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 better 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 symptoms developed by the 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. As a result, they run very low pulse rates, between 30-40 beats per minute, which are insufficient to sustain them. These babies have pacemakers put in and afterwards do very well. We don't know yet what the exact frequency of that syndrome is 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 identified, and nobody has done a prospective study of the large cohort 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.
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 better 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 symptoms developed by the 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. As a result, they run very low pulse rates, between 30-40 beats per minute, which are insufficient to sustain them. These babies have pacemakers put in and afterwards do very well. We don't know yet what the exact frequency of that syndrome is 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 identified, and nobody has done a prospective study of the large cohort 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.

So, having discussed those two things, we will talk more about the difficult lupus symptoms that you all are familiar with as an adult, how they might be different in children, and also how the children's emotional reaction to the subject of lupus might be different because of their age. Our youngest patient actually began to have lupus when he was only two months old. This is a little boy from Craig, Colorado, whose lupus was diagnosed in the newborn nursery. He was born prematurely and had a tremendous number of complications because of his very low birth weight. While still in the nursery, he developed kidney symptoms, low platelet counts and other things that announced the fact that he had lupus. He is now two years old. Actually, as far as the lupus in concerned, he is doing extremely well.

Now, these are some of the subjects we will cover: How does lupus differ in children in terms of the symptoms? Is the outcome the same? Is the management in children different?

The notion ten 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 now is very much the same and is as equally optimistic as is the situation for adults. So most theories indicate an 85-90% survivorship at 10 years of follow up. That 10-year period only indicates how long the studies have existed. It doesn't give an end point. Those participants may go on for many, many more years as the data becomes available. I will show you shortly that our experience here in the Rocky Mountain area has 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, spleen and lymph nodes. All these organs in children in general are more reactive than they are in adults. They often have to do with the response of the human body to outside stimuli to viruses, allergens, etc., and children's organs tend to enlarge more easily but then diminish again in size. So, perhaps the difference has more to do with biologic features of childhood than it does with any difference in the disease itself.

There can be more of a hereditary component in children with lupus. There are a small number of patients who have lupus as the result of congenital 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 lyses unwanted tumor cells within the body. This, in part, depends on the protein sequence that is known as complement. About 15 years ago it was first recognized that patients who looked as though they had lupus actually lacked these protein components. You may all recognize that complement, in general, are lab tests that are made frequently on lupus patients because they correlate with disease activity. When the disease is more active, the complement levels are depressed. The thing that was so striking about these children was that levels were not only depressed, they were totally absent. The way this lack of compliment manifested in the patients was very much like lupus in terms of rash, chest pain, kidney involvement and all the things that we know to associate with lupus. The gene carrying this deficiency must be received in a double dose by the child in order to express it. Where the heterozygotes were the only ones that had the defective gene in this regard, they do not express lupus. Only the ones who had gotten a double dose expressed lupus. This has been well worked out from the genetic point of view.

Perhaps the most striking critical differences are some of the side effects of therapy in the growing patient which are different than when you treat lupus in adults. In general, our therapy for 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, neurological or lung disease, something that is harmful to children or adults over the long term then steroids have to be used. If the symptoms relate more to skin rash or to arthritis, one can frequently use other medicines, known as non-steroidals, which can adequately manage the disease and can get the patient functioning, without the major side effects that steroids produce.

Diet for children with lupus is just a sensible sort of thing. There is nothing magical about it. We have all heard lectures on it from dietitians. As far as lupus is concerned, there is not any evidence that either a dietary deficiency or a dietary excess has anything to do with the causation of lupus. For that reason, as a therapy, diet is not helpful. In specialized circumstances, the patient has kidney disease or high blood pressure associated with their lupus,they should diet to modify or decrease their salt intake. But, otherwise, a well-balanced diet with a reasonable amount of protein is all that is really necessary.

I won't spend much time on sunscreens since you have heard from dermatologists who are far more expert than I am about the variety of sunscreens that are available. The incidence of photosensitive rashes in children is very similar to adults. That is, only about one-third of the cases with lupus actually have a reaction to ultraviolet light which can either produce a rash or actually flare their whole disease. One of the first things people hear about lupus is that everyone is photosensitive and that you have to stay in the house and wear large-rim hats and essentially sit under a rock for the rest of your life. But this is not the case for two-thirds of the people. In the first place, many people don't have a photosensitive rash. And, in the second place, with the modern sunscreens that can completely block out the ultraviolet spectrum that is responsible for this, most people can expect to live a normal existence as far as going out is concerned. Sunbathing by the pool may still not be a terrific idea, but otherwise it shouldn't modify the lifestyle very much. For children, they can go ahead with their plans for soccer, cheerleading or whatever else they plan to do.

