Thinking Out Loud!

My posts have been less frequent not because I lack things to post about but instead because I have ramped up Sam's home program and we have all been a little sick lately.

Sam picked up a respiratory infection during his visit to Pulmonary at Children's Hospital and decided to share it with most of the family. I am trying to get in to see all of Sam's doctors before we leave for our trip to California in June. His pulmonary appointment went fine. Sam's lungs are great when he is healthy but they take a really bad turn when he becomes ill. As his doctor listened to him breath she said it is amazing how good he sounds when he's healthy but it is equally amazing how quickly his lungs can take a dive when he gets sick. We discussed how Sam did with his last hospitalization for double pneumonia. I explained how we once again saw Sam do really well with the respiratory treatments up until the point that he turns a corner and then the treatment begins to irritate his condition more than it helps. Whenever Sam has been hospitalized we have found that a slow steady course of treatment is our best approach because if the treatment gets too aggressive or goes on for too long he begins to turn a very scary corner where his heart rate increases and his respiratory decreases almost as if his body is saying "Stop, I can't handle anymore." His doctor again acknowledged that our best line of defense with Sam is to limit his exposure and try to keep him healthy. We have tried preventative medications in the past such as a regularly scheduled dose from an inhaler but Sam's system did not tolerate it well and he became hyper, aggressive and manic. I am hoping that the nutritional protocol that has helped Sam tolerate supplements better will also help him with pharmaceuticals. It gets a little scary when your child has reactions not normally seen or the medication seems to have the opposite affect that a doctor would expect.

Yesterday we had an appointment with Sam's pediatrician. Due to Sam's medical issues our pediatrician is the director or point person for all Sam's other specialists. I rely on him to help us negotiate our way through hospitalizations and to help set Sam's treatment and therapy protocols. He helps Jeff and I make medical decisions for Sam and his opinion and medical expertise is very important to us. It is vitally important that we are all on the same page. Although Jeff and I are Sam's parents and probably know him best it is equally important to have a medical doctor who also knows and understands Sam and can speak on his and our behalf to physicians and surgeons that require more technical information. We talked over Sam's pulmonary appointment and I reviewed with him Sam's respiratory protocol during illness which includes Xoponex in nebulizer and inhaler form, Ipatropium Bromide and the oral steroid which we use as a last resort. We also reviewed Sam's continued use of the acapela and other breathing exercises to help increase his lung capacity.

We then focused our attention to Sam's hip and I sensed...some disagreement. So this is where I'm going to use this post to think out loud. As much as I like to live one day at a time and I like to focus on the here and now...Sam's hip issue is requiring me to research, think, pray, research some more, pray some more and do some "what if" planning. What if we go into Ortho in June and the x-ray shows no forward progress??? That's a big "What if". On the flip side if we go in and we see some white fluffy stuff on the x-ray I'm willing to sit back and give it a little more time even though Sam's current mobility is marginal. I would be thrilled to learn that the supplement protocol is not only helping Sam to be healthier but it is impacting his body's ability to grow new bone. If we could save Sam's hip I still think that would be the best outcome.

But what if...there is no bone growth?? We would be 1 year and 6 months post surgery. My gut is telling me that if there is no bone growth by this point all the waiting and hoping in the world isn't going to make it miraculously happen. And if we continued to wait...each year is another year that Sam is not able to enjoy a good quality of life.

Now some people would say "I should be happy". Sam can transfer himself, he can walk for short distances with a walker, he is still moving. But I'm not happy because I remember how active Sam used to be. He learned to ride a bike, he loved to hike, he went tubing, jet skiing and horse back riding, he was doing chores and he had more independence. Some people might even say "things change" and you have to accept this new reality just as you have had to accept Sam's other medical issues. But see...that's just it...I'm not really good at that.

I have spent 11 years working with Sam to enable a child that so many people would view as disabled. I am not in denial of Sam's multiple diagnosis and the challenges they present but I'm also not going to let those challenges stand in the way of Sam reaching his full potential. When Sam didn't crawl we worked with him to help his brain reform the connections and pathways that he was missing. When Sam didn't speak we checked and rechecked his hearing and finally got him a bone conduction hearing aid. And now that Sam struggles with walking again I want to do everything I can to help him regain his mobility.

