Joints (Knee, elbow, hip, ankle, shoulder and wrist)

Bleed in the joints occurs while playing or due to fall, direct blow and accident. It may also occur without apparent cause. Treatment will include Factor replacement and physiotherapy normally.

Signs and Symptoms

  • • Swelling, pain, and decreased functional use of a limb
  • • For infants and toddlers, the earliest sign may be the failure to use the arm or leg normally. The skin over the joint may be warm and the child may be fractious (irritable). As the child grows older he will be able to describe his sensation as a bleed occurs.
  • • Activity and mobility increase with age and many children with severe hemophilia may have experienced their first joint bleed in the weight bearing joints, i.e. ankles and knees by the age of three.

Treatment

  • • Factor Replacement
  • • Rest
  • • Physiotherapy. (Short-term splinting may be required)

Synovitis (Chronic joint swelling)

 How it Occures

All joints are lined with a tissue called synovium which produces a fluid that lubricates the joint. For persons with hemophilia, this lining often becomes thickened as a result of repeated bleeding episodes. Synovitis can be acute (lasting for only a couple of weeks) or chronic (a persistent joint swelling that does not go away). As more haemorrhages occur within a joint, the synovium becomes more thickened thus reducing the space within a joint, which in turn may lead to nipping of this membrane during movement, causing further bleeding. If this cycle of bleeding continues there will be eventual further damage (hemophilic arthropathy). Hemophilic synovitis is commonly seen between the ages of 6 and 16 years.

Signs and Symptoms

  • • Persistent swelling (large but not tense) - minimal or tenderness
  • • Full range of joint movement,
  • • Lack of rapid improvement with factor replacement
  • • No heat are the signs and symptoms of synovitis.

Treatment

  • • Aggressive prophylactic therapy
  • • Intense physiotherapy programmes, i.e. ice and exercises.
  • • Sometimes treatment by injection of rifampicin or radioactive substance in the joint is required
  • • Surgery may be indicated in some case to remove the synovium.


Management of chronic synovitis in the knee joint

  • • Apply splint in the manner shown in fig. for 6-12 weeks
  • • This is to be worn throughout the day and can be removed while going to bed
  • • Perform Isometric Thigh muscle exercise(tightening the patella) 10 times every hour.

Muscles

Muscles Bkeeds
Muscle bleeds occur either as a result of direct blow associated with pain and swelling or sports activity. They may also occur spontaneously.

Signs and Symptom

  • • Tingling sensation
  • • Stiffness
  • • Pain
  • • Redness
  • • Warmth
  • • Swelling.

Treatment

   Factor replacement
   Physiotherapy

Persons with hemophilia are prone to intra-articular and intra-muscular hemorrhages (hemoatomas). When muscle bleeds are not detected in time and treated properly the consequences can be disastrous leading to contractures. Common sites of muscle hemorrhages are the quadriceps, hamstrings, calf and forearm muscles. Bleeding into these muscles may affect a single muscle or a group, and those most frequently injured are the ones that extend over two joints, such as quadriceps, part of which passes over the hip and knee joints. These muscles are subject to stretching and forces with which the muscle fibres may not be able to cope and can therefore be easily damaged.

There are two types of hemoatomas that can be identified.

i.e. 1.Superficial hemoatomas and 2. Deep hemoatomas.


Superficial hemoatomas

Superficial hemoatomas rarely result in deformity as bleeding occurs in spaces between the muscles, where an increase in pressure on the underlying blood vessels and nerves is often avoided. The residual hemoatomas tends to follow gravity in downward direction, i.e. towards the hand or foot.

Deep hemoatomas

In these cases bleeding occurs deep into the muscle tissues where the blood is retained within the muscles. These hemoatomas tend to be very painful with gross swelling and loss of function. Bruising may or may not be present. Nerve compression may also occur as a complication.

Head

Bleeding inside the head is a common cause of death in persons with hemophilia. Bleeding may occur spontaneously or as a result of injuries, which may be slight, such as a minor fall or bump on the head. Symptoms of brain hemorrhage may not occur until several days after injury and include irritability, drowsiness, headache, confusion, nausea, vomiting and double vision. Medical attention should be sought immediately.

