Advice - Extension of reimbursement conditions for lumacaftor/ivacaftor (Orkambi®)

The National Health Care Institute has investigated whether the reimbursement conditions of lumacaftor in combination with ivacaftor (Orkambi®) can be extended. Lumacaftor/ivacaftor is already included in the Medicine Reimbursement System (GVS). The National Health Care Institute advises the Minister of Health, Welfare and Sport (VWS) to extend the reimbursement of the medicinal product lumacaftor/ivacaftor for the treatment of patients aged 1 to 2 years with cystic fibrosis. 

Indication for which reimbursement is requested

Lumacaftor/ivacaftor is used to treat cystic fibrosis (CF). CF is a pulmonary disease. It is a hereditary chronic disease in which the CFTR protein is less effective. CFTR is the abbreviation for cystic fibrosis transmembrane conductance regulator. The CFTR protein is a chloride channel in the mucus cells that ensures good and smooth mucus. Due to a CFTR protein error, this channel works less well. This makes the mucus tougher. Accumulation of the tough mucus in the lungs causes coughing, a reduced pulmonary function and recurrent pneumonia. Children with CF often have a stunted growth due to reduced nutrient intake and reduced metabolism. This is caused by the accumulation of tough mucus in the pancreas and intestines.

Lumacaftor/ivacaftor is already included in the GVS under additional reimbursement conditions, the so-called List 2 conditions. Lumacaftor/ivacaftor is reimbursed for patients with CF aged 2 years and older who are homozygous for the F508del mutation in the CFTR gene. Homozygous means that a patient has the same mutation in both copies of the gene, meaning they received it from both parents. 

The registered indication for lumacaftor/ivacaftor has recently been extended by the EMA to include the use in children aged 1 to 2 years with CF who have a homozygous F508del mutation. The marketing authorisation holder has now applied for reimbursement for this age group. At the moment, this group is receiving standard treatment. The standard treatment for CF patients consists of a combination of medicinal products that are aimed at combating pulmonary infections and inflammations (antibiotics), clearing of mucus (mucolytics) and improvement of the nutritional status (pancreatic enzyme supplementation therapy).

Advice from the National Health Care Institute

The National Health Care Institute advises the Minister of Health, Welfare and Sport to extend the reimbursement conditions for lumacaftor in combination with ivacaftor as follows: 

Only for the treatment of cystic fibrosis (CF) patients aged one year or older who are homozygous for the F508del mutation in the CFTR gene.

More information or questions?

If you have any questions about this advice, please send your question to the National Health Care Institute via warcg@zinl.nl. If you have questions about the reimbursement of a medicinal product, personal expenses or whether you should pay a contribution, please contact your health insurance provider.

How did the advice come about?

The Scientific Advisory Board (WAR) advises the National Health Care Institute about the assessment. Based on the assessment, the National Health Care Institute sends an advisory report to the Minister of Health, Welfare and Sport. The Minister makes the final decision whether or not to reimburse the medication from the basic health care package.

This report is a summary of recommendations by the National Health Care Institute. The original text is in Dutch.