Platelet Function Disorders are rare blood conditions that affect only 2000 – 5000 in the UK.
The term ‘Platelet Function Disorder’ (PFD) could be applied to any condition where platelets do not work properly.
Platelets are small particles in the blood that stick together at sites on injury (for example after a cut) to form a “plug” and stop the bleeding. If your platelets don’t work properly you bleed more than normal and this can vary from minor bruising to life threatening bleeding after surgery or an accident.
PFD stands for platelet function disorder(s), meaning that platelets do not work properly. Some patients with PFD have a normal platelet count, and some have a reduced platelet count (the medical term for this is “thrombocytopenia”). Most platelet disorders are acquired, meaning they appear spontaneously or as a result of an external factor; such as a change in the immune system, in kidney function, or as a result of medications. These conditions may disappear or “remit”. However, a small number of patients are born with inherited PFD, meaning that they will always have the condition.
This varies a lot according to the type of PFD. Some patients only get very minor bruising which can be painful and not look very nice, but is not life threatening. Some patients can have really severe bleeding such as nosebleeds that won’t stop and lead to anaemia (a low red blood cell count), bleeding from the bowel, or very heavy periods in young girls and women. The most serious type of bleeding is bleeding inside the head or brain, sometimes referred to as intracranial haemorrhage. Having surgery (or having children in women) is a time of particular worry and many patients need treatment prior to these events.
Patients with PFDs will be registered with the doctors and nurses at a haemophilia centre and will see them intermittently or if they need treatment. The haemophilia centre can often be a long way from their home, and attending can make patients miss time from work or school. Some patients carry a treatment pack with them at all times so they can give treatment quickly if they do develop a bleed.
Most patients with PFD in the UK carry a \’green card\’ to alert medical staff to the fact that they have a bleeding disorder. Simple things like going on holiday can be problematic, as patients with PFDs will need to find out where they should go if they develop a problem whilst they are away, and they may need to carry treatment with them. Some patients with PFDs are advised not to participate in sports with a lot of physical contact or risk of head injury, such as rugby or boxing. The label of having a PFD and the symptoms associated with it can cause embarrassment and stigma to some patients who just want to be \’normal\’.