The outpatient clinic at MHH Children's Hospital offers groundbreaking care for people with rare cancer predisposition syndromes.
Prof. Dr. Christian Kratz and Dr. Valentina Härter in professional discussion at the KPS outpatient clinic of MHH Children's Hospital. Copyright: Karin Kaiser/MHH
Genetic predisposition is the most significant known risk factor for cancer in children and adolescents, but it also plays an important role in adults.
It is estimated that around ten percent of cancer patients have an underlying cancer predisposition syndrome (CPS). In Germany, around 500,000 people are affected. The CPS outpatient clinic at MHH Children's Hospital has set itself the goal of improving clinical care pathways for people with rare CPS.
Individual counselling, early detection and therapy are crucial for those affected
A CPS is a genetic disorder that increases the risk of certain types of cancer. Those affected face health challenges that require individual medical care. Detailed counselling, the recommendation and implementation of targeted early detection examinations and treatment based on the latest findings are crucial for those affected. "With our KPS outpatient clinic, we want to offer people with rare KPS the best possible care. We currently care for several hundred children, adolescents and adults, with patients travelling from all over the country. We systematically collect data in order to continuously improve our recommendations. The latest research findings are incorporated directly into clinical care. In addition, those affected have access to special early detection examinations and the opportunity to participate in various scientific studies," explains Professor Dr Christian Kratz, Director of the Clinic for Paediatric Haematology and Oncology at MHH and Head of the KPS Outpatient Clinic.
‘We are grateful that the KPS outpatient clinic exists in Hanover,’ says Tanja Obermeier, deputy chairwoman of the International Bloom Syndrome Association and mother of a child with Bloom syndrome, an extremely rare disease associated with short stature, immune deficiency and a high risk of cancer at an early age, with a significantly reduced life expectancy.
Insufficient interdisciplinary care structures
While care pathways exist for people with hereditary breast and ovarian cancer and familial colorectal cancer, there is currently a lack of adequate interdisciplinary care structures for people with rare KPS.
MHH's KPS outpatient clinic, which works closely with the Institute of Human Genetics and the Institute of Diagnostic and Interventional Radiology, addresses this issue and makes a significant contribution to improving clinical care pathways for people with rare KPS. The focus is not only on caring for patients at MHH. In the outpatient clinic, those affected are included in various research projects with the aim of improving knowledge about CPS and care. Comprehensive care for those affected would require the establishment of a Germany-wide network of similarly oriented CPS outpatient clinics.
"In some cases, KPS is not diagnosed until one or more cancers have developed. The increasing use of genetic testing in cancer patients means that KPS is being diagnosed more frequently. However, such a diagnosis only makes sense if those affected receive appropriate counselling and if the recommended clinical consequences in the areas of cancer prevention, monitoring and therapy can also be implemented," explains Dr Valentina Härter, a doctor undergoing further training at the KPS outpatient clinic and at MHH Children's Hospital.
Expansion of specialised CPS outpatient clinics required throughout Germany
Professor Kratz points out that there are currently only a few medical infrastructures in Germany that offer adequate care for people with a CPS diagnosis. "There are several reasons for this shortfall: Most CPS are little-known rare diseases. In addition, clinical care pathways for people who are currently healthy but have an increased risk of developing a disease entity such as cancer are not well defined. Some CPS often require expertise from several disciplines. Another challenge is that the recommended examinations are often not reimbursed at a level that covers the costs. In addition, several generations of a family may be affected, but family-centred clinical care is rarely available," says Kratz.
Dr Valentina Härter and Professor Kratz's team recently published their experiences with the first 399 patients treated at the KPS outpatient clinic in an article in the journal The Lancet Regional Health – Europe. The team is supported by German Cancer Aid, the German Children's Cancer Foundation, the Federal Ministry of Education and Research, the Association for Children with Cancer in Hanover and the Gerdes Foundation. Further information on cancer predisposition syndromes can also be found at https://www.krebs-praedisposition.de.
The original article can be found here.
Text: Jana Illmer