If you ask Charles what his favorite things in life are, he will reply without hesitation that his wife Amber, and two sons Jamey (three) and Max (four) are the lights of his life. Although Charles shares a similar passion for family with many other fathers and husbands, Charles is unique because he suffers from severe hemophilia. Instead of wrestling with his young boys like most fathers, Charles found himself unable to do so due to the severe deterioration of his left knee. After having to consistently miss out on play time with Max and Jamey, it became clear that the only thing Charles could do to improve his situation would be to get a knee replacement.

Born in 1984 amidst the height of the HIV hysteria and inflicted with target joint bleeding in his knee, Charles’ parents decided to only infuse him with Factor 8 when absolutely necessary in an effort to protect him from HIV and tainted blood products. However, as a result of his parents’ cautiousness, Charles’ knee grew progressively worse and he experienced some of the most painful bleeds imaginable. To try to inhibit the bleeding, Charles visited a doctor who gave him some ill advice consisting of immobilizing his knee by casting it for 18 months. The results were tragic; Charles lost the majority of his range of motion, his muscles severely atrophied, and the growth in his leg was stunted leaving him with a severe limp and a shortened leg. Despite the struggles with his knee, Charles was able to escape the ‘80’s HIV free, and in 1992 underwent a left knee synovectomy which helped reduce the number of bleeds and pain but regretfully ensued in a larger loss of motion. Over the next 18 years, Charles soldiered on by keeping the rest of his body strong through lifting weights and working out at the gym.

It wasn’t until the Spring of this year that Charles decided he had had enough and that it was finally time for him to get his knee replaced. He started the search for other hemophiliacs who have had knee replacements and through his research was able to find a doctor who would treat the knee surgery and rehabilitation as equally aggressive as a normal patient; reminding him that he would be fully dosed up on factor 8 and therefore would have no reason to hold back. On June 29th 2011 Charles successfully had his left knee replaced and after staying in the hospital for only two nights he was able to walk out of the hospital on crutches with a brand new knee. The cast was removed on the third day of post op and Charles was able to immediately put weight on it. Range of motion therapy for Charles was aggressive with him opting to skip the passive motion machine after the first few hours and move on to an exercise bike to break up the scar tissue (a process that has been compared to the pain of child birth).

Due to the aggressiveness of both his therapist and Charles himself, Charles has achieved incredible results in regards to his range of motion. Today Charles’s knee can be completely straightened out to zero degrees and bent to the capacity of 123 degrees, whereas beforehand his range of motion was ten degrees straight and 90 degrees bent. His range of motion was not the only good result of surgery, the surgeons were also able to actually lengthen Charles’s leg and remove the bow he developed as a child by inserting a replacement that would normally be used for a twelve year old.

If you ask Charles about his progress he would tell you it’s based on three things: determination, hard work, and lots of Factor 8. Charles infused to 100% for an entire month after surgery and even now he infuses prophylactically to about 50% as he continues working out and building joint and leg strength. Charles now walks with a cane and is learning to walk with a normal heal/toe gait. He spends a lot of time on his “simple” bike at home accompanied by stretching exercises. He is mostly pain free except for the days when he pushes himself too hard.

Charles’s knee replacement has been a great success. After being told by many that he could never have a knee replacement because of his hemophilia, Charles persevered. Instead of being defined by his hemophilia, Charles has shown everyone that anything is possible; including wrestling with your boys.

X-Ray of my right ankle fusion. Notice the broken fibula!

My bleeding episodes occurred in my left ankle during my elementary school years. I remember my physical therapist fashioning different plastic molds in an effort to stop the bleeds and alleviate the pain. Crutches were part of my regular fashion statement at school and despite the jeers of some bullies and others accusing me of “faking it” I continued to put on my brave face and soldier on. Back then during the 70’s Hemophilia did not have home treatment protocol. Bleeds often became so severe and painful that they required in-patient stays at the hospital. I became a regular at Primary Children’s Hospital and spent many nights crying myself to sleep, my father rubbing my ankle or back to try and help me relax from the crushing pain.

Eventually my left ankle finally settled down when I was about 12 years old. Then I promptly became an avid sports fan and discovered the joys of pickup basketball and tag football games in my neighborhood.  My right ankle immediately decided it was its turn to bleed. Luckily we did have home infusion treatment in place by then and I was able to stop the bleeds before they were out of control. However, my right ankle along with my left knee continued to be target joints bleeding almost weekly with my left ankle occasionally joining in on the act.