Signs and Symptoms.

Some of the warning signs, such as a seizure or loss of consciousness, are unmistakable, and such obvious indicators are not apparent. Sometimes the signals are mild enough that one could confuse them with symptoms of flu or gastrointestinal upset:

  • • A persistent headache that does not respond to analgesics
  • • Confusion and difficulty in concentrating
  • • Drowsiness, Dizziness and stiff neck.
  • • Changes in behavious, lethargy, strange gait, slurred speech, visual problems (double vision) or sudden forceful vomiting.

Management

  • • Rush to nearest hemophilia treatment center
  • • Infuse factor concentrates to
  • • 100% for factor VIII - 50 units/kg
  • • 60% for factor IX - 60 units/kg
  • • CT scan to confirm diagnosis
  • • If bleed is confirmed, continue factors for 72 hours.
  • • Factor VIII - 25 units/kg every 8 hrs
  • • Factor IX - 50 units/kg every 12 hrs
  • • Thereafter depending on the individual clinical condition, factor infusion is to continue for the next 7-10 days
  • • Treatment to control fits should be started and continued for atleast 6 months
  • • Any head bleed is life-threatening, so even at the suspicion of one, infuse immediately, notify treatment centres and get to an emergency room
  • • Rapidity of treatment helps a lot in preventing complications. For instance, a young patient who had a mild head trauma (intracranial bleed) was treated so quickly that he did not suffer from neurological problems. 

 Back

Back Pain and Care in Pwh

Low back pain (LBP) is a very common problem in society to day. As bleeds into the spine are fortunately very rare, there is a tendency not to consider low back pain as a problem with hemophilia and to focus attention instead to peripheral joints. However, low back pain does occur in persons with hemophilia. It is to be noted that people with hemophilia are as likely to have LBP as any other people. Secondly back pain may result from increased strain on the low back if the spine has to compensate for problems elsewhere in the body, e.g., fixed flexion deformities of the knees of hips as a result of bleeds.

Risk Factors
Overweight - Being overweight will put increasing strain on the low back.
• Lifting - Repeated or heavy lifting of objects can place increased strain on the low back.
• Regular smoking - Evidence suggests that smokers are more likely to have low back pain. Smoking causes serious lung diseases involving expensive treatment.
Activities that involve prolonged sitting - Sitting at a desk or computer screen for long periods in poor postures and then slumping in front of the television in the evening can also be a cause of low back pain.
A sedentary lifestyle - If a person with hemophilia is generally unfit and does not undertake any exercise, he is more likely to have weak trunk muscles and can be at risk of developing low back pain.
Activities that involve prolonged driving - Prolonged driving can produce vibrations on the spine, which can be harmful. Car seats also may not provide sufficient support, which can lead to poor posture.
Increased strain on the lumbar spine - Hemophilia-related joint problems can cause additional strain on the low back. For example, an unresolved iliopsoas ( a muscle around the hip) bleed can cause a flexion deformity of the hip. This may be compensated by increased lordosis (arch) of the low back which can put increased strain on the small joints of the spine. Flexion deformities of the knees will throw the centre of gravity forward, also putting increased strain on the spine.

The majority of acute low back pain is not serious and settles down quickly. However, people with hemophilia who experience acute low back pain should seek advice from their hemophilia centre, as very occasionally the back pain may be indicative of a bleed. Treatment with factor replacement and appropriate diagnosis and management would therefore be essential. Advice should also be sought for promptly if the back pain is accompanied by fever or if there is any numbness or pins and needles in the legs, buttocks or back, or if there is difficulty passing water or opening of bowels. Assuming that no serious problems are identified the following advice may be helpful.

Bed rest may be indicated for 24-48 hours if pain is severe. However prolonged bed rest is not recommended as it can lead to increasing unfitness and deconditioning. When resting, it is important that the bed is firm but not too hard. A board underneath the mattress may be necessary. You should lie flat on your back, front or sides (whichever is most comfortable) to keep the spine straight, with one pillow and not sit propped up in bed. Often the most painful is flexion or bending forward and it is necessary to avoid this movement in the initial stages. Therefore, when getting out of bed it is important to turn onto your side first and push up into sitting using your hands rather than sit straight up from the bed.

