What is Haemophilia? Haemophilia is a rare life-long genetic blood clotting disorder that reduces the body ability to control clotting . It is a treatable condition without a cure. The most common of the rare are : Haemophilia A (problems that affect clotting Factor VIII ) Haemophilia B (problems that affect clotting Factor IX ) Both are classified as mild , moderate or severe , depending upon the amount of factor in the body. Please note they are different disorders, but both conditions can cause prolonged bleeding into spaces and areas not always visible, even from mild injuries or in fact no injury. Muscles and joints are commonly affected , which can lead to rapid damage and long- term disablilty . Some people may have several joint affected by changes in responces to bleeding , particularly where this has occured in childhood . These forms involve bleeding into the muscles and joints, which can lead to rapid damage and long-term disability. It is thought that around 85% of all bleeding episodes occur within the muscles and joints of the arms and legs. Most of these episodes occur within the hinge joints at the knees, ankles and elbows. Joints affected by repeated bleeding episodes are called 'target' joints. This condition may have more than one family / whanau member affected. Beware in approx 1/3 of cases it will appear without a family history . From that point onwards it will travel down affected person bloodline. In very rare events can be acqured , normally later in life , that requires a different treatment due to a different cause involving the immune system. Other Bleeding Disorders It is important to remember that other types of bleeding disorder, such as Von Willebrand’s Disease , are treated by the Auckland Haemophilia Centre. Rarer bleeding disorders such as Factor I, II, V, VII, X, XI or XIII deficiencies, platelet and fibrin disorders are also managed by the Centre. Vitamin K deficiencies and other anaemias are not . These disorders may affect women and men. They are not usually associated with muscle and joint bleeding except in very severe cases. More Information about Joint Bleeds Joint bleeds are called haemarthroses. These occur as a result of trapping and tearing of the membrane that lines the joint. This membrane is called the synovium. The synovium is a structure which normally creates the nourishing, lubricating fluid inside our joints. When this membrane is damaged, blood fills the joint. A diagram of this can be found by clicking this link. If the bleeding into the joint is not treated quickly, iron from the blood starts to affect both the cartilage and the synovium itself. Cartilage is a very strong structure that acts like a shock absorber on the surfaces of our joints. Iron from blood reduces the stability of cartilage, which can become drier, rougher and more fragmented. Iron deposits in the synovial membrane may also trigger the release of enzymes into the joint fluid which can result in damage to the cartilage. The hemophilic joint.pdf.pdf.pdf.pdf (PDF, 175.4 KB) The hemophilic joint. (a) Short-term recovery from a single hemarthrosis, and (b) Long-term damage caused by recurrent bleeding. Reprinted from TalkingJoints® [22] with permission from Novo Nordisk.

