This page gives an outline of visit to the Cystic Fibrosis service of The Latvia University Children’s Hospital (link to English pdf), taken from a report by Gary Connett, Consultant Paediatrician and honorary Senior Lecturer at Southampton University Hospitals Trust.
- Schedule of Visit
- Information about CF Care in Latvia (largely provided by Dr Svabe)
- Notes on the meeting with Arvis Eisaks Country Manager for Solvay Pharma
- Observations and Suggestions
CHI’s first visit to The University Children’s Hospital in Riga, Latvia was conducted by Drs. Gary Connett, Chris Rolles and Toni Rolles between 28thFebruary – 2nd March 2008. During this visit the team followed the following schedule:
Arrived late evening
08.30: Met at our hotel by Dr. Vija Svabe and travelled with her by trolley bus to the Children’s Hospital.
09.00-11.00: Discussions about CF care arrangements in Latvia with Dr. Svabe.
11.00-12.15: Dr. Connett’s Lecture to the Department (see attachment 1 for presentation).
12.15-13.30: Lunch in the hospital kitchen with Dr Svabe and hosted by Dr. Dzintars Mozgins the director of the hospital.
13.30-16.30: Joint consultations of CF children (see attachment 2 for case summaries).
16.30- 17.00 Meeting with Prof Dace Gardovska, Head of the Department of Paediatrics, a representative of the Governmental Health Ministry and other senior paediatricians.
9.00-10.00: Meeting with Arvis Eisaks, Country Manager for Solvay Pharma.
10.00-13.00 Joint consultations with CF children and their families.
The Children’s Hospital in Riga was established in 1899 by an Englishman called James Armistead.
Latvia has a population of approximately 2.3 million people and a land area similar to that of Wales. The population is 60% native Latvian and 25% Russian. There are two medical schools in Latvia but the drop out rate from these is high because in general a career as a doctor in Latvia is not well paid. The system of health care is complex but many of the CF medicines are state provided or else can be reimbursed through insurance schemes. Children do not pay for hospital consultations.
The only resource in Latvia for CF care is based in the Children’s Hospital in Riga. This is a large centralised tertiary referral centre. Dr. Svabe is the only paediatrician providing CF services. She spends half of her time on CF care and half on University teaching responsibilities. The CF service is within the paediatric respiratory service. There are between 8-12 paediatric pulmonologists/physiotherapists in Latvia.
Dr. Svabe has had links in the past with the CF service in Stockholm, Sweden. This was initiated in 1993 with interactions occurring approximately twice/year. The nurse and paediatric pulmonologist from Sweden have visited the Children’s Hospital although this link is not ongoing currently.
On the advice of the Swedish CF visiting pulmonologist a parent’s organisations was established. This organisation is not particularly active currently although we did have the opportunity to meet the convenor of this group.
There are 28 patients currently attending the tertiary centre aged 6 months to 24 years. The highest number of patients at any time has been 31. Approximately 15-18 of these diagnosed patients are cared for within the city of Riga and the remainder have been referred in from outside and travel to the capital for hospital consultations.
Dr Svabe thinks that, on the basis of cord blood sampling genetic studies, there should be up to 8 new cases of cystic fibrosis diagnosed each year in Latvia (there are approximately 20-22,000 births/year in Latvia). This is considerably greater than the number of cases that are currently referred to her for diagnostic sweat tests. Based on these figures there should be up to 200 patients attending the regional CF centre for care. Dr. Svabe does not believe that any other paediatricians are caring for children with a diagnosis of cystic fibrosis in Latvia.
Latvian civilians attend general practitioners as their first point of contact for health care problems. Unfortunately, general practitioners’ knowledge of paediatrics is often poor and there are no paediatricians working in primary care services. One can only assume that the majority of children with CF are presenting with failure to thrive in infancy and are dying of malnutrition or chest infections before diagnosis.
