Reason for request
Initial inclusion
Summary of opinion
Favourable opinion for reimbursement in “the treatment of obesity and the control of hunger associated with genetically confirmed Bardet-Biedl syndrome (BBS), loss-of-function biallelic pro-opiomelanocortin (POMC), including PCSK1, deficiency or biallelic leptin receptor (LEPR) deficiency, in children aged 2 to < 6 years.”
Clinical Benefit
| Substantial |
The clinical benefit of IMCIVREE (setmelanotide) 10 mg/ml is substantial in children aged 2 to < 6 years.
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Clinical Added Value
| minor |
Considering:
- the suggested efficacy of setmelanotide in terms of the proportion of responder patients (≥ 0.2 reduction in BMI Z-score) and the mean change from baseline in BMI, based on the results of the non-comparative RM-493-033 study conducted in 12 patients aged from 2 to under 6 years. After 52 weeks of treatment, 83.3% (n=10/12) of patients achieved a reduction from baseline in BMI Z-score of ≥ 0.2, and the mean percent change from baseline in BMI was
-18.4%,
- the expected beneficial impact associated with the reduction in BMI Z-score in these conditions with significant morbidity,
- the absence of robust data concerning the hunger score and change of eating behaviours of the children,
- the absence of robust data relative to quality of life, which is particularly impaired in these conditions, for both patients and their carers,
- the safety profile of setmelanotide, consistent with that already known in patients 6 years of age and above, characterised by hyperpigmentation disorders and injection site reactions as the most common adverse events, with patients treated with IMCIVREE (setmelanotide) required to undergo regular monitoring,
- the unmet medical need in these conditions, both for POMC deficiencies, including PCSK1 and LEPR deficiencies, and for Bardet-Biedl syndrome,
the Committee deems that IMCIVREE (setmelanotide) provides a minor clinical added value (CAV IV) in the treatment of obesity and the control of hunger associated with genetically confirmed Bardet-Biedl syndrome (BBS), loss-of-function biallelic pro-opiomelanocortin (POMC), including PCSK1, deficiency or biallelic leptin receptor (LEPR) deficiency in children aged 2 to < 6 years.
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