Pediatric aspects

Newborn babies

If the father or the mother has VWD, the risk for inheriting VWD in the newborn is 50% in most cases. Newborns with potential VWD can be born by vaginal delivery but instrumental delivery by forceps or vacuum extraction should be avoided. For severe cases of VWD with a VWD activity below 20% in the family, also, intramuscular injections and scalp electrodes, should be avoided and cord blood can be used to facilitate a quick diagnose. However, in most cases of suspected VWD, testing for VWD should be postponed since VW-activity in newborns can show doubled values compared to adult reference values (44-235%), misleading the diagnosis. For children without bleeding symptoms and family members with VW-activity above 20%, testing for VWD is recommended at the age of around one year. If the testing is done earlier, it is recommended to be repeated. Children with VW-activity above 20% can receive intramuscular injections, i.e., vitamin K or vaccinations.

Other pediatric issues

Diagnostics

In children, the bleeding score ISTH-BAT is recommended and can be used to capture bleedings [1,2]. A pediatric version is available with additional questions on neonatal bleedings. However, bleeding scores in children should be evaluated with caution since children often have not experienced any significant bleeding challenge. Therefore, despite a possible bleeding disease, the score can be low. For diagnostic subtyping of VWD, if possible, an adult family member should undergo the complete VWD diagnostics to minimize extensive blood draws, especially in RIPA analysis. Results of VWF levels should be interpreted with caution in children, especially if the blood draw was traumatic with a high stress level. When interpreting results, age-related values should be taken into account. However, general reference intervals for children, especially for infants, are missing for many coagulation assays. It is possible to find information about expected values obtained from healthy children in various pediatric text books and published studies, but the results should be interpreted with caution by laboratory expertise against the background of the local conditions regarding methods and other aspects.

Medication

For all medication, it is important to adapt the medication regularly due to growth and weight gain. Parents should be informed to avoid NSAID (Ibuprofen, Naproxen) for fever episodes in children with VWD and instead use Paracetamol. For inflammation or pain, also, celecoxib has been used. Regarding tranexamic acid, in some Nordic Countries, beside tablets, tranexamic acid is also available as oral solution and soluble tablets, which is useful in preschool children and can also be used locally, e.g. nose and mouth bleeds. DDAVP should not be used in children below 15 kg body weight. If DDAVP is administered to young children, fluid administration must be restricted, and electrolytes monitored closely. Beside i.v. - and s.c.-injections, which are dosed by body weight, also nasal spray is available for older children from about 5 years of age.

For teenage girls with heavy menstruations, oral contraceptives could be discussed or alternatively, small intrauterine contraceptive device suitable for that age group can be a solution, see chapter on women and VWD.

For children with severe VWD type 3, helmet and knee protection in the first years of life could be of value. A written emergency plan to daycare and school with information of disease and how to act in the case of bleeding is recommended for all children diagnosed with VWD.

As already mentioned in the pregnancy section, the hemophilia center should have a written plan for the pregnant mother as well as for the newborn. This must address all necessary steps for the newborn regarding diagnosis and treatment in case of bleeding etc.

1.

Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, et al. Normal range of bleeding scores for the ISTH‐BAT: adult and pediatric data from the merging project. Haemophilia 2014;20:831–5. doi:10.1111/hae.12503.

2.

Sanders YV, Fijnvandraat K, Boender J, Mauser‐Bunschoten EP, Bom JG van der, Meris J de, et al. Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric‐specific bleeding. American Journal of Hematology 2015;90:1142–8. doi:10.1002/ajh.24195.