Milroy’s Disease – DefinitionMilroy’s Disease is a condition that affects the normal function of the lymphatic system. The lymphatic system produces and transports fluids and immune cells throughout the body.
Impaired transport with an accumulation of lymph fluid can cause swelling (lymphedema). Individuals with Milroy disease typically have lymphedema in their lower legs and feet at birth or develop it in infancy. The lymphedema typically occurs on both sides of the body and may worsen over time.Milroy disease is associated with other features in addition to lymphedema. Males with Milroy disease are sometimes born with an accumulation of fluid in the scrotum (hydrocele). Males and females may have up slanting toenails, deep creases in the toes, wart-like growths (papillomas), and prominent leg veins. Some individuals develop non-contagious skin infections called cellulitis that can damage the thin tubes that carry lymph fluid (lymphatic vessels). Episodes of cellulitis can cause further swelling in the lower limbs.
How is Milroy’s Disease inherited?Milroy disease is inherited in an autosomal dominant manner. This means that having one changed (mutated) copy of the responsible gene in each cell is enough to cause symptoms of the condition. When a person with an autosomal dominant condition has children, each child has a 50% (1 in 2) chance to inherit the mutated copy of the gene.Most people with Milroy disease have an affected parent, but some cases are due to new mutations that occur for the first time in the affected person. About 10-15% of people with a mutation in the responsible gene do not develop features of the condition. This phenomenon is called reduced penetrance.
Causes of Milroy’s diseaseMutations in the FLT4 gene cause some cases of Milroy disease. The FLT4 gene provides instructions for producing a protein called vascular endothelial growth factor receptor 3 (VEGFR-3), which regulates the development and maintenance of the lymphatic system. Mutations in the FLT4 gene interfere with the growth, movement, and survival of cells that line the lymphatic vessels (lymphatic endothelial cells). These mutations lead to the development of small or absent lymphatic vessels.
If lymph fluid is not properly transported, it builds up in the body’s tissues and causes lymphedema. It is not known how mutations in the FLT4 gene lead to the other features of this disorder.Many individuals with Milroy disease do not have a mutation in the FLT4 gene. In these individuals, the cause of the disorder is unknown.
Risk factors of Milroy’s diseaseThe following are the risk factors for Milroy disease:. Age and sex. Family history. Immune system problems. Infections. Previous cancers.
Precursor conditions (non-cancerous conditions of lymphocytes)Signs and SymptomsThe main symptom is swelling of the lower leg (usually limited below the knee). It affects both legs but the right leg tends to be worse. Infant males may have a swollen scrotum (hydrocele).
It is important to note that swelling can occur at any site where the lymphatic vessels are abnormally small or absent. Gravity does play a role in the presentation as the fluid accumulates in the lowest parts of the body, like the legs. The swelling is generally soft and pitting. Certain affected areas may be hard with a “woody” feeling due to fibrosis.
Milroy`s Disease Diet Foods
The swollen area may be warm to touch.Cellulitis is common in patients with Milroy disease. The swelling in these cases is worse than just the disease on its own, there is usually redness of the skin, heat, and tenderness.
Lymphedema is a common side effect that may affect a cancer survivor months or even years after treatments such as surgery or radiation therapy. As part of the circulatory system, the lymph organs play many roles in the body including fluid balance and immunity.Lymph fluid is a lipid- and protein-rich fluid found within the lymphatic system that contains white blood cells and helps remove waste. When lymph nodes don’t filter and drain lymph fluid appropriately, the fluid will accumulate and lead to fluid retention and abnormal swelling around the extremities. Lymphedema can also affect other body parts such as the face, neck and abdomen.Some typical signs and symptoms of lymphedema include a tight or heavy feeling in your arms or legs, limited range of motion from swelling in extremities, pain or discomfort and hardening of skin.There is no specific medical nutrition therapy to cure lymphedema, but here are some tips to help manage symptoms, and possibly prevent lymphedema onset.
Maintain a healthy weight. There is an association between a high body mass index (BMI), or obesity, and an increased risk for lymphedema.
The more adipose tissue or fat one has, the more the lymphatic system will struggle to pass fluid. Excessive fat also promotes more inflammation. To successfully manage weight, balance meals with low-fat, nutrient-dense foods. Include lean protein, whole grains, fruits, vegetables and dairy in daily meals and snacks.
It is important not to follow fad diets that restrict any food group. Eating foods from each food group guarantees that the body receives all its vital nutrients for survival.
Monitor daily caloric intake. Phone apps like MyFitnessPal and Lose It!
Milroy`s Disease Diet Menu
Make tracking food consumption easy. Each person has their own unique circumstances, so it would be best to consult a dietitian for guidance. Exercise. Exercises that promote joint motion, stretching and strength training will help with circulation and improve lymph vessel activity.
Milroy Syndrome
Consult a medical specialist such as a physical therapist or certified lymphedema therapist to safely execute any exercise program. Avoid diuretics (“water pills”), alcohol and caffeine. While diuretic medications generally work well with fluid retention associated with venous insufficiency (such as conditions like congestive heart failure), they do not work well with excess lymph fluid-associated lymphedema. Alcohol and caffeine could also function like diuretics.
They both could dilate the lymph tissue and cause more swelling, and as a result, exacerbate the lymphedema. Don’t adopt a low-protein diet. Consumed protein has no connection with the protein in the lymph fluid. Low-protein diets may actually be harmful, as proteins contain essential amino acids that the body requires. Insufficient protein intake could result in malnutrition and muscle wasting. Each meal should include at least three ounces of lean protein such as eggs, fish, chicken, turkey and lean beef. Reduce salt intake.
Salt promotes fluid retention. There are no official guidelines for avoiding salt for lymphedema, however people who are sensitive to salt might exacerbate their symptoms if they eat high-sodium foods. Current recommendations for salt intake are less than 2,300 mg for healthy individuals and less than 1,500 mg for people who have cardiac disease, diabetes and kidney disease.In addition to good nutrition and exercise, there are many other strategies like compression therapy, massage therapy and proper skin care that can aid in the treatment of lymphedema. Talk to your doctor first if you would like to explore these options.Simone Gampel, RD, CDE, is a Registered Dietitian with Summit Medical Group in Berkeley Heights, NJ. She has worked in private practice, home care and large university hospitals.
She currently works with diabetic, bariatric, gastric and surgical oncology patients.