Novoeight

Ukraine

INSTRUCTIONS FOR MEDICAL USE OF THE MEDICINAL PRODUCT NovoEight® (NovoEight®)

Composition:

Active substance: turoctocog alfa;

One vial of powder contains 250 IU or 500 IU or 1000 IU of turoctocog alfa (recombinant human coagulation factor VIII (rDNA));

Excipients: sodium chloride; L-histidine; sucrose; polysorbate 80; L-methionine; calcium chloride, dihydrate; sodium hydroxide; hydrochloric acid.

Solvent: sodium chloride, water for injections.

After reconstitution, 1 ml of NovoEight® solution contains approximately 62.5 IU, 125 IU or 250 IU of turoctocog alfa (recombinant human coagulation factor VIII (rDNA)).

Pharmaceutical form. Powder and solvent for solution for injection.

Main physicochemical characteristics: lyophilized powder or friable mass, white or slightly yellow. Solvent: clear, colorless injection solution.

Pharmacotherapeutic group. Haemostatics. Coagulation factors. Coagulation factor VIII.

ATC code B02BD02.

Pharmacological Properties

Pharmacodynamics

Mechanism of Action

NovoEight® contains turoctocog alfa, a recombinant human coagulation factor VIII (rFVIII) with a truncated B-domain. This glycoprotein has the same structure as activated human factor VIII and the same post-translational modifications as the molecule isolated from plasma. It has been established that the tyrosine sulfation site at Tyr1680 (full-length native factor) — which is important for binding to von Willebrand factor — is fully sulfated in the turoctocog alfa molecule. When administered to patients with hemophilia, factor VIII binds to endogenous von Willebrand factor in the patient's blood. The factor VIII/von Willebrand factor complex consists of two molecules (factor VIII and von Willebrand factor) with distinct physiological functions. Activated factor VIII acts as a cofactor for activated factor IX, accelerating the conversion of factor X to activated factor X. Activated factor X then converts prothrombin to thrombin. Thrombin subsequently converts fibrinogen to fibrin, resulting in clot formation. Hemophilia A is an X-linked inherited coagulation disorder caused by reduced levels of factor VIII:C, leading to severe bleeding into joints, muscles, or internal organs, either spontaneously or following trauma or surgical procedures. Replacement therapy increases factor VIII levels in plasma, providing temporary correction of the deficiency and reducing the tendency to bleed.

It should be noted that annual bleeding rates (ABR) at different factor concentrations and across various clinical trials are not directly comparable.

Clinical Efficacy

Four multicenter, open-label, uncontrolled clinical trials were conducted to evaluate the safety and efficacy of NovoEight® for prophylaxis and treatment of bleeding episodes and during surgical procedures in patients with severe hemophilia A (factor VIII activity ≤ 1%). Three of these studies involved previously treated patients, and one involved previously untreated patients. A total of 298 patients participated in the studies: 175 adults and adolescents aged 12 years and older (≥150 days of exposure to the investigational product) without documented inhibitors, 63 pediatric patients under 12 years of age (≥50 days of exposure) without inhibitors, and 60 previously untreated patients under 6 years of age. Of the 238 previously treated patients, 188 continued into an additional safety assessment study. Treatment with NovoEight® was shown to be safe and provided the desired hemostatic and prophylactic effects. A total of 3,293 bleeding episodes were recorded in the 298 patients, and 2,902 (88.1%) of these were resolved with 1–2 infusions of NovoEight®.

Table 1. Use of NovoEight® and efficacy outcomes in previously untreated and previously treated patients

Parameter

Younger age group children

(0 to less than 6 years), untreated

Younger age group children

(0 to less than 6 years), treated

Older age group children (6 to less than 12 years), treated

Adolescents (12 to less than 18 years), treated

Adults

(≥ 18 years), treated

Total

Number of patients

60

31

32

24

151

298

Dose used for prophylaxis per patient (IU/kg BW)

Mean (SD)

45.2 (14.4)

41.5 (8.1)

38.4 (9.4)

28.5 (9.3)

28.5 (8.3)

32.8 (10.9)

Minimum; maximum

4.5; 363.8

3.4; 196.3

3.2; 62.5

17.4; 73.9

12.0; 97.4

3.2; 363.8

Dose used for treatment of bleeding (IU/kg BW)

Mean (SD)

43.6 (15.2)

44.0 (12.6)

40.4 (10.5)

29.3 (10.3)

35.0 (12.3)

37.5 (13.4)

Minimum; maximum

11.9; 118.9

21.4; 193.8

24.0; 71.4

12.4; 76.8

6.4; 104.0

6.4; 193.8

Effectiveness ratea, %

87.0 %

92.2 %

88.4 %

85.1 %

89.6 %

88.9 %

MT − body weight, SD − standard deviation.

a Efficacy defined as excellent or good.