The last point is the most important one. This is where the real difference in children can be found. 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 4 or 6 months. You can't tell as an adult that there is a difference in height. But after 5 years, the patient will always be below what would have been the predicted biologic height. What we would consider a more successful use of steroids is when the patient would be able to be managed on every-other-day steroids after a short period where a patient's growth was compromised because of daily steroids. For, if one can take less than 40 mg of prednisone every other day, then growth is not inhibited. So this is what we try to do with kids who have lupus which requires steroids. We try to get the disease under control as quickly as possible and then switch them over to every-other-day steroids, so that this main, irreversible side effect of long-term height can be avoided.

The other growth area that is equally important, as far as children are concerned, is emotional growth. Lupus is a disease because of its chronicity, its needs for medication and regular doctor visits, and its alterations of some performance of lifestyle. This is a disease that tends to sponsor dependency. As children grow up, one of the things that is most important for them to achieve in the formative stages is a sense of independence so that they can get to the point in their teenage years of being able to separate from their parents and go on and have their own careers and own lifestyles. This has to be constantly kept aware of: children need to have this normal emotional growth even though other factors about their illness are tending to sponsor dependency. Dependency is sponsored when the individuals around children with lupus seek to protect them from the outside world, undue stresses, embarrassment, or a wide variety of things. From a humane point of view, this makes some sense for the child who is acutely ill with lupus. However, in almost all situations, we can get them through that ill period to the point where they are feeling quite well. Then they should be allowed to return to age-appropriate kinds of activities to be able to come out of the isolation that existed during the early months of this disease. The protective barriers that parents, friends and teachers built around them should diminish and let them enjoy normal kinds of emotional growth. So that covers A and B: A.) "How we try to help people understand dependency," and B.) "What is legitimate protection and what may, in fact, be excessive or over protection?"
Denial is another mechanism that children may use similar to adults for that matter, as far as 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 children would like to believe that the disease never existed at all. What we try to do is to point out to them that they should lead a normal lifestyle when the disease is totally inactive or in remission; that, should the symptoms recur, they not deny its existence; and that they get back on therapy sooner than it took to diagnose the disease in the first place.

The last emotional problem that we see not uncommonly in children is depression. My own view of depression is that a lot of it stems from anger that turns inward. Children have as many reasons as adults have for being angry that they have the disease. I don't have a clear answer for children 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 uncommon at all for them to slow down, feel sad and depressed. We have spent a lot of time trying to get them through that early tough period, which is often complicated enormously since they need large-dose steroid therapy to revert their kidney disease or some other major organ involvement. Cosmetic deformities, associated with steroids, compounds the adolescent problem as far as self-esteem and self-image are concerned. These contribute to depression also. We have been able to successfully get a young person through these early feelings of isolation and depression, although I wish I knew some mechanism where we could prevent it from happening in the first place.

Now, our own experience at National Jewish, with patients in the Rocky Mountain area whose onset of lupus was less than 16 years of age, has been much the same as I related to you about the experience nationally and with adults. Our experience now is up to 45 patients. Of those 45, we have only had two patients that died. In terms of kidney disease, only one patient has reached the point of needing renal dialysis. Many of the patients' kidney disease is so inactive that they are off steroids. So the outlook is a lot better than was felt to be the case 10 or 20 years ago. This, I am sure, has been communicated to you all numerous times. The first thing that people used to find when they picked up an encyclopedia was that lupus was a fatal disease; that surely is not the case anymore. Most people should be able to expect treatment that results in a largely normal lifestyle.

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 understand 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. They were asked 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 me or of 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 to have a kidney biopsy. If their urinalysis showed activity in that organ, they were not told at the beginning that this would probably need a second or third biopsy at sometime in the future, years later, to understand whether the treatment had been successful. They were angry that they had not been given that information.

Almost all of the respondents related a feeling of isolation at being different whenever they looked back on their initial reaction to having lupus, some of the same feelings that I described to you before. All of the patients got through that period. So that their current view of the situation was that they were not that different; they no longer had 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 and you might expect a teenager, in particular, would be the least compliant (in terms of otherwise being flaky and not remembering to tie his shoes or other things). They do keep up with their medications very well. After that initial feeling of isolation, they discovered that lupus does not interfere with relationships. If they don't have to, they don't tell very many people about their lupus or what it is like and what kinds of things they are putting up 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 iron out, mostly on their part rather than on the friends' part, because other kids are remarkably accepting and compassionate about illness in another child. It was once thought that children could experience mean periods of teasing and abuse from one another. When it comes to chronic illness, that is really very seldom the case.