I want to think out loud for a moment about the concept of "quality of life". Prior to his diagnosis of Perthes and Hip Dysplasia Sam was an active 9 year old boy. He had learned to ride a bike, he loved to boat, jet ski and go tubing and he enjoyed playing basketball. Since his surgery Sam has gotten back on a bike but he is unable to push on the pedal with his left leg, so he no longer can pedal or enjoy a bike. Sam can still shoot a basketball but the effort of walking after the ball or bending to pick it up keeps him from enjoying this sport. Sam has gone boating, tubing and jet skiing but these activities cause him further hip pain. Currently Sam uses a walker to get around the house or for short walks into therapy. He uses the walker because his gait was getting worse and worse without it. If Sam walks without the walker he tends to hold his breath, curve his spine to the right and his limp is severe. Walking without the walker will lead to Sam choosing to crawl at some point. Sam still can not fully weight bear on his left leg and if he is standing he will choose to weight bear on his right leg and only uses his left side for balance. And those are just the physical issues.

Before the surgery Sam was moving further and further along in his homeschooling with the addition of chores. Sam was loading, unloading the dish washer, he was loading and unloading the washer and dryer, folding clothes, feeding and caring for Buddy, emptying the garbage cans and taking the garbage out, he enjoyed going to his Dad's shop and helping wash trucks. He was active and that activity kept him wanting to learn and do more. He understood the need and purpose of doing and learning those activities. We often broke up his academics with some type of physical activity like playing basketball or going for a walk. You can bargain an extra 10 minutes of math when you are promising a hike afterward. And then came surgery and a lot of these chores and activities had to be modified or removed. Our house is not handicap accessible so getting a wheel chair into the laundry or throughout the kitchen isn't really an option. And as creative as I can be my hands still feel tied at times as I deal with Sam's drastic change in mobility. If I'm being perfectly honest teaching a child with Sam's cognitive, speech and hearing issues is a pretty big challenge and yet we were being successful...add in a major physical challenge and that has really been a deal changer. I've moved furniture in the house to accommodate Sam's walker, I've come up with new chores and modifications to some of the chores, I have learned to work with Sam in whatever position he finds most comfortable...laying down, in the recliner, sitting on the floor or laying in bed. I have changed a lot of our academics over to the computer and Sam's Ipad for easy accessiblility. He is still learning...but a part of me can't seem to let go of how much easier all this would be if Sam was more mobile.

Soooo...let me invite you into my head for awhile. If we don't have bone regrowth we can A) Simply continue to wait, hope and pray. This would be an easy solution but would continue to limit Sam's mobility and his quality of life. We would still have to do surgery to remove his existing hardware to allow growth and he still has a leg length issue which would require some type of surgical intervention in the future.

B) Look into another surgical procedure like the external fixator but this isn't a guarantee for bone regrowth and I would question the chance for success because of Sam's lack of bone growth with the osteotomy. Every surgery has added risk for Sam. Due to his respiratory issues anesthesia is always a concern. Sam has to work on his physical rehab as well as his respiratory rehab. Sam has balance and sensory issues and the external fixator would most likely post challenges for Sam that other children may not have. Pin care is demanding and Sam fought through his incision care on the last operation. This could be the longest 5 months of pin care anyone has ever experienced. Also keep in mind we have cognitive issues and because of the amount of medical interaction Sam has had in his life he is medically defensive. I think Sam would be beyond frustrated if we went through all that and ended up at the same point we are now with no bone growth.

C) Go forward with a total hip replacement. In my mind...this takes us to a final operation and one that may give Sam the best chance for a better quality of life. This surgery would give Sam a new hip, hopefully better mobility and would take care of his leg length issue. But much like anything in life there is no guarantee. Total hip replacements come with their own risks and Sam's medical issues add to those risks. The following is an article on hip replacement that I have modified to reflect Sam's concerns.