A heat pad or hot water bottle may help to reduce the muscle spasm often associated with low back pain. It should not be too hot; pleasantly warm as otherwise a burn can result. The pad or hot water bottle should be wrapped in a towel rather than placed directly onto the skin and can be left in place for 10-15 minutes. Do not use heat if there is numbness of the skin or circulatory disturbances.

Sitting may be uncomfortable in the early stages and prolonged sitting should be avoided. When sitting, it is important to keep the spine supported with a small towel or cushion in the small of the back to maintain an upright posture and to sit on a firm surface rather than in a soft armchair

Gentle activities such as walking should be encouraged. Try to avoid standing for long periods in the early stages if it is uncomfortable. Some discomfort may be inevitable and should not be an indication to avoid all activity. However if severe discomfort occurs it may be necessary to undertake simple activities for short periods until the pain settles.

Take showers rather than a bath. This will avoid excessive bending and lying in a poor position which may cause an increase in pain.

Te Low Back Pain

Predisposing factors

Consider what might have caused the acute back pain. It may be one event such as lifting, alternatively, it might have occurred due to the cumulative effects of poor posture over a long period of time. By identifying the cause you may be able to prevent the problem occurring again.

Acute low back pain usually settles quickly. However, evidence suggests that after one episode of back pain there is a greater chance that it will recur. You should discuss your problem with a physiotherapist as he/she will be able to help you identify any predisposing factors and may suggest changes in your lifestyle that will help to avoid low back pain or may give you specific treatment to help relieve pain and exercises to do to ensure that you have a good mobility and muscle strength around your trunk.

Chronic Low Back Pain

Chronic low back pain can be very variable. It may be a mild ache noticed on certain activities, which does not interfere with lifestyle or it may be more severe, limiting activities ad affecting quality of life.

Management of chronic low back pain

Whatever be the type of low back pain, it is advisable to seek the help of a physiotherapist who will assess the factors causing pain and then decide on the appropriate course of action. This may mean addressing posture, losing of body weight, lifting correctly, becoming more active and undertaking regular exercise such as swimming. In some cases the physiotherapist will undertake specific treatments to relieve your pain including mobilizations, electrotherapy and teaching certain exercises as well as providing general advice. If the physiotherapist considers that your pain is due to stress on the spine, secondary to hemophilic problems affecting the hips or knees, treatment focused on these will also need to be performed. If several joints are involved then hydrotherapy exercises in water may be beneficial. Limited range of movement in the lower limbs due to arthropathy may mean that lifting cannot be carried out correctly by bending the knees and keeping the back straight. In these cases heavy lifting should be avoided to prevent undue strain on the low back.

Although low back pain can be uncomfortable and the pain is not relieved completely, the problem is seldom serious. There are a number of ways you can improve symptoms with simple attention to posture, regular exercise and lifestyle changes.

Eye Bleeds

Symptoms

  • • Pain around the eye
  • • Swelling
  • • Vision changes
  • • Change in colour in or around the eye

Prompt treatment with factor is necessary to ensure that there is no damage to the vision. The treatment centre as well as the eye doctor has to be consulted. Swelling and slow bleeding can be reduced by placing an ice-pack on the affected eye.

Nose Bleeds

Management

  • • Seat the patient upright
  • • Pinch the nose and advise breathing through the mouth
  • • Apply ice pieces, wrapped in a cloth to both the nostrils
  • • Once bleeding decreases or stops apply a Cyklokapron/transamin pack into the bleeding nostril

If bleeding continues infuse concentrate 10 units per kg; do ENT examination. Nasal packing may have to be employed.