Clinical data obtained prior to registration were confirmed by a post-marketing non-interventional safety study conducted to provide additional information on immunogenicity, efficacy and safety of NovoEight® under normal clinical practice. Overall, 68 previously treated patients (> 150 IU (international units)), including 14 patients under 12 years of age and 54 patients aged 12 years and older, received treatment on-demand (N = 5) or prophylactic treatment (N = 63) for a total of 87.8 patient-years and 8967 IU.

Surgery

A total of 30 surgical procedures were performed in 25 patients, of which 26 were major surgeries and 4 were minor surgeries. Hemostasis was successful in all surgical procedures; no treatment failure was reported.

Data on immune tolerance induction (ITI) were collected in patients with hemophilia A who developed factor VIII inhibitors. During clinical trials involving previously untreated patients (PUPs), 21 patients received ITI treatment and 18 patients (86 %) completed ITI therapy with a negative inhibitor test result.

Pharmacokinetics

All pharmacokinetic studies with turoctocog alfa were conducted after intravenous administration of a single 50 IU/kg dose of NovoEight® in patients with severe hemophilia A (FVIII ≤ 1 %) who had previously been treated. Plasma samples were analyzed using a one-stage clotting activity assay and a chromogenic assay.

The activity of NovoEight® in the FVIII:C assay was evaluated and compared to a marketed full-length recombinant FVIII product. The study demonstrated that results were comparable and consistent between both products, and that NovoEight® can be reliably measured in plasma without the need for a specific product standard.

Pharmacokinetic parameters of NovoEight® following administration of a single dose, based on the clotting activity assay, are presented in Table 2, and based on the one-stage chromogenic assay in Table 3.

Table 2. Pharmacokinetics of turoctocog alfa (50 IU/kg) by age – One-stage clotting activity assay – Mean (standard deviation)

Parameter

From 0 to 6 years

From 6 to 12 years

≥ 12 years

n = 14

n = 14

n = 33

Mean value (SD)

Mean value (SD)

Mean value (SD)

Increase in level (MO/dl)/(MO/kg)

1.8 (0.7)

2.0 (0.4)

2.2 (0.4)

AUC ((MO×hour)/dl)

992 (411)

1109 (374)

1526 (577)

CL (ml/hour/kg)

6.21 (3.66)

5.02 (1.68)

3.63 (1.09)

t½ (hour)

7.65 (1.84)

8.02 (1.89)

11.00 (4.65)

Vss (ml/kg)

56.68 (26.43)

46.82 (10.63)

47.40 (9.21)

Cmax (MO/dl)

100 (58)

107 (35)

123 (41)

Mean duration of action (hour)

9.63 (2.50)

9.91 (2.57)

14.19 (5.08)

AUC – area under the pharmacokinetic curve describing the time course of factor VIII activity; CL – clearance; t1/2 – terminal half-life; Vss – volume of distribution at steady state; Cmax – maximum factor VIII activity.

Table 3. Pharmacokinetics of turoctocog alfa (50 IU/kg) by age – Chromogenic assay – Mean (standard deviation)

Parameter

From 0 to < 6 years

From 6 to < 12 years

≥ 12 years

n = 14

n = 14

n = 33

Mean (SD)

Mean (SD)

Mean (SD)

Increment level (MO/dl)/(MO/kg)

2.2 (0.6)

2.5 (0.6)

2.9 (0.6)

AUC ((MO*hr)/dl)

1223 (436)

1437 (348)

1963 (773)

CL (ml/hr/kg)

4.59 (1.73)

3.70 (1.00)

2.86 (0.94)

t½ (hr)

9.99 (1.71)

9.42 (1.52)

11.22 (6.86)

Vss (ml/kg)

55.46 (23.53)

41.23 (6.00)

38.18 (10.24)

Cmax (MO/dl)

112 (31)

125 (27)

163 (50)

Mean duration of action (hr)

12.06 (1.90)

11.61 (2.32)

14.54 (5.77)

AUC – area under the pharmacokinetic curve describing the time course of factor VIII activity; CL – clearance; t1/2 – terminal half-life; Vss – volume of distribution at steady state; Cmax – maximum factor VIII activity.

