Lupus in Teenagers - - How They Cope and Accept It

Lupus in Teenagers - - How They Cope and Accept It
This presentation was given by Roger Hollister, M.D.
of the National Jewish Center for Immunology, Denver, Colorado.
Our thanks to the Lupus Foundation of Colorado

I was asked to speak about Lupus today and I would like to tell you something about the differences that occur in the very young child with lupus. Actually, I will spend the beter portion of this talk discussing some of the problems that teenagers have with lupus, which is, in fact, the most common age that children begin to have lupus.
I am not going to spend very much time speaking about the source of syptoms developed by offspring of mothers who have lupus. These children can have temporary symptoms such as rash or anemia which are due to passive transfer of the antibody across the placenta; these features in the offspring only occur for a short period of time as the antibody runs its natural "half" life and then the children are well thereafter.
The second syndrome that can occur in the offspring of lupus women is congenital heart block. This is an interference with a normal conduction mechanism that occurs in the heart between the various chambers: the electrical conduction is disturbed in these babies and as a result they run very low pulse rates, between 30-40 beats per minute which is insufficient to sustain them. These babies have pacemakers put in and afterwards do very well. We don't know yet at what exact frequency that syndrome occurs in the offspring of lupus women. The way the studies have been conducted up to now, the index cases have been identified in the pediatric cardiology clinic so that the children had to have the heart block to be known about. Nobody has done a prospective study of a large number of lupus women to see exactly what the risk is. the association is clear, but it is not yet possible to predict the probability of occurrence.
Are Lupus Symptoms Different In Children? Is Management of Lupus In Children Different? Is The Outcome The Same?
Here at the National Jewish Center for Immunology, our youngest patient actually began to have lupus when he was only two months old; lupus was diagnosed in the newborn nursery. He was born prematurely, had a tremendous number of complications because of his very low birth weight and while still in the nursery, he developed kidney symptoms, low platelet counts, and other things that announced that he had lupus. He is now two years old, and actually, as fat as the lupus is concerned, is doing extremely well.
The notion the years ago was that lupus was fatal in children and perhaps more fatal than it was in adults who acquired this disease. In fact, this is not the case. The outcome is now very much the same and equally optimistic as is the situation for adults. Most theories indicate an 85-90% survivorship at 10 years of follow-up. That 10-yeat peroid is only as long as the studies have existed. It doesn't hive an end point and those may go on for many, many more years as the data becomes avaible. I will show you shortly that our experiences here in the Rocky Mountain area have been much the same.
There are some clinical differences as far as children with lupus are concerned; there is apt to be more enlargement of the liver and spleen and the lymph nodes. All these organs in children in genreal are more reactive than they are in adults. This often has to do with the reponse of the human body to outside stimuli such as viruses, allergens, etc., and children's organs tend to enlarge more easily but then diminish again in size. So, perhaps the difference in the disease itself. There can be more of a hereditary componet.
There is a small number of patients who have lupus as the result of a congenital complement deficiency. Complement is one of the proteins that is contained in people's blood, which is very critical to the body's defense mechanism; the way one kills viruses or gets rid of bacteria is perhaps the way one loses unwanted tumor cells within the body. This in part depends on this protein sequence known as complement. About 15 years ago it was first recognized that patients who looked as if they had lupus actually had the complete absence of one of these protein components. When the disease is more active, the component levels are depressed. The thing that was so striking about the children was that complement levels were not only depressed, they were totally absent. The way this manifested in the patients was very much like adult lupus in terms of rash, chest pain, kidney involvement, and all the things that we associate with lupus. The gene carrying the deficiency must be received in a double dose by the child in order to express it, so children who only have one of these defective gens do not express lupus; it is only the children who have gotten a double dose.
Side Effects of Therapy
Perhaps the most striking critical differences are some of the side effects of therapy in the growing patient. In general, our therapy on children is much as it is for adults with lupus. We try to avoid steroid medication if possible; but it needs to be given if there is major organ involvement, no question about it. If there is kidney disease, neurological or lung disease, something that is really over the long term harmful to children or adults, then steroids have to be used. If the symptoms relate more to skin rash or to arthritis, onecan frequently use other medicines known as non-steroidals and adequately manage the disease and get the patient functioning well without having to have the major side effects that steroids produce.
Diet is just a sensible sort of thing; there is nothing magical about it. We have all had lectures in it in the past from dieticians. As far as lupus is concerned, there is not any evidence that either a dietary deficiency or dietary excess has anything to do with the causation of lupus. For that reason, diet as a therapy is not helpful. In specialized circumtances (if the patient has kidney disease or high blood pressure associated with lupus0, they should diet to modify or decrease the salt intake, but otherwise, a well-balanced diet with a easonable amount or protein is all that is really neccessary.
