Bleeding disorders and surgery

What to expect

ALEX CONNOLLY

Alex Connolly is the Clinical Nurse – Haemophilia at the Queensland Haemophilia Centre, Royal Brisbane and Women’s Hospital

Having surgery can be a daunting prospect at the best of times and people with a bleeding disorder might be more worried than others. However, just because you have a bleeding disorder that doesn’t mean you can’t undergo surgery. It does require a bit more planning and keeping your health care professionals in the loop on what is happening. Patients should be managed ideally at or in consultation with a comprehensive Haemophilia Treatment Centre. In an emergency situation this might not be possible but consultation with the staff at the Haemophilia Treatment Centre is a must. 

Any person undergoing surgery will have a pre-operative health assessment and again people with a bleeding disorder are no different. They do, however, require a consultation with a haematologist to develop a surgical plan and to determine the need for replacement therapy and what type. 

At the pre-operative assessment the doctor will determine if you need a blood test. An inhibitor screening test and or a factor level may be required to adequately determine a treatment plan.  When you are having surgery, in most cases a bolus dose of factor replacement is given about 60-90 minutes before your procedure. For people responsive to desmopressin (DDAVP) either an intravenous or subcutaneous dose is given. The bolus dose is to ensure a factor level peak while in surgery. This will limit bleeding during or immediately post-surgery. Patients with severe to moderate haemophilia may need a continuous infusion of factor replacement during and after surgery to keep their levels at optimum levels to decrease the risk of bleeding.

WHAT IS MINOR SURGERY AND WHAT IS MAJOR SURGERY?

Minor surgery is the type of surgery that can be often done as a day procedure and has minimal blood loss – procedures such as minor dental procedures, endoscopy/colonoscopy, removal of skin lesions and arthroscopy

Major surgery is surgery that has a significant risk of large volume blood loss or blood loss into a confined anatomical space. Procedures such as joint replacements, spinal, cardio vascular, abdominal and intracranial (brain) surgery are considered major procedures.

Both minor and major surgery requires factor replacement therapy. 

In the case of minor surgery this may be one to five days of therapy and in major surgery can be at least five to fourteen days. Both depend on the type of surgery and the severity of the bleeding disorder. 

When undergoing a dental procedure it is recommended that patients take some form of antifibrinolytic agents such as tranexamic acid as this will promote clot stability and ideally should be administered shortly before induction of anaesthesia. Alternatively, oral administration (1 g 3–4 times per day) may be commenced a day or two before surgery to ensure adequate blood levels are present at the time of operation.

POST-OPERATIVE MANAGEMENT

Depending on individual needs and type of surgery, a hospital stay may be necessary. This can be an overnight stay or several days or weeks. At times patients may be able to attend an outpatient or GP setting to receive factor replacement for a number of days post-surgery. Factor levels may be checked regularly to determine the adequacy of factor replacement. If you have mild haemophilia A, and have had intensive replacement therapy for the first time, an inhibitor test should be performed approximately four to twelve weeks after surgery.

Your surgeon might want to see you for a follow up appointment and it is important that you keep this appointment even if you feel okay. The haemophilia team might be able to see you then as well to see how you are progressing and if you need anything from the team, please ask!

THINGS TO KEEP IN MIND

• Inform your Haemophilia Treatment Centre (HTC) sooner rather than later when surgery is planned. It takes time to get factor organised particularly if the surgery is not at an HTC.
• Have your blood tests as required and let the HTC team know so results can be checked in a timely manner.
• Tell your specialist that you have a bleeding disorder. This may sound silly but you are the best advocate for yourself!
• The key to successful surgical management of the patient with a bleeding disorder is a multidisciplinary approach involving not only surgeons, anaesthetists and haematologists, but also laboratory scientists, specialist physiotherapists and haemophilia nurses. With careful planning, most surgical and invasive procedures can be carried out safely in persons with haemophilia and other bleeding disorders.
• A question not asked is a door not opened.

REFERENCES

1.  Australian Haemophilia Centre Directors’ Organisation (AHCDO). Guidelines for the management of haemophilia in Australia. Canberra: National Blood Authority, July 2016. 
2. Mensah PK, Gooding R. Surgery in patients with inherited bleeding disorders. Anaesthesia 2015;70 (Suppl 1): 112–120. 
3. Royal Brisbane and Women’s Hospital Services District. Procedure Haemophilia and Von Willebrands Disease. Brisbane: RBWH, 2014.
4. Marilee Goldberg. The art of the question: a guide to short-term question-centered therapy. New York: Wiley, 1998.