Avleigh's Journey

Avleigh has always struggled with her sleep from the time she was born. Imagine those first few week that you bring home your newborn… we’ve had this for the past 4 years! We’ve done all we can while keeping her safe to try to promote good sleep hygiene. We recently repeated a sleep study that identified her sleep apnea is progressing. Luckily the apnea is obstructive and a “simple” tonsillectomy can fix it. But is it really that simple? It would have been prior to her Von Willebrands diagnosis.

We met with her ENT specialist and went over all the details of what is to come with this procedure. Along with the pediatric sleep apnea, she has diagnosed her with laryngomalacia. This is the softening of the tissue of the voice box causing the tissues to fall over the airway opening causing more difficulty in breathing. During surgery she will remove the tonsils and adenoids but also perform a scope to determine if the “flap” needs to be clipped.

So what does all this mean for a child with Von Willebrands? It means our team of doctors had to be in communication to coordinate and determine a plan to keep her safe during one of the most dangerous procedures for a blood disease patient. You see, any procedure to the upper part of the body is a high risk for a person with blood disease. And in communication they were! When we left from the appointment everything was all lined up.

On February 4th, Avleigh will have her tonsillectomy. We will begin her medicine via her j-tube prior to the procedure and have her factor prescription on hand for the procedure. With this being her first procedure with this disease being identified, they are taking all precautions to ensure they keep the bleeding controlled. During the procedure they will also insert a temporary PIC line. This will assist us in administering her factor medicine post surgery. She will need to get the IV form of the medication for 10 days after the procedure. She is a very hard stick, so they have been proactive in lining up the PIC line in order to keep her as comfortable as possible with less sticks.

Once the surgery is complete, she will spend at least one night in PICU. They will monitor the bleeding and recovery very closely. The doctor said that she will be released when we both are comfortable that she is okay to go home. It could be one night for 14 nights… we will find out as she recovers. Prayers this procedures is successful and gives her the relief she needs for better nights rest!