The incidence of photosensitive rashes in children is very similar to adults; that is, only about 1/3 of cases with lupus actually have a reaction to ultraviolet light, which can either produce a rash or actually share their whole disease. One of the first things anybody hears os that everyone is photosensitive and you have to stay in the house and wear large-brim hats and essentially just situnder a rock for the rest of your life. This is not the case for 2/3 of people because they don't have a photosensitive rash! With the modern sunscreens that can completely block out the ultraviolet spectrum that is responible for this, most people can expect to live abnormal existence as far as going out, ect. Sunbathing by the pool may not be a terrific idea, but otherwise it shouldn't modify the lifestyle very much. Kids can go ahead with their plans for soccer or cheerleading or whatever else they plan to do.
Prednisone, in very ,very low doses--as low as 3 mg. a day--will totally inhibit growth. What this means is if you are on steroids for long periods of time, not only do you not grow, but you have lost that height irrevocably. This doesn't make much difference if you take Prednisone for four or six months, but if it goes on for five years, the patient will always be below what would have been the predicted biologic height. What we would consider a more successful use if steroids is a patient who had lupus and despite having a short period where growth was compromised because of daily steroids, the patient would be able to be managed thereafter on every-other-day steroids. If you can take less than 40 mg. of Prednisone every other day, growth is not inhibited. So what we ttry to do with kids who have lupus, which requires steroids, is to get the disease under control as quickly as possible and then switch them over to every-other-day steroids, so that this main side effect is irreversible in terms of long-term height can be avoided.
Achieving Independence
The other growth that is equally important as far as children are concerned is emotional growth. Lupus is a disease that tends to sponsor dependency because of its chronicity and its needs for medication, regular doctor visits, and alterations of some performance of lifestyle. One of the things that is most important for children to achieve in the formative stages is a sense of independence so that they can get to the point in the teenage years of being able to separate from their parents and go on to have their own careers and lifestyle. We have to be constantly aware of this. Children need to have this sort of emotional growth even though other factors about their illness tend to sponsor dependency. One of the reasons for this os that the individuals around the child with lupus seek to protect him from the outside wprld, from undue stresses, from embarrassment, from a wide variety of things. From the humane point of view, this makes some sense for the child who is acutly ill with lupus. In almost all situations, we can get them through that ill period to the point that they are felling quite well and then they should be allowed to return to the age-appropriate kinds of activities to be able to come out of the isolation that existed during the early months of this and to diminish the protective barriers the parents, friends, and teachers built around the child, and to let the child enjoy the normal kinds of emotional growth.
Denial is another mechanism that kids may use that is similar to that experienced by adults. Children may deny what their illness really represents and what the need for treatment may be, particularly when the disease goes into remission and the patient may be off steroids or other drugs that are used to treat it or the side effects. It is easy to understand that kids would like to believe that the disease ever existed at all. What we try to do is to point out to then that they should lead a normal lifestyle when the disease is totally inactive or in remission, and that should the symptoms recur, patientes should not deny its existence, and they should get back on therapy sooner than it tool to diagnose the disease in the first place.
Depression
The last emotional problem that we see not uncommonly in children is depression. A lot of it stems from anger, it turns inward, and kids have got as many reasons as adults do for being angry about having a disease. I don't have a clear answer for kids anymore than I do for adults as to "why me?" As they go through the early stages of understanding what lupus is about, and understanding the treatments that will be used, it is not uncoomon at all for them to slow down, to feel sad and depressed. We have spent a lot of time trying to get then through that early tough period which is often complicated enormously as they need large-dose steroid therapy to revert their kidney disease or some other major organ involvement.
Survey of Adolescents
About a year and a half ago, I surveyed the adolescents that we take care of, to try to see exactly what their view of having lupus was as a way of helping me to understanding the kinds of things that they were going through and how to help them. They were asked to list their views of the past as far as lupus was concerned, to reflect on how they currently view their situation, and also to give an estimate of what they thought they were going to do in the future now that they have lupus. their overwhelming criticism of myself or those around them about the past was that they needed more information; they did not feel that they had been told enough about the illness; in particular, they had not been warned enough about the drug side effects. Although they were told that they would likely need to have a kidney biopsy if their urinalysis showed activity in that organ, they were not told at the beginning that they would probably need a second or third biopsy at some time later to understand whether the treatment had been successful. They were angry that they had not been given that information. ALmost all of them related a feeling of isolation at being different, as they looked back on their initial reaction to having lupus. All of the patients got through that period, so that their current view of the situation was that they were not that different and they no longer has that sense of isolation that existed when the disease was first diagnosed. To most of them, taking medication was like the sunrise: there wasn't any big deal about it. they got used to doing it even though you might expect a teenager in particular would be the least compliant. teenagers do keep up with their medications very well.
After the initial feeling of isolation, the teens discovered that lupus does not interfere with relationship; unless it is necessary, they don't tell very many people about their lupus or what it is like, and what kinds of thing they are putting uo with. They share it with closest friends first, and they discover that this does not break up a friendship or mean a real difference in the relationship. That may take two or three months to sort of iron out, mostly on their part rather than on the friend's part, because other kids are remarkably accepting and compassionate about illness in another child.
