MECFA supports CF patients in Tunisia

Tunisia is home to about 250 people with cystic fibrosis (CF), which was first discovered in

1994. Tunisia's population is approximately 12 million. Healthcare is disproportionate.

Coastal communities benefit greatly from the presence of four university medical centres:

one in the north (Tunis), two in the middle (Sousse and Monastir), and one in the south

(Sfax). Most patients with CF (PwCF) are obliged to travel to Tunis for CF diagnosis and

treatment. Because of their low socioeconomic status and distance from hospitals, many

patients die before being diagnosed, while others are diagnosed too late. Practitioners';

understanding of the condition is inadequate, particularly in remote places. Furthermore, due

to consanguineous marriages and genetic variances, the frequency of CF appears to be

higher among tribes in Tunisia's south than in the north. Because there is no sweat testing

device or skilled clinicians in this area, many people may die without being diagnosed.

Currently, Creon is the only CF-specific medicine accessible in Tunisia, however it is not

covered by the national health plan. Families must pay out of pocket, and many are unable

to afford access.

In 2020, 123 children with cystic fibrosis participated in research to investigate the

prevalence and status in Tunisia. The typical age at diagnosis was 5 months, with a median

delay of 3 months. CF was diagnosed mostly through recurrent respiratory tract infections

(69.9%), malnutrition (55.2%), and/or chronic diarrhoea (41.4%). The mean sweat chloride

content was 110.9 mmol/L. At least one mutation was discovered in 95 instances (77.2%).

The most common mutations were Phe508del (n = 58) and E1104X (n = 15). Pseudomonas

Aeruginosa chronic colonization was present in 55 patients at a median age of 30 months.

Cirrhosis and diabetes developed in four patients at an average age of 5.5 and 12.5 years,

respectively. Sixty-two patients died at the median age of eight months. Phe508del mutation

and hypotrophy were linked to death (p=0.002 and p<0.001, respectively). (Sondess Hadj

Fredj et al., March 2020).

Conclusion, CF reduces life expectancy in Tunisia. There is an urgent need to establish

adequate management.

At the national level, Tunis' Béchir Hamza Children's Hospital is the largest paediatric

department overseeing PwCF. The hospital was established in 1938 and is the largest

children's hospital in the country. More than one-third of the CF population is treated at

Bechir Hospital, which is primarily handled by Dr Samia Hamouda. Regarding the CF Team

at the Hospital, there is one nutritionist, one gastroenterologist, and two physiotherapists on

staff; however, they do not work full-time for CF patients. Sweat testing, spirometry, and

routine microbiological culture are conducted. There is no dedicated outpatient room for CF.

Although there is presently no standardized CF Team, Dr Hamouda is eager to establish one

and raise the level of standard care at the institution. The background of the infrastructure

appears to be sufficient to establish a centre capable of initially serving Tunisia's whole CF

population.

Two paediatric departments in Sousse (Farhat Hached Hospital) and Sfax (Hedi Chaker

Hospital) have the necessary infrastructure to establish a reference centre for CF patients.

Each of these state hospitals has a paediatric pulmonologist and a paediatric

gastroenterologist who are knowledgeable with the condition.

MECFA recently donated diagnostics to Bechir Hamza Children's institution, Farhat Hached

Hospital, and Hedi Chaker Hospital, and is assisting each institution in establishing a

diagnostic program. Tunisia's Ministry of Health and Bechir Hospital are working to add

cystic fibrosis patients' data to the national health registry. MECFA has begun to support this

initiative by providing guidance and basic data sets.

MECFA has direct communication with Tunisia's Minister of Health, Dr Ali Al-Mrabet, via the

World Health Organization and MECFA board members. We had the opportunity to present

a three-phase approach that included diagnostics, access to critical drugs, and support for

creating centres and teams. We proposed donating diagnostics to three state hospitals and

implementing a two-year Quality Improvement program at Tunisia's Bechir Children's

Hospital. And we would assist BCH in subsequently carrying out the same program in Farhat

and Hedi Hospitals, establishing a CF Network in Tunisia. In exchange for our assistance,

we will request that the MOH offer dornase alpha, inhaled tobramycin, vitamins, and

enzymes as part of the national health programme.

