Lymphovenous Canada: How parents and children cope with lymphovenous disorders

Pam Hilliard, a physiotherapist for over 20 years with the Hospital for Sick Children, Toronto, answers questions posed to her. Her reponses here reflect her own personal opinions.

A number of children with lymphedema see me at the physiotherapy department at Sick Children's Hospital - mostly those who have primary lymphedema.

I also treat children with Klippel-Trenaunay syndrome which is a mixed disorder which may have a lymphatic component. Usually it involves gigantism, superficial vessel involvement where they may have a type of reddened skin (naeveus flammeus) and involvement of the deeper venous system. I also see children with lymph angioma - it is a mixture of a lymph component and a venous component. You get the swelling like you get with a lymphatic system involvement, but you may also get bleeding and deeper vessel involvement. Even though all of these children have conditions which might be called lymphovenous disorders, they require different treatment approaches which are often very individual.

Diagnosing the problem:

Children come in to see us at various stages and times in their lives - it depends a lot on when the diagnosis is made. This may be shortly after birth when the parent notices a larger limb, or later - sometimes in the teens. With the development of a condition such as hemangioma the enlargement and effect on the skin is usually noticed early on when parents go to a family doctor or a pediatrician. Sometimes children are referred for other tests. Sometimes a CT scan or a magnetic resonance imaging (MRI) may be ordered.

I'm usually involved when a physician here is seeing the patient. With someone who has that type of disorder, they will ask me to assess the patient as well. At this clinic it is usually goes through the plastic surgery department but I also get some patients through general pediatrics and occasionally through dermatology.

I see children between the ages of 0 and 18. Lymphedema may not be diagnosed until adolescence. With lymphedema pre-cox the condition usually occurs more when adolescents go into puberty, so I am really starting with children at that age. Although no one knows for sure why that is the case, it is generally believed that the condition is triggered by the change in hormones, although trauma, a change in stresses and other factors can also trigger it. These children bring in different issues and concerns than children affected by lymphedema at birth.

If we can find someone locally to work with parents and children - either at local hospitals or through other types of service providers, rather than having them come to us - that certainly makes sense. If they have infections that needs treatment, or if they can have their garments made locally, that is preferable. Usually they only need to come down to see us maybe once or twice a year once a regimen is established. It really depends on the individual child's needs.

Telemedicine is opening new doors for treatment in some northern cities. A "live" teaching session (or assessment and review of treatment) can be arranged between HSC staff and the family and local caregivers.

Shoes and clothes:

Clothing isn't quite as much of a difficulty for parents and children as footwear, although they sometimes have to be quite creative with clothing. Fortunately a lot of styles are baggy these days or mixed. You certainly don't have to go with skinny leggings if your style isn't skinny. Big oversized sweatshirts are quite acceptable.

Some people find certain shoe brands really work for them to accommodate extra depth and the larger foot. Adidas and Doc Martens come in wider sizes. Cowboy boots and kodiacs are unisex and aren't thought of as girls or guys. Sometimes parents may go a half a size larger to accommodate a larger foot and put an insert for the smaller foot to make it a little more functional.

Sometimes finding a shoe to fit is still a problem. We had a Portuguese shoe for toddlers that would open right down to the toe and would enable enough compression on the foot. They seem to be harder to find these days. Sometimes it's just a matter of lucking into the right person - a shoe manager who makes an extra effort to find the right shoes to fit the young ones.

If they have an interest in that kind of thing they go the extra mile to help them out. Occasionally, they have to get something custom-fitted which is more expensive. Fortunately that doesn't happen too frequently - what we are trying to do with the rest of the program is to get the size of the foot down so they don't have to go that route.

Parents' concerns:

For the small infant the concern for the parent is: "will they walk normally?" or "will this affect their motor development?", how their legs and arms will work when they start using writing instruments. Getting up, standing and walking, getting into shoes or boots. Pretty essential in winter. Sometimes shoes or boots have to be specially made. Those sort of issues are very important especially to the parent.

They are also having to deal with how they are going to cope with children once they get into school, once they get into swim and gym programs. Everyone deals with this differently. Some prefer to hide it, some are more open about it. One approach is to encourage parents to get their children into programs like dancing or gymnastics where the child learns to handle people perhaps looking or questioning - especially when they have a garment on.

