Our daughter Phoebe Mae Grover was plagued with upper respiratory infections almost from birth. Little did we know that these pesky colds would eventually turn out to be an immune deficiency requiring treatment with immunoglobulin.Phoebe's Story
Phoebe Mae was born December 14th, 2006 and weighed 8lbs 12oz. She looked a little blue at first, but they gave her a little oxygen and we thought things would be fine. An hour later we got the news that she had aspiration pneumonia and had been admitted to the neonatal intensive care unit (NICU).
This was just the beginning of our journey with her upper respiratory issues. She wasn't able to keep her oxygen levels up or maintain her temperature due to what we thought was pneumonia. She spent four days on oxygen fighting to breathe. She then all of a sudden started doing great, so we were sent home on day five of her NICU stay.
Before Phoebe was even a month old, we noticed she was working really hard to breathe and just didn't look right. We took her in and she had RSV, a common respiratory infection. She was admitted to the hospital on oxygen and antibiotics again, and she was there for almost two weeks. This pattern was off and on every month or so the first two years of Phoebe's life, with constant antibiotics, breathing treatments, respiratory infections and other breathing issues including asthma.Searching for Answers
Our pediatrician finally decided to go out on a limb and do some out of the ordinary blood work, particularly an immunology workup on her. When it came back, we were shocked to learn that she had low Immunoglobulin G (IgG), the antibodies that are responsible for keeping you healthy and fighting off infections. So off to Immunology we went, where they diagnosed her with Selective Antibody Autoimmune Deficiency NOS and started her on a daily dose of antibiotics to help her prevent infections. Sinus and respiratory infections continued even with the antibiotics. After several tests they did a CT that showed she had 98% blockage of her sinuses. She had a PICC line placed to administer stronger IV antibiotics for six weeks.
After that cleared up, she continued to get respiratory infections. So at age three they started Phoebe on IVIG. This is a treatment that is an IV every three to four weeks to replace the antibodies that her body doesn't produce enough of to keep her healthy. It involves four to six hours of immunoglobulin given by IV. A Better Treatment: SCIG
Phoebe would get severe migraines from her IVIG, and would still get really sick at the end of the month when the treatment would run out. We talked to our Immunologist and they suggested SCIG, which is short for subcutaneous immunoglobulin, meaning given under the skin. This is similar to the IVIG but there are many advantages. These include:
- Done at home weekly versus at the hospital monthly
- Two very small needle sticks in her tummy versus an IV
- Only takes 48 minutes versus four to six hours for the IV
- Less sickness at end of month because it is given weekly for a more consistent level of meds
Just before her fourth birthday we started her on SCIG. The first dose was done at the hospital for training, so we knew how to administer it ourselves. After that, we would be on our own. They put her on Hizentra
, which is a more shelf stable medicine for immunoglobulin replacement. Her first time with this treatment went pretty well even though she was quite shocked on the different way it was done.
Here is how we give her SCIG:
First we lay out all of our supplies on a sterile field
Next we attach the two needles to her stomach
Here they are attached and ready to be hooked up
Then we attach them to the tubing and tape over both to keep them in place
The SCIG is given by syringe pump attached by tubing to the two needles
Phoebe can relax at home during her treatment
When done, the tape and needles are removed
Since we have started this type of IgG treatment she doesn't get as sick as she once did. Overall, this is a better way for our busy lifestyle and multiple children. This has made our schedules so much simpler, and Phoebe does better with the process being at home and able to get up and move around in her own environment.
There is also now a link to immune deficiencies and mitochondrial disease, which two of my other children have. So now testing will begin on Phoebe to see if she also has mitochondrial disease.
Overall, Phoebe does really well with the SCIG and we all prefer it. In the beginning, it was a little scary to do it on our own, but now it's just another day in the life of Phoebe Mae.Hope Grover is a mother of five special children: Elliott (18), Andy (16), Austin (7), Adam (6), and Phoebe (4). The three youngest boys have Autism, and all four boys have ADHD. Austin and Adam also have confirmed Mitochondrial Disease. To learn more about Mitochondrial Disease, visit www.umdf.org. Phoebe has an autoimmune deficiency and at the moment is being watched for Mito as well. Keep up to date with Hope's kiddos at Caringbridge: www.caringbridge.org/visit/adamgroverHope says, "Every day is a blessing with my children. They have taught me to enjoy the little things and be thankful for each day that God has given us because we are never sure of the future. They have taught me to overcome obstacles no matter how big they may seem. With lots of determination and patience most can be overcome. We never know what tomorrow brings or how long we will have with our boys, so we cherish the things we do today. They are quite a challenge but worth every minute of it! God has blessed me with my children and they in return make my life complete."