Goals for quality improvement and Centre development in Tunis

• Establish a cutting-edge, multidisciplinary CF Centre in Tunisia. This pilot and

feasibility project will build a care centre capable of managing all clinical aspects of

CF patients, adhering to the best evidence-based treatment guidelines, and adapting

them to Tunisia's specific needs.

•  Train a multidisciplinary expert CF team in Tunisia. The training will take place under

the direct and constant supervision of the Marmara CF Centre in Istanbul, both in

Tunisia and Turkey. A CF team, including a CF nurse, physiotherapist, and dietitian,

as well as pulmonologists, will visit the facilities.

•  To significantly enhance clinical outcomes for CF patients at the Centre. We intend to

increase nutritional status (measured by BMI%) and lung function (measured by

FEV1% and FEF25-75%, respectively).

•  To prioritize quality improvement efforts. The Bechir Hospital team will collaborate

closely with Marmara CF Centre to build short- and long-term QI initiatives to achieve

their goal of improving care. QI activities will involve increasing BMI, enhancing

pulmonary function testing, and introducing Infection Prevention and Control (IP&C)

in the centre. We will start by training the Bechir team on quality improvement and

project development. Next, the two teams will collaborate on the pressing topics that

need to be developed, selecting one or two for research projects. Online education

and follow-up sessions will be scheduled.

•  Lead the implementation of a network of CF centres throughout Tunisia. Once the

pilot project has been completed, we will assist Bechir Hospital with QI and the

establishment of CF Centres at Farhat Hached Hospital and Hedi Chaker Hospital.

Following that, the two centres and the Ministry of Health will collaborate to establish

three regional CF Centres, resulting in Tunisia's National CF Centre Network. This

network will comply with and try to meet all the CFF's requirements for centre

certification.

•  Within 5-8 years, aim for a median life expectancy of 30 years. This will be

accomplished by establishing the National CF Centre Network, utilizing evidence-

based, cutting-edge healthcare delivery, adhering to strict quality improvement

principles, providing ongoing multidisciplinary team training, and launching a

significant clinical research endeavour.

Tunisia's present CF Care demonstrates that without a systematic and continuous

intervention, CF can quickly lead to mortality, imposing a huge economic and emotional

burden on patients and their families. There should be guidelines comparable to those in the

United States to assist in the development of interventions for prevention, diagnosis,

treatment, and rehabilitation to enhance these patients' outcomes and quality of life. Thus,

this pilot and feasibility study will contribute to the capacity of a national program and care

network for CF patients in Tunisia.

Program participants

The Marmara University Hospital CF Centre.

The Bechir Hamza Children's Hospital CF Centre team will get intense on-site training from

the Division of Paediatric Pulmonary Medicine and the MUH CF Centre (Prof. Bulent

Karadag, MD, CF Centre Director) during the first phase of the project. The Bechir CF Team

will then travel to Turkey to examine patients, provide treatment guidance, and begin quality

improvement initiatives. The MU CF team will establish open communication with the Bechir

CF team via secure IT to discuss patient management and care strategies. Online visits and

sessions will be scheduled.

Bechir Hamza Children's Hospital (CF Centre is under the Ministry of Health).

Bechir Hamza CF Centre will contribute to the initiative by forming a collaboration with the

MU CF Centre. Such a partnership ensures the regulatory framework and compliance with

the minimal standard of healthcare. A long-term goal is to create a network of CF care

facilities in Tunisia.

As the collaboration grows, Farhat Hached and Hedi Chaker Hospitals will be invited to join.

The collaborative partnership between Marmara CF Centre and Bechir CF Centre will be

extended to the two additional facilities, depending on their needs.