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It Makes Me Realize I'm Not Alone
Posted Oct 10, 2017
When Angel was born in May 2015, he appeared to be a perfectly healthy 7lb. 14oz. little boy. His mom Rachael and dad Alfonso brought Angel home to what they thought would be the typically chaotic life of a family of five.
At his two-month well check, Angel weighed almost a pound less than his birth weight. After some monitoring, the pediatrician diagnosed him with failure to thrive.
But it wasn’t long before he was repeatedly admitted to the hospital first, with an ear infection, then with a cough and eventually with pneumonia. Each time, the antibiotics he was prescribed were ineffective and Angel was getting sicker. He continued to lose weight and Rachael was taking him to the pediatrician every two days to monitor his weight.
When his weight fell to six pounds and Angel was still so sick he was using supplemental oxygen, Rachael decided to travel from their home in Williams, AZ, to Phoenix Children’s Hospital to look for answers.
Within 10 hours of arriving at PCH, Angel was placed in isolation in a room with special circulation to keep the infection levels low. “The suite,” as it was nicknamed, was double-doored. Rachael had to scrub her hands and arms, put on a gown, gloves and mask before she could enter the room to be with Angel. It took very little time before Rachael learned that Angel had Severe Combined Immune Deficiency, sometimes referred to and would need a bone marrow transplant before his first birthday. At the time, he was three months old.
Rachael had prepared for a week-long hospital stay away from home. It was clear, a week was not going to be enough. She shared what it was like for her during those first weeks. “I would never leave the hospital room. The first time I did leave to go downstairs to get something to eat, I came back and they said they almost lost him. He had so much mucus, his airways got clogged, and his oxygen level dropped below 60.”
Primarily, Angel was fighting off three viruses; rhinovirus, adenovirus, and coronavirus. The viruses that cause the common cold were life threatening for him.
About a month after Angel’s admittance, Alfonso moved the rest of the family to Phoenix. Rachael stayed by Angel’s side for most of his time at PCH trading shifts with Alfonso and other adult family members when they were available. For the entire time Angel was in the hospital, due to age restrictions, his siblings were not allowed to visit.
Because no one in Angel’s family was a match for the bone marrow transplant, he was put on the donor registry and the search was on.
For several weeks Angel was treated with experimental medications in an effort to clear the viruses and prepare him for the transplant. During that time, Rachael recalled, “Angel started having problems with his liver and kidneys and developed high blood pressure.”
In November 2015, the team found a donor for Angel. In an effort to clear the adenovirus which was causing so much difficulty for him, Rachael signed consent for an experimental t-cell infusion. Angel was injected with donor t-cells that had been treated with adenovirus. Rachael shared, “There was only 1 in 10 chance it would work but it was our only choice or the adenovirus was going to continue to cause complications.”
Angel’s medical team and family hoped to see those treated t-cells multiplying rapidly but his daily blood draws showed that wasn’t happening. Thinking they might have depleted the t-cells with their frequent blood draws, the doctors gave Angel a second infusion. And then, he caught RSV. “We thought it was going to be a huge setback,” said Rachael, “but we caught it quick, and he cleared the RSV virus in one week! By the end of January 2016, Angel was infection free. They could not detect the rhino-, corona-, adeno- viruses or RSV.”
Angel was almost ready for his bone marrow transplant. The donor was ready and the procedure was scheduled for a few weeks out. But, before it could take place, Angel would need a week of chemotherapy.
“That’s when everything went south,” recalls Rachael. “Up until the chemo, Angel was happy and social and no one could believe he was so sick. He just didn’t look sick.” She continued, “Once he had chemo, he started to look like a sick baby. He developed Mucositis where his mucus linings broke out in sores. As a result, he stopped taking a bottle and stopped eating, He needed NJ [feeding] tube. He didn’t want to move. His development took a step back.”
Transplant day finally arrived on February 22, 2015. Angel was still feeling the effects of chemo but by day 14 after transplant, tests showed the chemo had wiped out his broken immune system and the transplanted donor cells were creating a new immune system for Angel. After 8 months in the hospital, Angel was able to go home.
These days, Angel crawls everywhere and has started taking some tentative steps. He still has a high risk of infection and is on a lot of medication, some of which he will need long-term, and has frequent doctor visits. But, now that he has a port, blood draws are less painful for him and medication delivery is a bit more efficient.
To regain some of the skills he lost after chemo and the transplant, Angel is receiving services through the Arizona Early Intervention Program. He has graduated from OT, receives speech therapy every other week and his physical therapists are helping him develop his walking skills.
For the most part, Angel stays inside but when they do go out, Rachael makes sure he wears a mask or his stroller is protected with a weatherproof cover to keep germs at bay. Angel’s sister knows to change her clothes as soon as she gets home from kindergarten to make sure she keeps those school germs away from her brother.
Family and friends know the rules when they come to visit. No shoes in the house; make sure to get a flu shot; stay away if you’re not feeling well or if you’ve been sick recently; and wash your hands--a lot.
Rachael is active in the community helping educate legislators about SCID and the benefits of including a test for SCID in Arizona’s newborn screening. “If Angel was diagnosed at 10 days old with the newborn screening, he most likely would not have caught the viruses. He would have had his bone marrow transplant much sooner than he did and the cost of Angel’s care would have been so much less.” Even so, she says, “He got so lucky having his transplant at 10 months old. Most kids [with SCID] have so many complications by that time, they have no ability to fight it off.”
During an Office of New Born Screening meeting, Rachael connected with Vickie French, the Assistant Executive Director for Raising Special Kids. Rachael remembered the hospital social worker providing her with information about Raising Special Kids. Rachael shared with Vickie that of all the organizations she was referred to, Raising Special Kids helped the most. Rachael explained, “I have found the best way for me to heal is by talking it out and the only people I can talk to are the people who have gone through this. It makes me realize I’m not alone.”