Pamela’s Journey

Frankie is 12 years old, she has power to light up your day even when your down, she teaches us life is precious and short and to cherish every moment, despite all of her challenges, she awakens each day with a smile.

Francesca suffers from a Neurological disease called Rett Syndrome,she also has an additional diagnosis of Common Variable Immunodefiency.
Rett Syndrome causes her hands to wring to the point that her joints are swollen 3x the size they should be. (This movement is not a stim it is coming directly from her brain.) She suffers each day with severe GI dysfunction causing much pain on a daily basis. She has two separate tubes in her stomach one in her stomach for venting and meds, and a jejunal tube to release air from her intestines. Unfortunately Frankie also requires to be fed IV for 12 hour anywhere from 4-to all 7 days to sustain life. She also has severe lung issues, requiring a daily routine of 4 nebulizer treatments lasting 40minutes and 3 airway clearance treatments of 20 minutes daily. She is always monitored for seizures even though she hasn’t had one in 2 years. She is followed by Cardiology because she is at risk for Cardiac Arrest in her sleep. She has swallowing issues which makes her choke on her own saliva and sometimes aspirate. She suffers from bilateral knee/ankle rotation, she has brittle bones which resulted in her femur being fractured. She has a central line and developed thrombosis and requires blood thinner injections daily. She has apraxia, ataxia, dystonia making movement painful. Her biggest obstacle in enjoying life is she cannot regulate her body temperature, in heat she requires to be on oxygen and a cooling vest, if her body become too warm she goes into respiratory distress, so she can’t go outside in the summer a lot. She has poor circulation in her legs and feet, making them feel ice cold all time. Her Immunodeficiency basically means she can’t fight infections, she gets an IV infusion weekly to help give antibodies to fight infections. The everyday cold for us turns into a week hospital stay for her. She spends a good portion of her life as impatient mostly at Children’s Hospital of Philadelphia. She attends multiple doctor appointments with 10 specialists, and attends therapy to maintain movement. The simple things we take for granite like sitting on your own, she needs support or would fall, and she needs 100% total care for every aspect of her life. She requires round the clock nursing care. She is cognitively age appropriate she understands everything you say, but she locked in a body that won’t let her respond.

10 years ago my family’s life was forever changed by a phone call, her Neurologist had ran tests 4 months earlier, the news on the phone was Mrs Scarano we know what is wrong with Francesca, at first a sigh of relief thinking she can now get help to relieve all her medical issues, but the next sentence was, I am so very sorry to tell you your daughter has the worst disease she could have been diagnosed with, with tears holding back I asked is she going to die? We don’t know how long she can live, she won’t be able to talk, she will not be able to use her hands, and she will require round the clock medical care. That being said she already was trying to walk, crawled yelled Daddy and Mommy and called her brother D short for Domenico, she yelled at dog for barking, They said she will lose all of her skills as days go by, she will lose eye contact, develop seizures and other medical issues. I told her we need to redo the test this has to be a mistake, please not my beautiful daughter that I dreamed of , I couldn’t talk I fell down our stairs, I couldn’t even tell my husband what they said, my son sat in tears not knowing what was going on. After many phases first being acceptance, I got myself together and decided we would live for today and we would fight every day for her, that was many years ago, and we have never stopped.

Move forward as the years went by and I learned how to navigate thru many systems, Medical Assistance, MHMR, Therapy, IEP’s not easily I may add. In our path we were able to attend a handicapped preschool Children’s Association , for me probably the biggest turning point in finding peace, you see I never left preschool I waited in another room while she attended with her nurse, always there in case she had emergency. But I wasn’t the only mom doing that, hence comes where my support, and my lifelong and (lifeline) friends come in.

We learned from each other, we went out in public together not feeling isolated, we passed tips along, they were there to listen to me cry on my bad days and I was there to listen to them when there days came. 10 years later and those very same special needs kids are still best friends and the smiles they create when there together could never be forgotten. I still lean on each one of my friends for daily support as they do too. You can never know what we go thru and feel unless you are one of us, and believe me you don’t want to be.

In conclusion our beautiful daughter has taught me so much, that I am sure I would have never learned if it had not been for her, she brings more joy and happiness to our life than you could imagine.