What to do in an Emergency

Summary

Contact National PNH Service in Leeds:

  • PNH Service number (weekday working hours): 0113 206 8625

  • PNH Service on-call number (evenings, weekends & bank holidays): 07920 535918

Provide the following information upon arrival at A&E:

  1. Tell them you have PNH

  2. Tell them if you are on a Complement Inhibitor Therapy

  3. Describe Current Symptoms

  4. List All Medications

  5. Highlight Clot Risk

  6. Provide Specialist Contact Details

  7. Key Instructions for A&E Staff

Read on for more specific information on each of the points outlined above.

Who to Contact

In an emergency, patients with PNH should contact the National PNH Service in Leeds.

If you experience any of the following symptoms, call the National PNH Service on-call in Leeds immediately. Specialist haematologists will advise A&E staff on urgent treatment.

  • Severe anaemia (extreme fatigue, dizziness, shortness of breath, dark/red urine)

  • Blood clot symptoms (leg swelling/pain, chest pain, shortness of breath)

  • Signs of infection (fever, chills, confusion)

PNH Service number (weekday working hours): 0113 206 8625

PNH Service on-call number (evenings, weekends & bank holidays): 07920 535918

What Information to Give on Arrival at A&E

PNH is an extremely rare blood disorder so most A&E clinicians will never have managed a patient with PNH before. It’s important to tell A&E staff about your PNH diagnosis before any treatment starts as PNH can affect how your body responds to illness, infection, and some standard emergency treatments. Early awareness helps staff make safer, faster decisions. If you have a PNH safety card or keyring show it to staff so they can access information on who they should contact for advice.

Tell them you have PNH

Paroxysmal Nocturnal Haemoglobinuria (PNH) is a rare blood disorder that destroys red blood cells and increases the risk of blood clots.

Tell them if you are on a Complement Inhibitor Therapy

Where possible give the following details:

  • Drug name: e.g., Iptacopan (Fabhalta®), Eculizumab (Soliris), Ravulizumab (Ultomiris®), Danicopan (Voydeya®)
  • Last dose taken: when you last took the medicine
  • Administration method: oral tablet or intravenous infusion
  • Frequency: daily, twice daily, or every 8 weeks
  • Purpose: prevents destruction of red blood cells (reduces haemolysis and clot risk)

Important: Complement inhibitors significantly reduce the body’s ability to fight Neisseria meningitis & can mask signs of infection. A&E staff should monitor you closely for sepsis even if fever is mild.

Describe Current Symptoms

For example:

  • Fatigue, shortness of breath, dizziness
  • Dark/red urine (sign of haemolysis)
  • Abdominal pain or unusual headaches
  • Bruising, bleeding, or signs of clot (swelling, pain, or sudden shortness of breath)

List All Medications

For example:

  • Blood thinners (e.g., warfarin)
  • Supplements (iron, folic acid)
  • Any recent transfusions

Highlight Clot Risk

PNH increases the risk of blood clots. Let staff know if you are on anticoagulation therapy or have a previous history of thrombosis.

Provide Specialist Contact Details

Staff may need guidance on urgent transfusions, specific complement inhibitors or clot management.

  • PNH National Service (for urgent advice): St James’s University Hospital, Leeds (working hours 0113 2068625 / out-of-hours 07920 535918)
  • Supplements (iron, folic acid)Local haematologist: name of local haematology consultant and local hospital you attend
  • Scottish PNH Specialist: Dr Lindsay Mitchell, University Hospital Monklands

Key Instructions for A&E Staff

For example:

  • Monitor for infections carefully – complement inhibitors significantly reduce the body’s ability to fight Neisseria meningitis
  • Be aware that classic signs of infection (fever, elevated WBC) may be less obvious.
  • Urgent transfusion may be required if haemoglobin is dangerously low.
  • Contact the National PNH Service (working hours 0113 2068625 / out-of-hours 07920 535918)

Related Topics

What is PNH?

A helpful guide on Paroxysmal Nocturnal Hemoglobinuria (PNH) including common PNH terminology and symptoms.

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PNH Care in Scotland

PNH care in Scotland is based on a shared-care model. Find out more abut PNH care in Scotland on this page.

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PNH Treatment

There are a range of PNH treatment options available in Scotland from supportive care to advanced complement inhibitors.

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