Total hip replacement is one of the most successful and cost effective interventions in medicine. (Hmmm...please keep in mind we heard this about osteotomy too) It offers reliable relief of pain and considerable improvement in function in patients suffering with osteoarthritis or inflammatory arthritis of the hip. (Sam's diagnosis is Perthes or AVN which will lead to the conditions mentioned) Currently 300,000 hip replacements are performed annually worldwide. Two thirds of these are performed in patients over 65 years of age. Ninety to ninety five per cent of patients can expect to have their total hip replacement functioning at 10 years, and in 85% they will still be functioning at 20 years. (10 to 20 years without pain and better mobility is the goal. That would take Sam into his late 20's early 30's)

Although 85% of patients undergoing total hip replacement have a diagnosis of osteoarthritis, inflammatory arthritis, both seropositive and seronegative, is an important indication for total hip replacement and can offer tremendous improvements in quality of life even in the very young. In rheumatoid arthritis it has been estimated that the cost to the community of total hip replacement will be recuperated within an average 1.5 years because of savings made on medical and social care.

Quality of life after surgery approximates that of a healthy reference population. Improvements in pain, energy levels, sleep, social function are all observed. (Wouldn't this be great during Sam's early years when he is experiencing and learning more) Oxygen demands on activity are decreased and walking ability improves. (Two very important aspects when thinking about Sam) Most of these improvements are seen within three months of surgery. These gains in quality of life allow large numbers of patients to retain their independence and function more actively in society.

Indications for surgery

PAIN

Pain is the principal indication for hip replacement and is reliably relieved as early as one week after surgery. Pain from an arthritic hip is classically located in the groin and buttock. Radiation into the thigh may occur and at times pain may present in, or even below the knee. The pain is usually described as a dull ache that is difficult to localise. Activity aggravates the pain while rest relieves it. Increased activity during the day may be followed by pain extending into the evening. Night pain is particularly distressing to patients and an important surgical indication. (This is Sam's every day)

FUNCTIONAL LIMITATIONS

In osteoarthritis functional limitations are usually associated with pain but are rarely an indication for hip replacement in isolation. Walking and particularly stair climbing become difficult, with start up pain a particular feature. Capsular contractions and joint deformity cause a decreased range of motion in the hip, which typically leads to patients complaining of problems with pedicure and putting on shoes and socks or stockings. Functional limitations are usually more severe in patients with inflammatory arthritis reflecting the systemic nature of their disease. Functional improvements can be confidently predicted in patients with osteoarthritis and rheumatoid arthritis after total hip replacement. (Due to Sam's joint laxity he can put on his socks and shoes but walking and stair climbing are both major problems)

STIFFNESS

There are certain patient subgroups in which joint stiffness without hip pain is an indication for surgery. In ankylosing spondylitis hip stiffness, or even ankylosis, can leave patients with tremendous functional disability in the absence of pain. Hips commonly fuse in flexion, which in combination with a stiff thoracolumbar spine contributes to a stooped posture. These patients may become bed bound and if mobile often require walking aids. Hip replacement in this group leads to dramatic improvement in function allowing bedridden patients to ambulate and most patients to discard walking aids. (Here is where Down syndrome is a plus in Perthes and AVN, Sam's lax joints and low tone actually allow for less stiffness in the hip)

Patients who have had previous hip fusion, either spontaneous, usually from childhood infection, or surgical may also present with disability in the absence of hip pain. Disability in this group can be caused by back pain, pain in the knees or the opposite hip, as well as the disability imposed by the stiff hip. Although a hip replacement in this group of patients is technically demanding and associated with a higher complication rate than normal a moderately good range of movement can be achieved. Furthermore some surgeons feel that an arthrodesis should be taken down before another affected joint is replaced so as to minimise the risk of failure of the arthroplasty.

AGE

While most hip replacements are performed in patients between 60 and 80 years of age, older or younger age is not a contraindication to surgery. Hip replacement is occasionally performed in patients in their teens and early twenties. In this age group its most successful indication has been in relieving pain and improving function in patients with debilitating childhood inflammatory arthritis. In one study of patients 21 years or younger over 90% implant survival was seen at 10 years in patients with juvenile arthritis. This severely affected group of patients enjoyed a greatly improved quality of life over this period. Twenty five per cent of North American surgeons in one survey considered a patient’s age over 80 as a factor against total hip arthroplasty. We do not agree that older age should preclude a patient from total hip replacement and we do not think there is an upper chronological age beyond which patients should not be considered for total hip replacement. Elderly patients undergoing elective hip replacement for either osteoarthritis or rheumatoid arthritis have a greater life expectancy than average probably reflecting less co-morbidity in patients considered fit for elective surgery.

RADIOGRAPHIC CHANGES

The severity of the radiographic changes of arthritis within the hip joint usually but not always reflects the severity of the patients’ disability. However the decision to perform surgery is made on the severity of the symptoms not the severity of the radiographic changes. At times, particularly in osteoarthritis associated with a more pronounced inflammatory response, radiographic changes can be mild in association with severe symptoms. It is important to appreciate that mild radiographic signs of disease should not exclude a patient from consideration for total hip replacement. Occasionally, in the presence of mild radiographic changes, it is not possible to be certain that the hip is the source of pain. In this situation we find that an injection of local anaesthetic into the hip, performed under radiographic control, is a very useful diagnostic test.

RELATIVE CONTRAINDICATIONS

Factors considered as poor prognostic indications for total hip replacement are significant medical disease where the risk of surgery outweighs the expected benefit, psychiatric disease, dementia, or systemic infections. Though obesity and in particular morbid obesity are considered relative contraindications to hip replacement studies have shown that these patient groups can undergo hip replacement with low complication rates, with significant improvements in functional level, and at least in the short-term no increased risk of implant loosening. Local considerations against hip replacement include poor vascular supply, poor soft tissue cover, ulcers, and neuropathic disease of the hip.

SURGICAL ALTERNATIVES

In younger patients with non-inflammatory arthritis consideration should be given to alternative types of surgical intervention such as joint preserving procedures or possibly arthrodesis. Joint preserving procedures such as acetabular augmentation, femoral or acetabular osteotomy are usually considered if there are mechanical abnormalities within the hip such as acetabular dysplasia that predisposes to osteoarthritis. (We have already done this alternative) The aim is to improve the biomechanics of the hip, to improve the patient’s symptoms, and to delay the need for joint replacement surgery. (The biomechanics of Sam's hip are improved but he is lacking bone regrowth and he still has significant pain and discomfort) These procedures have high success rates provided the selection criteria are appropriate, and can be converted to total hip arthroplasty at a later date if indicated. Hip arthrodesis is most ideally utilised in the young active otherwise healthy patient with unilateral hip disease and a normal lumbar spine. When correctly indicated reliable relief of pain is achieved with patients complaining of little disability provided the hip is fused in a functional position. Low back pain, ipsilateral knee pain, and degenerative arthritis in the contralateral hip can limit the life span of an arthrodesis however these are usually relieved by conversion of the arthrodesis to a total hip replacement. Successfully functioning arthrodeses in 79% of patients at an average 35 year follow up illustrates the durability of this procedure.

Reasons for failure (Yep, this is the area that keeps me awake at night)

Failure of a hip replacement requiring revision surgery occurs at a rate of approximately 1% per year for the first 15 years. Beyond eight years acetabular component loosening is more common than failure of the femoral component. Late aseptic loosening of components is the commonest reason for implant failure causing approximately 75% of failures. The three next most common reasons for implants requiring revision are infection, technical errors at the time of surgery, and recurrent dislocation. If loosening is seen within the first two years after surgery infection should be suspected.

Although there is some overlap it is easier to appreciate risk factors for implant failure if we consider them under patient factors, implant factors, and surgeon factors.

PATIENT FACTORS

Age, sex, and aetiology of arthritis are the most important patient factors with a bearing on implant failure caused by aseptic loosening. The Swedish hip registry, which records national outcomes of hip replacements, has shown a significant increase in failure rates in men compared with women with the difference apparent as early as two years after surgery. This survey also showed that regardless of the underlying diagnosis the younger the patient the higher the rate of implant failure. Fractured neck of femur and avascular necrosis of the femoral head are the diagnosis that have the reputation for the worst outcome after total hip replacement. (These couple of sentences stop me in my tracks and cause me to completely reconsider if this surgery is appropriate. Sam is of course male and he has Perthes or AVN, those two factors are already against him now add in Down syndrome that brings joint laxity and low tone to the picture and my head begins to spin.)

