Living with lipodystrophy
Published Thursday, 17-Feb-2005 in issue 895
Adrianna Arce works in one of the San Diego clinics that specialize in the treatment of HIV/AIDS. She is a psychotherapist and sees regularly roughly 40 patients a week. She is well aware of one of the more insidious dangers of the disease: the ways in which HIV changes the appearance of those infected by it.
“It has always been one of the main characteristics of HIV,” she observes. “It’s the way in which it marks its victims. I’m sure most of us can remember the old days, before HAART [Highly Active Anti-Retroviral Therapy] therapy, when HIV was tantamount to a death sentence. The marking of the victim was much more extreme in those days, too. Karposi Sarcoma, which appear as purplish skin lesions, were more common, and of course, there was the wasting. No one could hide it. People wasted away to living skeletons; their faces were death’s heads. These deformations were frequently accompanied by virulent outbreaks of herpes simplex in the mouth and on the lips, thrush and rosea. It was like something out a medieval dance of death. The appearance was very macabre, and I’m sure it added a great deal to the popular terror of AIDS at the time. It played into the common perception of people being marked for death. It was so obvious, and I’m sure it also played into the religious idea of people being marked by their ‘sins.’
“At that time – I’m talking about the ‘80s – deviation from the social sexual norm was a taboo topic for the majority of people. I knew gays at the time who claimed to be perfectly at ease about their sexual orientation and seemed to be living an openly-gay lifestyle; this was while they claimed they just preferred not to discuss it in public. They said it was no one’s business, but suddenly there was no hiding it anymore. There was no mistaking the external symptoms. Everyone knew what the disease was, and how it was acquired.
“I remember attending a lecture by Dr. Kuebler-Ross; she’s famous for having formulated the seven stages of grieving when a loved one dies. She was also one of the first doctors to be engaged in the medical fight against AIDS. She said in this lecture that one of the great terrors of AIDS, the reason so many people feared it, was that everything became obvious. Adultery became obvious, sexual preference became obvious, child abuse became obvious. All the secrets people were most afraid to reveal came out.
“Anti-retroviral therapy has changed that to some degree. I mean, obviously, those infected are no longer dying in the same numbers. I was practicing in San Francisco in the ‘80s, and I can remember too well the days [when] it seemed you couldn’t go on the street without seeing men who were simply wasting away. Still, a serious consequence of HIV remains the way it changes the appearance. I have several patients with facial wasting: the prominent cheek bones with no fat to plump out the appearance of the face. Patients complain about the thinning in their arms and legs, or that the veins in the legs become more prominent. On the one hand, these changes can be more easily hidden just by covering them with clothing, by wearing long-sleeved shirts, but that’s far from an ideal solution. Some patients complain that they are no longer able to take the sun at the beach. They don’t want to expose their arms and legs, but they don’t like being pasty pale either. There is, of course, the problem of intimacy. They’re very uncomfortable with exposing their bodies to a lover, or even appearing in a group if they are not completely covered. It is a source of stress in their lives. It limits their freedom of movement and their spontaneity.
“Fortunately, I haven’t seen any patients with a noticeable buffalo hump. My female clients usually complain of a weight gain or difficulty in controlling their weight. I haven’t noticed facial wasting among my female clients, or, thank God, very often among my younger clients. It seems to be a problem limited to my male clients in the late 40-to-50 age group, and it bothers them because it makes them look older. It’s a period of time in everyone’s life when they are confronted with the problems of adjusting to aging – having to determine how they feel about it and the changes in their bodies and being treated differently by younger people. They’re taking leave of the privileges of youth. It’s a period of readjustment for everyone, and for these men it’s made doubly difficult because, in their case, it’s foreshortened. They can go from looking middle-aged to looking old in a short period of time, and it’s a shock.
“I don’t think anyone will be surprised to hear that it’s an especially difficult challenge for a gay man. Gay culture in general places a premium on youth and physical perfection. The ideal that gay men are constantly confronted with in advertising and in gay publications is the smooth-faced twenty-something with a ripped body. The self-esteem or the self-worth of a gay man is often based very strongly on his appearance. The consequences of HIV seem calculated to hit him where he’s going to feel it most.