There can be great difficulty for parents to schedule in pumps or message - it depends on how many other children there are and how busy they are. Some children deal with their condition better than others. In adolescence this is probably multiplied. They are having to deal with other things happening to their body and not looking that great. They feel uncomfortable with a large heavy limb, then you add compression or other treatment demands.

Special issues for adolescents:

I find compliance the hardest with adolescents. It is probably more difficult for children who develop lymphedema later than those who start out with it. Using an extremity pump can cut into an adolescent's social life. Some are quite active after school and with the school work they get some prefer to pump during the night. It depends on their tolerance - some find cannot tolerate it overnight so they pump in the evening. They can't do it every night but they try to do it perhaps every other night. Once a child has gotten their limb size down to as low as possible - the maintenance level - we encourage them to find the level of pumping they require to maintain their limb at this level - three times a week, once a week. Quite often they get to the point where they figure out what they can get away with to keep their limbs soft and pliable and in good condition.

Some young people put the pressure up very high in the pumps to keep their limb size down. This could be a problem - in some exceptional cases a band of fibrous tissue forms just above where the sleeve ends - causing a pooling of lymphatic material in the tissues. This is something we are looking into - whether this is the result of pumping at too high pressure will probably take more research. We would prefer people use lower pressures - I recommend 40-60 mercury pressure - the lower end for individuals who pump through the night - the higher end is probably a little more acceptable for shorter time periods such as two or three hours in the evening.

Getting teens to wear compression stockings may be a challenge. It is difficult to get them on - they are tight. A lot of it depends on how much the edema affects them. If it is difficult to get into the shoes they like or to participate in activities, they are likely to be compliant. But if they don't get the result, if they are teased or ridiculed for having something like that, the compliance drops. Sometimes they get away from it and then return to it as their values and mindset changes.

As part of our education we talk about how important it is to stay infection-free and how devastating some infections can be. But as in a lot of other conditions, children, in particular adolescents don't usually think beyond tomorrow and its difficult to get them to understand that the future means something. So what you are doing now with respect to treatment is to try and ensure a better future - sometimes that's very difficult. They see the now but don't really see the long-term gains. Looking good is sometimes more important at this age than long-term health problems.

Maintenance is also a hard thing to sell. When they get to a level where they are no longer increasing but not really decreasing it's hard to understand why it is important to keep it up. The idea of keeping the tissues soft and not allowing them to change and become hard, woody and fibrous so that down the road you don't have problems with infections, decreased function, or with those awful skin changes that can occur - that's harder to understand.

Treatment options:

I have some people who are constantly looking for an end to the problem - a solution that is lasting. It is a problem with any chronic condition to understand that this is for life. At this point in time we do not have a good solution. Surgery can bring bigger problems with it. We try to make it quite clear that this can be done but frequently it brings on more problems. Surgery is not a one-time solution that will take away the problem. I encourage people to take care of their condition as much as possible so that if something does come along they can take advantage of it. If you get to the point where your skin has changed and the tissue is fibrous and woody when something comes along you will be less likely to be able to take advantage of it.

Many patients appreciate having different treatment options to consider. Some parents have learned the manual lymph drainage massage techniques as an alternative to pumping and enjoy the opportunity to bond with their children.

If you are going to move lymphatic fluid out you have to use compression garments during the day in conjunction with pumping or massage when you are not wearing the garments. When your limbs are hanging down your chances of having the lymph building back up are quite high. If you have lymphatics which are restricted or non-functioning, it is important to wear the garments to keep the swelling down.

There are definitely different preferences for different people. Some people can keep the swelling down only wearing stockings - and that's preferable - the simpler the better. Many people can wear the below the knee stocking and can control their condition quite adequately. The longer leg stocking is a bit more of a problem. I have some children who go for the panty style or chap style which involves a waistband and may keep the stocking up more effectively. Some older children use a roll-on skin "glue" called "It Stays" to keep a thigh-high stocking up.

