Medical expertise helps young kidney patient thrive
Posted on 15 Sep 2021
Focal Segmental Glomerulosclerosis (FSGS) is a severe form of nephrotic syndrome. Professor Errol Gottlich, a paediatric nephrologist based at Mediclinic Morningside, explains further.
Conor van Baalen is a very busy eight-year-old, hell-bent on playing with his siblings and causing havoc. “He’s a mischievous little boy but we’ll take the grey hairs he causes us,” says his mother, Santie. “We’re just so grateful he has a second chance at life.” In February last year, Conor celebrated the first anniversary of his kidney transplant, and his family reiterate how indebted they are to his kidney donor (family friend Danie de Wet) and his doctor, Professor Errol Gottlich.
“Conor is a twin and at birth, both babies were completely fine,” Santie says. “However, at 18 months, Conor began swelling up, which I thought was a result of allergies. He looked like a little Oros man and was very miserable – the swelling affected every aspect of his life from sleeping to eating and socialising. When the paediatrician took one look at him and said it was kidney related, I felt as if someone had thrown cold water in my face. Conor was diagnosed with nephrotic syndrome (an abnormal loss of significant amounts of protein in the urine, generally of unknown cause). It was a massive shock, and the beginning of a roller-coaster ride for our family.”
The Van Baalens were referred to Professor Gottlich, a paediatric nephrologist at Mediclinic Morningside. “When I first saw him, Conor was fairly well and had no kidney dysfunction – but he was resistant to treatment,” Professor Gottlich says of his young patient. A kidney biopsy in July 2015 showed a very significant diagnosis called Focal Segmental Glomerulosclerosis (FSGS), a severe form of nephrotic syndrome, rarely affecting white children. “Although it is a fairly common form of nephrotic syndrome in black children, FSGS is rare in white children and because FSGS is often resistant to treatment, most sufferers go on to end-stage kidney failure,” Professor Gottlich explains. “The particular problem with FSGS in white children, is that, if it’s a non-genetic type of FSGS, there is a very high probability of it recurring after a kidney transplant. In black children, this is generally not the case.”
Conor’s generalised body swelling was associated with abdominal pain. This is because FSGS causes thick blood, which in turn causes pain as it circulates through thin abdominal vessels. “We started the cycle of trying to get control of his condition and used a whole lot of different immune-suppressant therapies, including cortisone and anti-rejection drugs,” Professor Gottlich explains. “However, although he did relatively well on and off, he never totally improved and as time went by, Conor’s condition progressed.”
Professor Gottlich adds that initially the swelling wasn’t the major symptomatic issue, but it made Conor cosmetically different and affected his functions and activities. In addition, the little boy had the trauma of repeated hospital admissions for treatment and tests, his growth slowed down and he was leading a very different life to that of his twin brother, Kody, and other siblings. “We tried to manage his condition but as expected, he became completely treatment resistant,” Professor Gottlich explains. “FSGS eventually starts scarring the kidneys, which is when a patient goes slowly but surely into kidney failure. In April 2018, when Conor was five years old, we withdraw most of his treatment because they were causing more side effects than benefits. We put him on growth hormones, managed his fluid intake as best we could and started him on a low-salt diet and daily blood pressure medication for chronic kidney disease. In March 2019, Conor went into end-stage kidney failure and we had to start him on peritoneal dialysis.”
Santie remembers this time very clearly. “Conor had an operation to have a catheter put into his abdominal cavity and I was trained how to change the dialysis fluid,” she recalls. “For eleven months, he had one litre of dialysis fluid every three hours, meaning I had to change his bag four times a day, from 6am to 6pm. The treatment made him lethargic and he wasn’t able to attend nursery school because he slept most of the day.” At the same time, Professor Gottlich started the extensive kidney transplant work-up, which included assessments by cardiologists and dentists, plus numerous X-rays and blood tests.
As Santie says, it was difficult for her and her husband to accept that they couldn’t be kidney donors for their son. “But Danie is a family friend and fellow amateur wrestler and he had offered to donate his kidney even before Conor started dialysis. He was a perfect match, and to this day, our entire family owes Danie a debt we’ll never be able to repay.”
The Van Baalens were understandably anxious to have the transplant happen as soon as possible, but Santie explains, it is tough getting permission from the Department of Health if the donor isn’t related to the patient. “Conor was getting weaker and weaker and three months before the scheduled transplant date, he was admitted to ICU for seizures due to uncontrolled blood pressure,” she recalls. “It was an incredibly stressful time, and we were also incredibly anxious about the donor’s wellbeing.”
Eventually, on 25 February 2020, Conor underwent a successful kidney transplant at the Wits University Donald Gordon Medical Centre. Four days later he was transferred to the Morningside Children’s Kidney Treatment Centre. “By six months post-transplant, everything had returned to normal and today Conor is very active, has a good appetite and has completely normal kidney function,” Professor Gottlich says. “While this is not an unusual ending, we were worried about re-occurrence. Of the five white children with FSGS who received kidney transplants in my practice, three have had a re-occurrence of the condition. Conor is one of the lucky ones.”
As Professor Gottlich adds, the fact that Conor has a non-identical twin bodes well for the future. Transplanted kidneys normally have a lifespan of 20-25 years – and as an adult, Kody would be a potential donor. In the meantime, Conor is firing on all cylinders, and has started Grade 1 with a bang.