In 1989 things were a LOT tougher.
Here are photos of our daughter Stephanie, born in Perth 1989. She has given permission to share these with you.
Basically Stephanie was born and diagnosed with PRS within hours of life. She spent 39 days in the neonatal nursery at PMH and was always very difficult to feed orally, with significant vomiting from the start, though she wasn’t fed orally until a week of age. She was predominantly fed via NGT although she vomited massively and the tube had to be replaced 2-6 times per day. IT was very stressful. I expressed for her for 7+ months and had to add an extra 40% calories so she could grow. Life was pretty hard for her, she ‘failed to thrive’ and her development was delayed. She was tube fed 3 hourly for 2 years and at night via a kangaroo pump and woke retching and gagging every morning for 2 years.
In order that her palate be able to be repaired she needed her NGT out of the way, so at 11.5 months she had a gastrostomy performed. This was changed to a button at 19 months. The button was not a huge success as the one way valve meant that she HAD to tolerate the volume, and so she vomited more (yep, still vomiting at this age). She lost 10% of her body weight in about 6 weeks, then got a bad fever, and then all her hair fell out. It was a very sad time.
Just after she turned 2 we asked for a psychologist to help us with a feeding program, to help her unlearn her oral aversion, where her mouth was just an exit point. She was showing some curiosity about food, but gagged so readily she couldn’t deal with it. It was a complex training process but after a week she voluntarily opened her mouth for a spoon and allowed it to touch her lips. She shuddered but did not throw up. I can still cry thinking about it. She rapidly over a few weeks began to accept tiny amounts of pureed foods, and swallowed them better each time. This new ‘eating game’ was fun! But she was still tube dependent for the majority of her calories.
With the agreement of her Gastroenterologist we decided to cease the night feeds (50% of her caloric intake) to induce hunger. The first night passed and she awoke without dry retching, for the first time in her life. It was a miracle. She started to tolerate more food per meal, and drink some milk from a sippy cup. We held an ‘eating girl’ party. She was 2 yrs and 3 months.
Stephanie had almost no babble, and at the age of 2 we (through PMH speechies) started teaching Steph to sign, so she could have some expressive language. It worked a treat, so I went to classes at the WA Deaf school for a semester to pick up more signs and we attended Deaf playgroup as well, so she could see other kids and adults signing. It became a second language for us. We moved to America for 7 months when she was 2yrs 4 months and continued speech and OT there, as well as seeing an ENT there. American signs were somewhat different but we continued doing it. When Steph was almost 3 she signed her first sentences that indicated that she was comprehending well, and had IMAGINATION that she could express. We were all so thrilled to bits. She continued to be signing non-verbal until around 4+ years of age, with a bit of babble and LOTS of speech therapy, then transitioned to majority of verbal language over the next year. She still signs a tiny bit now, but mostly for convenience across a room! Her speech is a bit slushy and some sounds still give her hassle, but just today at the Cleftpals meetup, she was chatting away and happy to share her experience of her frustrations and challenges, and her decision-making around choosing a second pharyngoplasty at age 21 to help improve the quality of her speech (it did). Stephanie tells of being unable to reliably say her name properly/intelligibly so she uses her middle name (Faye) as her ‘coffee name’ rather than try to repeat herself to baristas!
Steph has had many challenges in life with developmental and health issues. She had aide support throughout school and completed year 12 non-TEE. She has worked in hospitality in supported and open employment, and is currently doing admin work for a council. She lives by herself with her cat, and has some in-home support along with parental support. She has had 25 operations including gastrostomy, gastrostomy reversal, cleft repair, 2 x pharyngoplasties, major dental work x 5, grommets x 7. Her mouth was so small she was 18 before she could get her braces, and was delighted they came off the week before her 21st. Life continues to throw curve balls but Steph is enjoying being part of the FB groups for PRS and Cleftpals; telling her own story and forging her own identity as a person with PRS, and loves to look at photos of her life, hence the sharing of these pics. It is great to hear her perspective on her journey, and she gets value from hearing ‘old ‘ stories (to me) but she is discovering these stories as she was quite little when many of them happened.
Today she is 27 and a fantastic cook. She eats fairly well but is still bothered by reflux and textures. She is lettuce-phobic – it is a standing joke/bet in our house about salad consumption, jackpotting with each year she goes without eating salad.
I would love her life to be easier for her and for her to make friends more easily but she is a late-bloomer and is continuing to grow. She is a terrific person despite her struggles. I would change her situation FOR HER, but not for me as I have gained so much from being her Mum.
She truly is a remarkable young woman, with a lot to offer. We are so proud of her.