“I frequently find that a client, in those cases where he is confronted with the relatively sudden loss of his looks, will withdraw into a period of depression or mourning. Some of them isolate. They feel they have lost the thing that made them unique or worthwhile. They feel marked, especially as far as other gay men are concerned. Another gay man is more likely to recognize the cause of the wasting, and to know that he is dealing with someone who is HIV positive. The one suffering from HIV will often feel that he has no chance, that he will be unable ever to interest anyone in himself again. My job then, in a case like that, is to listen to the client’s concerns, to amplify his fears and present them to him as a reflection of his feelings to help him work through them. There is no short-cut in that case. We have to work through all the feelings of loss and the fear of rejection, and we have to discuss the actual reactions of the people around him, until the client is able to take leave of what he has lost and begin to look for his basis of positive self-image in some other area of his life. Even clients whose looks are not radically affected are haunted by these fears. They worry, ‘Do I look like that? Are others able to tell just by looking at me?’ The uncertainty constantly gnaws at [the] self-esteem. It can cause considerable uncertainty.
“Another problem I frequently face with clients is that they will put off starting therapy because they are afraid of how it will affect their looks. There’s a general body of opinion that the change in appearance is caused or aggravated by the medication, and some clients are so worried about the possibility that they are willing to put off starting therapy beyond the point where it is medically indicated. It’s a real dilemma. Once they begin therapy, there is generally no turning back. If they’ve been exposed to HAART therapy and then stop, there’s a considerable danger of developing immunity to those drugs, and that’s one treatment option fewer further on down the line. We have to walk a very fine line then.
“Clients are more amenable to therapy when it’s their decision, so we want to re-enforce the perception that they have the liberty of deciding when to begin treatment. They have to know it’s their choice, but we must make it clear all the same that there can be serious consequences if they wait too long. That, actually, is the situation where I personally feel most uncomfortable, because I don’t want to advocate, but I don’t want to see one of my clients hurting himself or herself by waiting. In that case, about all I can do is help in the decision process, help weigh all the pros and cons, help map out how they’re likely to feel about any consequences of their decision later and just help them come to an informed choice. It’s hard and sometimes it’s scary, but so far I think everyone has been able to make a reasonable decision, and I know that if they feel they have made the decision themselves, it’s a decision they’re probably going to stand by.
“Finally, I’d say there is a third category of clients that I’d pretty much equate with long-term survivors. Typically, they believed at some point that they were going to die, then their lives were saved by the advances in therapy. They probably spent a number of years – let’s say around five years or more – on disability until they began to feel confident enough to return to active employment, or some of them are being forced back to work because they can’t survive on their disability payments. I would put these clients together in a special group due to the high degree of general anxiety they feel. They’ve gotten used to a certain way of life, and now they’re attempting to re-integrate into the mainstream. They wonder if their skills are still current; they worry about their coping skills, and in addition, they have to worry about their appearance. They feel that – with all the other things they have going against them – they may appear unhealthy or frail, or someone may comment on how they look. It takes a great deal of courage under those circumstances to try to resume a life. They may feel that everything’s working against them. I feel the best I can do then is reassure them and encourage them to keep trying.”
Lipodystrophy in plain English
Adrianna is a psychotherapist, and as such, she is mainly concerned with the psychological effects of the physical condition of her patients. The physical causes of the problems she describes can be gathered under the term lipodystrophy. In plain English, lipodystrophy is an odd and unusual distribution of fat in the body’s tissue. It is frequently seen in conjunction with an HIV infection. Although it may not seem like such a serious consequence of HIV when compared with the problems faced by patients in the earlier days of the epidemic, it can have serious consequences for the patient’s life, as Adrianna’s observations show. It is not merely an insult to vanity.
Lipodystrophy can be divided into three main subclasses, or phenotypes: there is lipoatrophy, lipohypertrophy and a mixture of the two.
