Prevention of Bleeding
Platelet counts vary in patients with WAS ranging from below 10,000/cu.mm to 70,000/cu.mm with counts usually averaging around 35,000/cu.mm. The counts also vary from time to time in each individual patient. Some patients have "cycling" where the counts are periodically higher or lower with no apparent reason or pattern. Some parents notice an increase in platelet counts, while others notice a decrease in counts during the course of an infection. Parents, over time, are able to gauge the platelet count in these children by observing the pattern of their bruising and petechiae. The following are general guidelines written for WAS patients whose platelet counts are usually between 20,000-35,000/cu.mm. If any advice given is in conflict with the advice of the child's physician, please follow the advice of the physician. Given the variation in platelet counts, even in each patient over time and the unique bleeding pattern of each child, the decisions as to when to transfuse, what activities the child can participate in etc is best left to the specialist who follows the child regularly and their family to determine.
"An ounce of prevention is worth a pound of cure" applies especially well to these children. An estimated 20-25% of these children experience a major intracranial bleed1 at some time in their lives with a 50% mortality. These numbers are lower in patients with XLT2. Preliminary results of a recent study by Dr. Albert, M. et al showed that the incidence of a serious bleed in these patients was 13% with 1/6th of these bleeds being fatal. Even with the lower numbers, hemorrhages are still a major contributor to mortality and morbidity and and it is worth taking the time and effort to prevent injury as much as possible. While there is no perfect solution for injury prevention, parents have found the following general guidelines helpful.
Care must be taken to prevent serious injuries to the child, especially head injuries. Serious bleeding is most commonly associated with head and abdominal injuries, but fractures, especially of the larger bones can cause life threatening bleeds. Serious, spontaneous bleeding can occur, especially when the counts are below 10,000/cu.mm. Caregivers should remain watchful for signs of internal bleeding, such as headache, vomiting, dizziness, double vision, seizures or neck stiffness with a head bleed or abdominal pain when there is an abdominal bleed.
Listed below are some of the measures that can be taken to help prevent a bleed. This list may seem daunting. Please remember that most children are diagnosed in early infancy and there is time to set up all of these measures as the child grows. Most of them are precautionary measures that are taken over the years as the child grows.
Around the Home: Children, especially before school age, spend most of their waking hours at home and injury proofing the home can help keep them safe. Establishing clear ground rules at home gives children the boundary within which they can play and have fun. Don’t allow running through the house and on the stairs. Furniture contributes to a significant portion of injuries inside the home and appropriate care should be exercised when the child is near furniture. Padding of the corners and edges of furniture, fireplaces and counters is advisable. It is safer to remove furniture that the child will frequently go around such as tables and chairs. Secure flat screen TV sets and unstable furniture as they can tip over and cause crush injuries. Inspect toys that the child plays with, avoiding toys with sharp edges and corners. Thin, long objects such as popsicle sticks, forks and straws are best avoided especially when the child is younger. Walking around the home with sharp pencils, scissors etc is to be avoided. Extra care should be taken while using sharp tools such as knives and scissors. Sleeping arrangements should be reviewed, with padding for cribs and the use of side rails for older children who might fall out of bed. It might be safer to place the mattress on the floor and have the child sleep on it.
Watch for slip and trip injuries. Showers, tubs, liquid spills around the home, and pools are areas prone to falls. Use of non slip mats in the tub and shower and encouraging use of non skid shoes around the pool can help prevent some of these injuries. Ensure that area rugs and carpets are backed with a non skid material. Picking up toys and books off the ground, particularly on stairs and other high traffic areas, mopping up spills at home, encouraging a "no running" around the pool area, will help to prevent some of these falls. Siblings should be advised that there is to be no roughhousing with the affected child. Enlist their help to keep the home and the child safe.
Restriction of Activities: Given the variation in platelet counts, even in each patient over time and the unique bleeding pattern of each child the decisions as to what activity each child can participate is to be determined by the family and the specialists who are taking care of their child. Specialists may vary in their opinion of what activities are permissible. The other determining factors are the distance from the nearest major medical center, the family circumstances, and the temperament of the child. However, in order to maximize their safety, it is recommended that these children not participate in contact sports, rock climbing, swings, climbing heights, diving, some rides at amusement parks (particularly those that are marked for children over the height of 48", PE at school, two wheeled scooters, skateboarding, rollerskating and rollerblading, ice skating, riding in a vehicle where there is no seat belts etc Summing it up , it is safest to restrict the child to activities where the child's feet stay on the ground. Several of these recommendations come from the experience of parents, advice from hematologists and WAS experts and from the Platelet Disorder Support Association. The restriction of activities, the extra care and precautions that are needed can be frustrating for the child. Keeping them focused on what they can do and providing them with suitable alternative activities can help alleviate some of these feelings. Children on the whole are very resilient and adapt to circumstances. Given some encouragement and support, they adapt to these circumstances and find ways to have fun while staying safe.
Further reading on activities
So, what sport/activities can my child participate in? Swimming (no diving) is a great sport that gives a good work out while keeping them relatively safe. If the child works with a swim team it can promote sportsmanship and help boost their self confidence. As an added bonus, the chlorinated pools help keep the eczema under control. Table tennis, tennis and shuttle-badminton are other physical activities that are relatively safe. Some experts permit bike riding with helmets, while others do not. Once again, these activities should be planned in consultation with the specialist who cares for the child regularly. If the child is inclined, non physical sport activities such as chess and scrabble can be encouraged. These activities allow the children to be competitive while keeping them safe.
