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There are a number of different treatments that PNH patients may require. Some people, especially those with a low PNH clone may require little or no treatment. The treatments are generally supportive (blood transfusion, iron and folic acid, and anticoagulants) and specific (eculizumab, allogeneic bone marrow transplant) for PNH. Some of the more common treatments are listed below:


Allogeneic Bone Marrow Transplant

It is not common for this treatment to be recommended for PNH patients unless there is a severe bone marrow failure co-existing with PNH. It has many significant complications which may occur in some patients.


Anticoagulation

Some patients take medication to thin their blood (such as warfarin or heparin) to reduce the risk of developing blood clots.


Blood Transfusions

Blood transfusions can alleviate some of the symptoms experienced with PNH, including anaemia. A blood transfusion can usually be carried out in an outpatient clinic. Once your blood type is cross matched for antibodies, one unit of blood takes approximately two hours to transfuse into your body. This will usually alleviate the symptoms resulting from anaemia and haemolysis for a period of time (which differs for each person).


Folic Acid

Folic acid is a vitamin which the bone marrow needs to help it produce blood cells. This can be taken in tablet form.


Iron Supplements and Iron Removal

Iron levels can be both too high or too low in PNH and can depend on what other treatment someone is having. Some patients may need to take iron supplements such as ferrous sulphate and others may need to take medication to reduce the iron in their bodies.


Eculizumab (or Soliris)

This relatively new medication is otherwise known as “Soliris” and is administered by way of infusion once every two weeks on an ongoing basis. It was approved in England for treating PNH patients in 2007 and is funded by the NHS. Eculizumab is an antibody which blocks the activation of the complement part of the immune system. It therefore protects the red blood cells affected by PNH from destruction or stimulation by the complement part of the immune system.

Eculizumab has been proven to reduce the symptoms of PNH patients and improve their quality of life and their life expectancy. It also reduces the risks of many of the complications of PNH such as thrombosis, renal failure or pulmonary hypotension.

Terminal complement (which eculizumab prevents from forming) is required to prevent Neisseria Meningitidis which is a bacteria which can cause meningitis and other forms of meningococcal disease. Therefore patients being treated with eculizumab are predisposed to developing infections caused by the Neisseria group of bacteria. To reduce this risk, all PNH patients starting treatment with eculizumab are vaccinated with a vaccine against the meningococcal strains A, C, W and Y. The vaccine against serotype B (the most common serotype in the UK) is licensed and is used in patients treated with eculizumab. Patients are also advised to take 500mg of either penicillin or erythromycin twice daily as a prophylactic antibiotic.

Patients being treated with eculizumab are also provided with a patient safety card to carry with them at all times which states the symptoms of meningitis and tells them what to do if they experience any of these symptoms.

Eculizimab is administered at patients’ homes by nurses from an organisation called Healthcare at Home. Contact is made with the patient the day before the infusion is due and they are informed what time the Heathcare at Home nurses be attending the next day. Patients can nominate a day of the week and a general time that they wish to receive their infusion.

The nurse attends a patient’s home with the medication (or it is delivered separately and kept in the fridge) and administers an intravenous line into a vein and flushes the line with saline. The prescribed amount of eculizumab is then mixed with saline according to the prescription and this is then usually administered over a 30 minute period. After the eculizumab has been infused, the line is then flushed with saline again. Blood pressure and temperature are taken before and after the infusion in case there is any change which may indicate an adverse reaction.



Prescriptions

PNH patients may be prescribed folic acid, iron and prophylactic antibiotics as part of their treatment. Unfortunately PNH is not one of the medical conditions which allows patients to obtain exemptions from prescription charges. 


Specialist Treatment Centres

In England and Wales there are two NHS PNH specialist treatment centres called National Service Centres which are Kings College Hospital NHS Foundation Trust in London and Leeds Teaching Hospitals NHS Trust.

Here is a link to the National Service Centre website http://www.pnhleeds.co.uk.

In order to be able to treat patients in outpatient clinics closer to their homes, Leeds Teaching Hospitals NHS Trust has an agreement with eight other NHS trusts. The consultants and nurses from the PNH National Service Centre in Leeds travel to Outreach Clinics on a 12 weekly basis to see patients. The details of these Outreach Clinics can be found on the attached link:

http://www.pnhleeds.co.uk/patients/outreach-clinics/

The clinics are held in:

Birmimgham
Bristol
Lanarkshire
Liverpool
Manchester
Oxford
Peterborough
Southampton



What to expect from a PNH specialist centre:

Both the NHS PNH specialist treatment centres in Leeds and London can refer patients for counselling or other support services. The following specific services are available at Kings College Hospital:

PALS at Kings College Hospital

The Patient Advice and Liaison Service (PALS) is a service that offers support, information and assistance to patients, relatives and visitors. They can also provide help and advice if you have a concern or complaint that your clinicians have not been able to resolve for you. The PALS office is located on the ground floor of the Hambleden Wing near the main entrance on Bessemer Road.

Telephone: 0203 299 3601
Email: kch-tr.PALS@nhs.net


Counselling Service at Kings College Hospital

The counselling service at Kings College Hospital offers free and confidential sessions to all Kings inpatients, outpatients, their relatives and friends. If you would like to meet with one of the counsellors, you can contact them on 0203 299 1567 or 1567 from within the hospital.

 

 


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