When we first ventured into the adoption world in 2013, we had a list of needs we checked off as being “able to handle.” We were matched quickly with a toddler boy with a repaired heart condition. I often look at him now and think, “Wow, your medical needs are so easy.” We take him to the cardiologist once a year. He might need a valve replacement as a teenager or young adult. Easy peasy.
Then we ventured into the world of thalassemia, which sounds a little scary at first but once you’re in it, it’s really a very manageable need that doesn’t affect the child on a daily basis so much (besides daily medication.) Every 3 weeks our son goes to the children’s hospital and gets new red blood cells. He’s an active and healthy kid.
I can tell you that neither hemophilia nor older child adoption (or even adopting again!) were on my radar, so to speak. But God has a way of changing our hearts without us even realizing what’s happening. In March of 2016 we found ourselves embarking on the paper work trail to bring home a 10-year-old boy with hemophilia. We didn’t know exactly what type he had, although according to the medication they gave him, he had type A and based on the pictures we saw of his severely swollen knee and bulging elbows, we assumed he had severe type A. Turns out we were correct in our assumptions.
We researched about hemophilia and learned that basically all they need is the missing factor protein to be infused into their blood stream on a regular basis. We learned that people with severe hemophilia have less than 1% of the missing factor (in his case factor VIII) detectable in their blood. This missing protein makes it much more difficult for the body to form a clot when a bleed develops. Interestingly, approximately 60% of the population with type A hemophilia is in the severe category.
It sounds pretty heavy and kinda scary, but many hemophiliacs live active normal lives because they use a prophylactic treatment regime which involves infusing the missing factor intravenously two to three times a week. This usually prevents spontaneous bleeds, such as joint bleeds (we will get to that in a minute). If they were to have a bleed, either spontaneous or from an injury, they would need to infuse an extra dose of the factor and use the RICE method of Rest, Ice, Compression, Elevation. Hemophilia is not something that limits these boys on a daily basis and you would never know someone has hemophilia unless they tell you. They can play sports and work out and exercise. They are not going to “bleed out” in front of you from a cut. They do not bleed faster than people with normal factor levels, they just bleed longer and need help stopping the bleed sometimes.
Before we decided to adopt our son with hemophilia, I joined a Facebook group of other moms who had adopted boys with hemophilia (girls can have hemophilia but it is much more rare and usually boys are affected more severely). I read several blogs about having a son with hemophilia, specifically adopted. I read about the history of hemophilia and how is sometimes referred to as “the royal disease,” because it affected the royal families of England, Germany, Russia and Spain in the 19th and 20th centuries. I learned the sad and staggering statistic during the early 1980s more than 50% of the hemophilia population in the US was infected with HIV, as well as Hepatitis C. Many of those infected went on to develop the symptoms of AIDS in the following decade and thousands of hemophiliacs lost their lives. Thankfully, the medical community and the CDC learned a difficult lesson from that tragedy and the blood supply is now very safe and some patients use products that are synthetically derived without using human plasma. Even the plasma-based products are very safe and widely used today.
In all my research, the one thing I took away as a “worst-case scenario” or something you REALLY didn’t want to hear if you have a child with hemophilia is the word “inhibitor.” That’s a game-changer in regards to treatment and also quality of life. An inhibitor means that the person’s body has decided that the factor that is being infused into their blood (the very factor they are missing) is a foreign substance and the body works to reject it and kick it out; it develops an antibody. When this happens, the factor you just infused becomes essentially worthless as it does not bring the factor levels in the person’s blood up enough to have protection against bleeds. It is not known what causes an inhibitor to develop in some people and not in others. I learned that if a person is going to develop an inhibitor, it usually shows up during the first 50 infusions of the clotting factor. While inhibitors can be treated and “tolerized” (meaning the level of antibodies is reduced so that the infused clotting factor will indeed work), it is usually a long and arduous process to reach tolerization and not everyone will be able to tolerize their inhibitor. Even if you do get rid of an inhibitor, that doesn’t mean you won’t develop another one in the future. A severe hemophiliac with an active inhibitor will have to use other drugs to assist in the clotting process. These medications, inhibitor bypassing agents, do not work as well as factor products to control bleeds. It is also likely that the patient will have to take these medications much more frequently and in higher volume than someone without an inhibitor.
Adopting an older child was enough of a leap of faith for me to begin with. And while we knew about inhibitors, we had hoped that our son would not have an inhibitor since he had likely already received 50 doses of factor during his lifetime, even though he wasn’t being treated adequately. I asked the orphanage if the medication still worked to help his bleeds, and they said it did. So I figured that was the best we were going to get as far as an answer to the inhibitor question. His file contained some blood work, but no factor levels were given and there was nothing about an inhibitor showing up.
While bleeds in the head, throat, and abdomen are the most serious and life-threatening, they are rare. I learned the most common problems for people with bleeding disorders are the joint bleeds. If you don’t know anyone with a severe bleeding disorder, you likely have no idea what a joint bleed is. I know I didn’t. There are plenty of resources available online to learn all about joint bleeds and some educational videos where I found myself cringing as I watched them. The pain from these bleeds is intense and probably unlike any pain most of us have experienced. The joint fills with blood and becomes hot and swollen and unable to move. Eventually the blood is absorbed back into the body, but the damage is already done to the joint. With each joint bleed, a little more damage is done to the tissue surrounding the joint and the more likely it is to bleed again in the future. The cartilage erodes and arthritis sets in. This is why doctors emphasize the need for prophylactic treatment (prophy) for those with severe hemophilia. Most people with hemophilia are able to avoid joint bleeds from occurring often as long as they follow their treatment regime.
We had seen the picture in his file and knew his right knee looked awful, often referred to in the hemophilia community as “cantaloupe knee.” We also saw how the muscles of his arms and legs were atrophied and small, making his joints seem even bigger.
This is from the World Federation of Hemophilia and is basically what was going on with him: “If inadequately treated, repeated bleeding will lead to progressive deterioration of the joints and muscles, severe loss of function due to loss of motion, muscle atrophy, pain, joint deformity, and contractures within the first one to two decades of life.”
Unfortunately, his orphanage, for unknown reasons, had not made him “paper-ready” for adoption until he was 9 years old. He was brought to the orphanage at approximately 10 months old. He had been treated sporadically with factor while at the orphanage. I’m not an angry person, but I’ve struggled with why his file wasn’t prepared when he was much younger. I’ve had the thought that they didn’t prepare his file because they didn’t want him to leave… because he’s such a light in the world and a joyous soul. I have to believe that they cared for him and wouldn’t have knowingly let his body deteriorate like it did… and by the time he reached the age of 8 or so, they saw how he had trouble walking and how he couldn’t straightened out his knee anymore and how his elbows stayed swollen… and it was then they decided that he need to be adopted out of the orphanage. That’s the story I tell myself.
We were so excited to bring him home into our family and get him the medication and treatment he desperately needed. We prepared ourselves to learn how to infuse him intravenously and then once he was on prophy, he would begin to heal and not be in pain any longer. We knew we had an excellent HTC (Hemophilia Treatment Center) in Atlanta with some of the top doctors in the country. We found out that we would also be supported by an amazing non-profit called Hemophilia of GA that would be able to be our pharmacy for the factor medication and all the supplies we would need. They would also send out nurses to our house to teach us everything we needed to know. They would also cover our insurance deductible if we ordered factor through them. Hemophilia drugs are some of the most expensive drugs on the market. The cost for treatment is completely prohibitive without insurance coverage. There are several programs available nationwide to help families get their medication and also cover the deductible. We felt very comforted by all the support we would have as we dove into these new waters.