Pharmacokinetic parameters were comparable in patients under 6 years of age and those aged 6 to 12 years. Some differences in pharmacokinetic parameters of NovoEight® were observed between pediatric and adult patients. Higher clearance and shorter t½ in children compared to adults with hemophilia A may be partially explained by a larger plasma volume per kilogram of body weight in younger patients.

A single-dose pharmacokinetic study (50 IU/kg) was conducted in 35 hemophilia patients (≥ 18 years) across different BMI categories. Maximum plasma concentration (Cmax) and total plasma concentration (AUC) increased with increasing BMI, indicating that dose adjustment is required for patients with underweight (BMI < 18.5 kg/m²) and overweight (BMI ≥ 30 kg/m²); see section "Dosage and administration".

Table 4. Pharmacokinetic parameters of NovoEight® after administration of a single dose (50 IU/kg) according to BMI classa – One-stage clotting assay – Mean (standard deviation)

Pharmacokinetic parameter

Underweight, N = 5

Normal body weight,

N = 7

Overweight,

N = 8

Obesity, class I,

N = 7

Obesity, class II/III,

N = 7

Peak level increase (MO/dl)/(MO/kg)

1.7 (0.2)

2.0 (0.2)

2.4 (0.4)

2.3 (0.3)b

2.6 (0.3)

AUC ((MO*hr)/dl)

1510 (360)

1920 (610)

1730 (610)

2030 (840)

2350 (590)

CL (ml/hr/kg)

3.91 (0.94)

3.20 (1.00)

3.63 (1.24)

3.37 (1.79)

2.51 (0.63)

t½ (hr)

11.3 (2.0)

11.7 (3.5)

9.4 (2.9)

11.2 (3.5)

11.1 (2.7)

Vss (ml/kg)

56.8 (5.4)

44.8 (6.5)

39.6 (6.0)

42.0 (9.0)

35.0 (4.6)

Cmax (MO/dl)

100 (11)

121 (10)

144 (26)

140 (21)

161 (32)

Mean duration of effect (hr)

15.1 (3.0)

15.3 (4.8)

11.9 (3.7)

14.4 (4.6)

14.6 (3.7)

a BMI categories: underweight, BMI < 18.5 kg/m²; normal weight, BMI 18.5–24.9 kg/m²; overweight, BMI 25–29.9 kg/m²; obesity, class I, BMI 30–34.9 kg/m²; obesity, class II/III, BMI ≥ 35 kg/m².

b Data obtained from only 6 patients.

Table 5. Pharmacokinetic parameters of NovoEight® after administration of a single dose (50 IU/kg) according to BMI categorya – Chromogenic assay – Mean (standard deviation)

Pharmacokinetic parameter

Underweight, N = 5

Normal body weight,

N = 7

Overweight,

N = 9

Obesity, class I,

N = 7

Obesity, class II/III,

N = 7

Peak level increase (IU/dl)/(IU/kg)

2.2 (0.4)

2.9 (0.3)

3.0 (0.5)

3.2 (0.5)

3.5 (0.5)

AUC ((IU*hr)/dl)

1860 (700)

2730 (860)

2310 (1020)

2780 (1210)

3050 (730)

CL (ml/hr/kg)

3.28 (0.87)

2.25 (0.73)

2.84 (1.09)

2.58 (1.56)

1.94 (0.52)

t½ (hr)

11.7 (2.4)

11.5 (3.6)

9.7 (3.4)

10.4 (3.2)

10.5 (2.5)

Vss (ml/kg)

49.1 (10.4)

31.2 (4.5)

31.6 (5.8)

28.9 (5.1)

25.7 (4.0)

Cmax (IU/dl)

138 (29)

185 (24)

194 (31)

200 (33)

227 (32)

Mean duration of effect (hr)

15.5 (3.2)

15.2 (4.9)

12.6 (4.8)

13.5 (4.6)

13.9 (3.7)

a BMI categories: underweight, BMI < 18.5 kg/m²; normal weight, BMI 18.5–24.9 kg/m²; overweight, BMI 25–29.9 kg/m²; obesity, class I, BMI 30–34.9 kg/m²; obesity, class II/III, BMI ≥ 35 kg/m².

Preclinical safety data

Preclinical data indicate no special risk to humans based on the results of standard safety pharmacology and repeated-dose toxicity studies.

Clinical characteristics.

Indications.

Treatment and prevention of bleeding in patients with hemophilia A (congenital factor VIII deficiency).

NovoEight® can be administered to patients of all age groups.

Contraindications.

Hypersensitivity to the active substance or to any of the excipients.

