Surgery in patients with haemophilia and inhibitors
In patients with inhibitors, musculoskeletal functions often deteriorate more quickly in comparison to those in people who have not developed inhibitors, because hemarthrosis may be more serious and less susceptible to treatment, leading to the earlier onset of joint damage. Therefore the need for orthopaedic surgery is often be more acute than in patients with no inhibitors as it may alleviate a great deal of pain and improve quality of life.
Nevertheless, patients with inhibitors are often denied surgery or any invasive procedures as those imply high risk and are very expensive. However, nowadays it is possible to have optimal inhibitor management in place and this means that any type of major surgery can be carried out safely and with good results. Furthermore, in the long term it is likely that these surgeries can prove to be cost-effective.
“All but essential surgery is generally avoided in haemophilia patients with inhibitor antibodies, because of concern about the reliability with which haemostasis can be achieved and maintained in such patients. Orthopaedic surgical procedures, which are not required to preserve life fall under this category. As a result, patients with inhibitors may be denied operations, which could greatly enhance their quality of life and which are routinely offered to other haemophilia patients. While caution is appropriate in recommending surgery in any circumstance, we believe that the threshold for offering validated surgical procedures to patients with inhibitors should be re-evaluated in the light of current surgical and rehabilitative techniques, and the long experience with safe and effective factor VIII inhibitor bypassing agents”.
Teitel JM et al. Haemophilia 15: 227-239 (2009)
It should be noted, however, that any invasive procedure will require the relevant expertise and consultation within the entire multidisciplinary team, as well as carefully planned logistics.
In this article you will find some advice when considering, planning and practical aspects while undergoing surgery.
- There is no such thing as minor surgery in a patient with haemophilia and inhibitors. The risk of bleeding is high even in seemingly minor types of surgery: e.g. dental extraction, vasectomy, removal of skin lesions, therefore surgery should only be conducted at a centre with specific surgical experience of inhibitor patients.
- Good planning and teamwork is required:
- Availability of key clinical, laboratory and nursing staff throughout scheduled inpatient stay should be ensured;
- Availability of adequate stock of treatment product should be ensured;
- A patient should undergo surgery early in the week and should ideally be scheduled for the morning;
- A longer duration of stay in the hospital than with other patients should be foreseen.
- It is important to rule out other haemostatic problems ahead of time, e.g. check platelet count, prothrombin time etc.
- High cost needs to be budgeted for: a bill for major surgery can easily reach €400,000.
- Either aPCC or rFVIIa can be used during surgery, but a history of good response to the chosen bypassing agent is important;
- The use of epidural anaesthesia is not recommended to avoid potential bleeding around the spine. General anaesthesia will invariably be required though.
- There is no need for specific laboratory tests to monitor treatment with either of the bypassing agents.
- Physiotherapy should be scheduled for shortly after dosing with bypassing agent.
The recovery of inhibitor patients after surgery may take longer than in those patients without inhibitors. Therefore it is essential that proper rehabilitation programme of physiotherapy, tailored to the individual patient, is in place already before the surgery.