Lipoatrophy is an unusual loss of fat. It appears much as Adrianna has described it. There is a major loss of subcutaneous fat in the facial area. Subcutaneous fat is the layer of fat just beneath the skin. Even in healthy people there is considerable variation in the thickness of the subcutaneous fat layer. People with a thick layer of subcutaneous fat tend to have smooth, well-rounded features and plump-looking skin. When the layer of subcutaneous fat is thinner, a person’s features tend to seem sharper and more drawn. The skin lays very close to the bone. Subcutaneous fat disappears in people suffering from lipoatrophy. The face can take on a skull-like appearance. The cheeks are sunken, and the cheek bones appear very prominently. As Adrianna has remarked, lipoatrophy can occur in the arms and legs as well. As the subcutaneous fat is depleted in the limbs, the muscles and veins appear to be more cord-like and tautly drawn. The legs and arms appear to be skinnier and possibly weaker. Others areas affected by lipoatrophy are the buttocks and the breasts. As the buttocks lose subcutaneous fat, they become loose and flabby. The skin hangs from the bone; a person suffering from this condition can appear to have no butt at all when clothed. Lipoatrophy of the breasts can be described in a similar way. The skin becomes flabby and hangs loosely.
Lipoatrophy is not the same as the wasting that occurred in the early days of the AIDS epidemic. The wasting seen then was the result of a loss of muscle; lipoatrophy is a loss of subcutaneous fat.
Lipohypertrophy is an increase in the subcutaneous fat layer. This occurs primarily in the abdomen – which creates the impression of a large paunch known as a “protease belly”, the breasts and at the back of the neck, where it can lead to the so-called “buffalo hump” – a large swelling of fat along the neck, shoulders and upper spine. Lipoatrophy and lipohypertrophy can appear in the same patient in mixed form. It may happen that a patient loses fat in his face, arms, hips and legs, but gains fat on his abdomen and upper back.
Lipodystrophy may also result in the appearance of lipomas. These are odd accumulations of subcutaneous fat at random points on the body, and appear as swellings beneath the skin.
Lipodystrophy was not a major complication of AIDS before the advent of HAART therapy, for the simple reason that AIDS sufferers died too quickly for the effects of lipodystrophy to be a problem. Now that people are living longer with HIV/AIDS and are trying to live more normal lives integrated into society, lipodystrophy has become more apparent as a problem. As Adrianna has shown us, victims may feel concerned by their appearance or uneasy at how they feel others may react to them. They may feel at a disadvantage looking for employment or a place to live. It has to be admitted that for such a widespread problem that is of increasing concern, comparatively little is known about lipodystrophy. Medical science is still concerned with the most basic questions. Doctors are still struggling over how exactly to define lipodystrophy, and they are uncertain as to how often it actually occurs. Until they decide on a common definition, they can make little progress on the question of the frequency of lipodystrophy, since two doctors must be certain they are talking about the same thing before they can begin to agree that they have seen five, 10 or 100 cases of it. Unless doctors can agree on what lipodystrophy is, they cannot decide if they are treating a mild or severe case of lipodystrophy, or perhaps something which should be treated as a different syndrome altogether. It is not even clear what the cause of lipodystrophy is, or who is most at risk for developing it.
“It has to be admitted that for such a widespread problem that is of increasing concern, comparatively little is known about lipodystrophy.”
The cause of lipo
It is clear that a number of factors affect the development of lipodystrophy. The duration of the HIV infection and the duration of treatment both play a major role. Patients with a long history of infection and treatment are at greater risk of developing lipodystrophy. The disease progression also plays a role. It has been reported that patients with a long history of infection who wait with treatment until they are suffering from a low T-cell count (that is, they have a low number of disease-fighting cells in their immune system) are more likely to show signs of lipodystrophy. There has been some controversy as to whether it is better to start anti-HIV therapy early, before the infection has progressed very far, or whether it is better to wait until the patient is at some risk of developing AIDS before beginning treatment. Evidence cited at the Sixth International Conference on Lipodystrophy that links an increased risk of lipodystrophy to a delay in starting treatment until there is a low T-cell count would seem to indicate that this is an argument for starting treatment early.
