NZPAH Trust and Support Group

Supporting, educating and promoting awareness of PAH

Caleb’s Story

CalebCaleb and his identical twin Jayden were born prematurely on 12 May 2010 at 34 weeks (Caleb 3lb5oz/1580g and Jayden 4lb10oz/2250g).  Caleb’s birth weight was low for his gestational age – this was due to intrauterine growth restriction and twin-­to-­twin transfusion syndrome. When the boys were in NICU we were told Caleb had two holes in his heart ­ a patent ductus arteriosus (PDA) and an atrial septal defect (ASD).  Both holes are common in prem babies and we were told that the PDA would close once Caleb got to 40 weeks and the ASD should shut by the time he was 5 years old.


Jayden thrived once he was discharged from NICU but Caleb had ongoing issues including oral aversion ­ he had a NG tube for a year and then a MIC­KEY button.  In June 2011 he was diagnosed with mild Hypotonic Cerebral Palsy.  Just after being diagnosed with CP Caleb developed Bronchiolitis which then turned into pneumonia and a collapsed lung.
We saw numerous Paediatrician’s and consultants for various issues and each time I mentioned that Caleb was breathless, found it hard to crawl/walk up the stairs, he had an indrawing chest, sweated a lot, still had oral aversion but not one person we saw got to the bottom of all these issues until one Paediatrician finally referred us to ENT due to Caleb’s ongoing feeding and breathing problems.


Caleb had a Laryngoscopy and Bronchoscopy in March 2012.  From the L&B he was diagnosed with severe distal tracheomalacia and they uncovered that he had major issues with his heart.  Caleb had a catheterization in April and was diagnosed with a life threatening disease called bilateral Pulmonary Vein Stenosis (PVS) with hypoplastic LUPV, severe pulmonary hypertension (secondary to the PVS), anomalous RUPV to SVC and bilateral SVC's.


Caleb had the sutureless repair surgery on his heart in July 2012 (at Starship) to open the LLPV & RLPV and to close the ASD.  He had numerous pulmonary hypertensive episodes while in PICU so was put into an induced coma and he was ventilated for 17 days.  Caleb was in PICU for 3 weeks and then another 2 weeks in HDU and on the Heart ward.  Unfortunately while in PICU Caleb became dependent on Morphine and Diazepam so when he came home he was on a 6 week drug weaning programme.  


In October 2012 Caleb got pneumonia and collapsed lungs so spent 16 days in Waitakere Hospital.  During his stay there, Radio Lollipop had their official opening and Caleb was their 100th patient they saw so he had a visitor – none other than Sir John Kirwin!  JK spoke to us about Caleb’s medical issues and was very interested in how Caleb was doing and his prognosis.  JK chatted with Caleb, blew bubbles with him and gave him a soft toy which we appropriately named ‘JK’!


Caleb had an echo in March this year and his heart is looking better.  However, Caleb will have another Cath in the next couple of months as his Cardiologist wants to check the pressures in his lungs and also to look at the veins again.  She wants to make sure that Sildenafil is the right drug for Caleb going forward. Caleb is still on Sildenafil, Frusemide, Spironlactone and Omeprazole.


Caleb is still on Palliative Care (the PVS can come back at any stage) and we know that we are very lucky to have him with us so we try to make each day count for him and Jayden.