Known allergic reaction to hamster proteins.

Interaction with other medicinal products and other forms of interaction.

No interactions between human coagulation factor VIII (rDNA) and other medicinal products have been reported.

Special precautions for use.

Traceability

To improve traceability of biological medicinal products, it is essential to clearly record the name and batch number of the product administered.

Hypersensitivity

Hypersensitivity reactions of an allergic type may occur during administration of NovoEight®. The product contains traces of hamster proteins, which may cause allergic reactions in some patients. Patients should be advised to discontinue the medicinal product immediately and contact their physician if symptoms of hypersensitivity occur. Patients should be informed about early signs of hypersensitivity reactions, including rash, generalized urticaria, tightness of the chest, wheezing, hypotension, and anaphylaxis.

In case of shock, standard anti-shock therapy should be initiated.

Inhibitors

Development of neutralizing antibodies (inhibitors) to factor VIII is a known complication in the treatment of patients with haemophilia A. These inhibitors are typically immunoglobulins of the IgG class that interfere with the procoagulant activity of factor VIII. Quantitatively, they are measured in Bethesda Units (BU) per 1 ml of plasma using a modified assay. The risk of inhibitor development correlates with factor VIII exposure and is highest during the first 50 exposure days, but persists throughout the patient's life, although this risk is uncommon.

The clinical significance of inhibitor formation depends on their titre, with low titres posing a lower risk of inadequate clinical response compared to high inhibitor titres.

Overall, careful monitoring of all patients receiving treatment with factor VIII coagulation factor products is required for inhibitor development through appropriate clinical observation and laboratory testing. If the expected plasma factor VIII activity level cannot be achieved, or if bleeding cannot be controlled with an appropriate dose, testing for the presence of factor VIII inhibitors should be performed. In patients with high inhibitor levels, therapy with factor VIII may be ineffective and alternative treatment approaches should be considered. Management of such patients should be performed under the supervision of physicians experienced in treating patients with haemophilia and factor VIII inhibitors.

Precautions related to excipients

The medicinal product contains 30.5 mg of sodium per reconstituted vial, equivalent to 1.5% of the maximum daily sodium intake for adults recommended by WHO, which is 2 g.

Cardiovascular complications

In patients with existing cardiovascular risk factors, replacement therapy with factor VIII may increase the risk of cardiovascular complications.

Catheter-related complications

If a central venous catheter is required, the risk of catheter-related complications, including local infections, bacteremia, and thrombosis at the catheter site, should be considered.

To maintain traceability between the patient and the medicinal product batch, it is strongly recommended to record the name and batch number of the product each time NovoEight® is administered to a patient.

Children

The aforementioned warnings and precautions apply to both adults and children.

Use during pregnancy or breastfeeding.

Studies on the effect of NovoEight® on reproductive function in animals have not been conducted. Since haemophilia A is rare in females, experience with the use of factor VIII during pregnancy and breastfeeding is limited. Therefore, factor VIII should be used in pregnant women and women during lactation only if clearly indicated.

Ability to affect reaction speed when driving or operating machinery.

NovoEight® does not affect the ability to drive or operate machinery.

Administration and dosage.

Treatment should be initiated under the supervision of a physician experienced in the treatment of hemophilia.

Monitoring of treatment

During the course of treatment, appropriate monitoring of factor VIII levels is recommended to adjust the dose and frequency of repeat injections. Patients may exhibit varying responses to factor VIII, as well as differences in half-life and recovery. The dose calculated based on body weight may require adjustment in patients with underweight or overweight. Pharmacokinetic studies of the drug after a single dose administered to adult patients have shown that maximum plasma concentration (Cmax) and total plasma concentration (AUC) increase with increasing BMI. This suggests that dose adjustment may be necessary. Patients who are underweight (BMI < 18.5 kg/m²) may require an increased dose, while obese patients (BMI ≥ 30 kg/m²) may require a reduced dose. However, currently there is insufficient data to provide specific recommendations for dose adjustment; see section "Pharmacokinetics".

In particular, careful monitoring of replacement therapy through coagulation analysis (factor VIII activity in plasma) is mandatory during major surgical procedures.

When performing a one-stage clotting assay based on activated partial thromboplastin time (aPTT) in vitro to determine factor VIII activity in patient blood, both the type of aPTT reagent used and the reference standard applied in the assay may significantly affect the results of factor VIII activity measurement in plasma. There may also be considerable discrepancies between results obtained using one-stage aPTT-based clotting assays and chromogenic assays according to the European Pharmacopoeia. This is particularly important when changing laboratories and/or reagents used for analysis.

