Management of Bleeds & Injuries
Epidemiology & Disease Mechanism
Treatment of WAS
Coping with WAS
Management of Bleeds & Injuries
Management of Injuries
Even with the best of care, children are going to get injured and it is good to be prepared by having all the required supplies accessible and to be mentally prepared. It is helpful to have a letter from the child's physician stating the medical condition of the child, emergency measures that are needed and their 24 hour contact numbers. This letter can go a long way in an emergency setting to help emergency personnel to respond quickly and effectively to the needs of the patient. Parents, caregivers and the school should have a copy of the letter handy at all times (purse, glove compartment). Caregivers who are at home should be trained in the emergency management of the child and should have all the contact numbers and the home address handy (needed for 911 calls).
There are several factors that determine the management of an injured child. All of the suggestions below are only meant as guidelines. It does not replace medical advice given by the physician. The parents and the child's physician will have to decide on a plan of action that is appropriate for each child.
When to call 911?
A very good question which is not easy to answer. It depends on the the severity of the injury, the need for immediate medical care, platelet transfusions etc and the comfort level of the caregiver to handle the situation. While debating whether to take the child yourself or call 911, it is useful to remember that emergency personnel have the means to take care of some emergencies in the ambulance, they can call ahead and inform the emergency room of the patients condition and have a set up ready on arrival and they can call in air transport if required. It is important to take into consideration the distance from a major medical center, the time it might take for you to get the child there versus emergency personnel to get there.
Here are some of the possible reasons to call 911. It is not an all inclusive list and each child's care must be individualized to their particular circumstances. If in doubt it is better to be safe and call 911.
Any injury that can cause significant bleeding anywhere, especially in the head and abdomen, fractures.
Eye injuries. Loss of consciousness, seizures, vomiting after head injury.
Other bleeds that could prompt such a call are severe nose bleeds, significant vomiting of blood or significant bleeding anywhere.
Anything more than a mild crush injury (such as when a finger is caught in a door) should be treated as an emergency.
While waiting for the emergency personnel to come, one should provide basic first aid care-staunch bleeds using compression, ice and elevate injured arms, legs, toes and fingers, splinting a fracture etc. Please call the child's physician and let them know that the child is on the way to the ER. The physician may be able to get things set up in the ER prior to the arrival of the child.
Even minor head injuries, especially the ones that cause a bruise should be examined by a knowledgeable physician as soon as possible. It is best to call 911 in the event of a moderate to severe head injury with the goal of getting the patient to a major medical center within 15-20 minutes. It is safer to err on the side of caution. An ice pack can be applied to the injury to reduce bleeding into the scalp. This will not prevent or slow any internal bleeding that is occurring. Once the child is in the hospital he will be evaluated for the platelet count and the seriousness of the bleed will be assessed and further interventions such as CT Scans, transfusions etc are planned.
After evaluating the child, the physician may decide to send the child home. When the child is sent home after the evaluation for a head injury, it is important to follow a 24 hour head injury follow up care. Some of the red flags that may require immediate transport to the Emergency Room after being sent home are severe or persistent headache, vomiting, dizziness, loss of consciousness, double vision, seizures (see above link for a more comprehensive list).
A helmet can prevent help to 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 when a helmet was worn, be checked out by a physician.
Other Major injuries
Any significant injury to the abdomen and fractures necessitate an emergency transport to the ER. In suspected and in obvious fractures, ice the part and keep it as elevated as possible prior to and during transport.
Nose bleed are a common occurrence among patients with WAS. They can range from annoying to life threatening bleeds. If the nose bleed is moderately severe or if there is vomiting of blood, see the doctor right away. If it is mild to moderate, apply moderate and steady pressure around the middle of the nose with the thumb and forefinger, pressing the sides of the nose against the septum(the partition between the two nostrils). Tilt the head forward and have the child breathe through his mouth. Tilting the head forward prevents the blood from trickling into the back of the throat and causing a gagging sensation. These measures are difficult in younger children and it helps to keep them distracted with stories or their favorite TV show. Check after 5 minutes to see if the bleed has stopped. If it has not stopped, apply pressure constantly for 10-15 minutes and check again. If bleeding continues, it is time to see a doctor. The doctor may have to pack the nose with gauze in order to stop the bleeding. Medications that promote clotting such as amicar or tranexamic acid may help control the bleeding. Platelets may have to given to control the bleed and if the blood loss has been severe, the child may require a red cell transfusion.
Once the bleeding has stopped, follow the measures listed in prevention of nose bleeds (above) to help prevent the recurrence of bleeding. Avoid blowing the nose and strenuous activity for the next 24 hours as these can restart a bleed. Blood that has been swallowed during the course of the bleed can lead to the child having black, "tar like" stools over the next few days.
Dental Bleeds and Oral bleeds
Children with WAS can have prolonged oozing and bleeding after losing their baby teeth or even during the eruption of new teeth. If the bleeding is severe, a physician should see the child right away.