Whatever the situation, we need to stress excellent skin hygiene. Young people need to understand they must prevent open sores. Excellent foot care is essential in making sure there are no ingrown toenails, that toe nails are cut straight across, making sure the skin between the toes is kept dry and very clean to prevent fungus from developing. When the lymphatic system is not functioning or malfunctioning, these things tend to occur more often.

Another important aspect of treatment which is often overlooked is exercise and activity. Getting the calf muscles to act like a pump assists in the movement of lymph fluid. Swimming is especially good because of the hydrostatic pressure of the water on the limb. Riding a bicycle, using a trampoline or rebounder or doing step exercises are also good.

Distance and availability can be a barrier to using massage (manual lymph drainage or MLD) as an alternative therapy. There aren't that many registered manual lymph drainage specialists and in many of the places my clients live that isn't an option. MLD is unfortunately quite expensive as well and is not covered by public health care. If parents don't have private insurance programs, it may not be feasible. Ideally parents should be taught some MLD techniques to use at home.

Extremity pumps can allow young people with lymphedema more independence - particularly adolescents - they can set it up when it is convenient for them. These options should be discussed with your lymphovenous caregivers.

Financial barriers:

We work to try to eliminate any financial barriers that parents may be experiencing in getting their children the treatment they need. I have some parents who don't have insurance to cover the 25% of the garments their children need which the Assistive Devices Program in Ontario does not pay for. For one set of garments that may not be a huge issue. But usually children need to have their garments changed three times a year - in some cases more often if the child is really growing and changes have to be made in a garment. And if you get into looking at costs for pumps, the sleeves and for manual lymph drainage specialists, those costs can be quite high.

If a family is on social assistance, quite often if you go through the right channels and get the right letter they will pick up that 25% and we certainly provide whatever documentation is required to move that ahead. Sometimes people will need that for insurance purposes as well. Sometimes we or the family will approach Easter Seals or a local Rotary, Kinsman, Legions Kiwanis club - quite a few people have groups locally that can help them out. I had a girl who really wanted the pump but the 25% was just too much for her. To mum's credit and also the doctor, they were able to come up with funding through a couple of fund-raising groups/agencies for the pump and the sleeves. Some feel they prefer not to utilize these resource - they may feel embarrassed. This is entirely up to the individual family involved.

Long-term expectations:

Pumping or massage doesn't necessarily result in a normal looking leg. A lot depends on the natural body structure. It can depend on the severity of the condition and the parent and the child's reaction to and compliance with the treatment program. In some situations, the swelling is extremely well-controlled. If not completely normal-looking - it is certainly acceptable. The tissue is soft with minimal or no infections and inflammatory episodes. They are doing well. Then I have the other spectrum where children have a lot of problems - and you don't get as good a response. There may be some parents who blame themselves - parents are always second-guessing themselves if things aren't going quite perfectly.

When parents or children have difficulty coming to terms with the disorder we look at where the problem is, options, what alternate solutions are available, what they are dealing with. Sometimes things work better, for instance, simply if a garment is cut differently. If they can maintain their good skin condition by wearing a garment at night rather than the day - that sometimes works better. This doesn't always work as well with straight lymphedema but it sometimes works better with other types of conditions. If we can get away with it, it is sometimes better.

Sometimes parents or children appreciate having other health care professionals available to them - a social worker or a psychiatrist or even talking to other children through Ability On-line. Some people will appreciate having that made available to them - others will handle it locally rather than having Sick Kids arrange things.

Quite often it is extremely helpful for parents to talk to other parents about their experiences and concerns. I have had a number of families who have said they felt alone and would appreciate having another family to give them advice. They'd like to have a network to bounce around ideas and concerns. Getting people together and having a voice. Even to have an equipment exchange or sell-off so that when children are moving up in size they can trade their pump sleeves. Some people want to get involved in a group so they can delve into what kind of common denominator causes these disorders. To do more research in this area. Investigate innovative treatments. That's certainly part of the impetus behind trying to get a support group going in Canada.

Although this is a life-long condition, a positive family outlook and approach goes a long way in normalizing the life of a child with a lymphovenous disorder.

Pam Hilliard can be reached at pamela.hilliard@sickkids.on.ca

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