Dosage

The dosage and duration of replacement therapy depend on the severity of factor VIII deficiency, the location and severity of bleeding, and the patient's clinical condition.

The quantity of factor VIII units is expressed in International Units (IU), corresponding to the current WHO standard for factor VIII preparations. Factor VIII activity in blood plasma is expressed as a percentage (relative to the normal level in human plasma) or in International Units (relative to the international standard for factor VIII in plasma).

One International Unit (IU) of factor VIII activity is equivalent to the amount of factor VIII present in 1 ml of normal human plasma.

On-demand treatment

The calculation of the required factor VIII dose is based on the empirical finding that 1 International Unit (IU) of factor VIII per 1 kg of body weight raises factor VIII activity in plasma by 2 IU/dL. The required dose can be calculated using the following formula:

Required number of units = body weight (kg) × desired increase in factor VIII level (%) (IU/dL) × 0.5 (IU/kg per 1 IU/dL).

The amount to be administered and the frequency of administration should always be individually determined based on clinical efficacy.

In the event of the hemorrhagic events listed below, factor VIII activity should not fall below the indicated plasma activity levels (in % of normal or IU/dL) for the appropriate period. Table 6 can be used as a guideline for dosing during bleeding episodes and surgical procedures.

Table 6. Dosing guideline for bleeding episodes and surgical procedures

Severity of bleeding/surgical procedure type

Required factor VIII level (%)

(IU/dL)

Infusion frequency (hours)/duration of therapy (days)

Bleeding

Early signs of hemarthrosis, muscle hematoma, or oral bleeding

20−40

Repeat every 12−24 hours for at least 1 day until bleeding stops, as determined by absence of pain, or until healing

More severe hemarthrosis, muscle hematoma, or hematoma

30−60

Repeat infusion every 12−24 hours for 3−4 days or longer until pain resolves and function is restored

Lifethreatening bleeding

60−100

Repeat infusion every 8−24 hours until the life-threatening condition has resolved

Surgical procedures

Minor surgical procedures,

including tooth extraction

30−60

Every 24 hours for at least 1 day until healing

Major surgical procedures

80−100

(pre- and postoperative)

Repeat infusion every 8−24 hours until adequate wound healing occurs, followed by continued therapy for at least 7 additional days to maintain factor VIII activity between 30 and 60% (IU/dL)

Prophylaxis

For long-term prevention of bleeding episodes in patients with severe hemophilia A, the usual recommended dose is 20–40 IU of factor VIII per kg body weight every other day or 20–50 IU of factor VIII per kg body weight three times a week. In adults and adolescents (aged over 12 years), a less frequent dosing regimen may be considered (40–60 IU/kg every third day or twice weekly). Sometimes, particularly in younger patients, shorter intervals between administrations or higher doses may be required.

Surgery

Experience with the use of the product in children undergoing surgical procedures is limited.

Elderly patients

There is no experience with the use of the product in patients aged >65 years.

Children

For long-term prevention of bleeding episodes in patients under 12 years of age, recommended doses are 25–50 IU of factor VIII per kg body weight every other day or 25–60 IU of factor VIII per kg body weight three times a week. Dosing recommendations for children aged 12 years and older are the same as for adult patients.

Administration method

Intravenous injection.

The recommended infusion rate for NovoEight® is 1–2 ml/min. The rate should be adjusted according to patient comfort.

Instructions for reconstitution of the product prior to administration are provided in the Instructions for Use of NovoEight®.

Storage after reconstitution

Chemical and physical stability of the reconstituted product has been demonstrated for 24 hours at 2–8 °C; for 4 hours at temperatures not exceeding 30 °C, provided the product has been stored for no more than 9 months at room temperature not exceeding 30 °C from the date of manufacture until reconstitution. Storage for 4 hours at temperatures not exceeding 40 °C is acceptable provided the product has been stored for no more than 3 months at room temperature of 30–40 °C from the date of manufacture until reconstitution. From a microbiological standpoint, the reconstituted product should be used immediately. If not used immediately, the user is responsible for storage conditions and duration. When reconstitution is performed under controlled and validated aseptic conditions, the storage period of the reconstituted product should not exceed the time limits stated above.

Any unused reconstituted medicinal product stored for more than 4 hours at room temperature up to 40 °C should be discarded.

Instructions for Use of NovoEight®

READ THESE INSTRUCTIONS CAREFULLY BEFORE USING NOVOEIGHT®.

