Look How Far She's Come...

Look How Far She's Come...
11.20.09 3 lbs. 3 oz. 16 in.

Fiona's ABCs (diagnoses)

(this is a joke with medically complex kids since most things are abbreviated with letters it's like they have the "ABCs"!)

Omphalocele: her O was considered "giant" and contained most of her small intestine, entire liver, stomach, gallbladder, spleen, and part of her appendix. She has had 4 surgeries in total to fix it. Her first surgery was on September 22, 2010 and she had all 4 within a year. She now has full muscle closure and will sometime in the future get a belly button!

Heart: she was born with 4 holes in her heart- PDA (Patent Ductus Arteriosus), PFO (Patent Foramen Ovale) , ASD (Atrial Septal Defect), VSD (Ventricular Septal Defect). The PDA and the PFO both closed while she was still in the NICU. Over the past 2 years both the ASD and the VSD have been slowly closing on their own. The ASD is now fully closed and the VSD is just a tiny pin hole that will probably never need surgical repair. She is considered to have CHD (Congenital Heart Disease).

Lungs: immediately after birth she went into respiratory distress (RDS- Respiratory Distress Syndrome) and was intubated and put on a ventilator and oxygen. She has Pulmonary Hypoplasia which is basically incomplete development of the lungs. She also has BPD (Bronchopulmonary Dysplasia) which is a chronic lung disease resulting from her being premature and being on a ventilator. She got her trach at 9 weeks old and has been on a ventilator since birth. She is now only on the ventilator at night due to sleep-related hypoventilation and hypoxemia, which means that she takes very short, shallow breaths while she is sleeping and her body doesn't get enough oxygen (this also causes her to sometimes retain too much carbon dioxide). She also has central sleep apnea, which means that her brain periodically forgets to tell her body to breathe and her oxygen levels drop. While she is awake she usually has tachypnea- she breathes faster than normal. She only uses oxygen now when she gets a bad cold. She also has mild tracheomalacia- a floppy airway.

Urinary: she has VUR (Vesicoureteral Reflux)- urine backs up from her bladder into her kidneys which puts her at a higher risk for urinary tract infections. When she was born she had a Grade III in her right kidney and a Grade IV in her left. She is now a Grade II in her left and very minimal in her right.

Brain/Development: she had a Grade I- IVH (Intraventricular Hemorrhage- a brain bleed) at birth which resolved itself at about 3 months. She has normal congnitive function. She was pretty far behind physically by the time she came home. She receives physical, occupational, and speech therapy every week. She is doing much better and is about 6 months behind with both gross and fine motor skills. She just started actually saying a few words (at 2) and does about 75 signs. She is a little behind with her social skills, but is getting better. She has hypotonia (low tone in her muscles).

Feeding/Nutrition: she has had feeding issues since day 1. It was awhile before they started feeding her and an even longer time until her stomach and intestines tolerated full feeds so she was given TPN (Total Parenteral Nutrition) and IV fluids. Since she was intubated and didn't get her trach until she was 9 weeks old, she didn't get her first bottle until she was 10 weeks old. She was fed via NG (Naso-Gastric) tube until she was 7 months old. She has GERD (Gastroesophageal Reflux Disease) and post-tussive emesis which pretty much means that she throws up ALOT. She is considered FTT (Failure to Thrive) because she is so much smaller than average. She has dysphagia to solids and a hyperactive gag reflex which means that there are very few things that she can eat without gagging and throwing up. This has slowly been getting better, but is still an issue. She currently gets about a 4:1 mixture of Pediasure 1.5 and Scandishake with added sunflower oil and we work every day on trying to find new foods that she can eat. We try to pack as many calories as we can into as little volume as possible because she can only take small amounts at a time.

Medicines: she is currently on Amoxicillin for her VUR, Zantac for reflux, Diuril for her heart, Periactin which is an appetite stimulant, Melatonin for her sleep issues, Pulmicort which is a breathing treatment, and a multivitamin.