Mouth Bleeds

These tend to look more serious than they really are, but should always be examined carefully. Here are some things one can expect from mouth bleeds of all types:

  • • Because the mouth is moist and very active, clots can't form easily and they are likely to fall out before the clot is healed.
  • • Because the blood is mixed with saliva, it often looks like there is more blood than there really is
  • • Swallowed blood can upset one's stomach and cause nausea and vomiting
  • • If the bleed persists for few hours, hemophilia treatment center should be consulted.
  • • Bleeding due to a cut on the tongue requires attention from a doctor or a member of the hemophilia treatment team

Suggestions for treating mouth bleeds

  • • Eating a popsicle or ice-cube may help reduce pain
  • • Eating soft foods for a few days

Neck & Throat

Bleeding into the face, neck or throat can be serious because swelling can cause obstruction to breathing. Infections may also give swelling in the neck and it is sometimes difficult to be sure if the swelling is due to infections or bleeding. All neck swellings should be regarded as possible bleeds and treated.

Signs and Symptoms

  • • Swelling
  • • Change in skin colour
  • • Trouble breathing or swallowing
  • • Coughing or throwing up blood
  • • Change in voice tone

It is possible for these bleeds to be spontaneous, but they may also occur as a result of hard coughing, sore throat or dental care. One should be wary about the use of retractors which dentists use to pull the cheek out to clear the way for dental work. The tip of the retractors puts heavy pressure on the soft tissues of the throat which can result in a bleed.

Gastrointestinal Bleeds (stomach and intestines)

Bleeds in the GI tract have a wide range of symptoms.

  • • Feeling week, looking pale and having pain in the stomach area
  • • Vomiting fresh(bright red) or old(dark colored)blood, or passing a bloody or blackish stool

Management

  • • Admit in hospital
  • • Transmenic acid (Cyklokapron/Transanin) - 10 mg/kg intravenous/orally every 6 hrs
  • • Factor Concentrates - 20 units/kg, repeat 10 units/kg, 8 hourly for Factor VIII, 12 hourly for Factor IX
  • • Blood transfusion if required
  • • Treat the underlying cause according to the endoscopy findings.

Such bleeds can be caused by anything that pushes into the belly such as a kick or a sport-related accident.

If there is any suspicion of a stomach or gastrointestinal bleed, infuse first, then consult hemophilia treatment centre…All medications should be doublechecked since they may contain aspirin; the generic name of which is salicylic acid.

Urinary Tract bleeds

These are the bleeds that occurs in the kidney, bladder, urethras (the tubes that lead from the kidney to the bladder). These bleeds may happen for no apparent reason (spontaneously) or be caused by an injury. Most kidney bleeds occur without a known cause, while most bladder bleeds are usually due to an infection. These bleeds can be very serious if left untreated.

Signs and Symptoms

  • • Bright red or cola colored urine
  • • Lower back pain or pain that go from the back to the front and down the leg; the pain may be constant or it may come and go
  • • Painful or frequent urination (this could be a symptom of a bladder infection, which would require treatment with an antibiotic).

Some suggestions for treatment

If bleeding is from strain or injury, patient has to be examined for internal injuries and X-Ray may need to be taken to check kidneys.

Urinalysis (test on urine to look for sign of infection) is carried out to check for bladder infection

For spontaneous bleeds, more fluids has to be taken to flush the urinary tract. This will help prevent clots, which could damage your kidneys or ureters. It is recommended that the patient drink one 8 ounce glass of liquid (app.200ml) each hour during the day and one every couple of hours at night. Drinks with caffeine should be avoided because they cause restlessness.

  • • Resting flat on one's back
  • • Heavy lifting, pulling or pushing has to be avoided for 1-2 weeks
  • • Aminocaproic acid should be avoided as it prevents clots from dissolving which could lead to damage of kidneys.
  • • Transmenic acid (Transanin/Cyklokapron) should not be taken
  • • Factor concentrate: 20 units/kg, repeat 10 units/kg, 8 hourly for factor VIII, 12 hourly for factor IX

Small cuts

Small cuts are common among active young adults. Most will stop bleeding quickly and will not require the use of factor therapy.

To treat cuts and scrapes

If superficial

  • Clean the area with soap and water
  • Apply pressure for 5-10 minutes.
  • Apply ice wrapped in a cloth (Fibrin sealant/glue may be applied, but presently not available in India

If deeper

  • Clean the wound
  • Apply firm pressure, bandage if necessary
  • Infuse factor concentrate 10 units/kg
  • Suture one hour later (it is important that suturing should be done only after infusing factor concentrates)
  • Antibiotics for 5 days

Sports injuries

Sprained ankles are the most common sport injury seen in PWH and can be severe with multiple tendon and ligament damage. Following this type of injury there will usually be a joint haemarthrosis, which will require factor replacement, but it is also necessary for the physiotherapist to diagnose other potentially damaged structures and give appropriate treatment.