Some of the respondents 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; being able to succeed in school was something that was a strong point for them. The patients who were in remission barely thought about lupus anymore. 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.

And lastly and universally, with the hatred of steroids as a treatment, as far as their view of the future was concerned, very few adolescents thought it was going to alter their career choice. After being treated for their lupus, as I said, for an average of around 6 years, they discovered that it did not modify most of what they wanted to do or were able to do in their day-to-day life and their thoughts about going to college or what job opportunity they were ultimately going to see wasn't being modified very greatly by having lupus.

You are aware of the fatigue component of lupus and teenagers experience this, too. Interestingly, they can separate the fatigue that lupus produces from the fatigue that almost all adolescents share. They can tell you that 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. I was quite amazed that adolescents had that much insight into themselves as to the difference.

Adolescents have concerns about parenting. This is something that we deal with from the time that children reach a child-bearing age or when they have learned sex education in school or from parents. They want to know what the risk is to their offspring. We tell them about the sorts of things that I referred to earlier: the risk of their offspring having lupus is actually very small. We have one family with siblings who have lupus, but otherwise it really doesn't run in families. 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 that they are taking, as to whether this will affect their fertility or whether it will cause congenital defects. Happily that is rarely the case, regardless of what organ system is involved and what kinds of medications they are taking. The tendency of the medications to produce birth defects is actually very low. They are concerned about fertility, as to whether they will ever be able to bear children. Patients with lupus are equally fertile. The biggest problem is carrying the pregnancy to its completion. But now, 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.

I summarized adolescents' hatred of steroids and compared it to what physicians think about steroids. There are two groups of side effects, if one looks at steroids from two different perspectives. Doctors tend to be concerned about elevated blood pressure, diabetes (in some patients who are prone to develop it), osteoporosis (which is thinning of bone), and steroid myopathy (which is a prednisone effect on muscles). From a medical point of view, these side effects can lead to more complications and that is what doctors are concerned about.

Teenagers are concerned that the Cushingoid appearance that high-dose steroids can cause can also increase the activity of acne, can produce hirsutism (which is body hair in excess) and growth failure.

Steroids may change the menstrual cycle, and sometimes that is hard to sort out because very active lupus can also change the menstrual cycle. Lastly, adolescents are concerned about striae, which is the medical term for stretch marks. If they are a person who is prone to this kind of side effect, they need to be told, unfortunately, that there is nothing that can be done to prevent it. Once the stretch marks are there, neither we rheumatologists nor dermatologists have any good way of reversing that. In some young women, this is perhaps a major debilitating long-term side effect. If they get on every-other-day steroids or if they get off steroids completely, all of these effects are essentially reversible, but the striae remain as a stigma thereafter.

In closing, there have been a number of publications that have tried to imply that major stress can be so significant as to produce this disease. This is a hang up that we have gotten into in the 1940s with a number of rheumatic diseases in over interpreting them and being psychosomatic where the notion was that the emotional factors were primarily responsible for the expression of disease. Now, we have come a long way since then in interpreting the information and in realizing that many of the early studies saw differences in personality in patients with lupus or arthritis which were the result of the disease and not the cause of the disease. But still, as recently as 5 or 6 years ago, a paper from 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. They implied that the onset of disease was induced by the divorce rate in the parents or that a subsequent divorce induced a major flare. And so I thought it would be worthwhile to look at our young people with lupus in terms of trying to understand one definable stress event, mainly divorce, as far as expression of disease is concerned. Of the 30 patients that I reviewed, only 4 had parents who had divorced before the disease's onset, so that is 4 out of 30. The national divorce rate is 4 out of 10, and as you are probably aware, Denver does even worse than that and comes in at a rate of about 1 out of 2. So clearly, the divorce rate among 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 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 their 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. Medically, it doesn't look very different than lupus in adults. Outcome-wise it is not very different and quite optimistic. Treatment-wise it is different because of steroid's potential to inhibit growth and because children are in an evolving stage of emotional development, which needs to be taken into consideration on an ongoing basis so that they can wind up being the kind of adults that we would all wish them to be.

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