Milroy's Disease - a parent's view

I decided to write about my son's problem for two reasons; firstly when he was first diagnosed with his condition even the Doctors had to look on the internet for information! And secondly because as every day passes it is a day when I can sigh with releif that he has not done himself any damage, that he is developing properly and that the treatment we are carrying out on him seems to be helping.

So what is so wrong with him?, you ask. What could possibly be so terrible for you?

Milroy's Disease. To put it into medical terms congenital lymphoedema. Basically his lymphatic system in his lower right leg and foot, and left foot either does not exist or is very reduced. It means that the fluid that is normally moved from the body stays there making the limbs swell. If you have been pregnant and got fluid retention in your legs then you know what it is like. But he will be like that, forever.

When he was born he was absolutely perfect. The midwife put him in my arms for a few moments, then began the routine checks and saw that he had very swollen feet and right leg. She called the consultants but they did not know what it was either. My little baby was only a few hours old when they took him from me to run tests. They eventually bought him back to me, only to take him back to neo-natal for 'observation'. He was now 15 hours old and we still had no idea what was wrong with him. Still, he made his feelings perfectly clear on the matter. He would scream and scream until I went there, then he would quieten at the sound of my voice.

It wasn't until that evening when one doctor said he thought he knew what it was, but that he had never seen it in an infant. He told me about Milroy's disease, what it was and basically how little they knew about it. I had a quick look on the Internet and what I saw scared me silly! Still, after seven days we were let out of hospital (I had pre-eclampsia, another reason for keeping me in) and told that someone from paediatrics would contact us. They didn't! I had to chase them until someone decided to take Jack's case on. I think they were all a little afraid to look at him, although it is a teaching hospital, as they didn't know what they were facing. We saw several doctors and received some very useless (we found out afterwards) information until eventually we were sent to dermatology, who refered us through to a lymphoedema nurse. They normally deal with cancer patients who have had the lymph nodes removed and suffer from lymphoedema afterwards.

Then we actually found someone who had dealt with lymphoedema and could start to advise us. Firstly we were told to discount all of the things we had been advised to do by the other doctors; raising his feet, hard massage, putting on constrictive bandages - they could actually do him more harm than good! Lymphoedema is managed with the following things; manual lymphatic drainage, moisturisation, exercise and support stockings. As Jack was only a few months old the last two didn't count but we were advised on the the massage and moisturisation as follows.

This is the name for the special type of massage used on patients with lymphoedema. Basically you have to assume that the lymph nodes situated around the site of the swelling do not work so you have to ask another set to drain the collected fluid. Lymph nodes are found in the groin, the armpits, neck, chest and stomach. First you have to clear the ones you want to drain; for Jack it is under the arms. You start just lightly brushing the chest and back with your finger tips, encouraging the lymph nodes to work. Then, also with featherlight touch, you stroke from the feet up to the hips, trying to clear the fluid from the swollen areas. This has to be done for about 10-15 minutes at least twice a day.

The other problem with Lymphoedema is that the skin is stretched and taunt and becomes very dry. If it cracks there is a great risk of infection. Therefore we have to make sure that Jack's skin is kept very soft and supple with at least twice daily application of emollients. The places to really be careful of are behind the knees and the crease of the foot and leg. We also have to make sure that his skin is thoroughly dried after bathing as this can lead to skin problems too.

This is the most worrying part of Milroy's Disease. When lymphatic fluid is left in the soft tissues it becomes a great breeding ground for bacteria. If an injury is made on that limb it is easily infected, leading to cellulitis which if left untreated can cause very serious blood infections. Unlike other children, if Jack gets a cut on his leg or foot he wont need a plaster, he will need antibiotics. The same applies if he gets athletes foot, ingrowing toenails, mosquito bites, cracked heals, blisters, sunburn, insect bites, cat scratches and so on. We have a great list of things that he cannot do without running a risk of infection, such as:

• take a hot bath
• walk barefoot - anywhere
• wear elasticated socks or bootees, or children shoes or slippers
• go out in the sun without very high SPF
• get too hot

He will also find that his legs will become more tired than his brothers do, and he will have less stamina for normal boys things such as running or climbing.

Well, we are taking every day as we find it. He is just starting to crawl which is making his knees red and I check them almost constantly for any signs of the skin breaking. I live in fear of the day that he does cut his skin, for once he starts to take antibiotics the next time he gets a cut he wont have so much resistance, and so on. We are fighting to get the best care for him; the most important test to see how bad his legs are cannot be done until he is over 18 months. It means we will soon be leaving Scotland to move to the South Coast so he is near a specialist in London - we cannot face a 600 mile trip to see them if something goes wrong with a toddler in tow too.

It has made me realise that there is something more to life than work, money and everything that that includes. My time is spent making my boys happy, giving them the best I can and making sure they have the best start in life. Jack is very precious; to me he is perfect and I tell him so every day. Because he is different I know he will be a target for bullies in schools and I dread that time. We will try to make sure that he does not feel different from his brother and that he is just right no matter the size of his legs.

My advice to you is very simple. Make a fuss! Keep on at the doctors until you are seen, make sure they remember you, or your child, and make sure they refer you to dermatology or the lymphoedema nurses. It is really important that this illness is not put away somewhere because they don't know what it is. Make sure you are not forgotten or you run the risk of damaging your health in the future.

If you want to know more about lymphoedema please visit the Lymphoedema Society Network site at www.lymphoedema.org.