Hemophilia Team Helps Patient after Accident
Dr. Kilach preparing for the surgery
Despite the increased challenges created by the COVID-19 pandemic, the AMPATH hematology team recently coordinated a successful surgery with the orthopedic service for one of their patients with severe hemophilia.
Hemophilia is a bleeding disorder that occurs due to a missing clotting factor in the blood. The genetic inheritance is X-linked recessive, and males are predominantly affected. Persons with hemophilia take much longer to form a stable blood clot, which significantly increases risk of bleeding. Bleeding may occur spontaneously, due to injury, or as a result of surgery, and is usually treated by intravenous infusions of clotting factor concentrate to replace the missing clotting factor. Unfortunately, people who receive clotting factor concentrate to treat bleeding may develop antibodies or inhibitors against it, rendering this type of treatment ineffective. In these cases, additional treatment with an agent that bypasses the normal clotting pathway is required.
Meet Mr. H, a 41-year-old man living with severe hemophilia with an inhibitor. He has received care in the hematology clinic for eight years and is very well-known to our team. Up until last year he was remarkably healthy and active, despite some mild limitation to his mobility, from prior joint bleeds causing joint pain. He is married, has four children, and sells used clothes.
Mr. H after surgery with part of his care team and family.
In April 2019, Mr. H was involved in a boda-boda (motorcycle transport) accident, sustaining a closed, displaced fracture of the right femur. The hematology laboratory at MTRH has the capability of measuring clotting factor and inhibitor levels, which are essential before any surgical procedure. In the case of Mr. H, the hematology team diagnosed a high-level inhibitor and surgery was deemed to have an excessive risk of uncontrolled bleeding. Unfortunately, after several months, there was little evidence that the fracture was healing, and Mr. H rapidly became wheelchair-dependent and unable to support his family.
The hematology team continued to advocate for Mr. H, recognizing his bleak situation. They spent eleven months gathering an adequate supply of donated by-passing products from various sources and creating a plan for surgery. Mr. H. was admitted in June 2020 for the operation. This involved close cooperation with the orthopedic team, nursing, physical therapy and other clinicians, as well as pre-operative COVID-19 screening.
Dr. Carole Kilach, our medical officer, was present in the operating room for the entire procedure, making sure that he received his first infusion of the by-passing product right at the start of the operation, and additional treatment as needed to control bleeding. Immediately after the surgery, the team made sure that the patient continued to receive his factor every few hours. They worked with the nurses on the orthopedic ward to provide a factor infusion every six hours for eight days, at which point he had recovered sufficiently to receive twice daily infusions until discharge. Our clinicians took measurements and pictures of his leg routinely, and communicated consistently with the hemophilia team in Indianapolis, as well as the other disciplines at MTRH. During the second week, Mr. H was able to begin gentle physiotherapy, and was able to leave the hospital on crutches. He continues therapy as an outpatient and feels much more hopeful that he may be able to walk again.
This type of surgery, in a hemophilia patient with an inhibitor, is very difficult to manage successfully, even in a setting where more resources are available. It can only be accomplished when there is a team experienced in the management of patients with hemophilia. The successful outcome of surgery for Mr. H was due to the fact that we are fortunate to have a team at MTRH with a thorough understanding of hemophilia care, even very complex situations. The successful outcome of this surgery was due to their expertise, as well as their absolute commitment to the patient and refusal to give up until a way forward could be found.
Special thanks to the following team members: Carole Kilach, Anne Greist, Cyrus Githinji, Festus Njuguna, Chris Roberson, Samuel Mbunya, Lindsay Dow and Chris Mwaniki.