New Indication for Bardet-Biedl syndrome (BBS) – 9/15/23

On September 5, Rhythm issued a press release announcing the new ICD-10-CM code specific to Bardet-Biedl syndrome (BBS) ‒ Q87.83 ‒ effective October 1, 2023.

This new ICD-10-CM code specific to BBS has the potential to improve patient identification and ongoing monitoring, which may help providers and payers:

• Accurately account for BBS patients who have been lost to follow-up or misclassified under a similar disease
• Deepen knowledge of clinical presentation, disease prevalence and progression
• Help improve the multidisciplinary coordination of care needed in the management of this multisystemic disease
• Add clarity to the claim submission process

Ultimately, these improvements in specificity in coding can result in improved recognition and care for people living with this rare disease.

Previously, healthcare providers diagnosing BBS were limited to using ICD-10-CM code Q87.89 (Other specified congenital malformation syndromes, not elsewhere classified), a non-specific code that includes all the following congenital malformations:

• Bardet-Biedl syndrome
• Birt-Hogg-Dube syndrome
• Cantrell's syndrome
• Cerebro-hepato-renal syndrome
• Laurence-Moon syndrome

The change will be effective October 1, 2023.

Indication and Important Safety Information

Indication

IMCIVREE is indicated for chronic weight management in adult and pediatric patients 6 years of age and older with monogenic or syndromic obesity due to Bardet-Biedl syndrome (BBS).

Limitations of Use

IMCIVREE is not indicated for the treatment of patients with the following conditions as IMCIVREE would not be expected to be effective:

• Other types of obesity not related to BBS or other FDA-approved indications for IMCIVREE, including obesity associated with other genetic syndromes and general (polygenic) obesity

Important Safety Information

WARNINGS AND PRECAUTIONS

Disturbance in Sexual Arousal: Spontaneous penile erections in males and sexual adverse reactions in females have occurred. Inform patients that these events may occur and instruct patients who have an erection lasting longer than 4 hours to seek emergency medical attention.

Depression and Suicidal Ideation: Depression and suicidal ideation have occurred. Monitor patients for new onset or worsening depression or suicidal thoughts or behaviors. Consider discontinuing IMCIVREE if patients experience suicidal thoughts or behaviors, or clinically significant or persistent depression symptoms occur.

Skin Pigmentation and Darkening of Pre-existing Nevi: Generalized increased skin pigmentation and darkening of pre-existing nevi have occurred. Perform a full body skin examination prior to initiation and periodically during treatment to monitor pre-existing and new pigmentary lesions.

Risk of Serious Adverse Reactions Due to Benzyl Alcohol Preservative in Neonates and Low Birth Weight Infants: IMCIVREE is not approved for use in neonates or infants. Serious and fatal adverse reactions including “gasping syndrome” can occur in neonates and low birth weight infants treated with benzyl alcohol-preserved drugs.

ADVERSE REACTIONS

• Most common adverse reactions (incidence ≥20%) included skin hyperpigmentation, injection site reactions, nausea, headache, diarrhea, abdominal pain, vomiting, depression, and spontaneous penile erection

USE IN SPECIFIC POPULATIONS

Treatment with IMCIVREE is not recommended when breastfeeding. Discontinue IMCIVREE when pregnancy is recognized unless the benefits of therapy outweigh the potential risks to the fetus.

To report SUSPECTED ADVERSE REACTIONS, contact Rhythm Pharmaceuticals at 833-789-6337 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

Please see the full Prescribing Information for additional Important Safety Information.

© 2023, Rhythm Pharmaceuticals, Inc. All rights reserved.

Rhythm, IMCIVREE, and their logos are trademarks of Rhythm Pharmaceuticals, Inc. US-SET-2200172 08/2022