We traveled to China in August 2016 and the trip went relatively smoothly with a few bumps along with the way mostly related to his inability to walk long distances (he didn’t want to use the wheelchair we brought and didn’t want to be carried piggyback so we caused a few scenes, lol.) I loved him instantly upon meeting him. LOVED HIM. It was a dream adoption as far as the bonding and attachment part. We got home and went to his first visit at the HTC in September, less than a week after arriving home. He walked in with his limp (like he always had.)
The nurses took one look at his knee and felt it and asked the question my husband and I had asked him since we met him, “Does it hurt? Is it painful?”
And he gave them the same answer he have us. “No. Doesn’t hurt.”
They were pretty sure he was having an active bleed in that knee. The kind of bleed that should’ve put him in a wheelchair. He had always refused to say that he was in pain, but when they showed him the “faces chart” which uses smiley face to frowny face, scale of 1-10, for pain, he point to an “8” for his right knee. So, at that point I realized that either he didn’t want to admit he was in pain or he was so used to living with the pain that he was numb to it. They gave him some factor at the clinic and did some other blood work. The doctor went over what hemophilia was and what his treatment plan would look like and why…. I still have the piece of exam table paper where the doctor scribbled his notes… and then we he finished, he said, “But this is assuming he doesn’t have an inhibitor. If he develops and inhibitor, just forget all this… it’s a totally different ballgame.”
We went home but the knee didn’t seem to improve with the factor dose. A couple of days later we got our shipment of factor from the pharmacy and a nurse came to our house to infuse him. As she was giving him the factor, her phone rang. She disregarded it until she was finished. She then checked her voicemail and it was the clinic nurse calling to tell her NOT to give him the factor because they had the results back from his lab work… it showed he had a high titer inhibitor. Gut-punch. Not what you want to hear.
She explained that now it made sense why his knee didn’t improve with the factor they had given him and started talking about how they could work on getting rid of the inhibitor and how he would get different medication for now.
I was still processing it all as I looked at my new son whom I loved so much, whom I wanted to heal and to take away the physical pain he had lived with for so long, whom I wanted to make strong and whole… and it started sinking in that this road had just taken a sharp left where I had planned on going straight.
And when the doctor called me from his cell phone on that Friday afternoon and spoke to me about levels… and high titer and low titer… and what immune tolerance induction was, my head was spinning. I got off the phone and went to my husband who was in his home office and broke down into tears. Oh, how I wanted to help my son and how helpless I felt. It was like the hill we were climbing (which was already strenuous enough for me!) suddenly became a perilous, rocky mountain with no clear path.
For the next several months we dealt with trying to infuse intravenously, then realizing that sticking our new son, who had typical emotional issues related to adoption, with a needle on a daily basis was not a great plan… then getting a PICC line to infuse an inhibitor bypass agent each morning, yet still he suffered from a major joint bleed (usually in an elbow) every 2-3 weeks. He was nowhere near stable. The medication didn’t really work to stop the bleeds and he had to take prescription pain meds to sleep when he had a joint bleed and often that didn’t even help. He was better than he was in China, but only marginally. But in between those joint bleeds, he was loving life, making friends, playing outside, learning what family means.
It took about 5 months after our first visit to the HTC for things to calm down with his joints. We took him off the initial bypass agent and are using a drug that works in a different way. Still not as the same as factor replacement, and doesn’t stay in the bloodstream for long enough to offer good protection, but it seems to be working for now. We also decided to embark on ITI (immune tolerance induction) which is when you bombard the body with high doses of the factor that it is rejecting in an effort to get rid of the inhibitor, same theory as allergy shots in a way.
What this means is that every morning before school, I access his port (he got one in January after we realized he would need high volumes of medication every day for the foreseeable future.) I create a sterile field on my dining room table and put on a mask and sterile gloves. I used a special needle to access the port and then infuse the factor and the other medication he takes. The whole process takes about 25-30 minutes including mixing the medicine and waiting 10 minutes in between infusing each medication. And while they say it’s possible to have more bleeds when you begin ITI, he’s had less. His joints look better and he hasn’t had a major joint bleed since we started. We are still early in the process, but now I can say I feel like we’re floating calmly instead of swimming and sinking, swimming and sinking. And the perilous, rocky mountain doesn’t seem so daunting… and the view is AMAZING. It’s one step at a time… a marathon, not a sprint.
