2019 - The Birth Year

On January, 14, 2019, our precious little girl was born at 7:38 pm. Weighing 7 pounds and 5 ounces, she came fast! Just like River, she had newborn jaundice so she spent some time on the bili lights before heading home. Named Jovie from the movie Elf, but the meaning is why we chose it. Meaning of Jovie: "jovial", or happy, cheerful and friendly.

On February 1, 2019, not even a month after being born, Jovie went in for her first visit with Dr. Michael Schmitz. He diagnosed her with, what we already knew, Proximal Femoral Focal Deficiency (PFFD) of left lower extremity.

Proud big brother!

On February 26, 2019, we went for a visit with the amazing Colleen Coulter, a Physical Therapist, to discuss managing the PFFD and the physical therapy that would be required for Jovie pertaining to the leg lengthening. That same day, we also met with Rebecca Hernandez who would be making Jovie's shoe lifts once she was walking age.

As if she didn't have enough going on, Dr. Michelle heard a heart murmur and we scheduled a visit to see Dr. Megan McKane, MD, with Sibley on March 15, 2019. She had an EKG and Echocariogram performed which showed a trivial PDA and fenestrated ASD. We will just watch and see if it closes on its own; nothing to do now.

On March 28, 2019, we took Jovie to see Dr. Seth Marcus, MD, at GI Care for Kids Atlanta, regarding possible reflux. He suspected that she may have had a milk protein intolerance. We were instructed to keep her upright after feeds, smaller and more frequent feeds, and begin a 2-4 trial of strict maternal "casein"/"whey" elimination diet. She also began taking prevacid to help with the acid reflux.

After receiving her school pictures, I was concerned about the right head tilt and posterior skull flatness at 3 months old. On April 9, 2019, I emailed Colleen Coulter asked about Jovie's ear: Her right ear sticks out more than her left ear. Does this have anything to do with how her head leans towards her right shoulder (torticollis)? I've attached a picture. Is there anything we can do for that? Do we need to make another appointment to see you regarding her neck or ear?

Colleen's response:

Hello!

Jovie is so darn cute :)

Yes, right torticollis can effect facial features including the ear.

Would be delighted to see Jovie.

On April 11, 2019, she went back to CHOA and met with Rebecca Hernandez to get a head scan and was diagnosed with Right Plagiocephaly due to Right Torticollis (Congenital Musculoskeletal Deformity of Sternocleidomastoid Muscle). She began physical therapy exercises with Colleen every two weeks. We also had exercises to perform at home, also.

From April to September 2019, Jovie would visit Colleen and Rebecca approximately every two weeks for Torticollis and Plagiocephaly.

On June 7, 2019, Jovie had her 4 month recheck with Dr. Schmitz. This visit was to check her leg length to see what the difference would be.

On June 14, 2019, Jovie had a follow-up with Dr. Meghann G. McKane, MD, to address her Fenestrated Atrial Septum. An echocardiogram was performed and the hole still had not closed on its own.

On October 30, 2019, Jovie had a re-check with Dr. Schmitz. It was at this appointment that we were officially transferred to Dr. Jill Flanagan because she was considered to be a good limb reconstruction candidate (had enough bone to lengthen) as opposed to amputation. She had an x-ray at this appointment where she had a limb difference of 3.5 cm. Her right femur was 14.2 cm and her left was 10.7 cm. Expected difference with no lengthening surgeries would be 11.5 cm. We also saw Rebecca for another head scan and she was released.

When we met with Dr. Flanagan for an evaluation, we were told that PFFD was based on a spectrum and that Jovie was considered a Type A, which is the best case scenario.

• Type A — The femur bone is slightly shorter on the proximal end (near the hip), and the femoral head (the ball of the thigh bone that goes into the hip socket) may not be solid enough to be seen on X-rays at birth, but later hardens (ossifies). This deformity is sometimes called congenital short femur, because the child’s anatomy from hip to knee is contiguous and similar to their peers except for the one shortened bone. In some cases, children with type A deformities will also have an externally rotated femur, which could lead to bowing of the legs (genu varum).

Surgical "Life Plan" recommendations are as follows:

• SUPERhip reconstruction of the left hip at age 2 years. Hardware is used for this surgery and would be taken out after 6 moths.

• Recommend first leg lengthening at age 4. Goal length: 4 cm

• Second lengthening at approximately age 10. Goal length: 4-5 cm

• Can either perform an epiphysiodesis (surgical procedure of a physis to stop future growth) of the right femur to "make up" the rest of the difference, but if lengthening goes as anticipated, would prefer a third lengthening at/near skeletal maturity (age 14-15) to ensure equal limb length and decrease risk of complications of epiphysiodesis of a limb not affected.

• Recommend to continue normal baseline activity, as tolerated.

Dr. Flanagan later told that she recommends lengthening at age 4 and not at age 5 when she'd be in Kindergarten. Kindergarten is a no lengthening year! No lengthening 6th grade, 9th grade, and 12th grade either (Flanagan rules :)).