boosting folic acid kept Mwenda healthy and his haemoglobin levels manageable. At three years old, Mwenda hit his first crisis, suffering classic symptoms of acute chest syndrome, a common occurrence in people with Sickle Cell Anaemia. He had his first blood transfusion and spent a fortnight in a Lusaka hospital, emerging fully recovered. The family, including his father Sandras, a personal finance specialist and Mwenda’s brother, Mwai, now 11, thereafter moved to Cape Town, while contemplating how fortunate they were to access specialist facilities and services that are rare are in sub-Sahara Africa.

"Darkest 48 hours of my life," - Mwenda’s mom, Lelemba Phiri

Then came his second crisis, one which gave his family palpitations and nearly cost him his life in October 2017. Lelemba remembers it well: “Those were the darkest 48 hours of my life. At first, he said his stomach was sore, about a six, on a one-to-ten pain scale. We advised him to take some Disprin and we waited a bit. The next day, October 18^th, it was no better, so we went to hospital. Scans by a urologist diagnosed appendicitis, and an operation was immediately scheduled to prevent a rupture which, given his condition, would have had far more dire implications than normal. The op went well. His haemoglobin levels were slightly low, but this was to be expected post-operatively. Back home two days later, Mwenda complained of chest and leg pain, so I gave him some strong pain meds, the prescribed antibiotics and some soup for supper. He was pretty lethargic, had a bit of a temperature and just wanted to sleep. We had friends over for dinner and I planned to check in on him in an hour. When I went to try and wake him he was non-responsive and breathing very fast. We didn’t even call an ambulance – we bundled him into the car, difficult because he was unconscious and had the appendix removal surgical wounds. I just kept telling him to keep breathing in the vain hope that he could hear me. Blaauwberg Netcare Hospital staff were absolutely amazing. His pulse was out of whack, blood oxygen levels were around 55% and his lungs were crashing. It was major panic for us. They probably worked for about three hours trying to stabilise him. They took x-rays - it was machine after machine. Every little abnormal-sounding bleep scared us. It was freaky. Everybody was running about.

Doctors ponder Mwenda’s near-death post-anaesthesia response

Doctors got the teenager’s oxygen levels back up to about 80%. He came to and was transferred to the ICU. At around 04:00 he had his first blood transfusion. "We were there throughout," says Lelemba. “After daybreak, the physician told us he was in a critical condition. It hit us like a ton of bricks. He'd been doing so well. In the next two days he had six blood transfusions. His heart had swollen a bit from the low oxygen levels and one of his lungs had about 10% capacity and the other 50%. Finally, after two days, things stabilised and for the first time the x-rays showed his lungs weren’t getting any worse - and his temperature stayed below 38°C."

Later the Phiri family thrashed out with the doctors what had caused the severe reaction. Nothing was conclusive but a couple of theories emerged. One was that he had a bad interaction with the anaesthetic during his appendix-removal op. The doctors said perhaps they should have boosted his haemoglobin levels to above 7.5 grams per decilitre before the operation. With hindsight a pre-operative blood transfusion would have probably been better. It could also have just been the stress that anaesthesia puts on the body anyway. His Sickle Cell Anaemia may have caused his red blood cells to have broken down at a faster rate making him more anaemic, more quickly. Or it could have been an infection unrelated to the anaesthesia. “It was difficult to isolate the cause of his crisis. To this day we don’t know the answer,” says Lelemba. “It’s all seemingly related to Acute Chest Syndrome, the leading cause of death for patients with Sickle Cell Anaemia."

Phiri family praises Discovery Health Medical Scheme’s support and efficiency

The Phiri's cannot speak highly enough of the Blaauwberg Netcare Hospital staff and of Discovery Health Medical Scheme’s seamless support and cover. Says Lelemba, “At one point, Mwenda’s veins had collapsed and his nurse couldn’t put an urgent IV line in so she called the trauma nurse who, under severe pressure from six other medical staff observing, a screaming distressed child and visibly distraught parents watching, felt a spot in his arm and calmly said, ‘I can’t see it but I believe it’s here’, and with one try, she hit gold.”

As for Discovery Health Medical Scheme, Lelemba has this to say: "The humanity with which Discovery Health Medical Scheme handled us and the seamless support and cover , were both absolutely amazing and made it all well worth it. . When we were going into hospital, Discovery called us and said they’d heard about Mwenda’s admission and wanted to assure us we didn’t have to worry about anything. We should rather focus on what was happening. I thought that was super-thoughtful. Discovery Health Medical Scheme knew exactly what was going on and were talking to the hospital and granting all the approvals. When it came to e-mail correspondence with Discovery Health Medical Scheme, they always replied within a day. For a big organisation, the humanity caught me off guard. There was no pre-approval hassle with the emergency. It was a painless experience for us. Discovery Health Medical Scheme  went the extra mile with their humanity and speed of response."

Phiri family launches a highly successful child-health NGO

Since then Mwenda has been incredibly well. “By the 18^th of December 2017, we had the Mwenda Phiri Initiative in full swing. It’s my son’s brain-child, to raise awareness and funds for paediatric haematology and ICU services in Africa. The first donations will go towards children’s ICU equipment at the University Teaching Hospital in Lusaka, something of which my grandfather, who died earlier this year at 82 years old, would certainly be proud. The list of necessary items is long and includes baby bassinets, incubators, CPAP machines, arterial blood gas machines, vital signs monitors - you name it,” says Lelemba.

**Sickle Cell Disease, (SCD), is an inherited blood disorder that causes red blood cells to turn into a sickle shape under certain conditions. These cells die early, creating a shortage of healthy red blood cells and so causing anaemia. The sickled cells can block blood flow to major organs causing a lot of pain in what is known as a Sickle Cell Crisis. Most children with SCD are of African ancestry but children of Middle Eastern, Mediterranean and South Asian ancestry are also affected. SCD is not contagious. New and aggressive treatments for sickle cell disease are prolonging life and improving quality of life for those with SCD. As recently as 1973, the average lifespan for people with SCD was only 14 years. Today, life expectancy for these patients is 50 years and beyond.

Follow the Phiri’s paediatric haematology and ICU initiative here and here. You can also read more in Lelemba’s blog