NovoEight® is supplied as a powder. Prior to injection (administration), it must be reconstituted with the solvent provided in the prefilled syringe. The solvent is 0.9% sodium chloride solution (9 mg/ml). NovoEight® is administered intravenously (intravenous injection). The contents of the package (see below) are intended for reconstitution and administration of NovoEight®.

For administration, an infusion set (tubing and butterfly needle), sterile alcohol-impregnated swabs, gauze pads, and adhesive bandages are also required. These devices and materials are not included in the NovoEight® package.

Do not use the equipment without proper training from your doctor or nurse.

Always wash your hands before use and ensure your working area is clean.

During preparation and administration of the medicinal product directly into a vein, it is very important to follow aseptic and antiseptic procedures. Improper injection technique may lead to blood infection.

Do not open the equipment until you are ready to use it.

Do not use the equipment if it has been dropped or damaged. Instead, use a new package.

Do not use the equipment if the expiry date has passed. Instead, use a new package. The expiry date is printed after the words "Exp." on the carton, vial, vial adapter, and prefilled syringe.

Do not use the equipment if you suspect it is contaminated. Instead, use a new package.

Do not discard any part of the kit until after you have administered the prepared solution.

The equipment is intended for single use only.

Package contents:

1 vial of NovoEight® powder
1 vial adapter
1 prefilled syringe with solvent
1 syringe plunger (located under the syringe)

  1. Preparation of the vial and syringe

Fig. A

  • Take the number of NovoEight® packages you need.
  • Check the expiration date.
  • Check the name, dosage, and color of the package to ensure it contains the required medication.
  • Wash your hands and dry them with a clean towel or air dryer.
  • Remove the vial, vial adapter, and pre-filled syringe from the box. Leave the plunger rod in the box.
  • Warm the vial and pre-filled syringe to room temperature. You can do this by holding them in your hands until they feel as warm as your hands.
  • Do not use any other method to warm the vial and pre-filled syringe.

Fig. B

  • Remove the plastic cap from the vial. If the plastic cap is loose or missing, do not use the vial.
  • Wipe the rubber stopper with an alcohol swab and let it dry for several seconds before use to remove microorganisms as much as possible.
  • Do not touch the rubber stopper with your fingers, as this may transfer microorganisms.
  1. Attaching the vial adapter

Fig. C

  • Remove the protective paper membrane from the vial adapter.

If the seal of the protective paper membrane is broken or damaged, do not use the vial adapter.

Do not remove the vial adapter from the packaging with your fingers. If you touch the tip of the vial adapter, this may transfer microorganisms from your fingers.

Fig. D

  • Place the vial on a flat and firm surface.
  • Turn the protective cap over and attach the vial adapter to the vial.

After attachment, do not disconnect the vial adapter from the vial.

Fig. E

  • Gently squeeze the protective cap between your thumb and index finger, as shown in Fig. E.

Remove the protective cap from the vial adapter.

Do not disconnect the vial adapter from the vial when removing the protective cap.

  1. Attaching the plunger rod and syringe

Fig. F

  • Take the plunger rod by the wide end and remove it from the box. Do not touch the side surfaces or the front end of the plunger rod. If you touch the side surfaces or front end of the plunger rod, this may transfer microorganisms from your fingers.
  • Immediately attach the plunger rod to the plunger inside the pre-filled syringe by turning it clockwise until it stops.

Fig. G

  • Remove the syringe cap from the pre-filled syringe by bending it downward until it breaks off at the perforation.
  • Do not touch the cannula of the syringe under the cap. If you touch the syringe cannula, this may transfer microorganisms from your fingers.

If the syringe cap is loose or missing, do not use this pre-filled syringe.

Fig. H

  • Carefully screw the pre-filled syringe onto the vial adapter until it stops.
  1. Reconstituting the powder with solvent

Fig. I

  • Hold the pre-filled syringe, slightly tilting the vial downward.
  • Push the plunger to inject all the solvent into the vial.

Fig. J

  • Keep the plunger rod pressed and gently rotate the vial until the powder is completely dissolved.

Do not shake the vial, as this will cause foaming.

  • Check the final solution. It should be clear or slightly opalescent. Do not use the solution if visible particles are present or if there is a change in color. Use a new package instead.

J

It is recommended to use the reconstituted NovoEight® solution immediately after preparation to prevent contamination of the solution.

If you cannot use the reconstituted NovoEight® solution immediately, it may be used within 4 hours when stored at room temperature up to 40°C or within 24 hours when stored at 2–8°C. Store the reconstituted solution in the vial with the attached vial adapter.