How do I recognise a bleed? You may feel pain, heat, tingling or bubbling sensations together with swelling and stiffness. If you are a parent and your child is very young, you may notice difficulty using or straightening a joint. The joint may appear swollen and feel hotter than the same joint on the other side of the body. You may notice your child limping or avoiding putting the heel on the floor when walking or standing. Your doctor or specialist nurse will have told you more about how to recognise a bleed. I think I have had a bleed - What do I do? Steps to follow at home if you have a bleed: Administer factor replacement as soon as possible following a bleed Start to follow the PRICE regimen. You can download a copy of this regimen by clicking this link Contact the Haemophilia Centre to report the bleeding episode and arrange an appointment with your Nurse or Physiotherapist. Who do I call? Call the Auckland Haemophilia Centre on (09) 307 4949 x25285 (8.00am - 4.30pm Monday to Friday). Outside of normal working hours, weekends and public holidays: Children: Please go to the Children's Emergency Department, Level 2, Starship Children's Hospital at the Auckland City Hospital Site (Grafton). Adults: Please go to the Emergency Department at Level 2 of Auckland City Hospital. Please remember to tell hospital staff either that you have been diagnosed with Haemophilia or have a bleeding disorder. Please remember to bring your factor replacement product with you. What should I do when the bleeding has stopped? Bleeding usually stops within a few hours once factor has been administered. Once it has stopped, it is important that you start using your affected joint or muscle again as pain allows. This is to minimise stiffening of the joint and loss of muscle strength. If the bleeding is in your leg, it is a good idea to keep the weight off it for around 48 hours by using crutches. It is important to remember that rest is NOT the same as immobility. You need to move the joint little and often, increasing as pain allows. You may need to use ice, compression, crutches or other walking aids for several days after a bleeding episode, and it is always better to use crutches than to ‘hobble’ around on your leg, as this can cause further bleeding. Now is the time to start thinking about booking an appointment with your physiotherapist. Your nurse or doctor can refer you for rehabilitation. You may need to organise time off from work or school so that you can attend your appointment. How long will I have to wait? For new bleeding episodes, after you have contacted your specialist nurse, who will assist in providing acute treatment . The physiotherapist will aim to contact you within 24 hours of referral. You will be offered a physiotherapy appointment within 5 working days of contact. Why is treatment important? It is very important that the bleed is treated quickly. This minimises the damage that can occur at a muscle or joint following a bleeding episode. Rehabilitation is important soon after the bleed has been treated with factor and PRICE, as delayed treatment can increase the risk of further joint damage and may lead to changes in the whole limb which can increase the risk of further bleeding. Physiotherapy can also be very useful to those patients with existing long-term joint damage. Many older patients affected by haemophilia have significant joint damage, where pain is more often due to the arthritic changes that have occurred rather than ongoing bleeding episodes. It is important that patients with existing joint damage see a physiotherapist regularly to ensure that they are receiving the right forms of treatment, as pain related to joint bleeds and pain related to arthritis are managed in different ways and complete jouint scores . What to do if you Have a Bleed.pdf.pdf.pdf.pdf.pdf.pdf.pdf.pdf.pdf.pdf.pdf.pdf (PDF, 115.6 KB)

Protection Reduce weight bearing or stress on the affected joint or muscle by using crutches or other supports such as a 'collar and cuff' for the arm. You may need to avoid putting weight on the affected side completely for the first 48 hours; and possibly longer if it is a severe bleed. Ice Ice helps to reduce swelling, prevent further bleeding and eases pain. Use a gel cold pack or a ‘CryoCuff’ if you have one. If not, make an ice pack from a bag of frozen peas or crushed ice wrapped in a cloth. Cold wraps or packs should be applied to the affected area ‘little and often’ for around 10-15 minutes every 2 hours or so.You may need to continue using ice for several days after the bleed if your joint is still warm and swollen. Do not apply for more than 20 minutes at a time and do not place ice directly on the skin as it can burn. Compression The more swollen a joint or muscle becomes, the more pain you will feel. Compression reduces swelling, and therefore pain. If the muscle or joint is very swollen, you may need to wait a day or so before using a compression bandage and then apply as pain allows. Your nurse or physiotherapist can provide you with an elasticated bandage and will make sure it fits correctly, as additional damage can be caused where the bandage is too tight. Try not to allow wrinkles in the bandage and make sure to remove it at night before you go to sleep. Elevation This helps to reduce swelling and relieve pain by increasing the blood flow away from the injured area. The injured area should be raised above the level of your heart. When you elevate your leg, remove the compression stocking to allow normal, healthy circulation. Elevate ‘little and often’ for around 20 minutes at a time.