There is also the risk of bleeds affecting joints and muscles. Choosing a suitable sport for them is therefore difficult. Contact sports (cricket, football, wrestling, boxing etc) have a greater risk than non-contact sports such as swimming, cycling or golf. Other factors like the level at which a sport is played, and whether there is adequate preparation and training, should also be considered when deciding whether a sport is suitable or not. Advice from a trained physiotherapist will help take a decision.

There are both intrinsic and extrinsic risks that can lead to injury connected with sports:

Intrinsic risks

These relate to factors within the body. Body type, shape, overall weight and muscle type mean that some people are more suited to some sports than others. Some people are better able to do endurance activities than speed activities. Choosing a sport without considering these aspects may lead to injury.

Tight muscles can predispose to injury. For example, if the calf muscles remain tight following a bleed, this can reduce the amount of dorsiflexion available at the ankle (the movement of the foot towards the head). This can lead to compensatory movements in the foot that can alter the alignment of the lower limb when walking or running and can lead to pain in the ankle, knee or hip.

Joints that may have been subject to bleeds in the past may have degenerative changes that cause damage to the articular cartilage, which loses its shock-absorbing capacity. These joints are less resistant to trauma, particularly during high-impact weight bearing activities.

Extrinsic risks

These relate to factors outside the body, including foot wear and clothing, equipment, the environment, training programmes and physical contact.

Footwear: For all sports particularly those involving walking and running, well-fitting shoes with a good grip and adequate shock absorption are essential. Ill-fitting shoes with high backs, shoes that are worn out can cause problems and should be avoided.

Protective clothing: Depending on the sport, protective clothing may be essential in reducing the injury. For example, a helmet is advisable when riding a bike.

Equipment: The correct equipment is also important. For example, a tennis racquet needs to be the correct size and weight for the user.

Environment: The surface on which the sport is played also needs to be considered. Running on grass may be less jarring on the lower limbs than running on a hard surface such as a road. If the sport involves road running, the direction needs to be changed regularly as the camber of the road can place an even strain on the limbs.

Training: Adequate training is important for all activities and this includes warm-up and cool-down after activities. It is important to stretch the muscles before sport and ensure that there is sufficient flexibility and range of movement to perform the sport safely. Training can also ensure that the skills, techniques and co-ordination required for the sport can be learnt and practised in a controlled environment

Physical contact: Sports that involve physical contact with others can lead to injuries. Playing competitively in team games may involve a greater risk. It is important to ensure that timing of prophylaxis is adequate to provide some protection against bleeds, but this should not be used indiscriminately in order to partake in particularly risky activities.

The key management of injuries is prevention. However if injuries occur despite consideration of the intrinsic and extrinsic risk factors then treatment is required.

How do sport injuries get treated?

Acute injuries

It is likely that any acute injury will be associated with increased bleeding and therefore factor replacement should be given as soon as possible and should be continued until the injury has resolved. If there is severe pain, swelling, numbness, appropriate medical advice should be sought as soon as possible. Failure to treat adequately initially can lead to a slow recovery and the risk of permanent damage. Acute injuries should settle quickly. If symptoms persist advice from the nearest hameophilia centre should be taken.

The management of acute injuries is recognized by the acronym RICE, which stands for rest, ice, compression and elevation. (For details please see under section MUSCLE)

Chronic injuries

If minor aches and pains or repeated bleeds are associated with sporting activities, it is advisable to seek the opinion of a physiotherapist with experience of hemophilia. These injuries may be due to inappropriate activity, over-training, poor technique or intrinsic factors that may need to be addressed with appropriate exercise programes. In some situations alternative sporting activities may have to be considered.

It should be understood that risks are involved in some activities of sport. However, if the appropriate precautions are taken, then sport can be a rewarding experience and fun.