Two further considerations in estimating the danger of lipodystrophy are age, gender and ethnicity. Older people appear to be at greater risk for developing lipodystrophy, which lends credence to the theory that lipodystrophy is basically an acceleration of the aging process. The signs of lipodystrophy – the increased girth, thinner arms and legs and an increase of fat deposits in the upper back – are essentially the normal signs of aging. Lipodystrophy merely brings these changes on at an earlier age than is considered normal.
Lipodystrophy appears to affect men and women differently. Men tend to lose subcutaneous fat in the face, arms and legs, whereas women tend to gain weight all around. All races are at risk, but Caucasians seem to be at a slightly higher risk for lipodystrophy than African Americans.
Fat and HIV drugs
Little progress has been made in determining the cause of lipodystrophy, but this may be compared with our general ignorance of the role of fat in the human metabolism. It was thought until recently that fat was an inactive substance. It was considered to be merely a storage mechanism to enable the body to put away excess calories in times of plenty to be consumed later in times when food was not available. This idea was at the base of the simplistic idea that people become fat by consuming more calories than they burn, and fat loss is basically a process of consuming fewer calories and burning more or them. While the basic tenets of this idea remain valid, we know now that fat plays a much more active role in the human metabolism. Fat cells are able to influence the body to increase the amount of food stored as fat, so that weight gain becomes a self-supporting process.
Fat is present in the brain and surrounds our nerves. Fat plays some ill-defined role in the thought process. We know that when lipoatrophy begins, the brain also loses its supply of fat, and this has led some scientists to speculate that AIDS dementia – the breakdown of the mental process observed in some AIDS sufferers – is related to the loss of fat in the brain.
Certain medications seem to be more closely linked to the development of lipodystrophy. Zerit and Crixidine are frequently implicated in lipoatrophy, just as Viracept and Crixivir are frequently mentioned in connection with weight gain, but no one medicine is suspected of being the cause of lipodystrophy, and no matter what anti-retroviral regimen is used, the longer the treatment continues, the more likely a patient will be to develop lipodystrophy.
The relationship of some HIV medications to lipoatrophy may have to do with their high toxicity, or – to be more specific – with their mitochondrial toxicity. The mitochondria, as we all learned in high school biology, are the power houses of the cells. We all carry mitochondria in the nucleus, the central body, of our cells. Mitochondria have the unique ability to break down ATP, which we can think of here as cell food. Just as our digestive system is able to break down food and turn it into energy to drive the whole body, mitochondria are able to break down ATP and supply the cell with energy. Some HIV medications have the unfortunate side effect of killing the mitochondria, leaving the cells to starve to death. The high mitochondrial toxicity of Zerit, for example, may kill off a large number of fat cells leading to lipoatrophy.
Another widespread disadvantage of HIV medications is that they lead to insulin resistance. Our body needs insulin to process the sugars we consume so that we may use the energy contained in the sugar. As cells become insulin-resistant, they need more and more of it to extract the necessary amounts of nourishment. Insulin resistance is at the base of some forms of diabetes. In its extreme form, diabetes can leave the body to starve through an inability to extract nourishment from food. Fortunately, medications have already been developed to reduce insulin resistance. Metformin and Glucophage are two medications that have been shown to be effective in reducing abdominal fat deposits in HIV patients who are showing signs of insulin resistance.
Facts for fighting lipo
An important factor affecting lipodystrophy may have nothing at all to do with medication or being HIV infected. It may be the plain old couch-potato lifestyle shared by so many Americans. A regular routine of exercise and proper nutrition can do a lot to offset the development of lipodystrophy. Doctors recommend exercising three to four times a week for at least 30 minutes. The goal is to exercise long enough to increase the rate of heartbeat to promote cardiovascular health. A lipodystrophy-fighting diet resembles the diet we are all recommended to follow for general health reasons: avoid fatty foods such as butter, whole milk, ice cream or anything high in cholesterol. Red meats such as beef should be consumed with caution. A diet high in fruits, vegetables, beans and fiber will go a long way towards promoting health, but these are general health recommendations.
