We started that day with our semi-annual tradition of brunch at the Canadian Honker with Grandma and Grandpa. We've started every Memorial Day and Labor Day this way for years. This year, it was a perfect, sunny summer day and we had a wonderful time. I was reminded that last year we had just returned 2 days earlier from Haiti and we were so torn as we had said goodbye to those two precious girls who had stolen our hearts and we had no idea how long we would need to wait before we could bring them home for good. This year, it was so special to have them here and be able to celebrate 6 months of being all together as a family.
We continued the fun by spending the afternoon together as a family at the amazing Kasson pool. This was only the second time that Chelley and Naika had been swimming in a pool bigger than a wading pool and they were so brave!! I couldn't believe how comfortable they were in the water, and Chelley was even brave enough to go down the really big water slide! It was a relaxing and fun time together and the day would've been absolutely perfect if Chelley hadn't started experiencing some tummy pain. Little did we know that that tummy pain was the beginning of a new chapter in our lives and a new adventure that we didn't see coming.
Rewind to May 2013, on our first trip to GLA, when we were summoned into the orphanage director's office just before we left to return home. She asked to speak to us and told us that they had just discovered that both Chelley and Naika had sickle cell disease. We really had no idea what that meant. She explained the basics of the disease, that patients who have this disease have red blood cells that form in the shape of sickles instead of forming into spheres like healthy red blood cells. This sickling can cause the red blood cells to not flow properly through the blood vessels and they can get stuck and bunch up. This can cause pain in that area, and also in the organs that need that blood flow. She explained that oxygen levels in the body can be a problem and that our girls won't be able to climb mountains or fly in unpressurized aircraft. This didn't seem like a big problem for us as we weren't planning on doing any of those things anytime soon! She admitted that she didn't have a lot of experience or knowledge about the full impact of the disease and we let it go at that.
When we brought the girls home, we were so consumed with just surviving the transition, and the girls were healthy all winter long, so their sickle cell disease was not at the forefront of our minds. Their OMC pediatrician had wanted us to have them seen at Mayo since sickle cell is pretty uncommon in this area and OMC just doesn't have the knowledge and experience that Mayo does for these patients. However, we didn't have any insurance coverage for treatment related to their sickle cell and we couldn't afford to have them seen at Mayo. We didn't realize at the time how critical it is for them to have ongoing care for this disease and we also didn't realize that there were state programs that could help us get the care they needed.
Fast forward to Chelley's tummy ache that started at the pool on Memorial Day. As most parents of 5 children will do, we tried to figure out many different solutions at home to try to help Chelley. At first we thought it was that she swallowed too much pool water, then maybe thought that dehydration or overheating was the problem, but rest, water, and being in the air-conditioning didn't help. Over that evening and the next day, her discomfort continued and started to get worse, and we tried pain relief and other over the counter meds and nothing made it better. She would rest off and on and we hoped that we could just ride it out. We were afraid to go spend hundreds of dollars at the Drs office for them to tell us that she had some very common ailment that could be dealt with at home. Finally, on Wednesday morning, as she continued to be in pain and then spiked a fever, we received some advice from a wise Dr. friend that we should have her brought to the St. Mary's ER and that we could be dealing with something very serious.
So, we whisked her off to the ER, where they proceeded to do multiple tests, labs, xrays, and ultrasounds to rule out all kinds of big and bad things like pneumonia, gall stones, bowel obstruction, etc. Thankfully, they were able to rule out all of those things but they were concerned about the fever, which meant she was fighting some kind of infection and they obviously wanted to help her get the pain under control, so they admitted her to the hospital. And that's when our real education began about Sickle Cell Disease and all of the ways that it impacts our newest daughters' health both now and for the rest of their lives.
Throughout the course of Chelley's 2 day stay at St. Mary's, we saw the doctors of Hematology/Oncology multiple times and learned that Sickle Cell is a disease that can cause problems in many areas and that all kinds of organs and functions of the body have to be monitored on a regular basis in order to keep these patients healthy and free from sickness and pain crisis. A week after Chelley's 1st hospital stay, both Chelley and Naika needed to receive the pneumococcal vaccine to help them be able to fight off any potential exposure to pneumonia. The next morning, they both had fevers and soreness at the sight of the shot but to be safe, they both had to come to the ER for blood draws and antibiotics. The next day we had a several hour follow up in hematology. The following weekend was uneventful and all the girls enjoyed their first day of VBS at church but on that Monday afternoon, Chelley started complaining of arm and leg pain, which resulted in another ER visit and another 2 day stay at St. Mary's to try to help her deal with the pain again.
