Interview with Dr. Jeanne Franzone, Nemour’s Children’s Hospital, Wilmington, USA

In most countries, pediatric surgeons are not allowed to operate on adults with OI because of the way hospitals are organized. Usually, the surgery is performed by an adult physician who knows about one body part (like hip, fingers, knee, pelvis etc). What are the pros and cons of having OI-specialists, who can also operate adults? We asked three very experienced OI-surgeons for their opinion on this. First out is dr. Jeanne Franzone from the USA. 

Who are you and what is your relationship to OI?

My name is Dr. Jeanne Franzone. I am an Orthopaedic Surgeon at Nemours Children’s Hospital in Wilmington, Delaware in the USA.

Dr. Richard Kruse (Orthopaedic Surgery), Dr. Michael Bober (Orthogenetics) and I are Co-Directors of our Multidisciplinary Osteogenesis Imperfecta Program. Although we are at a children’s hospital, we are able to follow our OI patients and families during the prenatal period, delivery process and neonatal care and up through the age of 35 years old.

Tell us about your experience!

On an annual basis, we are fortunate to see hundreds of unique OI patients. At our center, we perform upper and lower extremity reconstructive procedures and OI spine procedures. Over the past two years, one quarter of our patient volume has been in the age group of 18-25 years.

What is the difference between operating children and adults with OI?

There are principles of OI orthopaedic surgery that apply to both children and adults, but also certain differences. Regarding the differences, we consider the preoperative work up, surgical procedure and recovery. The preoperative workup for adults is often more complex and requires many medical subspecialties as adults tend to have more medical comorbidities and conditions that require coordinated multidisciplinary medical care. Regarding the surgical procedure, it is critically important to address the specific reason for which the surgery is being done. What exactly is causing the pain or functional limitation?

Adults tend to have more difficulty healing osteotomies than young children do, and it is important to tailor the surgery to each patient and his or her specific issues. This impacts the postoperative recovery as well, as it is preferable to minimize the postoperative immobilization for adults. Adults often additionally have family and work responsibilities to consider. Another consideration for all surgeries is Pain management. By the adult years, many OI patients are familiar with what options have worked well in the past and it is helpful to communicate this information to the perioperative care team.

Do you sometimes refer to an adult specialist?

Yes – there are certain procedures such as a total knee replacement for which we may refer to an adult joint replacement surgeon. Our pediatric center is somewhat unique in offering total hip replacement procedures for young adults with skeletal dysplasia.

What are the pros and cons of having OI-specialists who can also operate adults?

We feel strongly that it is important to care for patients with OI as a whole person and to care for OI patients with a multidisciplinary team.

Should we advocate for having more pediatric surgeons doing surgery on adults with OI?

The topic of transition of care for young adults with OI is a very important one. Advocating for multidisciplinary medical and surgical homes for patients with OI into adulthood would help improve OI care.

Should it be a collaboration between a pediatric OI-surgeon and an adult specialised surgeon?

Collaboration of pediatric OI surgeons and an adult specialized surgeon can be very helpful.

Do you belong to any international networks for OI-surgeons? If yes – which?

Dr. Kruse and I have organized an International OI Surgeons Work Group, which is an international gathering of specialized OI surgeons representing 10 countries and 3 continents. The group conducts case-based discussions to discuss surgical care in a way that spans different healthcare systems and available resources and also conducts multicenter OI clinical research.

Check out the Interviews with Dr. Guus Janus from the Netherlands and Dr. Michael To from Hong Kong.