When I was first offered a position working with the special infectious disease (SID) team at Children's Memorial Hospital, I wondered if I had the experience necessary to work with children with Human Immunodeficiency Virus (HIV). I certainly did not learn about it during my bachelor's degree training at Indiana University or while completing my master's at the University of Iowa, and I had only a general idea about what HIV was and how it affected the body. I had imagined treating ill patients with demanding feeding and swallowing needs, and my family was concerned about the risk of transmission. Now, a year into my job, I enjoy the children and teens, appreciate the team dynamics, and understand there is essentially no risk of transmission for staff.
For some background, the SID team is led by Dr. Ram Yogev. The diverse staff comprises pediatricians specializing in infectious diseases, an adolescent medicine physician, fellows, pediatric nurse practitioners, nurses, a developmental psychologist, a dietitian, a child life specialist, social workers, education specialists, a speech-language pathologist, a phlebotomist, and a pharmacist. The clinic also is staffed with a van driver and many regular volunteers. Other affiliated staff members include a neurologist, translators, audiologists, and physical and occupational therapists (referred to as needed). Behind the scenes there are also many staff members that organize research protocols and study drugs, since many of our children are enrolled in national and local research projects. Currently, our team follows approximately 150 children and teens both at the main hospital and at a new teen clinic. The average age of this population has been getting older in recent years, thanks to the new medications and better understanding of the disease leading to longer life expectations. In fact, this year more than half our patients will be 12 years or older. We have approximately one patient death per year.
Populations and Treatment
There is a small but growing teen population with behaviorally acquired HIV. However, most of the population seen at Children's Memorial Hospital has vertically acquired HIV, which is transmitted from the mother to the child during pregnancy. When an HIV-positive mother is pregnant, contrary to popular belief, her child does not automatically become HIV-positive. Due to the blood barrier in utero, a baby typically becomes exposed to the virus only in the birth canal during delivery or from breast milk after birth. When a pregnant mother is identified as HIV positive, it is recommended that she receive medications that minimize her virus levels so that the baby will be exposed to less of the virus during delivery. She also will receive intravenous HIV medicine (AZT) during labor and delivery, and the baby is treated with AZT prophylactically for six weeks.
Babies are followed by the clinic until they are 18 months of age and have shed their mother's antibodies. At that time, children may transfer to a regular pediatrician or choose to stay with the primary nurse practitioner at the SID clinic for well-child follow-up visits. During the first 18 months children are referred to early intervention services if they appear to have developmental delays. Education is a large part of each team member's job. I currently follow 15 noninfected children who were born to HIV-positive mothers and have a variety of speech and language issues. Most of these children are seen for speech treatment at facilities throughout the Chicagoland area.
There are many controversial issues surrounding HIV and AIDS, and there has been considerable debate regarding the legal mandating of HIV testing for all pregnant mothers. Given our understanding of maternal-fetal transmission and effective measures for prevention, one can see how beneficial testing would be both for parents and their unborn babies. Mandatory testing could significantly decrease the number of HIV-positive children born in the states.
HIV attacks cells that help fight off germs in the immune system. In addition, it uses these cells like photocopiers to replicate itself in the body. In time, the high viral levels can inhibit the normal functions of the immune system. Anti-viral medications work in different ways to prevent viral replication. Modern drug regimens use combinations of highly active antiretroviral agents. Such regimens have prolonged survival among children with HIV infection with concomitant improvements in neurological status and quality of life.
Great variability is observed among children with HIV infection, based largely on age when infected, viral strain, stage of disease, and environmental stressors. The biologic effects of HIV infection can range from mild to devastating. They can occur during early infancy, continue into the preschool and school-age years, and may lead to global or selective delays in neurodevelopment. There are many strains of the HIV virus and people, once infected, can react differently. For example, we had a very sick 2-year-old who was diagnosed as HIV positive during an inpatient work up. Subsequently his parents and siblings also were diagnosed as HIV positive. Amazingly, both the untreated siblings (ages 7 and 9) had developed appropriately and shown no symptoms of the disease.
Even with the medical advances in managing HIV, the central nervous system remains a key target of HIV infection. Hence neuropsychological sequelae are frequently observed. Many studies have reported delays in the cognitive, language, and motor development of children with HIV infection, and the severity of the delay often correlates with the stage of disease. Delays also often occur in the presence of neuropathological findings, including cerebral atrophy and acquired microcephaly. Our children are followed on a regular basis for cognitive testing by a neurodevelopmental psychologist. Fortunately, the number of encephalopathic children seen at the clinic has significantly decreased in recent years to approximately 10 patients at present. Many of these children have motoric issues and require wheelchairs. Aside from the neurogenic causes of speech and language delays, many of these children also present with decreased breath support that affects their intelligibility and volume.
