Dr. Richard W. Kruse recently published a new book on surgery in OI. OIFE was one of the consultants in the writing of the book, to secure that the voices of people with OI were represented. But dr. Kruse is not only a writer. He’s a busy doctor involved in many different projects and activities both nationally and internationally – including developing a new delivery clinic for babies with severe diseases and the international expert group connected to Key4OI. Because of all these activities, we thought it was time for a conversation.

Who are you and what is your relationship to OI?
I’m Richard W. Kruse – a Professor of surgery, pediatric orthopedic surgeon, and codirector of the osteogenesis imperfecta clinic with Dr. Michael Bober MD PhD pediatric geneticist at the Nemours Alfred I DuPont hospital for children in Wilmington, DE USA. I have a subspecialty in the surgical treatment of OI and see children and adults with OI as part of my daily clinical and research work. I am a member of the Medical Advisory Council of the Osteogenesis Imperfecta Foundation in the United States. I co-chair an international task force working on a consensus paper in the surgical treatment of OI with Dr. Ralph Sakkers in the Netherlands. I have recently finished producing a textbook on OI for surgeons.

I have been working as an OI subspecialist for 26 years. We see OI patients daily. We are fortunate to be able to follow patients fracture care in our extensive fracture clinic which I supervise. We have a large multidisciplinary team and have a formal team meeting and multidisciplinary clinic twice a month. We, as a team, and with international collaborators, are all involved in clinical research. 

In addition we have a comprehensive multidisciplinary team caring for both children and adults up to age 35 with OI. We are all in the same clinical location so we find interdisciplinary communication to be a strong part of our program. 

Could you say something about your involvement in the Key4OI project?
The Key4OI project involve an international group of OI experts organized and led under project manager Dagmar Mekking, CEO of Care4BrittleBones. The goal is to identify and achieve outcomes of treatment that matter to patients with OI throughout their lifetime. The project involves worldwide representatives and seeks to improve OI care in patients of all cultures and socioeconomic status. 

The key question is:
“What data should be collected, evaluated and compared taking into account both patient reported outcome measures and clinically reported outcome measures to drive sustainable insights for healthcare improvement and research into both. The primary objective is to reach multidisciplinary and international consensus for a standard set of outcomes in OI care and research.”

You told us about your new delivery unit for babies, can you explain what it is?
Our advanced delivery center is designed to help healthy expectant mothers deliver their baby who will require immediate and complex care after delivery.  The deliveries will be scheduled inductions or scheduled C-sections.  The baby will receive comprehensive coordinated care from pediatric subspecialties immediately after birth and mom will get to be in the same hospital. We have had one delivery of a patient with OI who was subsequently cared for in our NICU and continues to receive ongoing follow up by our subspecialty team after discharge. 

Who can come to the clinic?
We welcome patients from all over the US and even outside the US. If patients are not local, the mother will need to transfer her OB/prenatal care to our adult hospital partner, Christiana Care, in the event that she develop conditions that preclude her from delivery at Nemours (pre-eclampsia, for example) and delivery would have to occur at our neighboring medical center with postnatal transfer to Nemours of the baby. Insurance needs to be vetted as well prior to acceptance for delivery at Nemours. 

I’ve heard someone say that OI type II is lethal, but that some are “just too stubborn to die”. What are your thoughts this?
This is a very complex situation. The difference between type II and type III OI is not absolute. If one is to use radiographic criteria to make this distinction, there is no doubt that there are some infants with radiographic type II OI who have survived with aggressive care. There have also been infants with type III OI by radiograph who did not survive despite interventions. Some physicians have considered making the distinction between type II and type III OI based upon survival itself and not radiograph. Our belief is that the type of care and support to be provided to an infant should be based on the wishes of the family after extensive discussions about possible outcomes. While there are some children who may not survive despite intervention, there are others who surpass prognostic expectations, and this can be hard to discern from radiograph alone. 

Are there any special recommendations on birth of a baby with mild or severe OI?
There does not appear to be a significant benefit for the numbers of fractures seen at birth based on method of delivery. Decision should be made on a case-by-case basis and determined by obstetrical and maternal factors such as previous delivery method and whether or not the mother has OI. 

There are not clear clinical guidelines regarding the mode of delivery for a patient with OI. While theoretically delivery by cesarean is potentially less traumatic, evidence suggests that cesarean delivery does not decrease fracture rates at birth in infants with nonlethal forms of OI, nor does it prolong survival for those with lethal forms. 

In one study, most deliveries still occurred by cesarean for the sole reason that the fetus had OI. Overall, this decision should be individualized and made jointly by OB and neonatology/pediatric specialists. In addition to fetal and maternal considerations, other factors important to the decision include timing and ability of resources for a controlled delivery and postnatal stabilization.

These kind of programs cannot be developed in all hospitals/countries. How can we make the birth of an OI-child in any country as good as possible?
If it is known that a child will be born with OI, having a medical care team in place and having family and team discussions about care and interventions before delivery is best practice. We here are working closely with colleagues worldwide as mentioned above to begin to address the disparities of care available to patients and families with OI. It is a total group effort requiring extensive international collaboration to not only understand and address cultural and socioeconomic aspects but to keep the patient’s view at the forefront in addressing barriers.

And finally – congratulations on your new book! Do you have any messages for OIFE Magazine?
My answer to this is a thank you to OIFE for leadership in advancing patient care and research. It is absolutely critical that patient driven organizations lead the effort. We as health care providers need to more completely understand how our actions and decisions affect your lives. Also we need to thoroughly understand the priorities of your individual situations to provide you optimal outcomes.

Additionally things will not improve without all our voices being heard by governments, insurance providers, and communities.