Do not freeze the reconstituted NovoEight® solution and do not store the solution in the syringe.

Do not store the reconstituted solution without consulting your doctor.

Store the reconstituted NovoEight® solution in a place protected from light.

If your required dose is greater than that contained in one vial, repeat steps from A to J with additional vials, vial adapters, and pre-filled syringes until you obtain your required dose.

Fig. K

  • Keep the plunger rod fully pressed.
  • Turn the syringe with the vial upside down so that the vial is on top.
  • Release the plunger rod to allow the solution to fill the syringe.
  • Gently pull the plunger rod downward to draw the solution into the syringe.
  • If only part of the reconstituted solution is needed, use the scale on the syringe to see how much solution you have drawn, as recommended by your doctor or nurse.

If air has accidentally been drawn into the syringe, expel it back into the vial.

  • Holding the vial upside down, gently tap the syringe to collect air bubbles at the top.
  • Slowly press the plunger to expel all air bubbles.

Fig. L

  • Unscrew the adapter with the vial.
  • Do not touch the syringe cannula. If you touch the syringe cannula, this may transfer microorganisms from your fingers.
  1. Injecting the reconstituted solution

NovoEight® is now ready for intravenous administration.

  • Administer the reconstituted solution as recommended by your doctor or nurse.
  • Administer the medication slowly over 2–5 minutes.
  • Do not mix NovoEight® with other solutions or intravenous medications.

Injection of NovoEight® through needle-free intravenous (IV) catheter adapters

Caution! The pre-filled syringe is made of glass and is intended for use with a standard Luer-Lock connection. Some needle-free adapters with internal spikes are incompatible with this pre-filled syringe. Such incompatibility may prevent drug administration and/or damage the needle-free adapter.

Injection of the solution through a central venous access device (CVAD), such as a central venous catheter (CVC) or subcutaneous port:

  • Use aseptic and antiseptic techniques. Follow instructions for proper use of your adapter and CVAD, consulting your doctor or nurse.
  • When injecting through a CVAD, a sterile 10 ml plastic syringe may be required to withdraw the reconstituted solution. This should be done immediately after step J.
  • If flushing of the CVAD is required before or after injection of NovoEight®, use 9 mg/ml sodium chloride injection solution.

Disposal

Fig. M

  • After injection, unused NovoEight® solution, syringe with infusion set, vial with vial adapter, and other used materials should be disposed of according to the pharmacist's instructions.

Do not dispose of these materials with household waste.

M

Do not disassemble equipment prior to disposal.

Do not reuse equipment.

Children.

The medicinal product can be used in children according to the instructions provided in the section "Method of administration and dosage".

Overdose.

There have been no reports of symptoms of overdose with recombinant coagulation factor VIII.

Adverse reactions

Summary of safety profile

Hypersensitivity or allergic reactions (which may include angioedema, burning and pruritus at the infusion site, chills, flushing, generalized urticaria, headache, rash, hypotension, lethargy, nausea, restlessness, tachycardia, chest tightness, paresthesia, vomiting, wheezing) have been observed rarely, and in some cases may progress to severe anaphylaxis (including shock).

Formation of antibodies to hamster proteins and associated hypersensitivity reactions have been observed very rarely.

In patients with hemophilia A, neutralizing antibodies (inhibitors) to factor VIII may develop. If such inhibitors form, clinical response will be inadequate. In such cases, it is recommended to consult a specialized hemophilia treatment center.

List of adverse reactions

In Table 7, adverse events are listed by System Organ Class (SOC) with preferred terms from the Medical Dictionary for Regulatory Activities (MedDRA).

Frequency is defined as follows: very common (≥ 1/10), common (≥ 1/100 to < 1/10), uncommon (≥ 1/1,000 to < 1/100), rare (≥ 1/10,000 to < 1/1,000), very rare (< 1/10,000); frequency not known (cannot be estimated based on available data).

Within each frequency group, adverse reactions are listed in descending order of severity.