Some of the respondents to the survey said that schoolwork was something that kept them going at a time when they were becoming "Cushingoid" from the steroids and they were going to the doctor because they weren't feeling too hot about themselves or how they looked. The patients who were in remission barely thought about lupus anymore and, unless the disease flared or came back into existence, it was not something that was constantly on their minds. They went about the other things that you would expect adolescents to be concerned with. Lupus did not hang heavy on their thoughts.
Concerns About the Future
Even considering the hatred of steroids as a treatment, as far as their view of the future was concerned, very few of the respondents thought that it was going to alter their career choice. After being treated for lupus for an average of six years, patients discovered that it did not modify most of what they wanted to do or what each was able to do in day-to-day life. Thoughts about going to college or what kinds of job opportunities they were ultimately going to see were not being modified very greatly by having lupus.
You are aware of the fatigue component of lupus and teenagers experience this, too. Interestingly, teens can separate the fatigue that lupus produces from the fatigue that almost all adolescents share. They can tell you that the adolescent fatigue is something that makes them want to sleep all the time unless a friend calls up and suggests some activity and then suddenly they are not tired anymore. Lupus fatigue is different because even though the friend calls, you still can't get up the energy to go ahead and do it and I was quite amazed that the adolescents had that much insight into themselves as to the difference.
Teens with lupus do have concerns about parenting, and this is something that we deal with from the time that children really reach a child-bearing age of when the have learned sex education in school or from parents; they want to know what the risk is to their offspring and we tell them about the sorts of things that I referred to at the beginning of the talk: the risk of offspring having lupus is actually very small. We have one family where the mother and daughter have lupus; we have one family with siblings who have lupus; but otherwise, it really doesn't run in families, and they don't need to fear the fact that their offspring might have it in terms of deciding whether to have children or not. The risk to their offspring is probably higher than it is in the normal population. They are concerned about the medications they are taking as to whether this will affect their fertility or whether it will cause congenital defects. Happily, there are very rarely such difficulties, regardless of what organ system is involved and what kinds of medications the patient is taking. the tenency of the medications to produce birth defects is actually very low. They are concerned as to whether they will ever be able to bear children; patients with lupus are equally fertile and the biggest problem is carrying the pregnancy to its completion. But with modern medical management, the patients are willing to wait until their disease is under reasonably good control before becoming pregnant and then the outcome is usually very good.
There are two groups of side effects and varying opinions about which group of side effects warrants more concern. Doctors tend to be concerned about elevated blood pressure and diabetes in some patients who are prone to develop that; osteoporosis, which is thinning of bone; steroid myopathy, which effects the muscles; and from a medical point of view, these can lead to more complications. teenagers are concerned about other side effects; the Cushingoid appearance caused by high doses of steroids; the increase in activity of acne; hirsutism. which is excess body hair; the growth failure we have already talked about, changed menstrual cycles; and striae, the medical term for strech marks. Teens need to be told that if they are prone to one or more of these side effects, unfortunately there is nothing that can be done to prevent these from happening. Once the strech marks are there, neither we rheumatologists nor dermatologists have any good way of reversing them. In some young women, this is perhaps a major debilitating long-term side effect. If one is put on every-other-day steroids, or if steroids are discontinued completely, all of these side effects are essentially reversible except for the striae, which remain thereafter.
Stress and Lupus
There have been a number of publications that have tried to not exactly write about what stress has to do with illness, but rather to imply that major stress can be so significant as to produce this disease. This is a hang-up that we got into in the 1940s with a number of rheumatic diseases. We overinterpreted them as being psychosomatic where the notion was that the emotiona; factors were primarily responsible for the expression of disease. Now, we have come a long way since then in interpreting the information and realizing that many of the early studies that identified differences in personality in patients with lupus or arthritis were the result of the disease and not the cause of the disease. But still, as recently as five or six years ago, a paper form the Rochester group suggested that stress factors, particularly in children with arthritis as opposed to lupus patients, were responsible for the onset of the disease was related to inducing a major flare.
As a result of the Rochester report, I thought it would be worthwhile to look at our young lupus patients in terms of trying to understand one definable stress event, mainly divorce as far as expression of the disease is concerned. Of the 30 patients that I reviewed, only 4 had parents who had divorced before the disease onset. The national divorce rate is four out of ten, and as you are probably aware, Denver does even worse than that and comes in at a rate of about one out of two. So clearly, the divorce rate amongst the parents of children with lupus was not even as high as the national rate; it was difficult to implicate that kind of stress in expression of the disease. Likewise, in the 10 years that we have been caring for these children, the disease itself has not been responsible for divorce within the family. there is no denying the fact that the care of a child with a chronic illness is a big stress and changes a lot about the parents' lifestyle and how they help their children. But if careful attention by physicians and friends and others around them is given, there is not any reason that the existence of a chronic disease in children should lead to a breakup of the marriage.
And so, those are the salient features of children and lupus that I wanted to cover with you. Medically, it doesn't look very different than lupus in adults. Outcome-wise, it is not very different and is quite optimistic. treatment-wise, it is different becuase of the potential of steroids to inhibit growth and becasue children are in an evolving stage of emotional development, which needs to ve taken into consideration so that they can wind up being the kind of adults that we would all wish then to be.