A rare family in Italy

The story of Federica

My name is Federica and I live in Italy with my husband and our 2 children, 10 and 8 years old, respectively.

Our children have both a rare disease. The first one has brachydactyly in her right hand and the second one has macrodactyly in her right foot. Most people and physicians know brachydactyly while macrodactyly is a little-know disease. This is the reason why I choose to talk about it.

It is characterised by a sometimes significant overgrowth of 1 or more fingers or toes (due to the enlargement of bone, fat tissue, tendons and nerves) and can manifest in two forms: the static and the progressive one. Only recently researchers identified the possible cause of isolated macrodactyly, i.e. a somatic gain-of-function mutation in the PIK3CA gene, and that many deseases that were thought to be separate entities belong to a single spectrum called PROS (which is the acronym for PIK3CA-Related Overgrowth Spectrum). Macrodactyly and the limb overgrowths that often accompany it belong to the PROS spectrum.

My child has been very lucky and she only suffers from a minor and static form. But many children have more severe and progressive forms and the only solution to date is surgery. In many cases more than one surgery is necessary and sometimes amputation or ray resection is needed, so that children can wear shoes.

We have founded a charity here in Italy to help children with the disease and their parents. This is our website. www.macrodattilia.it. Last december we had our first conference with specialists of different areas (surgeons, geneticits, physical therapists, experts in gate analysis, psychologists. Videos from the conference are available on our website. We are providing them with subtitles so that even English-speaking people can understand them.

Despite their rare disease my children are wonderful. They are lively, joyful and do every sort of activity. All our children are special! We will try to support research, so that maybe one day a medical treatment can be available for the most severe cases that cannot be properly handled through surgery.  I wish all the best to all of you!