Newcastle Parents Launch High Court Legal Action in Fight for Daughters to have Life-Saving Treatment

The Rich family Credit: Family photo

The parents of two Newcastle sisters battling a rare genetic disease have launched High Court legal action to overturn the decision not to recommend the funding of potentially life-saving treatment.

Nicole and Jessica Rich have been diagnosed with a neurodegenerative condition known as Batten disease, which could see them not live past the age of eight to 12. It is estimated that only 30 people in the UK have the disease.

The siblings, aged seven and three, currently receive access to a specialist treatment known as cerliponase alfa for their condition, one on a sibling trial and one as part of a compassionate use programme provided by Biomarin, the pharmaceutical company that developed the drug.

The treatment has proven to be extremely effective with it appearing to halt the degenerative effect of the disease in both of them. It is estimated the treatment could help people with Batten disease live for 60 years.

Jessica Rich Credit: Family Photo

However, advisory body the National Institute for Health and Care Excellence has recommended that the drug, which is funded by health services in 19 other countries including Wales, cannot be available on the NHS because they cannot be certain, with the lack of long term data that it would provide value for money.

The decision has prompted Nicole and Jessica’s parents, Gail and Matthew to hire lawyers to challenge this decision.

Their legal team has now applied to the High Court for a judicial review of the decision, on the basis that it is unlawful on several grounds and also amounts to a breach of key human rights legislation.

Nicole and Jessica Rich Credit: Family photo

NICE said it would reconsider whether reaching an agreement with the pharmaceutical company but has recently postponed its announcement.

Kirsty Stuart, specialist public law and human rights lawyer at Irwin Mitchell representing the family said: “This is a truly tragic case where at present, cost is deemed to be more important than children’s lives.

“Despite all of their challenges the family leads as normal a life as possible. All Matthew and Gail want is what any parent would want – to provide the best possible life and future for their children.

“Cerliponase alfa is the only type of drug currently available to help treat people affected by this awful disease. Matthew and Gail have been devastated by NICE’s original decision not to recommended its funding on the NHS. The delay in NICE announcing whether to change its decision has caused further frustration for the family.

“They would rather not be in this position but unless NICE recommends the drug’s use on the NHS, feel they have no choice but to bring this legal case, not only to help themselves but other families.”

Nicole had started developing symptoms including speech problems, seizures and falls. Following months of tests she was diagnosed with Batten Disease aged five. This led to Jessica undergoing tests, even though she was not displaying any symptoms.

Since she started her treatment Nicole has not shown any additional symptoms of Batten disease and Jessica has not developed any signs and remains healthy.

It is unclear what BioMarin will do when Jessica’s trial ends in May 2020 in terms of future funding and it has indicated that it would not be able to fund the treatment long term for Nicole or others on the compassionate use programme.

Gail, 41, and Matthew, 31, have a son Louis, eight, who does not have the disease.

Nicole Rich Credit: Family photo

The family have previously met with Matt Hancock, the Secretary of State for Health and Social Care, to lobby for cerliponase alfa to be free on the NHS.

Gail, a full-time mum, said: “Nicole and Jessica are adorable girls. We feel so blessed to have them and are amazed how they don’t let their condition define them. Nicole enjoys going horse riding and Jessica is a bundle of energy. She has started nursery and enjoys singing and dancing.

“All we want is to be able to give them the best chance in life. We are trying to remain positive but it is difficult knowing that if their treatment stops it will effectively hand them a death sentence.

“The fact that there is a treatment which works and will give not only our family but others years of happiness and memories is what makes this so difficult.”

Matthew, a production operative added: “What makes this even harder to try and come to terms with is the fact that 19 others countries fund the treatment. If it can available there why not in England? It just seems that those who have made the decision have shown a total lack of compassion.”

Nicole and Jessica Rich Credit: Family Photo

A NICE spokesperson said: