Sarah\'s Condition it Is Often

Sarah's Condition

It is often said that obtaining an early diagnosis of SLE (systematic lupus erythematosus) is one of the most important yet difficult aspects of promoting a positive treatment outcome for children with this disorder. For many months, eleven-year-old Sarah has been manifesting joint pain, headaches, and mouth ulcers. Yet she was only diagnosed by her pediatrician with systemic lupus after manifesting the typical 'butterfly' rash. "In reality, fewer than one-third of children with systemic lupus erythematosus will have this facial rash when they first see a physician…Many times they had begun to do poorly in school, lost interest in their friends, and become withdrawn. In several cases, SLE was recognized only because the primary care physician was wise enough to do a complete work-up prior to the start of psychotherapy" (Lehman 2002).

Ironically, despite this unfortunate lack of awareness on the part of many physicians, compared to leukemia, cystic fibrosis or muscular dystrophy, SLE is a relatively common disorder: "About 25,000 children and adolescents in the United States have lupus or a related disorder" (Lupus, 2009, Arthritis foundation). But because the public does not know about the possibility of manifesting lupus, especially in young children, teachers, parents, and even physicians are often not alarmed by the obvious signs. Sarah's parents are understandably frustrated by what they likely see as incompetence on the part of the medical profession -- the reasons that lupus is often undiagnosed should be explained to them by the nurse in a way that does not seem to excuse the doctors for failing to treat Sarah before she manifested the rash. However, it is essential that Sarah and her parents regain faith in the medical profession. Sarah's parents, teachers, and treatment team must all be 'on the same page' in terms of setting realistic goals for Sarah's treatment and management of her condition so she can lead as normal a life as possible.

Sarah herself must gain a sense of control over her disorder, and the psychological difficulties of confronting a life with a chronic illness must be acknowledged by those around her as well as the physical complaints of lupus. The depression that a diagnosis can often cause is manifest in Sarah's comments, "oh it's just another one of my headaches. I probably have a brain tumor like my friend Kim." Children with SLE may often experience depression because of their severe fatigue and difficulties in school. A nurse must convey the importance of positive self-care to Sarah and the ability to manage her illness while being realistic. Sarah's slightly low blood pressure and high pulse indicates nervousness and anxiety as well as a manifestation of her condition. The nurse should also be willing and open to talk about how illness has affected other people in Sarah's life, like her friend Kim and make Sarah see the differences between her condition and Kim's.

Another reason for misdiagnosis of children with lupus is that the early symptoms of systemic lupus erythematosus (SLE) are often vague and can be attributed to a number of causes. They may include anemia, bruising, fatigue, fever, and weight loss. Systemic lupus "is extremely varied in the way it starts…all of these findings may be caused by many different diseases" (Lehman 2002). Because of its strong estrogenic component, it most often occurs in women during the height of their reproductive years (usually from early adolescence to about age forty-five) although it can also be manifested in young boys and men. The age of the child can cause physicians to discount the possibility: "Many times, primary care physicians who are told that a young patient has systemic lupus" will dismiss the likelihood of a child having the illness, saying: "that's a really rare disease. I haven't seen a case of SLE since medical school" (Lehman 2002). For example, Sarah is eleven and lupus in children occurs most often at the age of fifteen and older. This could also have contributed to initial confusion over her diagnosis.

The ACR (American College of Rheumatology) guidelines for a definite diagnosis of SLE "are often incorrectly used as a guide for when to consider the possibility of SLE," although its eleven criteria (including the butterfly rash) were "created to allow physicians to describe patients who most definitely did have SLE" (Lehman 2002). The fact that laboratory results on tests for lupus are often vague, from the point-of-view of the clinician, further complicates matters. There are certain laboratory criteria that may indicate lupus, the first of which is positive antiphospholipid antibody test. But because the test is so sensitive, it detects almost every case of SLE but many of the children with positive tests do not have SLE (Lehman 2002).

Other laboratory indications are general blood abnormalities or detections for an immunologic disorder, including lupus anticoagulant; positive anti-double-stranded DNA; false-positive syphilis test and a positive anti-Smith test (such as anticardiolipin). (Lehman 2002). Blood and urine tests are given to assess kidney functions. A complement test is given to measure the level of complement, a group of proteins in the blood that help destroy foreign substances but are present in low levels in individuals with lupus. A test of the patient's erythrocyte sedimentation rate can show when bloods proteins clump together and become heavier than normal. The faster the blood cells fall, the more severe the inflammation. AC-reactive protein (CRP) test is for a protein that is elevated when inflammation is found in the body (Lupus, 2009, Children's Hospital of Boston). "Although ESR and CRP reflect similar degrees of inflammation, sometimes one will be elevated when the other is not," and they are often used to test a patient's response to medication. X-rays may also be used to check for inflammation that manifests themselves in the bones and joints (Lupus, 2009, Children's Hospital of Boston).

Once they understand why diagnosis may have been difficulty, Sarah and her parents must understand that lupus is a serious condition and is chronic in nature. Lupus is typically "characterized by periodic episodes of inflammation of and damage to the joints, tendons, other connective tissues, and organs, including the heart, lungs, blood vessels, brain, kidneys, and skin. The heart, lungs, kidneys, and brain are the organs most affected. Lupus affects each individual differently and the effects of the illness range from mild to severe. Lupus can potentially be fatal" (Lupus, 2009, Children's Hospital of Boston). But many individuals with lupus live happy, healthy, and productive lives -- the novelist and short story writer Flannery O'Connor is one such example.

Lupus symptoms may come and, and the child may have good days and bad days. It is essential for Sarah and her parents to remember that a few good days do not mean that Sarah no longer has lupus. Part of Sarah's treatment plan must include ways of coping with more frequent absences -- tutors and an understanding school and teacher who are 'on board' with managing Sarah's illness are important. But regarding pharmaceutical treatment, "if the lupus symptoms are mild" medicated treatment may not be necessary, "other than possibly nonsteroidal anti-inflammatory medication SAIDs) for joint pain" (Lupus, 2009, Children's Hospital of Boston). Sarah currently takes this in the form of ibuprofen, in the form of 200mg by mouth every six hours as needed for pain. Sarah also takes a daily regime of 5mg of prednisone, a corticosteroid used to control inflammation that is also often prescribed for patients, in a does of 5mg by mouth each day. Immunosuppressive medication (to suppress the body's autoimmune system) may be used in more severe cases or during severe flare-ups of inflammation (Lupus, 2009, Children's Hospital of Boston).

Sarah and her parents must be reminded of the importance of sunscreen given that lupus sufferers are prone to develop rashes and are highly photosensitive (Lupus, 2009, Children's Hospital of Boston). To prevent outbreaks, stress reduction, a well-balanced diet and immediate treatment of infections is also advised (Lupus, 2009, Children's Hospital of Boston). Non-drug interventions are preferred, given that all of the aforementioned drugs do have side effects -- ibuprofen can cause digestive discomfort and steroids can cause lower bone density, a particular concern in a child with a small frame like Sarah's, during her critical bone-building years. Even the acetaminophen Sarah has been taking in the form of 320mg by mouth every four to six hours (alternating with ibuprofen) for pain can cause liver problems if taken in excess. The chronic nature of Sarah's condition and the likelihood she will need to take such medication for long periods of time also suggests that keeping dosages as low as possible and as minimal as possible to reduce building up a tolerance to the drug would be beneficial.