Male patients under 50 years of age, particularly with osteoarthritis secondary to trauma or avascular necrosis of the femoral head have increased rates of aseptic loosening after total hip replacement. These patients often return to athletic or heavy manual activities and this is correlated with increased implant failure at 5–10 years. Despite this implant survivorship at an average 18 year follow up in men less than 50 years of age is 80–85%. (Okay, I feel a little better after reading this area because Sam is not extremely athletic nor do I have to worry about heavy manual activities but I still wonder what the lax joints and low tone will add to normal lifting and mobility with a total hip replacement.)

Female patients less than 55 years of age with rheumatoid arthritis are another higher risk group for aseptic loosening with 25% failure rates by 15 years. The changed biological activity of the host bone23 and increased bleeding at the time of surgery are probably contributing factors to implant loosening though the aetiology of increased loosening in rheumatoid arthritis is still not clear.

Deep infection occurs in around 0.5–2% of total hip replacements and is the cause for revision surgery in at least 7.5% of failures. Infection is more common in inflammatory arthritis, psoriatic arthritis, patients taking corticosteroid treatment, chronic renal failure, diabetes mellitus, high risk surgical patients, malnutrition, and older age. (This is an area that may also affect Sam because is a higher risk patient.)

Dislocation rates of 1–2% can be expected in the best of hands but are often greater than 5%. Most of these will be single dislocations occurring in the early postoperative period, which are successfully treated by closed reduction. One in 30 revision arthroplasties are performed as a consequence of recurrent dislocation. Dislocation rates are increased in patients over 80 years of age. Poor muscular tone, femoral neck fractures, acetabular dysplasia, cerebral palsy, muscular dystrophy, and intellectual impairment are all associated with an increased dislocation rate. (Yep, that's not sounding really good either.)

IMPLANT FACTORS

Probably the most debated topic in joint replacement surgery is that of whether implants should be inserted with or without cement. Non-cemented implants were introduced in response to loosening seen in cemented systems. However to date the long term survival of cemented implants remains superior to any non-cemented system. A comparison of countries where hip registries are maintained shows that the higher the percentage of non-cemented implants inserted the higher the implant failure rate.

Improved operative techniques and implant materials mean that survival of some earlier designs of hip arthroplasties continues to increase. Improvements in surgical and in particular cementing techniques have led to decreased failure rates of hip replacements. Unfortunately many new implants, which are often used in the absence of clinical review, are failing to match the survival rates of earlier designs. Furthermore virtually all implants, instrumentation, and surgical techniques are regularly modified. Although these modifications are introduced to improve results they often achieve the opposite. For example minor changes in surface finish or stem geometry have been found to have detrimental consequences. There are now over 60 different primary total hip prostheses available in the United Kingdom, with a tremendous variation in cost. Over half of these have been introduced in the past five years, and the vast majority have no published clinical results. This is clearly unsatisfactory as some or perhaps many may have poor results. Outside research centres, which must be carefully monitoring clinical data on newer implants we believe that surgeons should be inserting prostheses with published peer reviewed follow up of at least 10 years.

SURGEON FACTORS (This is always a major concern for Sam)

Surgical technique is extremely important in determining implant survival. Experienced hip surgeons generally agree that it is more important to choose your surgeon than the implant. Surgical approach, preparation of the implant bed, and cementing techniques all reflect on implant survival. In a study of surgeons in Florida it was found that patients treated by surgeons doing low numbers of arthroplasties had higher death rates and that costs and length of hospital stay were greater. Dislocation rates do not seem to differ with surgical approach but are higher when the surgery is performed by inexperienced surgeons. Surgeons need to be trained to perform total hip replacement and must be carefully supervised during their learning period. Unfortunately training is difficult as the operation is complex and their is little evidence as to which are the best techniques to use. Though hip replacement is a commonly performed procedure it is not an easy operation to perform well and the implications to the patient are enormous if it is performed badly.

To determine where total hip replacement is performed based on initial cost alone may well be a false economy. Doctors should be encouraged to refer their patients for total hip replacement to surgeons who have an interest in hip surgery. These surgeons should be aware of how their failure rates compare with other institutions, as is done in hip registries in Sweden, Norway, and Finland. This should lead to a progressive improvement in results.

Conclusion

Hip replacement has transformed the lives of hundreds of thousands of people with arthritis of the hip regardless of the underlying aetiology. Surgeons should select their patients for surgery carefully, use implants with long term follow up, and apply surgical techniques that have been shown to decrease implant failure rates.

