Raising Awareness

Evie had several congenital conditions relating to her heart, lungs and digestion. She was diagnosed antenatally with hypoplastic right heart syndrome, dextrocardia and a suspected tracheo-oesophageal fistula which meant she couldn’t swallow. After Evie was born she was prone to infections as she was so fragile due to her body trying to compensate for so many problems.

Tracheo -Oesophageal Fistula (TOF) and Oesophageal Atresia (OA) affect 1 in every 3,500 babies and yet there is little public knowledge of this condition. A valuable source of information to Greg and Jill was from the TOFs website. The website gives detailed information on the different types of TOF/OA which can occur. The charity has set up support groups for families living with a child who has to live with this condition.

Little Hearts Matters is a charity set up to provide support and information for babies who are born with half a heart, due to conditions such as Complex Pulmonary Atresia, Tricuspid Atresia, Hypoplastic Left Heart Syndrome (HLHS) or Double Inlet or Outlet Ventricle (Univentricular Heart). Their website is a valuable resource and explains all of the complex problems in detail.

Evie’s respiratory difficulties were mainly due to the complexity of her other problems. Her heart being on the right and having a large hole meant that her right lung did not fully form. Her airways were an unusual shape and ‘floppy’ which meant that she often breathed with a wheeze. Evie spent a considerable amount of time being ventilated (on life support) to provide pressure to keep her airways open. Evie also needed oxygen when she was getting closer to needing the Glenn heart surgery. The British Lung Foundation provides information on many respiratory conditions.

Pulmonary vein stenosis is often caused by problems with the heart and lungs but this is not proven. It is a progressive condition in which the veins narrow. Evie was diagnosed with this and underwent a high risk operation to try and correct it. It is a condition which is not fully understood and further information can be read here.

Necrotizing enterocolitis (NEC) is a life threatening bowel condition which affects 3000 babies in the UK every year. Evie developed NEC due to her poor cardiac output following open heart surgery to try and remove the vein stenosis. Necrotising enterocolitis is a serious illness in which tissues in the intestine (gut) become inflamed and start to die. This can lead to a perforation developing, which allows the contents of the intestine to leak into the abdomen, causing a very dangerous infection. Further information can be found on these hospital websites: Great Ormond Street Hospital and Southampton Children’s Hospital.