Needless to say, these last 4 weeks have been incredibly stressful and overwhelming for all of us. We've been learning some of what it means to have children with a chronic illness and it has seemed at times that our family life will be defined by these crisis and by having to worry about whether or not 2 of our daughters can do basic things like bike riding, swimming, and playing outside on a warm day without resulting in a stay in the hospital. It's been hard and sad to see sweet Chelley in so much pain and to know how to help the other 4 as they have had their lives disrupted significantly as well. Maria and Annika have had to bear a lot more responsibility for their younger sisters than usual, and Livie and Naika aren't always so receptive to the help they need from their older sisters. They miss Mom and Chelley and have had much more time than usual without me at home. Matt and I have felt the stress of the disruption to our family life, the financial stress of mounting hospital bills with no insurance coverage (initially...more on that later), as well as readjusting our expectations and realizing what we will need to make to do to make sure that Chelley and Naika get the care that they need.
Over the course of the last month, we've gotten several big doses of information and education about what we need to do to help Chelley and Naika as much as possible. For those of you who are interested, here's the cliff notes version of what we've learned:
As I mentioned before, sickle cell patients have red blood cells that form in the shape of sickles (like a crescent moon) and therefore those cells have a tendency to not flow properly through the vessels and they can get stuck and bunch up. When this happens, there can be pain and/or damage to any tissues or organs in the body that need blood flow...which is pretty much ALL of them. So, if the vessels bunch up on their way to the lungs, kidneys, brain, etc. that can cause problems for those organs. If the vessels get stuck on their way to tissues or muscles, that can cause pain like Chelley had the 2nd time where her arms and legs hurt so much that she couldn't use them and they hurt even when she was laying down and resting. These episodes cause intense pain, and patients are at an increased risk for organ damage, stroke, etc. Also, because of the lack of proper blood flow to the spleen, their spleen essentially stops functioning by the age of 1, which means they have far less ability to fight off infection than the rest of us do. This means that every fever of 101 or higher in our girls is potentially a BIG deal that means an immediate ER visit, blood draws, antibiotics, and possibly hospitalization for 48 hours to make certain that they don't have an infection in their blood. From what I understand, something that would not be a big deal for the rest of us can turn into a pretty dire situation pretty quickly for patients with sickle cell disease.
There is no cure for sickle cell but we've learned that there are some things that we can do to keep them as healthy as possible. Both of them need to be on a low dose of penicillin and folic acid daily for the foreseeable future to help them fight off infections. There is also a drug that they have just started this week that helps to increase the levels of a healthy kind of hemoglobin in their blood. All of the medical professionals that we have talked to have said that this drug will go a long way towards keeping them healthy and preventing these crisis from happening so frequently. Even just a few extra percentage points of that healthy kind of hemoglobin in their blood helps their red blood cells to flow smoothly and not get stuck, thus avoiding the pain and organ damage. They will most likely be on this drug for the rest of their lives. We've also learned that dehydration can bring on the pain crisis so we need to be vigilant about making sure that Chelley and Naika drink enough water, especially before any kind of physical exertion or heat exposure. I ask you...did they not spend the first years of their life in a place significantly hotter than Minnesota? How were they not in pain all the time? Only by God's grace, mercy, and power was their health and well-being preserved during their years in Haiti, when it wasn't even known that they had this major disease! Another thing we can do to try to head off a crisis is to make sure they tell us at the very first sign of pain so that we can try to get on top of the pain before it becomes unbearable.
One of the doctors that we saw in the hospital recently gave us hope that our family life won't always be like it has been the last 4 weeks. That was so encouraging to this mama who was starting to feel like nothing might ever be "normal" or "easy" again, relatively speaking of course, since raising 5 girls is not usually "easy". :) They said we just need to get a baseline for the girls, get them on the necessary meds, and have a workable plan in place for when they do start to have pain, and then we won't have an ER visit or hospital stay every week or two. For the time being, they both need to have monthly blood draws, labs, and Dr. appts at Mayo Clinic but that's much better than spending days in the hospital or ER.
So, that's where we are now on this new adventure to which God has called us. We have seen so many blessings, provisions, and reasons to be abundantly thankful during the stress of the last few weeks. Stay tuned in the next day or two for the sequel to this post, where I will share many of those blessings that our generous and faithful God has showered on us so far on this new leg of our journey.
I'll leave you with a few pictures from our Memorial Day festivities, as well as some cute ones from "sister visits" with Chelley in the hospital.