Sooooo...welcome to our world. Basically all of our options kinda...well...suck. These type of decisions are among the hardest because there are so many risks for Sam. I want Sam to enjoy his life, I want him to be happy and I want him to have great experiences. His current lack of mobility is very limiting. I don't think any of us can really understand how a physical disability changes our life until we ourselves experience it. When you add this physical disability to Sam's other challenges I often feel I am headed more toward a caretaker role than enabling Sam to do things for himself. I think anyone living with a child with special needs will agree that there are times in our lives when doing something for them is so much quicker and easier than enabling them to do it. In my world it was one of the hardest lessons to learn that I had to slow down, change my perspective and enable Sam. It isn't that Sam can't do things often times it is the way or the time it requires that can send my schedule, my reality into a whirlwind. For most parents waking their neurotypical children up for school is a chore. We try to get them to clean up, eat something healthy, brush their teeth, get their school items together and we tell them to have a good day. Sam hates being woke up, in fact the act of waking him up could be the beginning of complete defeat for the day. Because of Sam's hip issues his movement in the morning is slow and unsteady. The walker is a must but even with that Sam will often times have enough pain that he needs to sit or lay down for a moment or two. In the morning Sam can usually remove his shirt with ease and if sitting the socks are doable but the underwear is a challenge when you hang on to a walker with one hand and try to manipulate the other without the ability to turn or twist much from the waist. And of course when you remove clothes in this fashion they usually end up on the floor which requires a bend or squat which is not one of Sam's best moves in order to put them in the laundry basket (which I have learned to remove the cover for easier disposal). While Sam performs these task along with a trip to the bathroom which includes a decreased ability to aim due to balance issues and the need to put weight on the right leg, I am putting the shower chair in the shower, adding a towel to the seat to help with the sensory issues and turning it on to warm up. Sam comes over with his walker and I assist him stepping into the shower. I wait as he gets into a comfortable position and wait again for him to ask me to turn on the water. I have learned that when you have sensory issues jumping into a running shower is a futile debate. If you turn on the water before Sam is ready he will promptly turn it off. I am working on making a mark on the shower that Sam can line up and turn the water on his own for a good water temperature. We are working on Sam shampooing his hair but his limited movement reaching up on the left side is still an issue and his right side is his weaker side. (Are we having fun yet???) Sam's ability to bend and twist is limited so soaping is an assisted procedure with the water off. Water back on to rinse with audible instructions to scrub the shampoo out of his hair. Water off and sometimes Sam can come out unassisted and sometimes the hip won't cooperate. Sam drys off and heads to the bedroom to get dressed because dressing requires sitting and standing and tile floor can be slippery and uncomfortable. Sam returns to the bathroom to comb his hair (Mom tends to step in to help direct the comb in the right direction). Sam brushes his teeth and we are ready for breakfast. This procedure can take 20-25 minutes on a good day and much, much longer on a bad day and at this point he is only clean and dressed. We are working on having Sam assist with breakfast and we are making progress but this slows everything down as Sam talks and sometimes complains his way through it. Yes, it would be easier to undress Sam, bathe Sam, dress Sam, comb his hair, brush his teeth and make his breakfast but then how will Sam ever be able to do these things on his own??? It takes time and patience (bucket loads) to work through these things with Sam but it is also one of the most touching and gratifying experiences to see Sam do more and more independently. I would also love to tell you that once Sam has mastered a skill you can ramp up the speed of completing that skill. Hmmmm....haven't really seen that yet. Sam is always on "Sam time" which appears to be anywhere from 5 to 2 hours slower than "Sue time" aka "bucket load of patience". But I love this little boy and I acknowledge that God is definitely trying to enhance my own life skills and perceptions as I struggle to teach Sam.

I read this post to Jeff tonight so we could begin getting on the same page and I will email it to Sam's pediatrician. My plan right now is to pray for white fluffy stuff on the June x-ray and just in case that doesn't happen I will begin putting my list of questions together to conduct an extensive interview to locate the perfect surgeon for my perfectly imperfect, seriously silly, willingly unwilling, amazingly made little boy that rocks my world, challenges my perceptions, dominates my heart and cripples my mind and ability to sleep. I love you SAM!!!