[CIS PIDD] [cis-pidd] AT lung care

[CIS-PIDD]

Dear all.
I´m a Clinical Immunologist working in Aguascalientes, Mexico. 
I´ve been taking care of two siblings with AT. One of them (15yo female) has marked lung involvement (2 pneumonias  and bronchiectasis by MRI) with chronic cough (persistent crackles and recurrent wheezing)
She is unable to perform lung function tests and I´ve tried several respiratory therapies including ICS/LABA, LAMA, SABA/ICS with different dosage with mild clinical improvement with current therapy (SABA /ICS high dose) 
She is receiving oral betamethasone 0.5mg/day, Hydroxichloroquine 200mg/day, SABA/ ICS high dose, Monthly IVIG (500mg/kg) and lung therapy. 
Improvement was noted in ataxia and infections but the cough is a concerning issue affecting QoL and I am really concerned because clinically the respiratory features are getting worst every year. 
Would you suggest other therapies or treatments in this case?
Thanks on advance.
Sincerely.
 Dr. Aristóteles Álvarez Cardona.Alergia e Inmunología Clínica Pediátrica.Tel: (449) 3181833(449) 9109900 Extensión 2311Cel: (449) 1265561
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