Investigations have shown that language functioning, especially expressive language skill development, is compromised by HIV disease. Delays in the development of expressive skills are often an early indicator of HIV's effect on the central nervous system, and these frequently precede abnormalities detected on brain CT scans. In our experience, about 60% of the children have impairments in one or more speech or language domains (greater than one standard deviation below the mean). Children with HIV infection also are reported to experience a higher than average incidence of upper respiratory and chronic middle-ear infections, which also may contribute to speech and language delays.
Given these issues, all newly identified HIV-positive children are assessed by an SLP to determine their baseline functioning. A typical evaluation includes parental report, a standardized testing protocol (typically the Preschool Language Scale-3 or Clinical Evaluation of Language Function-3), an oral mechanism exam, and articulation testing if needed. Speech treatment services are then recommended if necessary, and referrals are made to the schools or local clinics.
School-age children and teens up to 16 years of age are seen on an annual basis, with the younger preschool children having assessments every six months. Individuals aged 16-21 are evaluated or screened annually if they present with delays or deficiencies. Those that present with typical language are not re-evaluated unless there is a change in their medical status or behavior. Annual testing allows us to follow the language trends of these children, and any sudden decrease in skills is seen as a red flag signaling possible changes in medical status.
Children also are seen by the SLP for any feeding or swallowing issues, and this is done in close collaboration with the pediatric nurse practitioner and the dietitian. Feeding issues are largely related to volume of intake, since medications can affect weight gain or weight loss. Some of our younger patients also require gastrostomy tubes for nutritional supplements and medicines.
It must be taken into consideration that exposure to HIV often occurs in the context of environmental factors, such as poverty and substance abuse. These issues pose additional risks to normal child development. Children may be living in homes with limited resources and with parents who are struggling with their own health. Many of the families of children who are HIV-positive have incomes below the national poverty level, so a variety of social services are provided to the families by the SID clinic and other area organizations. The wide range of services might include providing bus passes, negotiating with a utilities company, or delivering medications to homes on a monthly basis. In one extreme case, funding was found to provide a furnished house for a homeless family.
Adherence to medication is probably the primary issue with many of our families. If medications are not administered regularly, individuals can build up resistance to them, which leaves fewer options for long-term medical management. For this reason the doctors would prefer the child to take no medicines rather than taking their medicines only intermittently.
For various reasons families sometimes have difficulty making medicines a priority. It should be noted that the medicines do not taste good and some of the pills are quite large. In addition, most children have regimens that require them to take six pills twice a day. Thus it can often be a battle for caregivers to get their children to take their medicines. One family told us that they have found a pile of pills hidden in their child's room.
Teenagers also have a hard time with adherence and might, for example, not take the blue pill because they don't like how it makes them feel. When I first saw the size and quantity of some of these pill regimens I could understand the reasons for poor adherence-and why they are called "pill burdens." Recently, one of our teens counseled younger patients at camp about the importance of medicines, yet he is himself one of our least adherent teens. I truly believe that many of the teens want to take their medicines and understand in theory why they should, but they have a hard time with the reality of it.
Children who have HIV are receiving treatment from SLPs all over the country for speech and language delays while their diagnoses remain unknown to the schools (approximately 50% of our children receive some school-based services). Given the lingering stigma over HIV, schools might best serve these children when they are not forced to confront biases and questionable beliefs about the disease. Our team has educators that are sent to schools when a disclosure is made either by the family or by the child. The educators go into the schools and try to dispel some of the myths that might affect the treatment of the child. If an SLP is taking universal precautions, as they should for all children, there is no risk of transmitting HIV during a session.
Continuing Need for Education
Recently, a speech-language pathology student attended our clinic. We read academic journal articles and informative newspaper articles, and discussed HIV at length. Toward the end of eight weeks we were discussing one of our adult patients who was getting married and talking about having a family. The student said, "but she's HIV-positive. She can't have kids." Once again I explained issues regarding mother-to-child transmission. Then she said, "but you can't hug and kiss." Wrong again. There are still so many deeply held incorrect beliefs about HIV. To be a good mentor, nothing should seem too simple to take for granted. You cannot get HIV by kissing, hugging, touching, or from toilet seats. HIV is transmitted by semen, vaginal secretions, breast milk, or blood-to-blood contact.
I really enjoy my job. I enjoy working with such a variety of ages-babies through adolescents-and there are times when the clinic sees both the baby and the teen mother. The children and teens we serve at the clinic do not appear as sad sick patients with visible signs of a chronic illness. They play, they go to school, they date, and they are as busy as their peers. HIV is not an acute disease for the majority of our families.
There are, of course, some frustrations. It can often be difficult to get families to stay for their language assessment because it is not a high priority in their overburdened lives. Most families are trying to get in and out of the clinic in as little time as possible. Speech evaluations also are put off if there are bigger psychosocial issues to deal with. The most positive thing about my position is being involved in the overall care of a child as part of a team, where you regularly hear about the child's needs-including dietary, psychological, social, and medical issues-and are able to follow the child on a long-term basis witnessing milestones as they are achieved.