Treatment options for lipodystrophy are limited at the moment. Lifestyle changes such as increasing exercise and improving diet are always an option, but they will not result in a spectacular reversal of lipoatrophy. Changing medications is another frequently-tried option, but it is not primarily recommended as a means of dealing with lipodystrophy. Treatment options, after all, are still limited, and if a patient has found a regimen that controls the viral load, it would be unwise to change the regimen solely for reasons of appearance. Furthermore, there is no way of knowing if a change of regimen will bring an improvement; a new regimen can also bring new problems. A change in regimen is more to be recommended in an attempt to improve the metabolic parameters – the body’s response to the medication – rather then for any hope that different medications will improve the appearance.
Serostim, which is human growth hormone, is currently being tested for its ability to counteract some of the effects of an HIV infection. However, it should be remembered that serostim is being tested for AIDS-related wasting, which is muscle loss, rather than for lipodystrophy. Nonetheless, serostim has shown some ability to increase lean muscle mass, reduce deep abdominal fat, and may help reduce fat accumulation on the back of the neck. Not all the news is good, however. It can also cause swelling of the ankles and wrists, it can increase the risk of lipoatrophy, and at $30,000 for a 12 week course of treatment, it is not cheap. It may require repeated maintenance shots to prevent fat from returning.
The simplest and most direct help available to sufferers of lipodystrophy is cosmetic surgery, as for many others hoping to look better or younger whether they are infected with HIV or not. Modern cosmetic surgery is safer and less invasive than in the past, but it still must be approached with caution. For whatever purpose it may be undertaken, it remains surgery, and it can be dangerous.
Surely everyone has heard of liposuction by now. We generally understand this to be a process whereby the fat deposits on a certain part of the body are liquified and then removed. It is more effective than dieting for weight reduction because it is localized and, since fat cells are destroyed, the fat deposits cannot quickly come back. Liposuction would seem to be recommended in cases of lipohypertrophy, but its usefulness is limited. It can do little to help in the reduction of abdominal fat caused by medication, because this fat tends to be thick, hard, fibrous and interspersed with the muscle, so it is not easily sucked away. Liposuction is more effective against the buffalo hump, but here too, it is not a cure-all. Fat deposits on the back of the neck and random lipoma are more suited to liposuction, but they also have a tendency to re-form after a while, and repeated liposuction carries the risk of infection.
Another procedure that is already being performed on non-HIV patients is autologous fat transfer. In this procedure, fat is removed from a person’s stomach or dorsal area – where we generally have too much fat – and is then injected into an area where fat is lacking. It can be used for example in cases of facial hyperatrophy. It is generally considered to be effective and durable, but once again, follow-up treatments may be necessary to maintain the improvement. Fat in general has a tendency to be re-absorbed by the body, or if it is injected in one location, to migrate to another location. Also fat cells may easily do what they were made to do, which is to increase in size. Autologous fat transfer used to treat facial lipoatrophy has sometimes resulted in what may be viewed as a reverse condition. Abdominal fat cells injected into the cheeks may begin to swell as they store fat, leading to a condition known as hamster cheeks. Any fat used in autologous fat transfer should be taken from parts of the body where weight gain is not very apparent.
The newest craze in the treatment of lipodystrophy is a product called Sculptra, formerly known as New-Fil. It was endorsed by the FDA last August as the first drug to be expressly approved for the treatment of HIV-related facial lipoatrophy. Aventis, the French company that produces Sculptra, wasted no time in raising the price of their product to $960 for a two-vial kit. One treatment session with Sculptra requires two to three vials, and four to six treatment sessions are necessary before the effects supposedly become permanent. Add on an average doctor’s fee of $400 per session, and the patient is looking at a treatment cost between $7,000 and $9,000, not to mention any follow-up session that may be necessary if the treatment proves not to be so permanent.
“The self-esteem or the self-worth of a gay man is often based very strongly on his appearance. The consequences of HIV seem calculated to hit him where he’s going to feel it most.”
Those suffering from HIV/AIDS are demanding more research be done to discover what actually causes lipodystrophy, how it can be avoided and ways to reverse its effects. Early on, research concentrated on developing life-saving medications. Now that treatments have proven to be effective in this arena, focus has shifted to include quality of life.
Many are waiting for less toxic, more manageable combination drug therapies with the hopes that these new drugs will provide aggressive anti-retroviral properties while reducing side effects including lipodystrophy.