Emergency Care Kit: It is best to have an emergency care kit that stays with the child at all times. Suggestions for what it should include are - an index card that includes the child's full name, his medical condition, medications that the child is on currently, the emergency contact information of the parents and doctor. Medical Supplies include band aids, antibiotic ointment, gauze, ice pack (these are commercially available packs that become cold when crushed), self stay wraps (Co Band), bandage, Q-tips, a few plastic zip bags (various sizes available in craft stores) for use as ice packs, Olae's bandage, a pair of tweezers and a magnifying glass. Keep emergency medications such as epi pens, benadryl, anti bleeding medications such as amicar on hand.
Helmets: A protective helmet is recommended from when the child is actively mobile (beginning to walk) and to keep it on during times that the child is actively mobile. It is better to purchase a helmet at a place where they are tailor made for the child until such time that they are old enough to fit comfortably into a regular bicycle helmet. Your physician will be able to guide you on which helmet is the most suitable for you. Helmets during periods of injury prone activity is advised for as long as possible and recommended up until the age when the child has a good sense of equilibrium and the parents are confident that the children are capable of handling themselves. A helmet can prevent or minimize a serious head injury. However, a helmet can provide a false sense of security and it is best that all head injuries, even those that occur when a helmet is worn be checked out by a physician.
Travel: Car seats can help minimize injuries in the event of an accident. They should be used until the age of 4 years and 40 Lbs. A booster seat should be used until the age of 8 years and 80 Lbs. Lap and shoulder belts should be used consistently thereafter. The safest place for these children is in the middle of the rear seat. Parents should follow all safety rules and should be a good example for the children.
Medical Alert Tags: A Medical alert bracelet with the 24 hour emergency contact number of the physician is recommended. A necklace, bracelet or watch may be used. A clear but simple description of the condition, such as "Low Platelets" , "Asplenia" etc should be inscribed. The child's physician would be the best guide on the information to put on the bracelet. The information helps emergency medical staff to correctly and quickly attend to the child's needs. One can have a complete medical history in the Medical Alert Database that can be accessed by the emergency personnel, or have the history on a key chain flash drive that they can access. In the event of a road accident, a Medical Alert Sticker placed on the windshield can alert emergency personnel quickly that a person in the car needs immediate medical attention.
Prevention of Nose Bleeds: Some children with WAS are prone to nose bleeds and they can have significant bleeding requiring transfusion of platelets and red cells. A few things can be done to help to prevent a bleed. Teach the children to avoid picking their nose as this can start a bleed. Humidified air in the home, especially during the winter months is helpful to keep the nasal mucosa from drying out. Apply very small amounts of a water soluble lubricant in the nostril several hours before bedtime. Please consult with the child's physician before using any lubricant in the child's nose. Certain medications for colds can dry the nasal mucosa and predispose the child to a bleed. Teach the children to blow their nose gently if they have to. Efficient management of allergic rhinitis and sinus infections can help prevent some of the bleeds. Saline nasal sprays are an useful adjunct to medications in the management of allergies and sinus infections.
Dental and Lip Care: Keeping lips moisturized by drinking plenty of fluids and using a lip balm helps prevent bleeding from cracked and dry lips. Meticulous dental hygiene should be maintained with regular brushing and flossing. Using brushes with soft bristles can help reduce some bleeding while brushing. Regular dental checks should be a part of the routine and sealants can help prevent some cavities.
Some children with WAS, particularly children with Classic WAS may need antibiotic prophylaxis prior to dental procedures. It is best to avoid dental surgery and extractions in children with WAS. Cavities should be attended to promptly.These procedures may necessitate platelet transfusions, general anesthesia and hospital admissions. Starting amicar, prior to dental procedures can help reduce bleeding during the procedure3. It is best therefore to prevent the formation of cavities. A mouth rinse may be advised by the dentist to reduce plaque formation. In older children who have "trouble spots" with brushing, it helps to have them use a disclosure tablet (available with the dentist or on Amazon). These are tablets that the child can chew on and spit out. It stain plaques and areas that need brushing and the child brushes to remove the coloring. This helps remove plaques and shows children the areas they need to improve their brushing. Some children can benefit from additional visits, over and above the standard bi annual cleaning visits. Selecting a good dentist who is experienced in the care of children with bleeding disorders and who can build a good relationship with the child is important.
Further Reading on mouth care
Immunizations and other intramuscular injections: The thinnest needle possible should be used when the child is to have an intramuscular injection. This will vary as the child grows. However in young children, it is usually possible to give all vaccines through a 25 gauge needle (24 gauge is larger than 25 gauge and so on). Apply steady pressure at the site for 3 minutes after the injection. Applying an ice pack to the injection site for 30-45 minutes helps minimize the bleeding that can occur with immunizations. Tylenol can be given for the pain associated with immunization. It is best to avoid intramuscular injection of antibiotics as it may cause a hematoma to form.
Medications to avoid: There are several medications that can reduce the platelet counts in these children and the reactions to each medicine vary. For a complete list seehttp://pdsa.org/itp-information/itp-warnings.html There are several medications that reduce the effectiveness of the platelet function and they should be avoided unless otherwise specified by their physician(see above link for a complete list). One of the most commonly used medications-ibuprofen (Advil, Motrin) should be avoided, along with other non steroidal anti-inflammatory medications(NSAID). Most medications are passed on in the breast milk and nursing mothers should be familiar with what medication they can safely take. While some "alternative medicines" may be helpful, it is important to know that some of them may prolong bleeding time. It is best to discuss plans for the use of any "alternative medication" with the child's physician prior to use.
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