A painful worn out joint is the end result to all of this bleeding and an ankle fusion is the best remedy to fix it. I eventually could not walk at all without severe pain and tendonitis around my left ankle and decided to get my first fusion in 2006 at the age of 41. Ankle fusions are actually a pretty straight forward surgical procedure. Five long screws are drilled deep into the ankle and tibia from two incision points. One is laterally on the side of the joint and the other directly over the top.  They have to physically break your fibula during the surgery to better access the joint area. The joint is set at a right angle 90 degrees and soft splinted for two weeks. That first two weeks is the most painful but can be controlled pretty well with pain meds.  Following that you are put in a hard cast for an additional four-week period. There is no weight bearing at all during this six-week period, which makes it very difficult to function in your daily activities.  Finally you are placed in a walking boot for 4 – 6 weeks and about 8 weeks post-op you are finally allowed to put some weight on the ankle. The boot does a great job of keeping the joint stable and I did not experience hardly any pain at all while walking in my boot.

Both of my ankle fusions went like clockwork. Because I infused to 100% every day for the first few weeks I did not experience any bleeding problems. My ankle healed and the bone fused together around the screws eliminating the pain. There is no physical therapy for this procedure. You pretty much are on your own and the process of walking again generally stretches out the foot and works the post-op pain out over a period of several weeks. Your foot bones and toes take over the bulk of the bending when you walk so they will get sore from time to time and may possibly get worse over the long term, but generally speaking you will feel like you have a new ankle and best of all no more pain! Your ankle actually still bends although somewhat more limited but I now have two pain free ankles and am able to walk with a semi normal gait doing the things I enjoy including golfing and going on long walks with my family.

If you have an ankle joint that has basically been destroyed by a lifetime of bleeding and are experiencing severe pain don’t be afraid to get this procedure done – it is not an easy process but the end result is fantastic and you will be so glad that you did it. If you have any questions at all about this please email me! thefeel@mac.com

Twenty –one year old Andrew Schnitker has always loved football and hockey; and even though he has severe factor VIII hemophilia, these sports will continue to be a huge part of his life.  That’s right, because even though Andrew can’t play these sports due to two target knee joints, he has high hopes of becoming a sports broadcaster after his graduation next December.

Andrew grew up in Dallas, Texas, and also has a brother who has hemophilia.  His mom knew she was a carrier because her dad had hemophilia, so she knew Andrew would have hemophilia before he was born.  “The thing I like least about having hemophilia is having target joints; both of my knees are bad. I had a port a cath from age 5 to 13, and started prophylaxis then since I started developing target joints before I was five because I had an inhibitor,” he recalls.   Andrew’s parents kept up his prophylaxis and he eventually beat his inhibitor.

When he was a boy, Andrew loved to watch the Dallas Cowboys football games and remembers watching the Dallas Stars hockey team win the Stanley Cup with his dad.  He really wanted to play, but was never allowed to.  “Mom and Grandma wanted to keep me safe,” he says.

Since Andrew has always loved sports, and has been acting since he was five years old, he has found a way to combine the two into a career.  He has been studying communications at the University of Texas in Austin, and hopes to someday have a job in the sports field.  You see, there is more than one way to have a career in sports; Andrew hopes to possibly be a sports writer, editor, producer or reporter.  He says, “I love sports, it’s something that brings people together, and to see the drama unfold is a thrill.”

Andrew is the president of his fraternity and says he has spent the last four years teaching all of his fraternity brothers about hemophilia.   He says, “I would rather have people ask questions and learn about my bleeding disorder than try to hide it or be embarrassed by it.  It is a disorder that not a lot of people know about and I want to clear up their misconceptions.  I would never want to use this disorder as a crutch.”

Andrew currently does a Radio show on campus.  “I’ve been the sports director on 91.7 KVRX for a year.  I do the Longhorn College sports talk, it is an FM spot Tuesdays at 7:00PM,” he explains.   He started out as a guest on the show, and finally was given a show of his very own.  He jokes, “I just kept volunteering until they gave me my own show!”  He says, “I have also done an internship with ESPN radio.  I love it because you get to create a character.  You can show up in athletic shorts and a T-shirt, it is such freedom- so enjoyable.  It’s a stress release for me.” He says he is not sure what his eventual job will be, but for the summer he has an internship with the CBS affiliate, doing sports writing, producing editing, and maybe even some reporting.   He is excited about his future and says he can’t think of anything better than to get paid for doing something you love.  He even thinks he would be willing to move anywhere, just to get a job in sports.  He says, “It’s funny, when I was in school, I hated English, but loved geography and history.  Now I may be a sports writer!”

Andrew’s advice to other kids with hemophilia is, “Don’t put yourself in a box that says, “I am a Hemophiliac!” It does make you unique, but you just have different challenges.  Embrace it, but don’t let it change who you are.  And Andrew’s advice about going to college: “Choose a major that you’re interested in, or really like.  College will provide some of the best experiences of your life…learn as much as possible and get involved at your school.  Everyone in college wants to meet new people and learn about different cultures, so jump in and embrace it!  The more learning and growing you do during your college years the more likely you will be to find the job you really want!”  That sounds like good advice from a twenty-something who has it all together and will soon be reporting from the world of sports.

The following article was written by our first guest blogger – David Simmons. It chronicles his decision on getting a knee replacement despite having severe Hemophilia. 

