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Perspective interview - Maria Gizewska

Perspective interview - Maria Gizewska

PKU perspective - Professor Maria Gizewska

PKU patients are a community with common needs that are stronger than the separation caused by national borders, Maria Giżewska1 , Associate professor of pediatrics at the Pomeranian Medical University in Szczecin (Poland) insists. Vice-chair of the scientific committee of the ESPKU, she oversees a project to link PKU patients and professionals in West Pomerania in Poland and Meklemburg-Vorpommern in Germany.

Prof Giżewska spoke at the 3rd Dietitians’ Workshop in Istanbul (Turkey), giving delegates an insight into PKU in Poland.  She has been working with Polish PKU patients for over 20 years and currently has more than 200 patients in her care. Most of her patients are children and teenagers with classical PKU, but she also takes care of a number with milder forms of hyperphenylalaninemia, patients with BH4 deficiency, maternal PKU, and adults with PKU as well.

PKU in Poland

‘PKU is one of the most common IEMs in Poland’ the professor told us. ‘The incidence of the disease is 1:7000-1:8000 and right now we have about 3000 known patients treated for PKU. We have 8 centers involved in screening and treatment of PKU patients. They are treated by pediatricians experienced in PKU, all of whom are members of the Polish PKU society, so it is really quite a strong group of professionals involved in PKU.

‘The gold standard for PKU treatment in Poland is low-Phe diet and we recommend lifelong treatment with diet. BH4 therapy is only available for patients suffering from BH4 deficiency.’

Encouraging patients to adhere to diet for life presents challenges to professionals across the world and we asked Prof Giżewska what the approach is in Poland.

She said: ‘We believe that the very close relationship between the treatment team, the patient and their family from the very beginning of their treatment is crucial for this so we try to create a “PKU family” that includes the professionals, the patients and their families.

‘We also believe strongly that education can significantly improve the understanding of the disease in our patients so we organize meetings, patients’ conferences, summer camps and other activities.

‘Through everyday contact with the patients we reinforce the message that, even if right now they do not follow the dietary recommendation strictly, it is never too late to come back to a more strict diet if there is a need for it.’

Access to care

Prof Giżewska is passionate about the need to reduce the huge differences in the access to care for PKU patients that exist between nations.

She said ‘PKU is one of the most common IEMs and one might think that, since it has been known for so many years, the treatment is equal for all patients, but this is not true. We know that screening, treatment and follow-up of patients varies between countries, even in Europe. It’s hard to believe, but there are some countries where not all the newborns are covered by a screening test for PKU.

‘In some countries there are problems with following diet for life or lifelong follow-up of the patients. I strongly believe that all professionals, together with health care and parents’ institutions involved in PKU should do everything to decrease these differences.

‘The place where a PKU patient is born shouldn’t have such a significant influence on the quality of their care. We should offer all patients the best treatment options that are known to the professionals.’

But what can be done?  ‘First of all we need better education’ the professor said. ‘I am strongly convinced that education directed to professionals, patients, medical authorities and governments can significantly influence the awareness of the importance of early screening, good treatment and lifelong follow-up. The second thing that is essential to consider is economic support for countries who are struggling to fund the introduction of screening or lifelong treatment of PKU patients. Finally, we need to create international guidelines for the treatment of PKU.’

Optimism - working together

As vice-chair of the scientific committee of the ESPKU, Prof Giżewska is very involved in the ongoing work to create European guidelines for PKU. Given the cultural and dietary differences between different countries how straightforward, we wondered, was this process going to be.

Prof Giżewska does not downplay the challenges of agreeing the new guidelines but is still optimistic that good progress can be made. Her optimism is perhaps well-placed given the success of the ‘Pomerania’ project to link physicians and patients across the Polish–German border.

‘This is a common project between the University of Greifswald in Germany, the Medical University of Szczecin in Poland and the Institute of Mother and Child in Warsaw’ she told us.

‘The aim of the project is to improve and to increase the number of IEMs that are included in the screening panel for all the newborns born in Western Pomerania in Poland and Mecklenburg-Vorpommern in Germany.

‘This is a fantastic collaboration not only because of the improvements in screening but also the effects on treatment and the benefit from contact between the patients. It was just last November when we organized the first Polish–German meeting of PKU patients and their families’ Prof Giżewska said.

‘We invited the German PKU patients and their families to Szczecin and had a fantastic two days of being together. In April 2013, we’re going to have the second meeting, and this time Polish patients and their families are going to Germany, again for two days of lectures, and different activities intended to increase knowledge, certainly; but also to create the inter-personal contact between professionals and the patients involved in the PKU treatment on both sides of the Polish and German border.

‘It is very important for patients to see that they are not the only person affected by this disease, also that professionals are treating the disease similarly on both sides of the border. It’s of huge benefit for patients and their families to see that they have the same problems in everyday life with PKU, whether they are German or Polish.

‘It was fantastic to notice how they exchanged their recipes, how they exchanged their ways of coping with everyday difficulties. They noticed that PKU is the same disease no matter if it is in Poland or Germany. I think that this kind of cooperation can change the way patients feel about themselves.’

Next steps for PKU: access for all

‘Over the last 30 years,’ said the professor, ‘great improvement has been made in early screening and treatments available to PKU patients. But, we have to remember that there are still countries where optimal screening and treatment is still not offered. I strongly believe we have to work to rule this out because it should not be the case that the quality of care should depend on where you are born. All patients should be able to access the same quality of treatment.’

Maria Gizewska

Associate professor of pediatrics, Vice-chair of the scientific committee of the ESPKU
Pomeranian Medical University
Szczecin, Poland