So at the second opinion dr, he took xrays of Mason AND Micah (because Micah has trouble running and sometimes walks with a stiff leg on one side and we know he has EDS but he's never had any x-rays of anything leg-related until now).
I have tried to look at this problem from all angles and really, really want our policy to be "no surgery unless the recovery is super easy or unless it is a life-threatening problem." However I am not sure we can in this case. Hip dysplasia is not a life-threatening condition in and of itself; however it is apparently a very big QUALITY-of-life threatening issue. We do not want to skip surgery if it will lead to unmanageable pain and a worse surgery later that will be less likely to help relieve his pain. This article sums it up: "Early diagnosis of hip displacement is important because treatment is more successful when children are young. When hips become dislocated and painful, it is very difficult to treat and there are not many options for treatment. If the hip dislocates, the problems that the children will have are terrible and the pain can be difficult to treat. We recognise that some parents don't want to consider surgery, especially if it's before their child is in pain. Many parents agree to surgery as a 'last resort', and many choose to try complementary and alternative medicine options first to see if they will help the hips. However, we found in this study that if children miss the opportunity to have the surgery, it resulted in far worse outcomes for the children and more pain long term."
I asked his NIH team their opinion (whether benefit outweighs risk) and the deciding factor for them was to find out if the dislocation is symmetric. Since it isn't, it becomes more pressing to fix it; otherwise he will develop severe pain at his spine where the bones rub in sitting off balance.
We will need to just be covered in prayer for this surgery if he absolutely needs to have it, which it sounds like everyone we have consulted is in agreement that he does... here are some of the specific requests we have:
1. Doctor/hospital selection. The first doctor we saw has more experience and overall great reputation but he is at a different hospital than we have ever used for surgery and therefore they don't "know" Mason as well. It would be harder to collaborate with his other doctors there.The first dr also proposes a slightly more involved surgery, with possible need for a spica cast for 3-6 weeks to months depending on recovery. A spica cast is really out of the question for Mason because of his blowout diapers happening almost daily (and a cast change under sedation is required whenever it is soiled); there is no way we could have a cast change every day or two for weeks or months).
2. Pain relief. Narcotics cause major problems for Mason (GI--slowed motility/constipation/worse reflux and neurological issues (withdrawal, seizures, urinary retention which can cause cardiac problems) so we want to limit them but yet make sure he has plenty of pain relief. The doctor mentioned the possibility of a caudal block but he wasn't sure Mason is a candidate with his other issues. We don't want him to be on a ventilator long at all as he produces so much mucous from it that he needs to be suctioned extremely often, which causes bleeding inside due to fragile mucosal tissue, which is frankly miserable.
3. Complication avoidance. Our biggest concerns are life-threatening complications arising from surgery for a non-life threatening condition. We are concerned about blood supply (Mason has "pathologically small veins" and a previous unrelated procedure failed due to lack of blood supply; because this surgery causes major blood loss and frequent need for transfusions, we are concerned about avascular necrosis, blood clots/stroke (his labs show that he tends toward issues with thick blood, and his blood clots during collection, etc,), anemia, etc. The immobility is not a good combo. He also has immune deficiency which makes bone or wound infection a very real possibility.
4. Sleep. Mason is a side sleeper but this surgery will require him to sleep on his back for at least 1 month. He can't sleep on either side due to the wounds on both legs. He needs good sleep to avoid seizures and right now won't lay on his back at all due to fear of medical procedures in that position, etc. Please pray he adjusts quickly and easily.
5. Rehab...It sounds like it could be 8-12 months after surgery for Mason to be just "back to baseline" and that's if everything goes very smoothly. Pray it doesn't take that long to get back to sitting, rolling and using stander/gait trainer comfortably, and that he can exceed that once the hips are in place. Pray especially that they will STAY fixed and never need another surgery (with EDS, muscle wasting from immobility can cause the joints to get even looser, which is another big reservation for us). We don't know if Mason will walk regardless of the surgery (it usually doesn't affect outcomes much with mobility, but is mostly a pain prevention surgery).
6. Stress. Just thinking about this ordeal makes me want some nitroglycerin handy. Please pray for all of us to lean hard on Jesus, to rest in His presence and strength, and trust Him to carry our burdens.