While South Africa has a cystic fibrosis, (CF), registry of 442 patients drawn from four centres of excellence, more data is urgently needed to begin making further inroads on the disease.
This was said by Dr Carla Els, a paediatric pulmonologist from Linksfield Hospital and past chairperson of the Cystic Fibrosis Association of South Africa. She was speaking at the 25th centenary gathering of the South African Rhinitis Working Group, SAARWG, entitled “Allergic Rhinitus – Back to Basics and the Future,” held at a Magaliesberg venue from November 15th to 17th this year.
Dr Els was presenting on cystic fibrosis and the upper airway, her central focus being on identifying and managing a patient with a blocked nose and allergic rhinitis after they failed initial therapy. Elaborating on the SA CF Registry Initiative, she said it was launched in April 2018 and embraced the Charlotte Maxeke Academic Hospital (Adult and Paediatric), linked to Mill Park Private Hospital, a combination of Groote Schuur Hospital, UCT Private Hospital, Red Cross Children’s Hospital and Tygerberg Hospital, Durban’s Albert Luthuli Academic Hospital, and the Port Elizabeth Hospital.
The data showed that the biggest CF population registered (62%) was white and six to 18 years old, with 54% of all patients’ female, 27% mixed-race and just five percent black. The median diagnosis age was six months old while 15% had meconium ileus – a bowel obstruction that occurs when the meconium in a child’s intestine is thicker and stickier than normal meconium, creating a blockage in a part of the small intestine called the ileum. Most infants with meconium ileus have CF. She added that at least one sweat test had been conducted in 201 patients (61%) while there was missing diagnosis information in 35 patients (11%), mostly adults or those how moved practices or clinics. A cross sectional annual review data was available in 306 people, with chronic pseudomonas infection in 97 (32%), ‘unable to establish pseudomonas status’ in 61 (20%), one death and no transplants in that year (2017). Ten were not seen for F/U that year and the status of one patient was unknown.
Dr Els listed key events for notifying regional CF co-ordinators and centres of excellence as new diagnoses or referrals, deaths, transplants and pregnancies or births.
CF ‘right under our noses’
Stressing the critical importance of the nose in the link between CF and AR, she said that she told her patients that the nose was the ‘start of all evil.”.
“We need to treat the nose to keep the chest healthy. We should all be thinking about CF in allergic rhinitis. CF patients who don’t want to eat always have mucous in their mouth. We also underestimate their psychological mechanisms of coping. In my practice if I can’t control the chest, I look at the nose. We don’t do that often enough. They (CF patients), hardly ever complain about their noses,” she added.
Dr Els said that while presenting symptoms were numerous, they were not always indicative. Often when the nose was good, lung function was better. However, the biggest problem she encountered among her CF patients was that they seldom treated themselves if they had a runny nose or were coughing. She said a combination of treatment strategies and surgery was often worth considering. Nasal saline irrigations were the mainstay in her practice. Antibiotics helped improve quality of life while nasal steroids were also very helpful. Decongestants helped with the absorption of medicines while all patients seemed to love mucolytics. However only topical steroids were proven to work and should be continued, with high-dose rinses very effective, especially when combined with surgery. Dr Els warned against referring patients to surgeons who had no experience in CF and said the 11-18-year-old group was a proven target cohort. Follow-up 10 years after endoscopic surgery showed that there was no different in their facial measurements, emphasizing how safe it was to use such procedures in children. As in other areas of disease, low socio-economic groups fared worse, especially where there was limited access to safe running water and healthcare facilities. She stressed that more aggressive treatment of the nose was required in 11 to 17 years-old’s, adding that post-surgical medical treatment was vital.
Tell CF patients to manage their noses
She added, ‘sinusitis is not a curable aspect of CF but if CF patients can just be persuaded to manage their noses, it will make a world of difference,”
A combination of many therapies made a difference for CF patients, but endoscopic surgery was a ‘must,’ she emphasized. There was a significant correlation between the health of paranasal sinuses and lungs. Interestingly, non-CF, CRS patients had distinct niches of micro-organisms in their upper versus lower airways and CF-CRS patients had indistinguishable niches at both anatomic sites. The evidence was that the sino-nasal cavity acted as a reservoir for bacterial translocation to the lower airway, she added.