A FAMILY are calling for the a ban on producing immune therapy from UK patient’s blood to be overturned - so they can treat their eight-year-old daughter's rare disease.

Immunoglobulin production has been banned in the UK since the 1990s due to the fear of transmitting the human form of ‘Mad Cow disease’.

The treatment is still used in the UK; however, it is imported from elsewhere in the world.

Megan Steadman's family from Aberbargoed are concerned that if this ban is not overturned, there could be serious consequences for her and countless others in a similar situation.

Megan was born with Hyper IgD syndrome (HIDs), a form of Mevalonate Kinase Deficiency, a rare auto-immune disorder that occurs with gene mutations for mevalonate kinase, an enzyme that is important to the production of cholesterol and isoprenoids. Symptoms include periodic fevers, chills, malaise, abdominal pain and diarrhoea and cycles are usually between 20-30 days.

Megan’s mum Victoria Stoneman said: “She was born with the condition and suffered with system wide inflammation, joint pain, sore stomach, frequent ear infections and cyclical high temperatures of over 40 degrees and neutropenia. Cycles ranged from monthly to weekly at its worst.”

At the age of two, Megan was diagnosed with HIDs and has since been “under the ‘amazing’ team at Noah’s Ark/UHW”, added Ms Stoneman.

South Wales Argus:

(Eight-year-old Mehan Steadman from Aberbargoed)

Around the age of four, Megan began immunoglobulin treatment with the hope of reducing her symptoms and the length of time between episodes.

The condition left Megan with neutropenia, a low count of white blood cells, leaving her susceptible to infections and lacking energy.

This improved with the transfusions and Megan’s Immunology nurse trained Ms Stoneman to give the infusions at home to lessen the impact of travelling on her daughter.

However, this didn’t fully manage her condition, so aged just six, Megan underwent a stem cell transplant and needed immunoglobulins more to rebuild and strengthen her immune system following a course of chemotherapy.

Ms Stoneman added: “We’re hoping that eventually she may not need immunoglobulin infusions but there’s always a chance she may need this support for the rest of her life.

“It’s really the protection immunoglobulin treatments give that make it so important for immune deficient patients.”

“Megan has yet to be affected by the plasma shortage, however patients are having to have a change of product which may either not work as effectively or cause reactions.

“The personal concern for me is that should Megan need this for the foreseeable future, then she will be affected and whist currently her infusions work, any change may not be as effective, leaving her susceptible to infection.

“My wider concerns are that the plasma availability will not keep up with the patient demands. Immunology research seems to be racing ahead with more effective methods of diagnosis, a better understanding of how diseases work and fine-tuning of treatments this means that the patient base requiring immunoglobulin treatment will only increase.”

A Welsh Government spokesperson said: “Discussions are ongoing, on a cross Wales basis, to determine the best way to respond to the worldwide shortage of immunoglobin and ensure the most appropriate supplies are available for patients in Wales.

“Clinicians and staff involved in obtaining these products for the NHS, supported by the Chief Medical Officer, are actively engaged with companies that supply the products to secure the best possible supplies for Wales and keep disruption to patients to a minimum.”