Table 7. Frequency of adverse reactions to the medicinal product in clinical studies

Organ system classes

Frequency in previously treated patients (PTPs)

Frequency in previously untreated patients (PUPs)

Adverse reaction

Blood and lymphatic system disorders

Uncommon

Very common

Inhibition of factor VIII

Psychiatric disorders

Uncommon

Insomnia

Nervous system disorders

Uncommon

Headache, dizziness, burning sensation

Cardiac disorders

Uncommon

Sinus tachycardia, acute myocardial infarction

Vascular system disorders

Uncommon

Arterial hypertension, lymphedema, hyperemia

Common

Flushing, superficial venous thrombophlebitis

Skin and subcutaneous tissue disorders

Common

Rash, erythematous rash

Uncommon

Rash, lichenoid keratosis, skin burning sensation

Musculoskeletal and connective tissue disorders

Uncommon

Muscle and joint rigidity, arthropathy, limb pain, muscle and bone pain

Common

Hemarthrosis, muscle hemorrhage

Respiratory, thoracic and mediastinal disorders

Common

Cough

General disorders and administration site conditions

Common

Injection site reactions

Common

Hyperthermia, erythema at catheterization site

Uncommon

Fatigue, hot flush, peripheral edema, hyperthermia

Investigations

Common

Elevated liver enzymesg

Common

Positive test for factor VIII antibodies

Uncommon

Increased heart rate

Gastrointestinal disorders

Common

Vomiting

Injury, poisoning and procedural complications

Common

Incorrect dose administered

Common

Infusion-related reaction

Uncommon

Contusion

Device malfunction

Common

Device occlusion

a Based on the total number of individual patients in all clinical studies (301), of which 242 were previously treated patients (PTPs) and 60 were previously untreated patients (PUPs).

b Frequency rates are based on data from studies involving all factor VIII products in patients with severe haemophilia A.

c Injection site reactions include injection site erythema, injection site extravasation, and injection site pruritus.

d Elevated liver enzymes include alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, and bilirubin.

Description of selected adverse reactions

In clinical studies of NovoEight® involving previously treated patients, a total of 35 adverse reactions were reported in 23 out of 242 patients. The most common adverse reactions were injection site reactions, complications related to incorrect dosing, and increased liver enzyme levels. Two of the 35 adverse reactions occurred in one out of 31 patients under 6 years of age, none were observed in patients aged 6 to 12 years, one event occurred in one out of 24 patients aged 12 to 18 years, and 32 events occurred in 21 out of 155 adult patients (aged 18 years and older).

Children

In clinical studies involving 63 children aged 0 to 12 years and 24 adolescents aged 12 to 18 years with severe haemophilia A, no differences in the safety profile of NovoEight® were observed between paediatric and adult patients.

In a study involving previously untreated patients aged 0 to 6 years receiving NovoEight®, a total of 46 adverse reactions were reported in 33 out of 60 patients. The most common adverse reaction was factor VIII inhibition (see section "Special precautions"). A high risk of genetic mutations was identified in 92.3% of all patients and in 93.8% of patients with confirmed high inhibitor titres. No other factors were significantly associated with inhibitor development.

Reporting of adverse reactions and lack of drug efficacy

Reporting of adverse reactions after drug registration is important. It allows continuous monitoring of the benefit-risk balance of the medicinal product. Healthcare professionals, pharmacists, patients, and their legal representatives should report all suspected adverse reactions and lack of drug efficacy through the Automated Pharmacovigilance Information System at https://aisf.dec.gov.ua/.

Shelf life.

The shelf life of the finished medicinal product is 30 months.

Do not use the medicinal product after the expiry date stated on the carton, vial, and syringe label. The expiry date refers to the last day of the specified month.

Storage conditions.

Store the medicinal product at 2–8 °C. Do not freeze. Keep out of the reach of children. During the shelf life, the product may be stored at room temperature (not exceeding 30 °C) for up to 9 months from the date of manufacture until reconstitution begins; the product may also be stored at room temperature (30–40 °C) for up to 3 months from the date of manufacture until reconstitution begins. The product must not be returned to the refrigerator. The date and temperature of removal from refrigeration must be recorded on the carton.

Store in the original outer packaging to protect from light.

Incompatibilities.

Since compatibility studies have not been conducted, this medicinal product must not be mixed with other medicinal products.

Packaging.

One 5 mL glass vial (Type I) containing powder, stoppered with a chlorobutyl rubber closure and sealed with an aluminium cap with a removable plastic cap, supplied with a solvent (0.9% sodium chloride solution) in a 4 mL pre-filled syringe (5 mL capacity) with a polypropylene plunger stop, bromobutyl rubber plunger, bromobutyl rubber needle cap, polypropylene plunger rod, together with a sterile vial adapter in individual packaging, all contained in a cardboard box.

Prescription status.

Prescription only.

Marketing Authorisation Holder/Manufacturer.

Novo Nordisk A/S.

Manufacturer's address and location of operations.

Novo Allé, Bagsværd, 2880, Denmark.