Chalazion / Hordeolum under therapie with Trikafta®

Question

Hello,
my two-year-old daughter has been taking Trikafta® (=Kaftrio/Kalydeco®) since October 2025. Since December, she has been suffering from meibomian gland dysfunction and constantly gets hordeola. She also now has a chalazion that has become encapsulated and isn't healing. Several forums, and a case study on PubMed, have shown that many people have experienced this since starting Trikafta. Our clinic is against discontinuing or reducing the dose. This can't go on like this. Why is this happening, when Trikafta is supposed to improve secretions, and what else can we do?

Answer

Dear Sir/Madam,

Meibomian glands are sebaceous glands located in the eyelids. They produce lipids, or fats, which are a fundamental component of the lipid layer of the tear film. These lipids protect the tear film from microbial influences and the evaporation of the aqueous phase, thus acting as tear film stabilizers.

To what extent can these glands be affected by a dysfunction of the CFTR channel, which is impacted in cystic fibrosis? The function of the CFTR channel is well understood in glands that produce aqueous secretions, such as in sweat glands (high chloride content in sweat when dysfunctional), or in the lungs, liver, etc., with the well-known consequences of thick secretions due to salt and water deficiency. However, the function of the CFTR channel in glands that produce fatty secretions is not yet as well understood.

To what extent can these glands be affected by a dysfunction of the CFTR channel, which is impacted in cystic fibrosis? Chloride transport through CFTR channels has been demonstrated in human and animal (rat) meibomian gland cells.

The hyperviscous mucus secretions associated with dysfunctional CFTR proteins can obstruct these glands and cells, leading to degeneration of the lacrimal acinar cells, meibomian gland dysfunction (MGD), and reduced glandular secretion.
(References: Moshirfar M, Brown AH, Sulit CA, Corbin WM, Ronquillo YC, Hoopes PC.
Corneal Refractive Surgery Considerations in Patients with Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders.
Int Med Case Rep J. 2022 Nov 9;15:647-656. doi: 10.2147/IMCRJ.S381078. eCollection 2022.PMID: 36388243 and:

Schneider-Futschik EK, Zhu Y, Li D, Habgood MD, Nguyen BN, Pankonien I, Amaral MD, Downie LE, Chinnery HR.
The role of CFTR in the eye, and the effect of early highly effective modulator treatment for cystic fibrosis on eye health.
Prog Retin Eye Res. 2024 Nov;103:101299. doi: 10.1016/j.preteyeres.2024.101299. Epub 2024 Sep 6. PMID: 39245300).

Thus, glandular dysfunction with the increased occurrence of styes/hordeola would be expected more readily in untreated CF.

But what do the data say about CFTR modulators and the occurrence of styes and/or chalazia?

To date, three different case series on the occurrence of chalazia and/or styes during concurrent CFTR modulator therapy have been published. A treatment algorithm can be found in Lieu N et al. Paediatr Respir Rev 2026 – you can discuss this with your doctors and forward them the reference.

Literature:
1) Lieu N, Singh J, Solomon M, Hunt S, Simonds S, Boyton C, Pandit C, Fitzgerald DA, Selvadurai H.
Chalazion and hordeolum in pediatric patients with cystic fibrosis on elexacaftor/tezacaftor/ivacaftor.
Paediatr Respir Rev. 2026 Mar;57:27-30. doi: 10.1016/j.prrv.2025.11.002. Epub 2025 Nov 17.PMID: 41298194
2) Kates MM, Luckett JP, Vicinanzo MG, Gutierrez H.
Chalazion and Hordeolum Development in Patients with Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor Therapy.
Ophthalmology. 2025 Jun;132(6):733-734. doi: 10.1016/j.ophtha.2024.12.035. Epub 2024 Dec 25. PMID: 39730105
3) McCaffrey C, Joshi N
461 Unseen consequences: Hordeolum and chalazion as a potential side effect of CFTR modulators
Abstracts of the 2025 North American Cystic Fibrosis Conference
Journal of Cystic Fibrosis 2026; 24S2 (2025) S264

In the studies conducted for the approval of the modulators, the afore mentioned changes in the meibomian glands did not occur more frequently. Although one would expect that, based on function, styes and chalazia should occur less often with a "corrected CFTR channel," this is now being described in the case series. This could have several reasons. Firstly, it may not yet be fully understood how the CFTR channel actually affects glands that produce fatty secretions. Perhaps it has an effect not only on the gland itself but also on the skin and the microbiome that colonizes it (i.e., the bacteria that are on the skin and can lead to inflammation of the gland). Thus, the modulators could also have effects on the skin and the microbiome.

Secondly, styes and chalazia are a problem in 4% of "healthy" children and recur at a certain age. The 4% of children who suffer from this condition may have a different sebum composition and/or skin microbiome, and therefore experience styes or chalazia more frequently. If modulators are to have an effect, the rate of children taking modulators and suffering from this condition would have to be higher or significantly higher than 4% to account for the background effect that also occurs in healthy children. This would, of course, require extensive data and/or a study specifically addressing this question and including a control group not taking modulators.

In published case series, the problem of styes and/or chalazia sometimes led to discontinuation or a reduction in the dose, while in other cases the therapy was continued and the problem was successfully managed with conservative treatment.

This can only ever be a case-by-case decision that you should make together with your doctors.

It is important that the therapy of choice is (even after healing of the acute state) to perform a rigid hygiene of the eyelid daily and consequently (warm compresses, which first liquefy the meibomian gland secretions, then express the meibomian glands). An ophthalmologist can provide guidance on this; they also have leaflets available on meibomian gland dysfunction. Unfortunately, there is limited data on its effectiveness, especially in children.

All other treatment options are exceptional. Topical antibiotics and steroids show inconsistent results and can have side effects in children. New therapies (probiotics, omega-3 supplementation, intense pulsed light) are promising but require further research. Intralesional steroid injections have high success rates but carry the risk of rare complications. Surgical excision remains effective for persistent lesions. Individualized treatment approaches are recommended, taking into account underlying conditions (vitamin A deficiency, Demodex infestation, rosacea).

References:
Kornhauser T, Elhusseiny AM, Pemberton JD.

Management strategies for chalazia in pediatric patients: A scoping review.

Eur J Ophthalmol. 2025 Jul;35(4):1481-1494. doi: 10.1177/11206721251330146. Epub 2025 Mar 29. PMID: 40156262

We hope this information has been helpful.

Daniela d'Alquen

17.05.2026