Over the past 7 months since we adopted Andrew, we have experienced the highest of emotions and the lowest. He is a resilient and positive kid. He is loved by everyone. He’s made great friends at school, his teachers adore him, the hemophilia clinic nurses and doctors love him. He performed a Chinese song in the school talent show after only being in the school 3 months. He’s learning to swim, kinda, lol. He plays basketball and football at recess with the boys (yes, I tell him often to “BE CAREFUL.”) He LOVES sports. God bless him, he was born to be a sports star in his heart. He looks at his new muscles he’s developing through typical kid activity as well as physical therapy and says, “Feel my muscle, is so big.” He is relishing in all of these new adventures in which he was never allowed to participate in China.
As with most adoptions, especially older children, there have been emotional meltdowns. When he first came home, he had very little ability to regulate his emotions. Often times, his most difficult moments were those where he was in pain from a bleed… completely understandable. Yet, it is hard to watch your loving, joyful, kind boy spiral down out of control to where he screaming, yelling, crying, trying to make the pain worse, etc. It’s hard, but I get it. I know when he acts like that, it’s not really him… it’s not my Andrew. And through my husband and I talking about it with him over these past several months, he is slowly learning that it’s good to seek out help when you’re hurting, and hugs aren’t just for happy times, but also for sad times. We talk to him about how holding anger in his heart doesn’t make things better, even though we know why he feels that way.
The last time he had a joint bleed, we had one of the most cathartic moments I’ve ever experienced in my life. He was up in his room, shutting down and shutting out everyone even though he wanted the attention and for us to know he was in pain. Yet, he wouldn’t accept our attempts to console him, or support him, or just to hold him. He doesn’t even know why he is angry, but this is how he dealt with pain at the orphanage. No one helped him, no one was there to hold him or rub his head and sing to him… he had to deal with all of that pain from all of those joint bleeds pretty much on his own. He would get an ice pack from a nanny but that’s about it. And when I went up to his room that day, I told him as I choked back my own sobs that I was so sorry he had to do this all on his own in China, and I was so sorry he didn’t have a mommy and daddy to help him with his hemophilia all of these years, and it wasn’t fair and it made me mad and very sad, too. With that he broke down… and all his hurt came out… he leaned into me and let me console him and hug him and be the mom he had needed for so long.
Through all of the challenges, or “opportunities to show grace” as I like to think of them, I can honestly say adopting our older son with severe hemophilia with a high titer inhibitor has been one of the most joyful blessings of my life. There hasn’t been a minute that I’d go back and do it differently. In fact, I’m thankful we didn’t know all that we were signing up for! If I had been given a picture of what we would have to tackle before we said “yes,” I don’t know if we would have said “yes.” And THAT would have been a tragedy.
Andrew’s treatment regime is just part of our daily routine now. Is it more involved than we originally thought it would be? Yes. So what do you do? You adjust your expectations and just do what you gotta do, as we say. And now that his bleeds are more under control for the time being, we feel like we are coasting along, finally… for now. I have high hopes for his future and the future of hemophilia treatment. There are exciting new drugs in the trial phases that will drastically change treatment of hemophilia for the better.
When I think about his adoption, the word that comes to mind is “thankful”…
Thankful he survived in China without proper treatment
Thankful that we have different medication options here to treat him
Thankful he bonded so easily with our entire family
Thankful he makes me feel like I hung the moon for him
Thankful he always apologizes to us after he loses control of his emotions
Thankful he teaches me every day what BRAVE looks like
Thankful that I get to witness pure JOY pour out of him as he lives his life to the fullest…knowing he is loved unconditionally.
Red Thread Advocates 