The following symptoms should be considered medical emergencies:
Nonstop bleeding of the tongue, cheek, or floor of the mouth;
Bruising or swelling of the tongue, throat, or neck; and/or
Difficulty breathing or swallowing
If a bleed occurs due to the the loss of baby teeth or eruption of a new tooth, cut and fold a small piece of gauze and have the child bite down on the gauze, applying pressure on the bleeding site. In younger children a parent may have to hold the gauze and apply pressure. Sometimes, pressure may have to be applied for 10-15 minutes to stop the bleeding. If the bleeding continues, one option is to try to use Avitene3, which is a shredded gauze like substance used to control bleeding for mucosal surfaces. A small amount can be applied to see if it helps to control the bleeding. If the bleeding continues and it is mild to moderate, amicar or tranexamic acid can be started with the permission of the physician to see if that helps. Pressure needs to be continued throughout this time. If bleeding continues despite these measures, it is time to see a doctor. One of the remedies that has been effective for patients with hemophilia is the use of amicar popsicles4(recipe given below). There is no data available in WAS patients, but this may be worth a try after consulting with the doctor. If this method of preparation is used the medication is at the tip of the popsicle and the child eats it first. It is important to clearly label these popsicles and keep them out of the reach of children. It takes one to two weeks for the bleeding site to heal completely. In the meantime, it is important to maintain good oral hygiene by brushing gently or use a cotton swab. The child should be fed soft foods that are not hot. Avoid drinking with straws and rinsing the mouth as this may dislodge the clot.
1. Defrost your child’s favorite flavor popsicles.
2. Mix 1 ounce of defrosted popsicles with 1 dose of Amicar (follow medication label for proper dosage) and pour the mixture into one 2-ounce mold. This puts most of the Amicar at the tip of the popsicle, the first part your child will eat.
3. Fill the remainder of the mold with additional popsicle juice.
4. Repeat the process for the remaining molds and freeze.
A mouth injury can take as long as two weeks to heal completely. To promote healing, serve your child soft foods, either served cold or at room temperature. To keep the teeth clean during the healing process, either brush gently or use a cotton swab. During healing, don't let your child use straws to drink from and do not rinse the mouth too vigorously, for either may dislodge the clot.
Minor GI Bleeds
Children with WAS can have bleeding when they vomit. If the bleeding is minor the blood can appear as small streaks. More severe bleeds can be in the form of large amounts of bright red blood. Vomiting of what appears to be "coffee grounds" also indicates bleeding in the stomach or the upper part of the small intestine(duodenum). It helps to keep the vomiting under control with diet and/or medications because recurrent vomiting worsens the bleed. Ice cold water or crushed ice can help lessen the bleed. Medications that promote clotting may be advised by the physician and can help. Anything more than a minor bleed needs immediate medical attention. Children with WAS can have hematorrhea (blood in the stool). Once again the bleeding can be small amounts appearing as streaks or "threads" of blood if the bleed is relatively minor, to more copious amounts with larger bleeds. A "black, tarry stool" (referred to as melena) shows that there is bleeding in the stomach or upper parts of the small intestine, or swallowed blood from a nose bleed. A minimum of about 100 ml of blood is needed to cause black, tarry stools. If the bleeding is minor, it can sometimes be controlled by dietary measures. If bleeding persists, a physician needs to be consulted to determine the cause of the bleeding and to treat it. In addition to the treatment to stop the cause of the bleeding the physician may try a clot promoter to help control the bleed. Endoscopic investigations may be required to evaluate repeated GI bleeding episodes.
Relatively Minor Injuries
In the case of an open, bleeding wound, clean the wound and apply pressure with a clean gauze. The time that pressure needs to be applied to stop the bleeding varies with each individual. Something as minor as a paper cut may require between 2-5 minutes of pressure and pressure may have to be applied for as long as 15 minutes. Check after the first couple of minutes to ensure that the pressure is working. If, after applying pressure there is still a bleed, it is best to see a physician.
Blunt force trauma can cause large and unsightly bruising. Some of the larger ones can be painful Most smaller hematomas do not cause a significant blood loss, but larger ones can. They can also get infected. Prompt icing and elevation of limbs can reduce the size of the hematoma. Redness around the area and disproportionate pain can indicate an infection and prompt treatment is needed with antibiotics. Ice can be applied for 30 minutes (sometimes longer) and repeated several times over the day as needed. An elastic wrap or Glad's "Press and Seal" or Saran Wrap can be used to keep the ice pack in place over some areas. The ice pack is more comfortable for the child if the plastic bag is wrapped in a thin cotton undershirt. It is useful to have a bag of frozen dry seed such as corn in the freezer at all times. This molds to the area of injury better and some children prefer it. If the hematoma continues to get significantly bigger despite icing, a trip to the physician is warranted. When an extremity is injured such as the hand or the leg, it is best to ice, rest and elevate in order to minimize the bleeding.