Aug 10, 2005 Latest post:
Jun 13, 2017
James Ciannello is almost 13 years old and has been living with many medical issues since the small age of 3 weeks old. James medical history includes Gastroenterology- Eosinophilic Esophagitis, Reflux, Feeding/Swallowing Issues, Early & Late Dumping Syndrome, Hypoglycemia. Immunology- Common Variable Immune Deficiency. Pulmonology- Severe Asthma, Bronchiectasis, Exercise Induced Asthma, Arytenoid Prolapse, Laryngomalacia, Vocal Cord Dysfunction, Small Volume Lung Aspiration, Chronic Bronchitis, Constant State of Exacerbation. Allergy- Multiple Food & Drug Allergies, Cold Urticaria. Dermatology- Eczema, Ichthyosis Vulgaris, Juvenile Plantar Dermatosis. Rheumatology- Enthesitis, Chronic Leg Pain, Plantar Fasciitis. Cardiology- very mild pulmonary regurgitation. James has a Mic-key button which is a type of feeding tube and currently hooks up for 8 hours a day receiving Elecare Jr. unflavored. James has a Port, central line for his infusions and he also frequently requires supplemental oxygen.
James has had about 30 hospital admissions over the years between CHOP, RWJ, NIH, and NJH. I have stopped counting the amount of tests or procedures but has been under general anesthesia close to 30 times including 4 operations and 21 scopes with biopsies.
Eosinophilic Esophagitis is a GI disorder where his esophagus is inflamed as a result of too many eosinophils. His body reads food as a foreign invader (bacteria) and attacks it with eosinophils which cause scar tissue and damage to the GI tract. The scar tissue in time leaves permanent damage. Eosinophils are a type of white blood cells. James' body rejects food. This disorder causes James to have a strict limited diet, very few foods. When we introduce a food to James, one food has to be eaten for about 2 weeks to watch for a reaction both allergic and from an EE standpoint. We usually do about 2 or 3 foods and then James has to have an Endoscopy with biopsies. James has done 30 food trials over the years. He has also had 21 endoscopy's to date. Unfortunately, eosinophils are only able to be seen under a microscope and at present time, the only true test for diagnosis/management is an Endoscopy.
Currently, James has eosinophils in his esophagus, lungs, and liver that we are aware of based on biopsy results.
James also has CVID, common variable immune deficiency. His body makes very few of his own antibodies. Antibodies fight off infection. This makes James susceptible to getting sick easily. James has a nurse come to our house every 3 weeks to administer his IVIG, Privigen, which is human plasma that infuses antibodies to help keep him free from infection being he cannot make them on his own. Usually takes about 3 hours for the infusion. James started receiving infusions December 2005.
James lungs are very complex and remain a daily source of struggle for James. He has severe asthma, exercise induced asthma, bronchiectasis, arytenoid prolapse, laryngomalacia, vocal cord dysfunction, and small volume lung aspiration. James requires the use of oxygen, frequent breathing treatments, averaging 7 to 10 times in 24 hours and still struggles to breath daily. We also use the AerobiKA and chest vest therapy. He recently started a new treatment, Mepolizumab where he receives a SubQ injection every 4 weeks.
James remains allergic to more than 50 items, food and medicine. If James does not test positive to a food and we trial the food, it can still make James body sick and reject the food which is from the GI disease.
James has cold urticaria which means when his skin is wet or sweaty and then he goes into a cooler room or a breeze hits him, he face and neck area break out into hives. Every time he bathes, hives.
James currently overnight feeds through his mic key button (feeding tube) 8 hours of Elecare daily which is an amino acid elemental formula as well as water. He is eating White Potato, French Fries, Rice, Chips, Applesauce and Orange Popsicle.
James has leg pain almost daily and was diagnosed with Enthesitis which could help explain why he is often in pain. It causes inflammation at the sites where tendons or ligaments enter into the bone. This is often felt in his heel, hip, legs, and knees.
James care is mostly at Children's Hospital of Philadelphia under Dr. Ritu Verma- GI, Dr. Kate Sullivan-Immunology, Dr. Terri Brown-Allergy, Dr, Hank Mayer- Pulmonology, Michele Shuker- Nutrition, Dr. James Treat- Dermatology, Dr. Karen Zur- ENT, Dr. Ravishankar- Cardiology, Dr. Blinman- General Surgeon, and Robin Cook- Dietitian as well as the feeding team and day medicine. His other care here in New Jersey is with Dr. Gerard Fritz, his primary.
James also has a doctor, Dr. Josh Milner down at National Institute of Health in Bethesda, Maryland.
James also has been seen by Dr. J. Tod Olin out at National Jewish Health in Denver, Colorado.
James also sees Dr. Perry Wolk-Weiss at The Get Well Center in Bound Brook, NJ
We have also tried homeopathic with James over the years but without change. James has trialed supplements and the most recent trial was something called NAET but unfortunately does not have seem to have helped in 9 months time of trying.
Virginie, from Home Solutions was our home care nurse who had been treating James from 2005 until 2015.
Jeff, from Prompt Care is our new home care nurse that we started with March 2015.
And I can't forget to mention our wonderful pharmacist, Mike Ryan from Devines in Dunellen.
We would like to thank everyone for your prayers. God is good and we should always trust in him!