School nurses aid in care of children with primary immunodeficiency

For children with undiagnosed primary immunodeficiency (PI), the school nurse can serve as a gateway to the discovery of the condition, and for those students already diagnosed, the school nurse plays a critical role in their care.

To spread awareness of PI and inform school nurses of how important they are in the lives of children with PI, Dr. Beth Younger, a pediatric nurse practitioner, assistant professor of pediatrics at Johns Hopkins School of Medicine, and long-time member of the Immune Deficiency Foundation’s (IDF) Nurse Advisory Committee (NAC), visited the School Nurses Association of Carroll County in Maryland and provided a presentation on PI for their annual education meeting.

“They were riveted. I’ve never spoken to a group that was so excited and attentive. They were interested and engaged and had lots of questions,” said Younger, who spent the evening educating about two dozen nurses. “My goal was to get them thinking of PI. Everyone thinks it’s this rare diagnosis, and the reality is it’s out there, and if you look, you will find it.”

Younger took special care to define PI and explain the 10 warning signs of PI in children, as described by the Jeffery Modell Foundation (JMF), another advocacy organization dedicated to supporting persons with PI. Consider the child with chronic, recurrent, severe, and unusual infections who may also have autoimmune issues like inflammatory bowel disease, type 1 diabetes, rheumatoid arthritis, and autoimmune cytopenia, in which the immune system attacks healthy blood cells, Younger told the school nurses.

Younger pointed out that the school nurses won’t act as diagnosing clinicians but rather as a bridge to parents in starting the conversation about the possibility of PI.

“They wanted to know what to say to parents, and I told them just explain the red flags like continually missing school three times a month or kids with constant fever, runny nose, and a wicked cough. Things that are that simple. The longer you are in practice, the more gestalt for this you develop. You understand this is not quite normal, this is something outside the spectrum. Maybe something else is going on,” said Younger.

“Just talk to a mom and say, ‘Wow, this kid is sick a lot. Has anyone thought about why that might be?’ No one’s committing to a long-term conversation about it; just make them aware of the pattern and discuss the warning signs.”

Younger reviewed types of PI, including common variable immune deficiency (CVID), with the nurses. About 20% of patients with CVID are diagnosed under the age of 16, she told the school nurses, and they often develop sinus infections, have a predisposition for autoimmune diseases, and have a higher incidence of lymphoid cancers. She also touched on more rare PIs like severe combined immunodeficiency (SCID), chronic granulomatous disease (CGD), and Wiskott-Aldrich syndrome (WAS), as well as X-linked agammaglobulinemia (XLA), and complement deficiencies.

Younger reviewed treatments, described symptoms such as fever that could mean a medical emergency for a child, and discussed the implications of vaccines on children with antibody deficiencies (such as with CVID and SCID), complement deficiency or a missing spleen, and phagocytic dysfunction (such as with CGD).

“There are specific things to look out for with specific diseases. Make sure you know what the plan is and what they need to be aware of. Very frequently, I’ll write letters to principals and let them know that they have a child with a PI and describe the ramifications. Sometimes, it’s a sit-down meeting with the school, sometimes, it’s a letter, but we all have to be on the same page. Everyone needs to know what it looks like, and the school nurse is part of that conversation,” said Younger.

Younger advised the nurses that when caring for a child with PI, it’s imperative to:

• Never administer a vaccine or treatment without a parent or guardian’s permission.
• Understand the implications of the diagnosis, including precautions for the child and what concerns need immediate reporting.
• Establish a therapeutic relationship with the child and their parents or guardians.
• Determine a communication link with parents or guardians about issues like illnesses at school; know the best route of contact and who to contact.
• Discuss with parents or guardians if they want other students or parents to know about their child’s PI.
• Monitor environmental accommodation if needed.

Carroll County elementary school nurse Jeanne Patterson, mother to an 18-year-old son with PI who participated in an IDF Teen Escape, helped coordinate the presentation. School nurses handle everything from everyday boo-boos to chronic conditions for students, said Patterson. They participate in medical plans and accommodations and educate staff on classroom cleanliness and childhood illness. Patterson understands the role a school nurse serves in raising the index of suspicion for PI and in supporting kids in the school environment post-diagnosis.

“I know the nurses felt very informed about PI after the presentation, and it helped them think differently about children who are ill a lot,” said Patterson. “It also served as a good reminder to keep the lines of communication open with parents.”