The biochemistry department in the Children’s Hospital has the only sweat testing equipment in Latvia. This uses the nanoduct method. The more traditional Gibson and Cooke method (as still used in Southampton and many other regional centres in the UK) was rejected by the Latvian association of biochemists. Up until 10 years ago Dr Svabe performed all sweat tests and the titration of analytes herself. Research grants have funded genetic studies for CF in Latvia but newborn screening is not currently available. Cascade screening of relatives of index cases is available. Seventy-five percent of CF genes in Latvia are the most common DF508 mutation.
Most patients are on a similar treatment regime. All those with pancreatic insufficiency receive Creon 25,000 (or 10,000 through a reimbursement scheme). Creon micro (a formulation for infants) is not available.
Children receive the fat soluble Vitamin E and many take Acetylcysteine by nebuliser two to three times a day and also by mouth. This is a “mucolytic” agent often used in eastern European countries but for which there is little scientific evidence of benefit.
Patients do not have access to nebulised antibiotics such as Tobramycin and Colomycin or the mucolytic agent rhDNase although these drugs do occasionally become available as a result of budgetary negotiations for limited periods of up to 3 months at a time.
Intravenous antibiotics are available. These include Piperacillin, Gentamicin, Amikacin and Ceftazidime. Oral Ciprofloxacin is also available.
Most patients use outdated nebuliser systems that were originally donated from Sweden. This equipment is not state provided.
Dr. Rolles began this meeting by discussing the history of CF developments in Russia, India and more recently Lithuania.
Arvis Eisaks explained that currently Creon is Solvay’s 3rd most important product in cash terms in Latvia but approximately 90% of the local market is over the counter prescribing for digestive complaints and gastroenteritis rather than proven pancreatic insufficiency. There has been a long tradition of using pancreatic enzymes in this way in the Baltic states and a relatively poor understanding among the local population and some doctors as to the proven indications for the use of these medications.
Unfortunately, therefore, Solvay currently had only a small interest with regard to the use of Creon for CF in Latvia. However, with improved diagnostics to identify the large number of children who are currently escaping diagnosis, and access to appropriate care, this could lead to an increased market for Creon and the situation could change. He was keen to support a project to address this issue.
Discussions centred around educating primary care physicians (“the gate keepers”) but Dr. Eisaks pointed out that the problems were probably more related to secondary care paediatricians not considering the diagnosis of CF when individuals are referred to them. Dr. Connett pointed out that Prof Gardovska, the Head of Paediatrics and Infectious Diseases had talked about her meeting yesterday, concerned with care pathways for the febrile child. A similar problem based approach with an algorithm for the child with failure to thrive might usefully be developed that includes early consideration of the need for a sweat test and/or CF genotyping. This could be promoted either through Latvian Health Care Journals, inclusion of such materials in the undergraduate syllabus or Post Graduate Educational Meetings.
Dr. Rolles stated that the CF Trust might be willing to help with the provision of parent information that could be translated into Lithuanian. Further discussions considered the possibility of project planning across all of the Baltic States to increase the “patient power” to raise CF issues at a political level more effectively.
The Children’s Hospital in Riga is clearly a centre of excellence for the delivery of tertiary specialist paediatric services. This centralised model of health care for rare and life threatening conditions has enabled paediatricians to develop good services to meet the needs for such children throughout the whole of Latvia. However these services do depend upon clear referral pathways to ensure that sick children access the most appropriate specialist service and it would appear that this is not currently happening for the majority of CF children in Latvia. Whilst the services for CF have developed to a high standard within this institution they have depended almost exclusively on the hard work and dedication of a single paediatrician, Dr Svarbe. CF care in Latvia is currently critically dependent upon the continued efforts of this one individual and there does not appear to be any other paediatrician receiving specific training in this speciality. There is clearly a need to train a larger number of interested paediatricians in CF care to support Dr. Svabe and to plan for the continued provision of CF services into the future.
The current model for specialist care for CF in Latvia is very much consultant lead and there is no multidisciplinary team akin to that which has been developed in many other countries. There is no doubt that CF sufferers would benefit from a care package delivered by an expanded team including input from physiotherapists, dietitians and nurses with specialist skills in the management of this condition. This might facilitate the move to a less hospital centred approach to care and keep patients more healthy within their local communities – in effect, as out-patients.