HAEMOPHILIA A (FACTOR VIII) REPLACEMENT PRODUCTS For people with Haemophilia A there are three different factor replacement products available: CSL (Biostate) FVIII: a plasma derived product ordered through Blood Bank. Advate (Takeda): a recombinant short acting factor VIII replacement product. Adynovate (Takeda): a recombinant, long acting factor VIII replacement product. Hemlibra (Roche) : monoclonal antibody , long acting . Pharmacy can fill scripts. Advate and Adynovate are kept in the Haemophilia Centre fridge, Building 8, Level 5, Auckland City Hospital and there's also a afterhour supply in Bloodbank. For a supply of Advate or Adynovate, Monday-Friday 0730-1600 hours please contact the Auckland Haemophilia Centre ext: 25285/0211954262 . Afterhours please contact bloodbank. HAEMOPHILIA B (FACTOR IX) REPLACEMENT PRODUCTS For people with Haemophilia B there are two different factor replacement products available: Rixubis (Takeda): a recombinant, short-acting factor IX replacement product. Alprolix (CSL): a recombinant, long-acting factor IX replacement product Alprolix and Rixubis are kept in the Haemophilia Centre fridge, Building 8, Auckland City Hospital or afterhour Bloodbank . It is important to check which factor product each person uses, for several reasons: If someone has developed an Inhibitor and has received therapy for this, i.e. has been 'Immune Tolerised' on a certain product, we don’t know if changing the product will have an effect on the Inhibitor. People may respond differently to different products. For example, a patient may get different Factor rise in their blood to a recombinant product and a plasma derived product. INHIBITORS When a person develops an inhibitor, the usual replacement products are ineffective, and to stop bleeding episodes, FVIIa (NovoSeven) or FEIBA is used. Novoseven is kept on the Haemophilia Centre clinic bench and FEIBA is stored in the refrigerator or afterhours from the bloodbank. If you would like more information on the types of clotting factors used by the Auckland Haemophilia Centre, please call the Centre for advice on (09) 307 4949 x25285/0211954262.

If you are registered with the Haemophilia Centre, you can contact nursing staff directly on (09) 307 4949, extension 25285/0211954262. If you have recently moved to Auckland, contact the specialist nurse at the Haemophilia Centre for advice on how to register with the Centre and how to access general haemophilia services.

Your specialist nurse or doctor will refer you for physiotherapy. It is important to remember that all bleeds should be reported, so that each bleeding episode can be treated promptly. As soon as you are able, let your specialist nurse or doctor know that you have had a bleeding episode so that a referral can be made and actioned quickly. In order to ensure that your muscle or joint makes a speedy recovery, make sure to attend your appointments and do the exercises you have been prescribed. You may need to organise time off work or school in advance of appointments so that you are able to attend. Please do bring relatives or whanau to physiotherapy appointments. Attending with a partner or family member can help to provide support during assessment or rehabilitation appointments.

Physiotherapy services for children are provided in a dedicated children's therapy area on Level 3 of Starship Hospital on the Grafton site, Park Road, Auckland. Adult physiotherapy appointments are provided in Outpatient Physiotherapy on Level 1 of Building 7 at the Greenlane Clinical Centre, Greenlane Road, Auckland. Your physiotherapist will aim to offer you an appointment around two days after you have reported a bleed to your specialist nurse. You will be asked some questions related to your haemophilia and how this condition affects your life. Some people feel that this can be a little easier with the support of whanau. Please bring family members to the appointment with you if you wish. Your physiotherapist will also examine your muscles and joints. If it is your first assessment or you have not been seen for around one year, you will have several different types of measurements taken, including the movement of your joints and the strength of your muscles. You may be asked to remove your shoes and socks so that the therapist can examine your pattern of walking. Please wear comfortable shorts to your appointment if you are able to, as most assessments will include an examination of your hips, knees and ankles. Based on your assessment, your physiotherapist will then give you advice and exercises to carry out. A series of physiotherapy appointments may be required. The therapist may also use other treatments such as ultrasound (using high-frequency sound waves). You may be referred for further specialist assessment, for example, to an orthotist who can assess your feet for special footwear. It is important that you attend each of your appointments and do all the exercises as often as you are advised to for maximum benefit. Please do let your physiotherapist know if you are having problems.

It is important to record when you use Factor, either for prophylaxis or when treating a bleeding episode. This should be done every time you administer Factor. Failure to complete a monthly reporting form may put continuing home supply at risk. People with Haemophilia managed by the Auckland DHB Haemophilia Centre are required to return a Factor Usage Reporting Form every month. This can be done by completing a form by hand and sending it to the Centre.