I had always known, following an inability to get rid of a stubborn limp after college and upon receiving several opinions from orthopedic surgeons, that I would someday require total knee replacement surgery.  My left knee had become a “bone on bone” situation after many episodes of repeated bleeding into that joint as a child.  Over time, the amount of cartilage remaining in the joint slowly diminished.  I remember one physician who took a glancing look at my x-ray and simply exclaimed, “ugh”!  Being a 34 year old severe hemophiliac, I naturally had concerns about how such an invasive procedure would affect my life, my ability to work, and to participate in activities that are important for fathers to participate in with their children.  Are the results guaranteed?  Certainly not, as with any surgery, the procedure comes with certain risks.  However, the potential benefits of the surgery, in my opinion, far outweighed any risks involved, even for a hemophiliac.

Approximately one year prior to my knee surgery, which took place in December 2008, I had contemplated the surgery but ultimately did not execute.  One of the main reasons I decided not to go forward with the surgery at that time was my physical condition.  At the time, I weighed nearly 220 pounds and generally felt tired and out of shape.  Over the next year, I committed myself to following a regular exercise regiment which I still follow today.  I never knew how much exercise can make a difference in the way you feel on a day-to-day basis.  Yes, there is one form of exercise you can perform as a severe hemophiliac with a blown knee…swimming.   As I continued swimming, I gradually benefited from more energy each day, a lower weight, and a trimmer waistline.  I felt that it was important to be in good physical condition to have the best chance for the knee replacement surgery to be a success.

Even though I knew I needed the surgery sometime in the next few years, I had no idea that the surgery would be occurring so soon.  An opportunity had emerged at work whereby the senior managing partner at the commercial real estate firm I work for would be gone for an extended period overseas.  Since I report to this partner, it was the perfect opportunity for me to work-from-home, to the extent possible, while recovering from the surgery.  I was told that it is often a six-week period that patients are not able to return to work.  The idea was met positively at work, so I decided to go ahead with the surgery; however, getting on a surgeon’s schedule on short notice at the end of the year is easier said than accomplished.  Many patients choose to have surgery over the Christmas holidays because they have extended vacation time and also want to get the surgery done prior to the end of the year to avoid paying the full amount for deductibles and co-pays in connection with their health insurance policies. I had to beg and plead with the various doctors, nurses, and rehabilitators to get everything lined up within a two-week period of time or else my window of opportunity would soon close.

With regard to the surgery experience, it was about what I expected.  I was nervous prior to the surgery, but everything went very well and the surgeon gave a good report to my wife and mother, both waiting anxiously in the waiting area.  Part of the precautions taken for any surgical procedure taken on a hemophiliac involve having clotting levels at 100% for a sustained period of time.  I was very concerned about the frequency of my infusions of Factor VIII, and was becoming aggravated with the nursing staff who didn’t completely understand how important the timing was for the infusions.  They would often show up 30 minutes to an hour after the scheduled time.  Although I’m sure an hour either way doesn’t make much difference, in my mind, I wanted everything to be right on schedule.  After all, I really needed my knee to work for me.

 Did it hurt?  Well, I was fine until the anesthetic injected into the knee during surgery wore off.  It was Christmas day and my wife and children were there with me, in the hospital room, opening presents.  I remember how difficult it was to tolerate the pain in my knee while trying to hide may pain from my children in an effort to keep the focus on them.  After a narcotic mix finally arrived, I had some relief.  The difficult part of the pain experience persisted for about a week after surgery.  It was difficult to be totally comfortable and physical therapists always expected more that you could deliver.  However, the paid did subside a bit, and I did slowly begin to gain more range of motion in my knee.  After a 4 day hospital stay, I went home.

My rehabilitation at home involved an in-home visit from a physical therapist a couple of times per week followed by outpatient physical therapy sessions for about two additional months.  The sessions involved low impact repetitions of muscle strengthening exercises followed by stretching.  The stretching exercises were sometimes painful, because the therapist would often need to “break through” a point in the range of motion where I had difficulty meeting the goals.  For the first few weeks, I used a walker to get around the house.  After a while, I ditched the walker for a cane.  Finally, I ditched the cane.

Now, my knee freely swings through a normal walking stride without pain.  Although my muscles surrounding my knee are still much smaller than the muscles surrounding the other knee, they will eventually gain mass and strength.  Whatever limp I once had is now severely diminished.  The pain I once experienced in my knee on a daily basis and at night when I would sleep is now gone.  I am now able to walk longer distances, stand on my feet longer, climb stairs, play low-impact sports and other activities I could not perform prior to the surgery.  Although the prosthetics will always need to be reevaluated by x-ray and precautions taken to avoid any infection around the knee, I feel that the absence of the daily pain that I once had and my increased physical ability far justify any issues that I may have to deal with concerning the prosthetics.  Overall, I am very happy with the outcome and would make the same decision were I to have the choice again. 

Written By David Simmons