Whilst there is a microbiological basis for antibiotic prescribing in Latvia, the only specimens processed currently are either expectorated sputum or lavage specimens obtained by fibre optic bronchoscopy. This means there is no information obtained about the presence of infecting bacteria in young non-expectorating CF sufferers unless they are bronchosocped (most children cannot expectorate until at least the age of 7). It is not practical to carry out such tests very often (i.e. once or twice a year). The visiting Swedish CF team had recommended laryngeal aspirate specimens in non-expectorating children but this invasive specimen collection method has understandably not been taken up because it is poorly tolerated by patients. Most CF centres apart form those in the Scandinavian countries do not use these types of specimens. The use of cough swabs as an alternative means of detecting infection in non expectorating children would be a major advance. This would enable treatment of early infections before the progression to severe complications such as advanced bronchiectasis. Nebulised antibiotics have been perceived as being poorly effective in the Latvia clinic. This might be for a number of reasons including their relatively late introduction for treating patients after established infection in more severe disease, poor compliance and/or poor drug delivery from out of date nebuliser systems. In the UK and many other countries, the introduction of nebulised antibiotic therapy, coupled with the provision of good nutrition, is believed to have been one of the major advances in CF care. It would seem preferable to opt for this approach to preventing CF lung infection with relatively inexpensive nebulised antibiotics such as colomycin and the soon to be generically available tobramycin rather than mucolytic agents such as acetylcysteine. There is now good evidence for the use of relatively inexpensive hypertonic saline as an agent to promote mucociliary clearance and perhaps the increased use of this agent could be usefully explored as an aid to clear airway secretions with physiotherapy. Nebulisers are not state funded. PARI-LC nebulisers are now cheap and highly effective costing around £40 to purchase in the UK. Providing this sort of equipment to the Latvian CF service would be a hugely cost effective means towards ensuring that prescribed nebulised therapies are effectively getting into patient’s lungs.
There is clearly a major problem with under diagnosis of CF throughout Latvia. This is probably happening at a primary care (GP) level but the diagnosis must also be being missed by paediatricians, to whom sick infants are being referred but they do not consider this diagnostic possibility. As already alluded to in the discussions with Dr. Eisaks, there are many vital opportunities to increase awareness about CF throughout Latvia. In particular a care pathway could be developed whereby children presenting with failure to thrive enter a diagnostic process that includes sweat testing. This “gold standard” diagnostic test needs to be more readily available to all paediatricians as an investigation that they can request when faced with poorly grown children and/or those with chronic respiratory symptoms. An alternative or complementary consideration would be the introduction of newborn screening throughout Latvia for CF. Genetic studies indicate that there is a very high carrier rate of common CF mutations and so screening test based on a few genotypes is likely to be highly successful in diagnosing the majority of patients with severe early onset phenotypes. This should prove cost effective in avoiding the initial presentation of severely affected individuals with advanced disease and therefore needing more prolonged in patient stays. There is clearly a well developed genetics unit with a motivated team, which could successfully take such an initiative forward.
There are clearly opportunities to link more closely with the pharmaceutical industry as already alluded to in the discussions that occurred with the Country Manager of Solvay Pharma. Similarly, more could be done to help develop the local parent’s group. Accessing materials and information from the UK CF Trust would be of benefit here.
Our visit to Latvia was most enjoyable. We were looked after extremely well and there was genuine interest in our visit coupled with a desire to try and improve the paediatric service where possible. Professor Dace Gardovska, the Head of The Department of Paediatrics has requested a copy of my report from the visit and I will forward this to her as well as to Dr. Vija Svarbe who provided most of the above information. I particularly enjoyed sharing consultations of patients with her and was impressed by her clear documentation, knowledge and dedication to the care of CF children. I appreciated the opportunity to establish such a link and find such common ground in a foreign country.
Consultant Paediatrician, Honorary Senior Lecturer
Southampton University Hospitals Trust, UK