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Updated:
23 September, 2024
Practical patient care for the management of patients with hemophilia
Practical Patient Care for Patients with Hemophilia
By Nina Jurander, specialist nurse at the Coagulation Center, Sahlgrenska University Hospital in Gothenburg.
Reviewed by Fariba Baghaei, senior physician at the Coagulation Center, Sahlgrenska University Hospital in Gothenburg.
Assessment of a Patient with Hemophilia
A hemophilia patient does not stop bleeding without treatment. This applies regardless of whether the hemophilia is mild, moderate, or severe. Therefore, the Coagulation Center/clinic should always be contacted in case of bleeding, even when the patient is in a unit where bleeding is commonly occurring, e.g., nosebleed in the ENT department or hematuria in urology.
Hemophilia Patient in the Emergency Department
Hemophilia patients should be prioritized until bleeding is ruled out. The coagulation on-call doctor should be contacted via switchboard when the patient arrives at the emergency department. The patient often carries a bleeding risk card with details of their hemophilia and contact information for their clinic. Factor concentrate is the first treatment for bleeding and should be administered without delay. The dose and preparation are prescribed by the coagulation on-call doctor.
IV Access
Avoid placing a peripheral venous catheter (PVC) in blood vessels that the patient uses for their treatment. PVCs are larger than butterfly needles and can damage blood vessels. Use the smallest possible PVC to preserve the veins, as the patient requires lifelong intravenous treatment.
Other IV Access
The patient may need medication before the insertion or removal of other IV lines, such as drains or central venous catheters (CVCs).
Patients with hemophilia are often experienced in mixing their own medication and administering their injections intravenously using a butterfly needle. Feel free to consult the patient.
Blood Sampling
Always apply pressure for several minutes after puncture, even if the venipuncture was unsuccessful. Hemophilia patients can develop a bleed that is not visible until later and can cause compartment syndrome. Avoid multiple sampling attempts and assess the necessity. Avoid capillary sampling, even in children.
Medications
Avoid aspirin or other medications that affect platelet function. For pain relief, prioritize paracetamol. NSAIDs should be avoided as much as possible.
Intramuscular Injections
Avoid intramuscular injections. Consult the coagulation on-call doctor if an intramuscular injection is necessary.
Treatment with Intravenous Factor Concentrate
The preparation and dose are prescribed by the coagulation doctor.
Information on how to mix, administer, and store the factor concentrate is available via the package leaflet, by scanning the QR code on the package, or through instructional videos on FASS.se.
If coagulum is observed in the syringe, stop the injection and contact the coagulation clinic for advice on further management.
During home treatment, factor concentrate is administered into a peripheral vein via a butterfly needle or into a subcutaneous venous port. If the patient has a PVC or CVC, the factor concentrate can be administered through them, but the factor preparation should be administered alone, without any other drip or medication. The access line should always be flushed thoroughly with NaCl after the injection is completed.
The subcutaneous venous port should be flushed with at least 20 ml NaCl after the injection is completed. For children weighing less than 20 kg, flush with 20 ml; for children under 10 kg, use 10 ml. When using a factor concentrate containing both factor VIII (FVIII) and von Willebrand factor (vWF), the dose is prescribed based on FVIII, even if both FVIII and vWF contents are listed on the package. The on-call doctor at the Coagulation Center/clinic should be contacted to access factor concentrate from the emergency stock.
Other Treatment for Hemophilia
Patients treated with subcutaneous monoclonal antibodies may require additional intravenous factor concentrate for trauma, procedures, or surgery. For minor bleeding, Octostim may be sufficient for some patients instead of intravenous factor treatment. In most cases, patients should receive additional treatment with Tranexamic acid. Whether it’s factor concentrate or other medications, treatment should always be prescribed by a coagulation doctor.
Blood Sampling for Factor Concentration (Factor VIII/Factor IX/vWF)
Blood samples are taken in citrate tubes (filled to the line) and inverted at least five times. The tubes must be delivered to the laboratory staff within 30 minutes. Both pre- and post-samples are usually measured to evaluate effectiveness and possibly prescribe additional medication. On the laboratory requisition, indicate the time for the pre- and post-sample, the time of the last medication administration, the medication given, how many units of the medication were administered, and the patient’s weight. Be sure to label the sample tube carefully to avoid confusion between pre- and post-samples. It is important that the citrate tube is completely filled to the line and that the sample tube label is marked with the time of sampling.
Pain in Joint/Extremity
Never attempt to straighten a joint where a hemophilia patient has pain or reduced mobility. The restriction of movement and pain are likely due to a joint bleed, which can worsen if forced.
During Surgery
Anesthesia and Surgery
Factor concentrate should always be administered before anesthesia, as intubation and insertion of a laryngeal mask pose a high risk of life-threatening airway bleeding. For planned surgeries, a treatment plan from the Coagulation Center/clinic should always be in place. In emergency cases, the patient’s doctor at the Coagulation Center/clinic should always be contacted. Remember not to attempt to straighten a bent joint during anesthesia.
Epidural Anesthesia
May be administered after treatment with factor concentrate. Contact the Coagulation Center/clinic for a treatment plan before insertion and removal of the catheter.
Spinal Anesthesia
May be administered after treatment with factor concentrate. Contact the Coagulation Center/clinic for a treatment plan before puncture.
During Dental Treatment
Dental Treatment
For planned surgeries, a treatment plan from the Coagulation Center/clinic should always be in place. In emergency cases, the on-call coagulation doctor at the Coagulation Center/clinic should always be contacted. Factor concentrate treatment should be given as close as possible to the dental procedure.
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